A unique combination of Generalised Pustular Psoriasis (GPP), Acrodermatitis Continua of Hallopeau (ACH), and psoriatic arthritis (both axial and peripheral) in a young male – a case-based review in literature

A unique combination of Generalised Pustular Psoriasis (GPP), Acrodermatitis Continua of Hallopeau (ACH), and psoriatic arthritis (both axial and peripheral) in a young male – a case-based review in literature | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report A unique combination of Generalised Pustular Psoriasis (GPP), Acrodermatitis Continua of Hallopeau (ACH), and psoriatic arthritis (both axial and peripheral) in a young male – a case-based review in literature Ritasman Baisya, Sukdev Manna, Sneha Dhali This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7199418/v1 This work is licensed under a CC BY 4.0 License Status: Under Review Version 1 posted 9 You are reading this latest preprint version Abstract Background - The simultaneous occurrence of generalised pustular psoriasis (GPP), acrodermatitis continua of Hallopeau (ACH), and Psoriatic arthritis (PsA) in the same patient is infrequently documented in the literature. However, diagnosis of PsA, especially axial PsA, is often missed in everyday clinical practice. Case - A 21-year-old male presented with a generalised scaly, pus-filled rash affecting his face, chest, back, and upper and lower limbs. He also reported experiencing inflammatory polyarthritis in his toes and fingers for the past month, along with right-sided lower back pain during this same period. Additionally, he had a history of intermittent pustular rashes on his nails over the past year, worsening during this episode. Examination revealed a generalised pustular psoriasis rash, with a Psoriasis Area Severity Index (PASI) score of 38.5. There was also evidence of nail psoriasis with (ACH) and a nail psoriasis severity index (NAPSI) of 48/80. His Disease Activity Index for Psoriatic Arthritis (DAPSA) score was recorded at 34. He also had hepatitis and neutrophilic leukocytosis. Following the initiation of therapy with Adalimumab and methotrexate, he experienced significant improvement in both cutaneous and musculoskeletal symptoms, and his systemic features related to hepatitis were also resolved. Conclusion - This is a unique case of GPP with ACH with non-radiographic axial and peripheral Psoriatic arthritis with significant improvement with methotrexate and biological therapy. It highlights the importance of multidisciplinary management of any psoriasis cases. GPP ACH PsA Adalimumab Methotrexate Figures Figure 1 Figure 2 Figure 3 Introduction Psoriasis is a chronic inflammatory skin condition associated with multiple systemic involvements, predominantly arthritis. Generalised pustular psoriasis (GPP) is a variant of psoriasis that can present with diffuse systemic features and nail changes, mainly acro-pustular dermatitis. (1) There is a notable scarcity of literature on musculoskeletal involvement in GPP , yet it remains a common occurrence in clinical practice. The connection between GPP and acrodermatitis continua of Hallopeau (ACH) is also underreported. In this case, we present a young male who experienced GPP and ACH, accompanied by axial and peripheral psoriatic arthritis, and he made significant improvements following the initiation of biologic therapy. This case emphasises the critical need for a multidisciplinary approach in managing psoriasis, integrating the expertise of both rheumatologists and dermatologists for optimal patient outcomes. Case presentation A 21-year-old male presented to the Rheumatology outpatient department with complaints of swelling in his right fourth and fifth toes for the past month. He also experienced low back pain during the same period, with symptoms worsening in the second half of the night and early morning stiffness lasting more than 30 minutes. Additionally, he noticed multiple erythematous pustular rashes on his face, chest, and back, which were significantly itchy and scaly and often filled with purulent discharges for the last 3 months. He had observed yellowish discolouration of his nails for the past year with occasional purulent discharge. He also complained of intermittent high-grade fever, malaise and prostration. There was no family history of similar rashes or joint pain. On examination, he was febrile, cutaneous examination displayed a scaly erythematous pustular rash over his face, chest, and back, (Figure 1) . He also had an extensive scaly pustular crusting rash on his scalp. All these cutaneous features were in more favour of generalsied pustular psoriasis (GPP). Examination of his nails revealed pitting, the oil drop sign, onycholysis, subungual hyperkeratosis, and acrodermatitis continua of Hallopeau (ACH). (Figure 2) The musculoskeletal examination showed tenderness in the left shoulder, wrist, the fourth distal interphalangeal joint, the right second proximal phalangeal joint, and the interphalangeal joints of the fourth and fifth toes, along with swelling in the same areas. He had evident dactylitis in right 4 th , 5 th toes. Investigations revealed neutrophilic leukocytosis (total leukocyte count – 10140 /microliter with differential for neutrophil 86%), pyuria (pus cells 12 -15 /high power field), elevated C-reactive protein (CRP) levels (4.3 mg/ml) and transaminitis ( Aspartate transaminase – 136 U/L, Alanine transaminase – 242 U/L). Conventional radiography of the sacroiliac joint was normal. Magnetic resonance imaging (MRI) of the sacroiliac joint showed bilateral bone marrow oedema, suggesting active sacroilitis (right greater than left) in the short tau inverse resonant (STIR) sequence (Figure 3) with no erosion in the T1 sequence, suggesting non-radiographic sacroilitis. The abdominal ultrasound displayed a "starry sky" appearance, suggesting hepatitis. According to the criteria (2) set by the European Rare and Severe Psoriasis Expert Network (ERASPEN), he was diagnosed with GPP with Additional Complications and systemic involvement, including fever, hepatitis, leukocytosis, and pyuria, as well as Psoriatic Arthritis with non-radiographic sacroiliitis (based on (ClASsification criteria for Psoriatic Arthritis (CASPAR criteria) (3). At his initial visit, his Psoriasis Area Severity Index (PASI) score was 38.5, the nail psoriasis area severity index (NAPSI) was 48/80 for fingernails, and his Disease Activity Index for Psoriatic Arthritis (DAPSA) score was 34. Considering his systemic involvement and high disease activity, treatment with a TNF inhibitor (Adalimumab—40 mg subcutaneously every 15 days) was initiated alongside methotrexate (20 mg per week). After the initiation of therapy, his musculoskeletal pain drastically improved, and his nail and skin changes significantly resolved. On Day 28, his PASI score was 0 and NAPSI 8/80 (Figures 1 and 2—follow-up visit), with arthritis in remission. The patient had regular follow-ups, and after receiving four doses of adalimumab, he could not continue that medication and was placed solely on methotrexate ( 15 mg /week ) . Six months after the initial visit, he reported no rash, significant nail improvement, reduced joint pain, and overall improved quality of life. Discussion In this case report, we have described a patient with acute-onset generalised pustular psoriasis, acrodermatitis continua of Hallopeau (ACH), with significant axial and peripheral arthritis and systemic features who was successfully treated with a TNF inhibitor and methotrexate. GPP and PsA - GPP is a rare condition, representing less than 3% of all psoriasis cases. (4) GPP associated with psoriatic arthritis (PsA) is infrequently found, and information about this combination is sparse. Most reviews and expert opinions suggest that the prevalence of PsA in GPP ranges from approximately 4% to 30%. (5) However, cases of axial involvement are often overlooked, and types of peripheral arthritis were generally omitted from these reviews. A retrospective study conducted in Japan (4) indicated that spinal involvement is actually prevalent in GPP with PsA, despite the smaller sample size. This highlights the importance of including rheumatologists in expert discussions to adequately address musculoskeletal issues in GPP. We have performed a comprehensive review of the literature available in the PubMed (Medline), Web of Science, and Embase databases using MeSH keywords (GPP and PsA), revealing a scarcity of literature regarding the link between PsA and GPP cases. Our analysis includes a detailed descriptive comparison of various case reports and articles in Table 1 (4, 6-12) A retrospective analysis (4) in Japan involved seven patients with GPP and PsA, 20 patients with GPP only, and 148 with PsA only. Findings showed that skin lesion severity and laboratory abnormalities were milder in GPP+PsA patients compared to GPP-only. Additionally, GPP+PsA patients had greater musculoskeletal involvement than PsA-only patients (n = 148). They were significantly more likely to report severe musculoskeletal manifestations (p = 0.006). Furthermore, the prevalence of spinal disease (p = 0.031), dactylitis (p = 0.024), enthesitis (p = 0.046), and bone erosion and deformation (p = 0.022) were significantly higher in GPP+PsA patients than in those with only PsA. GPP and systemic involvement - Typically, GPP is a multisystem disease characterised by repeated flares consisting of the sudden onset of an extensive erythematous skin rash rapidly covered with pustules, associated with general symptoms such as fever, malaise with asthenia, myalgia, and arthralgia. (1) Investigation-wise, the most common features are a high serum C-reactive protein level, leukocytosis with neutrophilia, and elevated liver enzymes (neutrophilic cholangitis). (1) Viguer M et al (13) found that 90% (20/22) of patients with GPP had at least one abnormal liver examination result. Half exhibited significant liver disturbances: jaundice in four cases (18%), elevated gamma-glutamyl transferase (over five times normal) in ten cases (45%), high alkaline phosphatase (over double normal) in seven cases (32%), and increased aminotransferases (over three times normal) also in seven cases (32%). These abnormalities normalised with psoriasis remission, suggesting a link between severe liver issues and skin manifestation intensity. Our patient also showed very high Transaminases, which were subsequently improved. However, this review was predominantly focusing on arthritis in GPP, and hence, Table 1 included patients with GPP and PsA with systemic features. ACH and PsA - Acrodermatitis continua of Hallopeau (ACH) is a rare and debilitating variant of pustular psoriasis, characterised by sterile pustules on the fingers, toes, and nail beds, alongside erythematous atrophic skin. (14) In some instances, ACH may precede, coincide with, or follow the onset of GPP. Coexistence of ACH and psoriatic arthritis has been infrequently reported. Our patient presented with a GPP rash with a very high PASI score, exhibiting acro pustular psoriasis (ACH) and systemic features, including hepatitis. A comprehensive analysis of various studies and case reports of ACH with PsA is detailed in Table 2. (15-23) Genetic background – In 2011, mutations in the IL36RN gene were first identified in nine Tunisian families of GPP and later in patients with ACH. (24) These mutations lead to a functional deletion of the interleukin-36 receptor antagonist, which enhances the effects of interleukin-36 and activates the NF-κB signalling pathway. The deficiency of interleukin-36 receptor antagonists (DITRA) is now recognized as a new category of auto-inflammatory diseases. (24) Other mutations include a gain-of-function mutation in CARD14 (25) and loss of function mutation AP1S3. (26) In this case report, we have not done genetic testing due to financial constraints. Treatment response—GPP and ACH present a challenging clinical scenario with limited effective treatment options. The periods of remission between flare-ups complicate the conduct of therapeutic trials. Topical treatments are generally ineffective. Preferred choices for systemic therapy include methotrexate, retinoid, and cyclosporine. (27) Although anti-TNFα biologic therapies have been used in several cases, their efficacy often diminishes over time, requiring additional medications or dose increases to maintain their effectiveness. Phase III studies have demonstrated significant clinical efficacy of the anti-IL-17 drugs secukinumab, ixekizumab, and brodalumab in treating patients with GPP and erythroderma. (28) The Th17 response is crucial in GPP and may be worsened by uncontrolled IL-36 signalling. Due to their similarities, this is relevant to ACH. The Th17 response leads to the recruitment and activation of neutrophils involved in the disease (29) . Methotrexate is effective in treating generalized pustular psoriasis (GPP) by inhibiting immune cells in the epidermis (30) . However, it has a slower onset of action, requiring several weeks before its effects are noticeable. Therefore, it is primarily used as maintenance therapy for chronic GPP cases (31) . A proposed dosage schedule for methotrexate based on the severity of GPP is as follows: for mild cases, 6 mg per week; for moderate cases, 7.5 mg per week; and for severe cases, between 7.5 and 15 mg per week (30) . In this particular case, we used a TNF inhibitor (Adalimumab) in combination with methotrexate, and the patient demonstrated a significant response. Due to financial constraints, the patient was subsequently maintained on only methotrexate and showed a sustained response after six months. This case-based review has several limitations. The patient lacked a histopathological diagnosis and genetic screening. Follow-up lasted only six months because the patient was lost to follow-up. Nevertheless, the patient presented acutely with severe skin, nail, joint, and systemic symptoms, showing a remarkable response to TNF inhibitors and sustained remission with methotrexate, which is noteworthy in the literature. Conclusion This was a challenging case because