Pemphigus with features of both vulgaris and foliaceus variants, associated with antibodies to 160 and 130 kDa antigens

Abstract

We report an unusual variant of pemphigus in a 44-year-old man. He presented with scaly and crusted erosions associated with pruritic vesicles and erythema mainly on the chest, abdomen, back and face. Histology showed acantholysis with neutrophilic spongiosis in the granular layer and subcorneal region of the epidermis. Intercellular IgG in the epidermis was positive on direct immunofluorescence. Indirect immunofluorescence showed intercellular antibodies at a titre of 1 : 2 in the suprabasal epidermis. Circulating autoantibodies to 130 kDa and 160 kDa antigens were detected in the patient's serum by immunoblot analysis using epidermal extracts. These two antibodies eluted from individual nitrocellulose membranes reacted with the intercellular space in the epidermis on indirect immunofluorescence. This observation suggests that these antibodies correspond to desmogleins 3 and 1, respectively. The clinical symptoms almost completely disappeared after 28 days treatment with oral prednisolone (30 mg/day), leaving brown pigmented flecks on the lesional sites. These findings suggest that this patient's pemphigus has features of both the vulgaris and foliaceus variants, with antibodies against desmogleins 3 and 1.

Pemphigus Vulgaris with No Mucosal Lesions Showing Pemphigus-Foliaceus-Like Skin Manifestations: Is There a ‘Cutaneous Type’ of Pemphigus Vulgaris?

“…In addition to case reports of the coexistence of PF and PV [10] and the transition between PV subtypes [11] , our case may provide a unique opportunity to consider a broad spectrum of pemphigus. A cutaneous-type PV may be tentatively defi ned as PV that shows the clinical appearance of PF with suprabasilar skin blisters and/or erosions and no mucosal involvement, and possesses both antiDsg1 IgG and anti-Dsg3 IgG during at least part of the period of active disease.…”

Cutaneous type pemphigus vulgaris: A rare clinical phenotype of pemphigus

“…However, it has been reported that some rare cases do not fall into these 3 phenotypes of pemphigus. [5][6][7][8][9] We report 4 cases of pemphigus that show cutaneous lesions without mucosal involvement, suprabasilar acantholysis identified histologically, and detection of both antiDsg1 and anti-Dsg3 IgG autoantibodies with a tendency of predominant index values of anti-Dsg1 as determined by enzyme-linked immunosorbent assay (ELISA).…”

Diagnosing Pemphigus foliaceus: A Retrospective Analysis of Clinical, Histological and Immunological Criteria

“…Desmoglein (Dsg) 1 is now regarded as the target of PF antibodies, though it plays a pathogenic role also in PV [9, 10]in which Dsg-3 is more important. More recently, a highly sensitive enzymatic assay has become available for both Dsg-1 and -3 [1].…”