Abstract
INTRODUCTION Erythrodermic Bullous Pemphigoid, first described by Tappeiner et al in 1982, is a rare variant of Bullous Pemphigoid characterized by erythroderma along with blister formation with only eight patients reported so far. The present case is the youngest to develop erythrodermic bullous pemphigoid.
CASE DESCRIPTION 45-year-old Indian woman presented with erythroderma for 31/2 months and bullous eruption for 2 weeks. It started with erythema and scaling in the neck accompanied with bullous eruption which ruptured in 2–3 days.
Histopathological examination showed sub-epidermal blistering. Direct immunofluorescence (DIF) of perilesional skin demonstrated linear deposition of C3 at dermo-epidermal junction . DIF for IgG and IgA was negative. The patient was treated with 2.5 mg/kg body weight/day prednisolone and 2 mg/kg body weight/day azathioprine orally along with symptomatic treatment. She was also given three daily intravenous pulses of 100 mg dexamethasone to combat new lesions and
resistant nature of the disease. The treatment led to almost complete disappearance of lesions. Despite this improvement, the patient expired following septicemia.
DISCUSSION Erythrodermic BP, is a rare variant of BP characterized by erythroderma along with blister formation. Clinical presentation of the present patient differed in respect of age (45 years) from most of the other reported cases.
It was also observed that onset of erythrodermic bullous pemphigoid at an early age may be associated with resistance to treatment.
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References
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