the patient exhibited multiple severe manifestations, including GPP, ACH with sacroiliitis, peripheral arthritis, and dactylitis, alongside systemic symptoms, all of which improved successfully with biologic treatment and methotrexate. Clinicians should thoroughly analyse all domains in any psoriasis case, as musculoskeletal involvement, particularly axial issues, is often overlooked. Abbreviations GPP Generalised pustular psoriasis ACH Acrodermatitis continua of Hallopeau PsA Psoriatic arthritis CASPAR criteria ClASsification criteria for Psoriatic Arthritis NAPSI Nail psoriasis area severity index PASI Psoriasis Area Severity Index DAPSA Disease activity psoriatic arthritis score Declarations Funding – None Consent to participate – Consent was taken Consent for publication - Written informed consent was obtained from the patient for publication of this case report and accompanying images Ethical approval and its number – Not applicable Data availability – Not applicable References Bachelez H (2018) Pustular psoriasis and related pustular skin diseases. Br J Dermatol 178(3):614–618 Navarini AA, Burden AD, Capon F, Mrowietz U, Puig L, Koks S et al (2017) European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol 31(11):1792–1799 Taylor W, Gladman D, Helliwell P et al (2006) Classification criteria for psoriatic arthritis: development of new criteria from a large international study. Arthritis Rheum 54(8):2665–2673 Manome-Zenke Y, Ohara Y, Fukui S et al (2022) Characteristics of Patients with Generalized Pustular Psoriasis and Psoriatic Arthritis: A Retrospective Cohort Study. Acta Derm Venereol 102:adv00685 Elewski B, Lebwohl MG (2025) Management of Chronic Generalized Pustular Psoriasis: A Review and Expert Opinion. J Psoriasis Psoriatic Arthritis 2:24755303251318976 Kimura U, Kinoshita A, Sekigawa I, Takamori K, Suga Y (2012) Successful Treatment With Adalimumab in a patient with Psoriatic Arthritis and Generalized Pustular Psoriasis. J Dermatol 39:1071–1072 Vieira Serrão V, Martins A, Lopes M (2008) Infliximab in Recalcitrant Generalized Pustular Arthropatic Psoriasis. Eur J Dermatol 18(1):71–73 Seki K, Amano T, Nakayama Y (1997) Ryumachi 37(4):568–573 Freeman S, Kumar, Rk, Owen Ms (1979) Generalized Pustular Psoriasis with arthritis and pericarditis: Case Report. Australas J Dermatol 20:1–6 Sb H (2005) Kim Ni. Generalized Pustular Psoriasis Following Withdrawal Of Short-Term Cyclosporin Therapy For Psoriatic Arthritis. J Eur Acad Dermatol Venereol 19(4):522–523 Fukunaga Y, Kanbara T, Ikezawa Z (2008) A Case of Generalized Pustular Psoriasis Accompanied by Psoriatic Arthritis and positive Anti–Cyclic Citrullinated Peptide Antibodies. Japanese J Dermatology 118:1953–1959 (In Japanese) Chung M, Elewski B (2025) Treatment of Generalized Pustular Psoriasis with Bimekizumab. SKIN J Cutan Med 9:2112–2115 Viguier M, Allez M, Zagdanski AM, Bertheau P, de Kerviler E, Rybojad M et al (2004) High frequency of cholestasis in generalized pustular psoriasis: Evidence for neutrophilic involvement of the biliary tract. 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J Dermatol 33(11):787–791 Marrakchi S, Guigue P, Renshaw BR, Puel A, Pei XY, Fraitag S et al (2011) Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 365:620–628 Jordan CT, Cao L, Robertson ED et al (2012) Rare and common variants in CARD14, encoding an epidermal regulator of NF-jB, in psoriasis. Am J Hum Genet 90:796–808 Setta-Kaffetzi N, Simpson MA, Navarini AA et al (2014) AP1S3 mutations are associated with pustular psoriasis and impaired Toll-like receptor 3 trafficking. Am J Hum Genet 94:790–797 Robinson A, Van Vorhees AS, Hsu S et al (2012) Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol 67:279–288 Imafuku S, Honma M, Okubo Y et al (2016) Efficacy and safety of secukinumab in patients with generalized pustular psoriasis: a 52-week analysis from phase III open-label multicenter Japanese study. 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Exp Dermatol 32(8):1204–1218 Table Table 1 – A comprehensive review of GPP cases with Psoriatic arthritis in the literature Author Country Gender /age GPP features Axial features Peripheral + dactylitis Systemic Treatment Kimura et al, (3) (2012) Japan Case report 36 year female Pustules ( lake of pus ) Psoriatic plaques , no nail No Sacroilitis /enthesitis DIP , wrist , left knee , rt foot Fever Raised ESR , CRP ,MMP Adalimumab Zenke Y et al (2) (2022) Japan Article 7 GPP + PSA – 4 male Median age 39 years No separate description Spinal involve -5 Enthesitis > 3 in 3 patients PA > 4 in 3 Dactylitis >3 in 2 patients No separate description Management was not included Serrao V et al (4) (2008) Portugal Case report Male Pustular eruption No mention Severe arthritis Fever Methotrexate with steroid Seki K et al (5) (1997) Japan 31 Male Pustular eruption No mention Symmetric polyarthritis Fever Methotrexate Freeman S et al (6) (1979) Australia Case report 52 , F Pustular eruption Sacroilitis Knee , shoulder , limb ,foot Pericarditis ---------- Hong S B et al (7) (2005) Korea Case report 27 , M Pustular eruption on plaque psoriasis Not mentioned Polyarthritis Fever Raised ESR ,CRP Withdrawal of cyclosporine precipitate GPP , MTX Fukunaga Y et al (8) (2008) Case report Japan 28, M GPP , same time arthritis Not mentioned Elbow , knee , wrist , ankle ------ --------- Chung MD et al , (9) (2025 ) Case report USA 39,M GPP & plaque psoriasis Not mentioned Polyarthritis Fever Heart failure , obesity Infliximab Bimekizumab Additional Declarations No competing interests reported. Cite Share Download PDF Status: Under Review Version 1 posted Editorial decision: Revision requested 24 Nov, 2025 Reviews received at journal 23 Nov, 2025 Reviewers agreed at journal 10 Nov, 2025 Reviews received at journal 08 Nov, 2025 Reviewers agreed at journal 08 Nov, 2025 Reviewers invited by journal 22 Oct, 2025 Editor assigned by journal 08 Aug, 2025 Submission checks completed at journal 06 Aug, 2025 First submitted to journal 03 Aug, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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15:18:42","extension":"html","order_by":13,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":71292,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-7199418/v1/f29048428a624b57b7bb8d7a.html"},{"id":95528181,"identity":"5a69b77f-3a6b-416d-8697-667cc5182f78","added_by":"auto","created_at":"2025-11-10 10:15:40","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":94531,"visible":true,"origin":"","legend":"\u003cp\u003eGPP rash on front, back, and face (a, b, c) and resolution of the rash after 28 days of adalimumab injection (d, e, f)\u003c/p\u003e","description":"","filename":"skinrashGPPFigure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7199418/v1/cfa0f50ddb481b9607c915c8.jpg"},{"id":95527561,"identity":"06b3fae1-0dc0-43a4-8515-7b0712d0065f","added_by":"auto","created_at":"2025-11-10 10:14:10","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":56135,"visible":true,"origin":"","legend":"\u003cp\u003eACH rash on fingers at initial presentation (A), post-adalimumab day 3 (improving trend) (B), resolution at day 28 (C)\u003c/p\u003e","description":"","filename":"ACHrashegypt.