Onychomadesis: A marker of disease activity in bullous pemphigoid?

A 65-year-old male patient, a known case of bullous pemphigoid, presented with multiple tense, fluid-filled bullae over the trunk, and extremities for the past 7 months. He had onychomadesis of all fingernails on the right hand, left index fingernail, and left ring fingernail [Figure 1a and b]. The patient had periungual disease activity on most nails with onychomadesis. The diagnosis of bullous pemphigoid had been made earlier based on histopathology and direct immunofluorescence. However, he was not on regular treatment. We made a final diagnosis of bullous pemphigoid with onychomadesis and started him on prednisolone 0.5 mg/kg/day with good improvement. Onychomadesis is the separation of the proximal nail plate from the nail matrix and nail bed. The proximal nail fold, nail matrix, nail bed, and hyponychium express the target antigens of bullous pemphigoid.^[1] In addition, IgG and C3 deposition have been noted in the basal membrane zone of the nail bed of patients with bullous pemphigoid.^[2] The prolonged inflammation of the nail matrix and periungual bullae might be implicated in the development of onychomadesis. Onychomadesis may also point toward disease activity in patients with bullous pemphigoid.

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Figure 1:

(a and b) Onychomadesis of all fingernails on the right hand, left index fingernail, and left ring fingernail with periungual inflammation.

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