Localized Pemphigus Vegetans without Mucosal Involvement
A 62-year-old healthy woman presented to our dermatology out-patient department with single painful red colored elevated lesion covered with pustules on the left breast for 6 months. There was no history suggestive of any systemic involvement. There was no history of fever, any drug intake, eye or mucosal complaints, no past or family history of similar illness, no history of any topical application. On examination, a single well-defined polycyclic erythematous plaque of size 6 × 7 cm with pustules concentrated mainly at the periphery of the plaque was present on left breast. KOH mount for fungus was negative, but based on clinical appearance probable diagnosis of tinea corporis was kept, and she was started on oral Fluconazole 150 mg once a week and terbinafine cream local application twice daily for 2 weeks. On subsequent follow-up after 2 weeks, there was no improvement, but the lesion in the left breast had progressed and involved the entire left breast to form a single well-defined moist, verrucous vegetating plaque of size 8×10 cm with multiple pustules, yellow to brown colored crusts at places, and some raw oozy areas. Plaque was surrounded by a broad rim of erythema []. She also developed similar verrucous lesion in the left groin on subsequent 2 weeks follow-up []. Rest of the mucocutaneous examination was within normal limits. Nikolsky's sign was negative and Tzanck smear showed eosonophils, but no acantholytic cells. Gram stain for bacteria showed only mixed inflammatory infiltrate with eosinophil predominance and acid fast bacteria staining was negative. Pus culture from the pustule was sterile. Routine laboratory studies including complete blood count, C reactive protein, erythrocyte sedimentation rate, liver and renal function tests, and chest X-ray were all within normal limits. Human immunodeficiency virus serology was negative. There was no evidence of inflammatory bowel disease. Biopsy specimens were obtained from the right breast with the differential diagnosis of pemphigus vegetans, pyoderma gangrenosum.\nPatient was started on oral prednisolone 40 mg once daily with which she showed dramatic response within a week. Plaque healed completely with hyperpigmentation at 2 weeks [Figures and ].\nHistopathological examination [Figures –] showed suprabasal cleft with acantholytic cells, numerous eosinophils, intraepidermal eosinophilic abscesses, and dense inflammatory infiltrate in the dermis. As the biopsy was taken from an early pustule, that might be an explanation for the absence of hyperkeratosis and papillomatosis in the present case. Direct immunofluorescence (DIF) revealed intercellular Immunoglobulin G (IgG) deposits in the epidermis [].