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Atypical Bullous Pemphigoid – three non-blistering presentations.
Authors and F O’Sullivan, L Barnes¹, M Murphy

Departments of Dermatology, South Infirmary Victoria University
Hospital, Cork, and St James’s Hospital¹, Dublin
Bullous pemphigoid is the most common autoimmune blistering dermatosis seen in elderly populations. The classic presentation of tense pruritic blisters developing on either healthy or erythematous skin is relatively easily recognised. However rarer clinical variants exist. We report three atypical presentations of bullous pemphigoid; prebullous pemphigoid, nodular pemphigoid and “invisible” pemphigoid. Case 1: A 79-year-old lady presented with a six week history of an intensely pruritic generalised rash, treated unsuccessfully with moderate potency topical steroids and anti-histamines. Examination revealed widespread urticated erythematous plaques without blistering. A clinical diagnosis of prebullous pemphigoid was made. Biopsy showed acute spongiosis with oedema and an eosinophil rich infiltrate. Subepidermal clefting was noted but without blistering. Direct immunofluorescence however revealed linear IgG and C3 deposition along the basement membrane zone. She was treated with high potency topical steroids and her rash has resolved. Case 2: A 73-year-old nun presented with an eighteen month history of a pruritic rash. Examination revealed excoriated nodular lesions, particularly on the lower limbs and feet. There were no bullae. Due to the prolonged intractable nature of the nodules a biopsy was performed which showed non specific features consistent with chronic dermatitis. However direct immunofluorescence displayed deposition of linear C3 along the dermo-epidermal junction. Circulating basement membrane antibodies were also present (titre 1:2560). A diagnosis of pemphigoid nodularis was made and she responded well to oral steroids. Case 3: A 78-year-old man presented with a four month history of pruritus. On examination, he had a few excoriations but no primary rash. Standard pruritic screen was negative; he was treated with oral doxepin with some initial improvement. Three years later he represented with persistent pruritus, again without any primary rash. Histology was non specific showing a mild lymphocytic dermal infiltrate, no increase in eosinophils, but direct immunofluoresence showed linear IgG and C3 along the DEJ, prompting a diagnosis of ‘invisible bullous pemphigoid’. This has been rarely reported in the literature1. Three year later he has remained itch free on mycophenolate mofetil and 5mg prednisolone without ever having developed a blister. These cases illustrate the importance of diagnostic biopsies with immunofluoresence in elderly patients with pruritic skin disorders. 1. Alonso-Llamazares J, Rogers RS 3rd, Oursler JR et al. Bullous pemphigoid presenting as generalized pruritus: observations in six patients. Int J Dermatol 1998: 37(7): 508



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