Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

Abstract

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.

Paraneoplastic Pemphigus

“…disease, but a syndrome characterized by the presence of mucocutaneous and non-cutaneous pathology associated with neoplasia [3]. More than 260 case reports of PNP have been documented since that time [1,4,5] and about 20 of the reported patients were children or adolescents.…”

IgA Pemphigus in a Child – a Case Report

“…Patients with the SPD type sometimes show irregular erythematous skin lesions with vesiculopustules, erosions and desquamation resembling the features of subcorneal pusular dermatosis (Sneddon-Wilkinson disease) (15). The IEN type demonstrates a characteristic clinical feature, the so-called "sunflower-like" configuration (16). A herpetiform appearance has also been reported (17).…”

“…Acantholysis in IgA pemphigus is much milder than in classic pemphigus. Characteristically, the clefts and pustules localize in the subcorneal region in SPD type IGAP, whereas they are present in the entire or mid epidermis in IEN type IGAP (3,16). In the upper dermis, a superficial perivascular and interstitial infiltrates of lymphocytes, neutrophils and sometimes eosinophils are present (19) Desmocollin 1 (Dsc1) has recently been identified as the target antigen of the SPD type (15).…”

“…In SPD type, IgA deposition is seen predominantly in the upper epidermal layers, whereas in IEN type, it is seen throughout the epidermis or restricted to the lower epidermis. IgG or complement component C3 is also sometimes deposited, but is weaker than IgA (3,5,16,23).…”

“…Enzyme-linked immunosorbent assay (ELISA) is used for the diagnosis of IGAP and for detection of autoantibodies in individual patients (16,27). The specificity and sensitivity of Dsc and ELISA is not very high compared with immunofluorescence study using Dsc-transfected COS-7 cells (4,28).…”