{"paper_id":"15d732eb-6193-494c-a13c-920ad2c85a5f","body_text":"UC Davis\nDermatology Online Journal\nTitle\nDanazol-induced Stevens-Johnson syndrome in a patient with systemic lupus \nerythematosus\nPermalink\nhttps://escholarship.org/uc/item/24v513b9\nJournal\nDermatology Online Journal, 21(1)\nAuthors\nKoh, WL\nTay, YK\nKoh, MJA\nPublication Date\n2015\nDOI\n10.5070/D3211025452\nCopyright Information\nCopyright 2015 by the author(s).This work is made available under the terms of a \nCreative Commons Attribution-NonCommercial-NoDerivatives License, available at \nhttps://creativecommons.org/licenses/by-nc-nd/4.0/\neScholarship.org Powered by the California Digital Library\nUniversity of California\n\n \nFigure 1. Bullae, erosions and atypical targets on the palms  \n \nVolume 21 Number 1  \nJanuary 2015 \n \nLetter \nDanazol-induced Stevens-Johnson syndrome in a patient with systemic lupus erythematosus \nWL Koh1, YK Tay1, MJA Koh2 \nDermatology Online Journal 21 (1): 17 \n1Department of Dermatology, Changi General Hospital, Singapore \n2Dermatology Service, KK Women’s and Children’s Hospital, Singapore \nCorrespondence:  \nDr Wei Liang Koh \nE-mail address: Koh_Wei_Liang@hotmail.com \nAddress: Department of Dermatology, Changi General Hospital, 2 Simei Street 3 Singapore 529889 \nTelephone number: +6593697325 \nFax number: +6567816202 \n \n \n \n \nAbstract \nA patient with Stevens-Johnson syndrome related to danazol is presented.  \nKeywords: Stevens-Johnson syndrome, Danazol, Systemic lupus erythematosus \nLetter to the Editor \nCase synopsis \nA 19-year-old woman with a 5-year history of \nsystemic lupus erythematosus (SLE), presented with \n2 days of facial rash and oral ulcers. This eruption \nwas significantly different from the malar erythema \nthat characterized her earlier SLE flares. Her long-\nterm medications included oral tacrolimus 2 mg/day \nand prednisolone 15 mg/day. Significantly, she had \nbeen started on oral danazol 200 mg/day for \nautoimmune haemolytic anaemia, 2 weeks prior to \nthe current admission.  \nOn examination, the patient was febrile. There were \ndiscrete to confluent, dusky purpuric macules, \npapules, and targetoid lesions, over the forehead, \ncheeks, neck, and arms. Similar papules and plaques \nwith central bullae were seen on the palms (Figure \n1). Erosions were noted on the lips, hard palate, and \nvulvae. The conjunctivae were normal. The \ncutaneous lesions affected 3% of her body surface \narea. Systemic examination was unremarkable except \n\n \nFigure 2. Lesional biopsy showing subepidermal blister with full thickness \nepidermal necrosis and adjacent interface changes: basal vacuolar alteration, \nnecrotic keratinocytes and exocytosis of lymphocytes. (Haematoxylin and \neosin, original magnification x 40) \nfor mild conjunctival pallor. Lesional skin biopsy from the right arm showed a subepidermal blister with full thickness \nepidermal necrosis with interface changes adjacent to the blister (Figure 2). Alcian blue stain did not show increased dermal \nmucin. These changes were consistent with Stevens-Johnson syndrome (SJS). Direct immunofluorescence (DIF) from \nperilesional skin was negative.  \nA diagnosis of danazol-induced SJS was made and \ndanazol was stopped. She was started on intravenous \nmethylprednisolone 1 g/day for 3 days followed by \nintravenous hydrocortisone 100 mg 8 hourly for 3 \ndays. The eruption was also treated with \nbetamethasone valerate 0.1%/ clioquinol 3% cream. \nRe-epithelisation was noted 10 days later. She was \ndischarged on oral prednisolone 30mg/day, presently \ntapered to 10 mg/day. \nDanazol is an attenuated androgen indicated for \ntreating endometriosis, fibrocystic breast disease, and \nhereditary angioedema [1]. It has been used \nsuccessfully as a second-line agent for the treatment \nof SLE-associated hematological abnormalities such \nas thrombocytopenia and autoimmune hemolytic \nanemia. [2, 3] Its mechanism of action is unknown \nbut androgens appear to display potent \nimmunoregulatory and anti-inflammatory properties \nin modulating the development of SLE in animal \nstudies\n [2]. The main reported side effects include \nweight gain, virilization, hepatitis, and rashs. \nAlthough SJS has been listed as a rare reaction in the \nproduct insert, causal relation has not been confirmed \n[1]. We believe this is the first case report of danazol-induced SJS. It has been suggested that SJS/ toxic epidermal necrolysis \n(TEN) happens at a higher frequency in patients with collagen-vascular disorders [4]. However, there is no clear evidence at \npresent that SLE by itself is a risk factor. SJS/TEN-like SLE was a possible consideration in the differential diagnosis in our \npatient but the clear drug relationship, acute clinical course, genital involvement, and rapid improvement upon cessation of \ndrug suggest otherwise [5]. Both drug-induced SJS/TEN and SJS/TEN-like SLE can present with similar clinical and \nhistological findings. DIF is negative in drug-induced SJS/TEN and may or may not be positive in SJS/TEN-like SLE [5]. \nIndeed, some authors consider Rowell’s syndrome (erythema multiforme with SLE), SJS/TEN-like SLE, and SJS/TEN as part \nof a same clinical spectrum, which may exhibit full-thickness necrosis of the epidermis (acute syndrome of apoptotic pan-\nepidermolysis) [4, 5]. It is important to be cognizant of this rare, but severe cutaneous adverse reaction when prescribing \ndanazol. \nReferences \n1. Danazol capsule – detailed prescribing information. In: Danazol capsule full prescribing information, dosage and side \neffects. MIMS.com USA. Available at: http://www.mims.com/USA/drug/info/Danazol%20Capsule/?type=full. Accessed \nAug 23, 2014.  \n2. Letchumanan P, Thumboo J. Danazol in the treatment of systemic lupus erythematosus: a qualitative systemic review. \nSemin Arthritis Rheum 2011 Feb; 40 (4):298-306. [PMID: 20541792] \n3. Avina-Zubieta JA, Galindo-Rodriguez G, Robledo I, et al. Long term effectiveness of danazol, corticosteroids and \ncytotoxic drugs in the treatment of hematologic manifestations of systemic lupus erythematosus. Lupus 2003; 12(1):52-7. \n[PMID: 12587827] \n4. Ziemer M, Kardaun SH, Liss Y, Mockenhaupt M. Stevens-Johnson syndrome and toxic epidermal necrolysis in patients \nwith lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature. Br J \nDermatol 2012 Mar; 166(3):575-600. [PMID: 22014091] \n5. Lee HY, Tey HL, Pang SM, Thirumoorthy T. Systemic lupus erythematosus presenting as Stevens-Johnson syndrome and \ntoxic epidermal necrolysis: a report of three cases. Lupus 2011 May; 20(6):647-52. [PMID: 21148602]","source_license":"CC0","license_restricted":false}