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

Abstract

Background: Clinical, histological and immunological criteria distinguish pemphigus foliaceus (PF) from pemphigus vulgaris (PV), but whether and how often they are concordant in the same patient is unknown. Methods: Seven clinical records were selected from two hospital settings for having a diagnosis of PF and the initial serum and histopathological specimens still available. Controls were 8 PV records selected in the same way. Histopathological slides were re-evaluated. Stored sera were studied by indirect immunofluorescence (IIF), Western blot and ELISA. Results: Acantholysis was superficial in all PF patients and deep in all PV patients. Mucosal lesions were not exclusive of PV. IIF was positive in 43% of PF patients. Western blot revealed desmoglein 1 in 86% of PF patients and in 25% of PV. ELISA revealed anti-desmoglein-1 antibodies in up to 71% of PF and in 62% of PV patients, in 1 failing to detect anti-desmoglein-3 antibodies. Conclusions: Histopathology remains the most reliable criterion for diagnosing PF. Western blot and ELISA, especially in combination, may be only of confirmatory value.

Immunopathological Diagnosis of Pemphigus foliaceus / Reply

“…Following up these cases we found that all of them proved positive also in IIF in the further course of the disease. We do not understand such a difference in the IIF results of our study and that of Delmonte et al [1] since both our groups used monkey oesophagus as a substrate. Histopathology is useful for the differentiation of PV and PF; however, it cannot replace a highly reliable DIF method.…”

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We read with interest the paper by Delmonte et al [1] on the histological and immunological criteria for diagnosing pemphigus foliaceus (PF). Since our experience in this field is quite different, we would like to present our findings.

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Comparison of enzyme-linked immunosorbent assay test with immunoblot assay in the diagnosis of pemphigus in Indian patients

“…[13] Delmonte et al reported raised anti-Dsg 1 antibody in 62% of PV. [14] In a Thai study, anti-Dsg 1 and Dsg 3 antibodies were found in 85.7% and 100% cases respectively in PV while in Iranian patients they were detected in 76.7% and 94.5% patients respectively. [15,16] In PF, both mean anti-Dsg1 and 3 titers were high in our study.…”

Three Cases of Transition from Pemphigus vulgaris to Pemphigus foliaceus Confirmed by Desmoglein ELISA

“…PV was confirmed by histology at presentation. Repeat biopsies would have strengthened the evidence for transition to PF as one study reported that the absence of mucosal erosions could not differentiate PF from PV reliably and that histological criteria for PF had to be fulfilled for the diagnosis of PF [13]. One PV patient in that study had antibodies to Dsg1 and Dsg3 on immunoblotting but only anti-Dsg1 antibodies were present unequivocally on Dsg ELISA testing (the anti-Dsg3 ELISA value was in the ‘gray zone’).…”