BULLOUS PEMPHIGOID PRESENTING AS GENERALIZED PRURITUS - OBSERVATIONS IN 6 PATIENTS

Background Bullous pemphigoid is a chronic immunobullous disease of th e elderly. Classically, tense, pruritic blisters develop on normal or erythematous skin. These may be preceded by a prodromal pruritic, urti carial, or eczematous eruption. Occasionally patients may develop gene ralized pruritus without blisters as a prodrome of bullous pemphigoid. Methods The records of the patients were reviewed. Biopsy specimens w ere studied by light and immunofluorescence microscopy. Serum specimen s were studied by indirect immunofluorescence techniques including the salt-split skin technique. Results We studied six elderly patients pr esenting with generalized pruritus as the dominant or single presentin g feature of early bullous pemphigoid. Two of the six had rare vesicle s at presentation, All had excoriations and one each presented with mi nimal urticarial or eczematous papules. Routine skin biopsies were lar gely nonspecific. All patients had confirmation of their diagnosis by either indirect or direct immunofluorescence testing or both. Ail six patients had their disease completely controlled by their treatment, C onclusions The clinical presentation of the six patients in our series and the eight previously reported patients should be regarded as an u nusual prodromal manifestation of bullous pemphigoid characterized by generalized pruritus without primary skin lesions. This presentation c ould be described as ''pruritic pemphigoid,'' because it joins the rem arkable clinical finding of generalized pruritus with the underlying d iagnosis of bullous pemphigoid. Elderly patients with severe or persis tent unexplained generalized pruritus merit immunofluorescence testing to exclude bullous pemphigoid as the cause of the generalized pruritu s. Establishing an early diagnosis permits the prompt institution of e ffective therapy with dapsone or systemic corticosteroids with an exce llent prognosis for complete control of the disease.