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7199418/v1/67e28c0695dee9dc6253a044.jpg"},{"id":95527649,"identity":"1086592b-8dbb-4866-a657-2aa6cd922d8b","added_by":"auto","created_at":"2025-11-10 10:14:26","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":124981,"visible":true,"origin":"","legend":"\u003cp\u003eMRI of sacroiliac joint – STIR sequence showed hyper-intensity in bilateral SI joints (right \u0026gt; left)\u003c/p\u003e","description":"","filename":"STIRMRIimageinPSAegypt.png","url":"https://assets-eu.researchsquare.com/files/rs-7199418/v1/2052c637c1e5c3beda4f8a2a.png"},{"id":95531554,"identity":"8690e060-4729-4f8c-814d-81e34f19852f","added_by":"auto","created_at":"2025-11-10 10:23:16","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":707167,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7199418/v1/5ecafe14-360b-4237-834f-b645dd5f63d5.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003eA unique combination of Generalised Pustular Psoriasis (GPP), Acrodermatitis Continua of Hallopeau (ACH), and psoriatic arthritis (both axial and peripheral) in a young male – a case-based review in literature\u003c/p\u003e","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePsoriasis is a chronic inflammatory skin condition associated with multiple systemic involvements, predominantly arthritis. Generalised pustular psoriasis (GPP) is a variant of psoriasis that can present with diffuse systemic features and nail changes, mainly acro-pustular dermatitis. \u003csup\u003e(1)\u003c/sup\u003e There is a notable scarcity of literature on musculoskeletal involvement in GPP , yet it remains a common occurrence in clinical practice. The connection between GPP and acrodermatitis continua of Hallopeau (ACH) is also underreported. In this case, we present a young male who experienced GPP and ACH, accompanied by axial and peripheral psoriatic arthritis, and he made significant improvements following the initiation of biologic therapy. This case emphasises the critical need for a multidisciplinary approach in managing psoriasis, integrating the expertise of both rheumatologists and dermatologists for optimal patient outcomes.\u003c/p\u003e"},{"header":"Case presentation ","content":"\u003cp\u003eA 21-year-old male presented to the Rheumatology outpatient department with complaints of swelling in his right fourth and fifth toes for the past month. He also experienced low back pain during the same period, with symptoms worsening in the second half of the night and early morning stiffness lasting more than 30 minutes. Additionally, he noticed multiple erythematous pustular rashes on his face, chest, and back, which were significantly itchy and scaly and often filled with purulent discharges for the last 3 months. He had observed yellowish discolouration of his nails for the past year with occasional purulent discharge. He also complained of intermittent high-grade fever, malaise and prostration. There was no family history of similar rashes or joint pain.\u003c/p\u003e\n\u003cp\u003eOn examination, he was febrile, cutaneous examination displayed a scaly erythematous pustular rash over his face, chest, and back, (Figure 1) . He also had an extensive scaly pustular crusting rash on his scalp. All these cutaneous features were in more favour of generalsied pustular psoriasis (GPP). Examination of his nails revealed pitting, the oil drop sign, onycholysis, subungual hyperkeratosis, and acrodermatitis continua of Hallopeau (ACH). (Figure 2) The musculoskeletal examination showed tenderness in the left shoulder, wrist, the fourth distal interphalangeal joint, the right second proximal phalangeal joint, and the interphalangeal joints of the fourth and fifth toes, along with swelling in the same areas. He had evident dactylitis in right 4\u003csup\u003eth\u003c/sup\u003e, 5\u003csup\u003eth\u003c/sup\u003e toes.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eInvestigations revealed neutrophilic leukocytosis (total leukocyte count – 10140 /microliter with differential for neutrophil 86%), pyuria (pus cells 12 -15 /high power field), \u0026nbsp;elevated C-reactive protein (CRP) levels (4.3 mg/ml) and transaminitis ( Aspartate transaminase – 136 U/L, Alanine transaminase – 242 U/L). Conventional radiography of the sacroiliac joint was normal. Magnetic resonance imaging (MRI) of the sacroiliac joint showed bilateral bone marrow oedema, suggesting active sacroilitis (right greater than left) in the short tau inverse resonant (STIR) sequence (Figure 3) with no erosion in the T1 sequence, suggesting non-radiographic sacroilitis. The abdominal ultrasound displayed a \"starry sky\" appearance, suggesting hepatitis.\u003c/p\u003e\n\u003cp\u003eAccording to the criteria (2) set by the European Rare and Severe Psoriasis Expert Network (ERASPEN), he was diagnosed with GPP with Additional Complications and systemic involvement, including fever, hepatitis, leukocytosis, and pyuria, as well as Psoriatic Arthritis with non-radiographic sacroiliitis (based on (ClASsification criteria for Psoriatic Arthritis (CASPAR criteria) (3). At his initial visit, his Psoriasis Area Severity Index (PASI) score was 38.5, the nail psoriasis area severity index (NAPSI) was 48/80 for fingernails, and his Disease Activity Index for Psoriatic Arthritis (DAPSA) score was 34.\u003c/p\u003e\n\u003cp\u003eConsidering his systemic involvement and high disease activity, treatment with a TNF inhibitor (Adalimumab—40 mg subcutaneously every 15 days) was initiated alongside methotrexate (20 mg per week). After the initiation of therapy, his musculoskeletal pain drastically improved, and his nail and skin changes significantly resolved. On Day 28, his PASI score was 0 and NAPSI 8/80 (Figures 1 and 2—follow-up visit), with arthritis in remission. The patient had regular follow-ups, and after receiving four doses of adalimumab, he could not continue that medication and was placed solely on methotrexate ( 15 mg /week ) . Six months after the initial visit, he reported no rash, significant nail improvement, reduced joint pain, and overall improved quality of life.\u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eIn this case report, we have described a patient with acute-onset generalised pustular psoriasis, acrodermatitis continua of Hallopeau (ACH), with significant axial and peripheral arthritis and systemic features who was successfully treated with a TNF inhibitor and methotrexate.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eGPP and PsA - \u0026nbsp; GPP is a rare condition, representing less than 3% of all psoriasis cases. (4) \u0026nbsp;GPP associated with psoriatic arthritis (PsA) is infrequently found, and information about this combination is sparse. Most reviews and expert opinions suggest that the prevalence of PsA in GPP ranges from approximately 4% to 30%.