Abstract
A 49-year-old male presented with erythroderma of 3-month duration with a flexural onset. No mucosal lesions were present, and skin biopsy showed features of acantholysis and suprabasal clefting. Direct immunofluorescence test was negative in repeated examinations. The patient was presumptively managed as pemphigus foliaceus with immunomodulators though the level of split was suprabasal rather than subcorneal, and the patient responded well to the treatment. However, his elder sister presented to the department with clinical and histopathological features of classical Hailey–Hailey disease (HHD) which resembled the pathological features found in the index case. This prompted us to review the diagnosis and retrospectively diagnose the index case as HHD presenting with erythroderma.
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