Bullous pemphigoid presenting as generalized pruritus: observations in six patients

Abstract

Background: Bullous pemphigoid is a chronic immunobullous disease of the elderly. Classically, tense, pruritic blisters develop on normal or erythematous skin. These may be preceded by a prodromal pruritic, urticarial, or eczematous eruption. Occasionally, patients may develop generalized pruritus without blisters as a prodrome of bullous pemphigoid.

Methods: The records of the patients were reviewed. Biopsy specimens were studied by light and immunofluorescence microscopy. Serum specimens were studied by indirect immunofluorescence techniques including the salt-split skin technique.

Results: We studied six elderly patients presenting with generalized pruritus as the dominant or single presenting feature of early bullous pemphigoid. Two of the six had rare vesicles at presentation. All had excoriations and one each presented with minimal urticarial or eczematous papules. Routine skin biopsies were largely nonspecific. All patients had confirmation of their diagnosis by either indirect or direct immunofluorescence testing or both. All six patients had their disease completely controlled by their treatment.

Conclusions: The clinical presentation of the six patients in our series and the eight previously reported patients should be regarded as an unusual prodromal manifestation of bullous pemphigoid characterized by generalized pruritus without primary skin lesions. This presentation could be described as "pruritic pemphigoid," because it joins the remarkable clinical finding of generalized pruritus with the underlying diagnosis of bullous pemphigoid. Elderly patients with severe or persistent unexplained generalized pruritus merit immunofluorescence testing to exclude bullous pemphigoid as the cause of the generalized pruritus. Establishing an early diagnosis permits the prompt institution of effective therapy with dapsone or systemic corticosteroids with an excellent prognosis for complete control of the disease.