\u003csup\u003e(5)\u003c/sup\u003e\u0026nbsp; \u0026nbsp;However, cases of axial involvement are often overlooked, and types of peripheral arthritis were generally omitted from these reviews. A retrospective study conducted in Japan \u003csup\u003e(4)\u003c/sup\u003e indicated that spinal involvement is actually prevalent in GPP with PsA, despite the smaller sample size. This highlights the importance of including rheumatologists in expert discussions to adequately address musculoskeletal issues in GPP. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eWe have performed a comprehensive review of the literature available in the PubMed (Medline), Web of Science, and Embase databases using MeSH keywords (GPP and PsA), revealing a scarcity of literature regarding the link between PsA and GPP cases. Our analysis includes a detailed descriptive comparison of various case reports and articles in Table 1 \u003csup\u003e(4, 6-12)\u003c/sup\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eA retrospective analysis \u003csup\u003e(4)\u003c/sup\u003e in Japan involved seven patients with GPP and PsA, 20 patients with GPP only, and 148 with PsA only. Findings showed that skin lesion severity and laboratory abnormalities were milder in GPP+PsA patients compared to GPP-only. Additionally, GPP+PsA patients had greater musculoskeletal involvement than PsA-only patients (n = 148). They were significantly more likely to report severe musculoskeletal manifestations (p = 0.006). Furthermore, the prevalence of spinal disease (p = 0.031), dactylitis (p = 0.024), enthesitis (p = 0.046), and bone erosion and deformation (p = 0.022) were significantly higher in GPP+PsA patients than in those with only PsA.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eGPP and systemic involvement - Typically, GPP is a multisystem disease characterised by repeated flares consisting of the sudden onset of an extensive erythematous skin rash rapidly covered with pustules, associated with general symptoms such as fever, malaise with asthenia, myalgia, and arthralgia. \u003csup\u003e(1) \u0026nbsp;\u003c/sup\u003eInvestigation-wise, the most common features are a high serum C-reactive protein level, leukocytosis with neutrophilia, and elevated liver enzymes (neutrophilic cholangitis). \u003csup\u003e(1)\u003c/sup\u003e\u0026nbsp; Viguer M et al \u003csup\u003e(13)\u003c/sup\u003e found that 90% (20/22) of patients with GPP had at least one abnormal liver examination result. Half exhibited significant liver disturbances: jaundice in four cases (18%), elevated gamma-glutamyl transferase (over five times normal) in ten cases (45%), high alkaline phosphatase (over double normal) in seven cases (32%), and increased aminotransferases (over three times normal) also in seven cases (32%). These abnormalities normalised with psoriasis remission, suggesting a link between severe liver issues and skin manifestation intensity. Our patient also showed very high Transaminases, which were subsequently improved. However, this review was predominantly focusing on arthritis in GPP, and hence, Table 1 included patients with GPP and PsA with systemic features.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eACH and PsA - Acrodermatitis continua of Hallopeau (ACH) is a rare and debilitating variant of pustular psoriasis, characterised by sterile pustules on the fingers, toes, and nail beds, alongside erythematous atrophic skin. \u003csup\u003e(14)\u003c/sup\u003e In some instances, ACH may precede, coincide with, or follow the onset of GPP. Coexistence of ACH and psoriatic arthritis has been infrequently reported. Our patient presented with a GPP rash with a very high PASI score, exhibiting acro pustular psoriasis (ACH) and systemic features, including hepatitis. A comprehensive analysis of various studies and case reports of ACH with PsA is detailed in Table 2. \u003csup\u003e(15-23)\u0026nbsp;\u003c/sup\u003e\u003c/p\u003e\n\u003cp\u003eGenetic background – In 2011, mutations in the IL36RN gene were first identified in nine Tunisian families of GPP and later in patients with ACH.\u003csup\u003e\u0026nbsp;(24) \u0026nbsp;\u003c/sup\u003eThese mutations lead to a functional deletion of the interleukin-36 receptor antagonist, which enhances the effects of interleukin-36 and activates the NF-κB signalling pathway. The deficiency of interleukin-36 receptor antagonists (DITRA) is now recognized as a new category of auto-inflammatory diseases.\u003csup\u003e\u0026nbsp;(24) \u0026nbsp;\u003c/sup\u003eOther mutations include a gain-of-function mutation in CARD14 \u003csup\u003e(25)\u003c/sup\u003e and loss of function mutation AP1S3. \u003csup\u003e(26)\u003c/sup\u003e\u0026nbsp; In this case report, we have not done genetic testing due to financial constraints.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTreatment response—GPP and ACH present a challenging clinical scenario with limited effective treatment options. The periods of remission between flare-ups complicate the conduct of therapeutic trials. Topical treatments are generally ineffective. Preferred choices for systemic therapy include methotrexate, retinoid, and cyclosporine.\u003csup\u003e\u0026nbsp;(27)\u003c/sup\u003e\u0026nbsp; Although anti-TNFα biologic therapies have been used in several cases, their efficacy often diminishes over time, requiring additional medications or dose increases to maintain their effectiveness. Phase III studies have demonstrated significant clinical efficacy of the anti-IL-17 drugs secukinumab, ixekizumab, and brodalumab in treating patients with GPP and erythroderma.\u003csup\u003e\u0026nbsp;(28) \u0026nbsp;\u003c/sup\u003eThe Th17 response is crucial in GPP and may be worsened by uncontrolled IL-36 signalling. Due to their similarities, this is relevant to ACH. The Th17 response leads to the recruitment and activation of neutrophils involved in the disease \u003csup\u003e(29)\u003c/sup\u003e.\u003c/p\u003e\n\u003cp\u003eMethotrexate is effective in treating generalized pustular psoriasis (GPP) by inhibiting immune cells in the epidermis \u003csup\u003e(30)\u003c/sup\u003e. \u0026nbsp; However, it has a slower onset of action, requiring several weeks before its effects are noticeable. Therefore, it is primarily used as maintenance therapy for chronic GPP cases \u003csup\u003e(31)\u003c/sup\u003e. A proposed dosage schedule for methotrexate based on the severity of GPP is as follows: for mild cases, 6 mg per week; for moderate cases, 7.5 mg per week; and for severe cases, between 7.5 and 15 mg per week\u003csup\u003e\u0026nbsp;(30)\u003c/sup\u003e. In this particular case, we used a TNF inhibitor (Adalimumab) in combination with methotrexate, and the patient demonstrated a significant response. Due to financial constraints, the patient was subsequently maintained on only methotrexate and showed a sustained response after six months.\u003c/p\u003e\n\u003cp\u003eThis case-based review has several limitations. The patient lacked a histopathological diagnosis and genetic screening. Follow-up lasted only six months because the patient was lost to follow-up. Nevertheless, the patient presented acutely with severe skin, nail, joint, and systemic symptoms, showing a remarkable response to TNF inhibitors and sustained remission with methotrexate, which is noteworthy in the literature.