Cicatricial pemphigoid of Brunsting-Perry. Immunofluorescent studies

Abstract

Six patients with a vesiculobullous eruption of the type described by Brunsting and Perry as benign pemphigoid were studied by direct and indirect immunofluorescence. All six showed linear deposits of IgG but not IgM or IgA at the epidermal-dermal junction. One case also showed C3 deposition and one patient had circulating antibasement membrane zone antibodies in a titer of 1280. These data provide strong evidence that this condition belongs to the cicatricial pemphigoid-bullous pemphigoid spectrum of disease.

Epidermolysis Bullosa Acquisita-a Sign of Cicatricial Pemphigoid?

“…The findings on direct immunofluorescence of the skin were very similar to those of pemphigoid. IgGj which was present at the BMZ in all the patients with EBA, is the commonest immunoglobulin class found in the skin in bullous pemphigoid (BP) (Jordan, Triftshauser & Schroeter, 1971) and CP (Bean et al, 1972;Holubar, Honigsmann & Wolff, 1973;Michel et al, 1977). Other immunoglobulin classes including IgM and IgA may sometimes be found in BP (Jordan et al, 1971;Provost & Tomasi, 1974) 3nd in CP (Holubar et al, 1973;Griffith et al, 1974;Michel et al, 1977;Rogers et al, 1977).…”

“…The poor response to systemic corticosteroids in three of five patients with EBA is consistent with this feature of CP. BP is sometimes responsive to dapsone (Person & Rogers, 1977b) and one of the patients with CP reported by Michel et al, 1977 was controlled with sulphapyridine. A patient with EBA who responded to dapsone and other sulphonamides has been reported (Purdy & Fairbrother, 1972).…”

Cicatricial Pemphigoid

“…A similar group of cases was reported by MacVicar and Graham''; they suggested the name localized chronic pemphigoid. Michel et al 5 have recently studied similar cases using immunofluorescence and demonstrated in vivo fixed basement membrane zone immunoglobulins and complement.…”

Localized Pemphigoid

“…Since Brunsting and Perry (I) first described localized pemphigoid as "benign pemphigoid", some cases of this disorder have been reported (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). Direct immunofluorescence studies showed that usually IgG and C3 are deposited at BMZ in localized pemphigoid (6,7,9,11,12,17). Only rarely was IgA also deposited (4,17,18).…”

“…Only rarely was IgA also deposited (4,17,18). Indirect immunofluorescence studies showed that circulating anti-BMZ antibody of the IgG class was usually represented (7,II). However, that of the IgA class has never been demonstrated.…”