Dyshidrosiform Bullous Pemphigoid: Case Reports and Review

Abstract

Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla, and groin. It is caused by circulating and tissue-bound autoantibodies directed against bullous pemphigoid antigen 1 or bullous pemphigoid antigen 2 or both. Dyshidrosiform bullous pemphigoid is a rare variant of bullous pemphigoid, and it usually presents as itchy, potentially hemorrhagic, or purpuric blisters on the palms and/or soles of elderly individuals; subsequently, typical bullous lesions of bullous pemphigoid appear on other body sites. In our study, we report the features of two men with dyshidrosiform bullous pemphigoid and review the characteristics of individuals with this rare subtype of bullous pemphigoid. Including the men whose condition is described in this paper, at least 72 patients with dyshidrosiform bullous pemphigoid have been reported so far. However, complete features of the condition have not been described for all of the individuals.

A case report of dyshidrotic bullous pemphigoid developing after partial anterior circulation ischaemic stroke

“…DBP is a clinical variant of BP distinguishing feature of which is the initial or persistent palmoplantar localization of the lesions. Bullae or vesicles can be haemorrhagic in nature, and purpuric lesions are common [ 2 ]; as in the case described, some patients might have prodromal symptoms, mainly eczematous or papular eruptions [ 3 ].…”

“…DBP has been associated with neurologic, psychiatric and cerebrovascular disorders. There are only three cases reported in the literature of DBP in patients with a past history of stroke [ 3 ]; however, to the best of our knowledge, no previous case has been reported of DBP developing immediately after a cerebrovascular event. Stroke as a risk factor for developing BP has been studied in the literature.…”

Dyshidrosiform Bullous Pemphigoid

“…These included dapsone (eight individuals whose dose ranged from 50 to 200 mg daily, median 150 mg daily) [ 4 , 6 , 17 , 20 , 23 , 25 , 28 , 29 ], oral antibiotics (three individuals), such as erythromycin [ 22 ], doxycycline [ 16 ] or tetracycline [ 28 ], and nicotinamide (one individual) [ 28 ]. Topical corticosteroids (17 individuals) were also used as an adjunctive therapy [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 ].…”

“…Immunosuppressant drugs, either alone or as a corticosteroid agent, were also used to treat dyshidrosiform bullous pemphigoid. Azathioprine was used in four individuals; the dose ranged from 100 to 150 mg daily (median, 100 mg daily) [ 22 , 24 , 33 , 35 ]. Cyclophosphamide was used in one individual; the daily dose was 100 mg [ 22 ].…”

“…Pruritic tense subepidermal blisters usually develop in flexural areas that are proximally located, such as the axilla and the groin. The blisters can be localized or widespread [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 ].…”