\u0026nbsp;\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThis was a challenging case because the patient exhibited multiple severe manifestations, including GPP, ACH with sacroiliitis, peripheral arthritis, and dactylitis, alongside systemic symptoms, all of which improved successfully with biologic treatment and methotrexate. Clinicians should thoroughly analyse all domains in any psoriasis case, as musculoskeletal involvement, particularly axial issues, is often overlooked.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eGPP\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eGeneralised pustular psoriasis\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eACH\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eAcrodermatitis continua of Hallopeau\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003ePsA\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003ePsoriatic arthritis\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eCASPAR criteria\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eClASsification criteria for Psoriatic Arthritis\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eNAPSI\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eNail psoriasis area severity index\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003ePASI\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003ePsoriasis Area Severity Index\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eDAPSA\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eDisease activity psoriatic arthritis score\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eFunding –\u0026nbsp;\u003c/strong\u003eNone\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to participate –\u0026nbsp;\u003c/strong\u003eConsent was taken \u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication -\u0026nbsp;\u003c/strong\u003eWritten informed consent was obtained from the patient for publication of this case report and accompanying images\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical approval and its number –\u0026nbsp;\u003c/strong\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability –\u0026nbsp;\u003c/strong\u003eNot applicable\u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eBachelez H (2018) Pustular psoriasis and related pustular skin diseases. Br J Dermatol 178(3):614\u0026ndash;618\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eNavarini AA, Burden AD, Capon F, Mrowietz U, Puig L, Koks S et al (2017) European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol 31(11):1792\u0026ndash;1799\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eTaylor W, Gladman D, Helliwell P et al (2006) Classification criteria for psoriatic arthritis: development of new criteria from a large international study. Arthritis Rheum 54(8):2665\u0026ndash;2673\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eManome-Zenke Y, Ohara Y, Fukui S et al (2022) Characteristics of Patients with Generalized Pustular Psoriasis and Psoriatic Arthritis: A Retrospective Cohort Study. Acta Derm Venereol 102:adv00685\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eElewski B, Lebwohl MG (2025) Management of Chronic Generalized Pustular Psoriasis: A Review and Expert Opinion. J Psoriasis Psoriatic Arthritis 2:24755303251318976\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKimura U, Kinoshita A, Sekigawa I, Takamori K, Suga Y (2012) Successful Treatment With Adalimumab in a patient with Psoriatic Arthritis and Generalized Pustular Psoriasis. J Dermatol 39:1071\u0026ndash;1072\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eVieira Serr\u0026atilde;o V, Martins A, Lopes M (2008) Infliximab in Recalcitrant Generalized Pustular Arthropatic Psoriasis. Eur J Dermatol 18(1):71\u0026ndash;73\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSeki K, Amano T, Nakayama Y (1997) Ryumachi 37(4):568\u0026ndash;573\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eFreeman S, Kumar, Rk, Owen Ms (1979) Generalized Pustular Psoriasis with arthritis and pericarditis: Case Report. Australas J Dermatol 20:1\u0026ndash;6\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSb H (2005) Kim Ni. Generalized Pustular Psoriasis Following Withdrawal Of Short-Term Cyclosporin Therapy For Psoriatic Arthritis. J Eur Acad Dermatol Venereol 19(4):522\u0026ndash;523\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eFukunaga Y, Kanbara T, Ikezawa Z (2008) A Case of Generalized Pustular Psoriasis Accompanied by Psoriatic Arthritis and positive Anti\u0026ndash;Cyclic Citrullinated Peptide Antibodies. Japanese J Dermatology 118:1953\u0026ndash;1959 (In Japanese)\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eChung M, Elewski B (2025) Treatment of Generalized Pustular Psoriasis with Bimekizumab. SKIN J Cutan Med 9:2112\u0026ndash;2115\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eViguier M, Allez M, Zagdanski AM, Bertheau P, de Kerviler E, Rybojad M et al (2004) High frequency of cholestasis in generalized pustular psoriasis: Evidence for neutrophilic involvement of the biliary tract. Hepatology 40(2):452\u0026ndash;458\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eWen P, Liu C, Wang T, Jiang X, Wang P, Wang S (2024) Successful treatment of acrodermatitis continua of Hallopeau coexisting with generalized pustular psoriasis with spesolimab: a case report. Front Immunol 15:1338285\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eAssan F, Husson B, Hegazy S, Seneschal J, Aubin F et al (2022) Groupe de Recherche sur le Psoriasis de la Soci\u0026eacute;t\u0026eacute; Fran\u0026ccedil;aise de Dermatologie. Palmoplantar pustulosis and acrodermatitis continua of Hallopeau: demographic and clinical comparative study in a large multicentre cohort. J Eur Acad Dermatol Venereol 36(9):1578\u0026ndash;1583\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKim M, Yang S, Kim BR, Youn SW (2021) Nail involvement features in palmoplantar pustulosis. J Dermatol 48(3):360\u0026ndash;365\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eBenzian-Olsson N, Dand N, Chaloner C, Bata-Csorgo Z, Borroni R, Burden AD et al (2020) ERASPEN consortium and the APRICOT and PLUM study team. Association of Clinical and Demographic Factors with the severity of Palmoplantar Pustulosis. JAMA Dermatol 156(11):1\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eHuang CM, Tsai TF (2020) Clinical characteristics, genetics, comorbidities and treatment of palmoplantar pustulosis: A retrospective analysis of 66 cases in a single center in Taiwan. J Dermatol 47(9):1046\u0026ndash;1049\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eIgari S, Yamamoto T (2023) Acrodermatitis continua of Hallopeau with psoriatic arthritis treated with biologics and apremilast. JEADV Clin Pract 2:601\u0026ndash;604\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eRitasman Baisya Acrodermatitis Continua of Hallopeau in A Case of Psoriatic Arthritis. J Clin Images Rep. 2023 SRC/JCIR-120.