Linear IgA disease in an adult with unusual clinical features
A 19-year-old male, resident of Jalandhar presented to the skin OPD with complaints of fluid-filled lesions in both the groins of 2 weeks duration. The blisters ruptured due to friction in 2-3 days time forming raw areas. These raw areas did not increase in size but were associated with discharge of clear fluid. Mild burning sensation was present on the lesions. Similar lesions also appeared on the arms, neck, and face over the duration of 3-4 days and followed the same course. He also developed multiple oral ulcers during the same period. No constitutional or systemic symptoms were present. Patient had an episode of enteric fever 3 months back which resolved in 2 weeks time following treatment with a course of tablet ciprofloxacin. There was no history of similar illness in the past or in related family members. There was also no history suggestive of diabetes, weight loss, or any other major illness in the past.\nOn examination, he had bilateral tender inguinal lymphadenopathy. Rest of the general examination was normal. Dermatological examination revealed multiple bullae distributed over crural regions, genitalia, perianal area, face, neck, and ears [Figures -]. There was sparing of scalp, axilla, palms, and soles. The bullae were tense and contained clear fluid. Bulla spread sign and Nikolsky's sign were negative. Multiple erosions were interspersed among the bullae in above areas and few of these erosions had overlying honey colored crusts. He also had multiple urticarial lesions on the back [] and a single annular plaque with a rim of vesicles on the dorsum of the right hand. Few oral erosions were also present.\nInvestigations revealed normal hematological and biochemical parameters. ELISA for HIV was negative. X-ray chest and USG abdomen were normal. Tzanck smear from the lesion showed no acantholysis and pus culture grew Staphylococcus aureus. Skin biopsy revealed a subepidermal blister with inflammatory cells predominantly comprising neutrophils, with a few lymphocytes and eosinophils []. The dermis revealed superficial perivascular lymphomononuclear infiltrate. Direct immunoflourescence done on perilesional skin showed a linear homogenous deposition of IgA with the absence of IgG and C3 along the basement membrane zone []. The diagnosis of LAD was thus confirmed. The patient was started on Tab Dapsone 100 mg twice daily and 75% of lesions regressed in 72 h with complete remission within 2 weeks [].