Pemphigus Vegetans Localized To Surgical Site Scars: A Singular Case Scenario.

Dear Editor, Pemphigus vegetans is a rare variant of pemphigus vulgaris, constituting 1–2% of pemphigus. It is characterized by vegetative plaques in intertriginous areas and the oral mucosa.[] The age of onset is fifth to sixth decade of life, with a female-to-male ratio of 1:3. There are two types of pemphigus vegetans, namely, the Hallopeau type and the Neumann type. The lesions are usually flexural, although non-flexural lesions are also reported. We hereby present a case of Neumann-type pemphigus vegetans presenting only at sites of surgical scars. A 50-year-old lady came to a dermatology outpatient with complaints of a painful, irregularly raised lesion with blood-tinged pus discharge over the right axilla for 2 months. Lesions initially eroded, gradually increasing in size to form elevated plaques. One month later, a similar lesion developed at the cesarean scar site. The patient was diagnosed with pemphigus vulgaris 9 years ago and was treated with steroids and immunosuppressants. Surgery was performed for growth over the right axilla 3 years ago. Subsequently, episodes of similar lesions occurred only at the right axilla and lower abdominal scar sites. The patient is a known case of systemic hypertension. On mucocutaneous examination, a well-defined, soft, friable vegetating plaque of size 20 × 15 cm with blood-tinged purulent discharge and surrounding erythema was seen over the right axilla, extending to the arm [Figure 1a]. A similar vegetating lesion of size 10 × 3 cm was seen over the lower abdomen at the Pfannenstiel incision site [Figure 1b]. Multiple erosions were seen over the bilateral buccal mucosa, palate, and gingiva, and a fissured tongue was noted. Erosions were also noted over labia majora. Perilesional Nikolsky sign was positive. Histopathological examination of the right axillary lesion showed hyperkeratosis, acanthosis with pseudoepitheliomatous hyperplasia, suprabasal bulla with acantholysis, and an upper dermal neutrophilic and eosinophilic infiltrate [Figure 2a]. Direct immunofluorescence revealed intercellular immunoglobulin (IgG) and C3 deposits in the epidermis in a fishnet pattern [Figure 2b]. The diagnosis of pemphigus vegetans was made based on the clinical features, histopathology, and immunofluorescence findings. She was treated with oral prednisolone and cyclophosphamide, to which the vesiculobullous lesions responded; however, vegetative lesions did not resolve. Hence, intralesional triamcinolone (10 mg/mL) was given once every 4 weeks. Significant improvement was noted with each session, and the oral steroid and cyclophosphamide could be tapered [Figure 3]. The patient is on regular follow-up. Pemphigus vegetans present with two subtypes: a mild Hallopeau type and a severe Neumann type. In the Neumann-type, vesicles and bulla rupture, forming hypertrophic granulating erosions that bleed easily and are chronic. They exude serum and pus, with pustules seen at the edges. Few lesions in non-intertriginous areas are dry, painful, and hyperkeratotic and show fissuring. In the Hallopeau type, pustules progress to vegetative plaque, which is usually self-limiting. Oral mucosa may be involved with cerebriform changes on the tongue, which is a diagnostic clue for pemphigus vegetans.[] Histopathology of early lesions shows eosinophilic spongiosis and eosinophilic microabscesses, with suprabasal clefts and acantholytic cells. A biopsy of older and persistent lesions shows hyperkeratosis, acanthosis, and pseudoepitheliomatous hyperplasia with variable infiltrates of neutrophils, eosinophils, and lymphocytes. Eosinophilic infiltrates and microabscesses are more prominent in the Hallopeau type.[] Circulating autoantibodies can be detected by indirect immunofluorescence or using enzyme-linked immunosorbent assay (ELISA), which can be used to monitor disease activity. The mechanism of koebnerization in pemphigus is controversial. Trauma to the skin or mucosa leads to epitope spreading and overexpression of antigens causing acantholysis even in low titer of antibodies. On healing, poorly differentiated keratinocytes are vulnerable to the antibodies. Hence, it is a result of genetic, environmental, and immunological factors. Balighi et al. reported two cases of pemphigus vulgaris at the site of trauma.[] These lesions mimic pyodermatitis-pyostomatitis vegetans, vegetative pyoderma gangrenosum, pemphigoid vegetans, IgA pemphigus, paraneoplastic pemphigus, Hailey–Hailey disease, halogenoderma, blastomycosis-like pyoderma, cutaneous tumors, such as endometriosis and squamous cell carcinoma, and deep fungal infections [Table 1].[] A skin biopsy helps confirm the diagnosis. Cutaneous diseases documented at the surgical site include pemphigus vulgaris, pyoderma gangrenosum, psoriasis, and erosive pustular dermatosis.[] Systemic corticosteroids are the mainstay of treatment. Adjuvant immunosuppressants and immunomodulatory agents may be used. In our patient, as there was no significant response to 4 months of cyclophosphamide and oral steroids, intralesional triamcinolone was given, which resulted in an excellent response after six sessions. Similarly, Cetinarslan et al. reported a case of pemphigus vegetans in the umbilical and inguinal regions treated successfully with four sessions of intralesional steroid at a dose of 10 mg/mL every 2 weeks.[] Other topical measures include the application of gauzes soaked in steroids twice daily in the intertriginous region and the application of pure silver nitrate in pencil form. Resistant pemphigus vegetans can be treated with surgical excision, carbon dioxide lasers, and tumor necrosis factor-alpha (TNF-α) inhibitors.[] The localization of pemphigus vegetans at the sites of surgical scars, as seen in this patient, is an interesting presentation in the background of a long-standing pemphigus vulgaris. Intralesional corticosteroids are an efficient treatment modality with excellent clinical response and negligible systemic side effects. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1 Amagai M. 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