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eTouraj Khosravi-Hafshejani, Zhou Y, Dutz JP (2019) Acrodermatitis Continua of Hallopeau with Psoriatic Arthritis. J Rheumatol Apr 46(4):437\u0026ndash;438\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eLefkir S, Slimani S, Brahimi N, Ladjouze-Rezig A (2015) Successful treatment of Acrodermatitis continua of Hallopeau associated with psoriatic arthritis with adalimumab. Eur J Rheumatol 2(2):78\u0026ndash;79\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eJo SJ, Park JY, Yoon HS, Youn JI (2006) Case of acrodermatitis continua accompanied by psoriatic arthritis. J Dermatol 33(11):787\u0026ndash;791\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMarrakchi S, Guigue P, Renshaw BR, Puel A, Pei XY, Fraitag S et al (2011) Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 365:620\u0026ndash;628\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eJordan CT, Cao L, Robertson ED et al (2012) Rare and common variants in CARD14, encoding an epidermal regulator of NF-jB, in psoriasis. Am J Hum Genet 90:796\u0026ndash;808\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSetta-Kaffetzi N, Simpson MA, Navarini AA et al (2014) AP1S3 mutations are associated with pustular psoriasis and impaired Toll-like receptor 3 trafficking. Am J Hum Genet 94:790\u0026ndash;797\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eRobinson A, Van Vorhees AS, Hsu S et al (2012) Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol 67:279\u0026ndash;288\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eImafuku S, Honma M, Okubo Y et al (2016) Efficacy and safety of secukinumab in patients with generalized pustular psoriasis: a 52-week analysis from phase III open-label multicenter Japanese study. J Dermatol 43:1011\u0026ndash;1017\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eGalluzzo M, D'Adamio S, Teoli M, Bianchi L, Talamonti M (2019) Biologic therapy for acrodermatitis continua of Hallopeau: Successful treatment with secukinumab and review of the literature. Dermatol Ther 32(3):e12899\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eUmezawa Y, Ozawa A, Kawasima T et al (2003) Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity. Arch Dermatol Res. ;295\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKodali N, Blanchard I, Kunamneni S, Lebwohl MG (2023) Current management of generalized pustular psoriasis. Exp Dermatol 32(8):1204\u0026ndash;1218\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"},{"header":"Table","content":"\u003cp\u003eTable 1 \u0026ndash; A comprehensive review of GPP cases with Psoriatic arthritis in the literature\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"705\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eAuthor\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eCountry\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003eGender /age\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003eGPP features\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eAxial \u0026nbsp; \u0026nbsp; features\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003ePeripheral + dactylitis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003eSystemic\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003eTreatment\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eKimura et al, \u003csup\u003e(3)\u003c/sup\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e(2012)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eJapan\u003c/p\u003e\n \u003cp\u003eCase report\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003e36 year female\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003ePustules\u003c/p\u003e\n \u003cp\u003e( lake of pus )\u0026nbsp;\u003c/p\u003e\n \u003cp\u003ePsoriatic plaques , no nail\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003cp\u003eSacroilitis /enthesitis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003eDIP , wrist , left knee , rt foot\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003eFever\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eRaised ESR , CRP ,MMP\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003eAdalimumab\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eZenke Y et al \u003csup\u003e(2)\u003c/sup\u003e\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e(2022)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eJapan\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eArticle\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003e7 GPP + PSA \u0026ndash; 4 male\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eMedian age 39 years\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003eNo separate description\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eSpinal involve -5\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;Enthesitis \u0026gt; 3 in 3 patients\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003ePA \u0026gt; 4 in 3\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eDactylitis \u0026gt;3 in 2 patients \u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003eNo separate description\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003eManagement was not included\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eSerrao V et al \u003csup\u003e(4)\u003c/sup\u003e\u003c/p\u003e\n \u003cp\u003e(2008)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003ePortugal\u003c/p\u003e\n \u003cp\u003eCase report\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003eMale\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003ePustular eruption\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eNo mention\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003eSevere arthritis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003eFever\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003eMethotrexate with steroid\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eSeki K et al \u003csup\u003e(5)\u0026nbsp;\u003c/sup\u003e\u003c/p\u003e\n \u003cp\u003e(1997)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eJapan\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003e31 Male\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003ePustular eruption\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eNo mention\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003eSymmetric polyarthritis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003eFever\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003eMethotrexate\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eFreeman S et al \u003csup\u003e(6)\u003c/sup\u003e\u0026nbsp; (1979)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eAustralia\u003c/p\u003e\n \u003cp\u003eCase report\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003e52 , F\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003ePustular eruption\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eSacroilitis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003eKnee , shoulder , limb ,foot\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003ePericarditis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; ----------\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eHong S B et al \u003csup\u003e(7)\u003c/sup\u003e\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e(2005)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eKorea\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eCase report\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003e27 , M\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003ePustular eruption on plaque psoriasis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eNot mentioned\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003ePolyarthritis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003eFever\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eRaised ESR ,CRP\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003eWithdrawal of cyclosporine precipitate GPP , MTX\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eFukunaga Y et al \u003csup\u003e(8)\u003c/sup\u003e\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e(2008)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eCase report\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eJapan\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003e28, M\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003eGPP , same time arthritis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eNot mentioned\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003eElbow , knee , wrist , ankle\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; ------\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e---------\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 90px;\"\u003e\n \u003cp\u003eChung MD et al , \u003csup\u003e(9)\u003c/sup\u003e\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e(2025 )\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 87px;\"\u003e\n \u003cp\u003eCase report\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eUSA\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 65px;\"\u003e\n \u003cp\u003e39,M\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003eGPP \u0026amp; plaque psoriasis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 81px;\"\u003e\n \u003cp\u003eNot mentioned\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 95px;\"\u003e\n \u003cp\u003ePolyarthritis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 88px;\"\u003e\n \u003cp\u003eFever\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eHeart failure , obesity\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 106px;\"\u003e\n \u003cp\u003eInfliximab\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eBimekizumab\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"egyptian-rheumatology-and-rehabilitation","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"","sideBox":"","snPcode":"43166","submissionUrl":"https://submission.springernature.com/new-submission/43166/3","title":"Egyptian Rheumatology and Rehabilitation","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Open","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"GPP, ACH, PsA, Adalimumab, Methotrexate","lastPublishedDoi":"10.21203/rs.3.rs-7199418/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7199418/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eBackground - The simultaneous occurrence of generalised pustular psoriasis (GPP), acrodermatitis continua of Hallopeau (ACH), and Psoriatic arthritis (PsA) in the same patient is infrequently documented in the literature. However, diagnosis of PsA, especially axial PsA, is often missed in everyday clinical practice.\u003c/p\u003e\u003cp\u003eCase - A 21-year-old male presented with a generalised scaly, pus-filled rash affecting his face, chest, back, and upper and lower limbs. He also reported experiencing inflammatory polyarthritis in his toes and fingers for the past month, along with right-sided lower back pain during this same period. Additionally, he had a history of intermittent pustular rashes on his nails over the past year, worsening during this episode. Examination revealed a generalised pustular psoriasis rash, with a Psoriasis Area Severity Index (PASI) score of 38.5. There was also evidence of nail psoriasis with (ACH) and a nail psoriasis severity index (NAPSI) of 48/80. His Disease Activity Index for Psoriatic Arthritis (DAPSA) score was recorded at 34. He also had hepatitis and neutrophilic leukocytosis. Following the initiation of therapy with Adalimumab and methotrexate, he experienced significant improvement in both cutaneous and musculoskeletal symptoms, and his systemic features related to hepatitis were also resolved.\u003c/p\u003e\u003cp\u003eConclusion - This is a unique case of GPP with ACH with non-radiographic axial and peripheral Psoriatic arthritis with significant improvement with methotrexate and biological therapy. It highlights the importance of multidisciplinary management of any psoriasis cases.\u003c/p\u003e","manuscriptTitle":"A unique combination of Generalised Pustular Psoriasis (GPP), Acrodermatitis Continua of Hallopeau (ACH), and psoriatic arthritis (both axial and peripheral) in a young male – a case-based review in literature","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-11-08 15:18:37","doi":"10.21203/rs.3.rs-7199418/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-11-24T20:18:27+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-11-23T10:11:06+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"252348467350369429898688057075868838445","date":"2025-11-10T10:34:40+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-11-08T09:25:48+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"177171704524587030791224614026330891368","date":"2025-11-08T09:00:00+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-10-22T19:38:04+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-08-08T23:15:36+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-08-06T06:52:31+00:00","index":"","fulltext":""},{"type":"submitted","content":"Egyptian Rheumatology and Rehabilitation","date":"2025-08-03T10:09:53+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"egyptian-rheumatology-and-rehabilitation","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"","sideBox":"","snPcode":"43166","submissionUrl":"https://submission.springernature.com/new-submission/43166/3","title":"Egyptian Rheumatology and Rehabilitation","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Open","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"7db327a8-5261-416a-a336-537395002ef1","owner":[],"postedDate":"November 8th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"under-review","subjectAreas":[],"tags":[],"updatedAt":"2025-12-30T19:38:25+00:00","versionOfRecord":[],"versionCreatedAt":"2025-11-08 15:18:37","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7199418","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7199418","identity":"rs-7199418","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}