Abstract

Hailey-Hailey disease (HHD) or Familial Chronic Benign Pemphigus (FBCP) is an uncommon autosomal-dominant bullous disorder characterized by recurrent vesicular and erosive lesions at friction prone inter-triginous sites. It runs in families. Diagnosis is by strong suspicion in any chronic intertriginous dermatoses with similar disease running in parents & siblings with typical histopathology showing extensive acantholysis described as characteristic dilapidated brick wall - appearance. Often treated with various therapeutic options with various periods of remission. Here we describe two cases with itchy red rash in disease prone areas over several areas with impartial relief, strong family history, on examination showing typical clinical picture, confirmed by biopsy. Both are managed with non-steroidal drugs like oral MgD3, low dose oral naltrexone with short course of intermittent topical mid potent steroid with antifungal / antibacterial combination creams leading to a dramatic improvement. These twin case series highlight the ways to avoid unnecessary delay in diagnosis so as to cut short, prolonged use of steroids (oral & topical) especially in flexural dermatoses prone for striae atrophicans. Non - steroidal / non immunosuppressive therapy given in them helped in avoiding long term deleterious side effects.