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


OBJECTIVE
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].


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.


CONCLUSIONS
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.
Department of Dermatology, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India; Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.
Joyeeta
J
Chowdhury
Department of Dermatology, Mata Gujri Memorial Medical College, Kishanganj, Bihar, India.
Pijush Kanti
PK
Datta
Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.
Satyendra Nath
SN
Chowdhury
Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.
Nilay Kanti
NK
Das
Direct immunofluorescence; Mahajan's score; pemphigus area and activity score; pemphigus foliaceus; pemphigus vulgaris