A Clinicopathological Study of Pemphigus in Eastern India with Special Reference to Direct Immunofluorescence

Abstract

Background: Pemphigus is a group of chronic autoimmune vesico-bullous disorders in which the epidermis and the basement membrane zone are the focus of attack resulting in cutaneous and mucosal blister formation. Direct immunofluorescence (DIF) test is a very sensitive test for the diagnosis.

Aim: To study the clinico histopathological patterns of pemphigus in eastern India. The study also aims to correlate DIF with clinical and histologic findings as well as severity of skin involvement [scoring systems].

Materials and methods: Total 41 patients were studied over a period of 1 year in the Post-graduate centre of Dermatology in Eastern India. DIF, histopathology and clinical data were correlated.

Results: In our study Pemphigus vulgaris (PV) was the predominant type with 32 cases followed by 8 cases of pemphigus foliaceus (PF) and a single case of IgA pemphigus. Mean age at presentation was late middle age. Majority of the patients, 26 (63.41%) initially had cutaneous involvement followed by mucosal involvement. In this study group 36 (87.80%) patients showed acantholytic cells on histopathological examination. Most patients of PV showed suprabasal blister 20 (62.50%) followed by intraspinous 5 (15.62%) and subcorneal 5 (15.62%) blister. In majority 28 (87.50%) of the PV patients IgG and C3 antibodies were deposited throughout the epidermis. The strength of antibody positivity was strong in most of the patients (71.87%). In cases of PF mostly IgG 6 (75%) antibodies were deposited in the upper epidermis. DIF intensity had poor correlation with disease activity/severity except in PF.

Conclusion: Almost 85.36% cases of pemphigus were diagnosed clinicopathologically. But 6 cases couldn't be diagnosed accurately on clinicopathological basis and in them DIF was confirmatory. Two cases of pure mucosal PV and 1 case of IgA pemphigus was confirmed by DIF. Two cases of bullous pemphigoid clinico-histologically mimicking PV were also excluded by DIF. So it appears from our study that DIF is confirmatory for diagnosis of pemphigus in all cases.

Keywords: Direct immunofluorescence; Mahajan's score; pemphigus area and activity score; pemphigus foliaceus; pemphigus vulgaris.

Figure 1

(a) Pure mucosal pemphigus vulgaris. (b) Inconclusive histology of mucosal pemphigus. (c) Direct immunofluorescence showing IgG deposition in epidermis

Figure 2

(a) Flaccid vesicles over upper and lower limbs. (b) Histopathology showing intra-epidermal blister in × 400. (c) Direct immnunofluorescence showing IgA deposits in epidermis

Figure 3

(a) Multiple flaccid and tense bullae in a generalized pattern. (b) Mucosal erosions in the same patient. (c) Histopathological examination from bulla in × 400 showing intra-epidermal split. (d) Direct immunofluorescence showing linear IgG deposit in basement membrane zone

Figure 4

(a) Erythematous scaly lesions over cheeks. (b) Histopathology showing inflammatory infiltrate in dermis in × 400. (c) Direct immunofluorescence showed IgG deposit in upper epidermis