A Clinicopathological Study Of 22 Cases Of Pemphigus
Dr Leena J B , Assistant Professor, Fr Muller Medical College , Kankanady, Mangalore.
Background : The term pemphigus refers to a group of autoimmune blistering diseases of the skin and the mucous membranes . Pemphigus affects 0.1- 0.5 patients per 1,00,000 population per year . Aim: To evaluate the clinical findings in association with histopathological findings, to study the histopathological findings in various vesiculobullous lesions of skin and to confirm with immunofluorescence wherever possible. Materials And Methods: Histopathological evaluation of biopsies received from the department of Dermatology was done in the Department of Pathology, JSS hospital, Mysore, from August 2006 to July 2008. Results: A total number of 648 skin biopsies were received, out of which 22 cases were of the pemphigus group of diseases. Pemphigus vulgaris accounted for 81 %( 18) of cases and pemphigus vegetans accounted for 9.01 %( 2) of cases. The less common types were pemphigus erythematosus and IgA pemphigus constituting 4.1 %( 1) of the cases. Pemphigus vulgaris formed the most common variant. The male to female ratio was 1.35:1. The age incidence was between 21-70 years .The trunk and extremities were the frequently involved sites and had classical flaccid bullae in 90.9% of cases. Oral mucosa was involved in 66% of the cases of pemphigus vulgaris. Apart from the common histopathological changes in pemphigus vulgaris, we also observed: 1.Spongiosis of adjacent epidermis (11 cases) 2. Acantholysis in adnexa (4cases) 3. Hair shaft and sebaceous gland inside bulla (biopsy artifact)(3cases). Both the cases of pemphigus vegetans showed hyperkeratosis, papillomatosis and acanthosis, along with suprabasal bulla containing inflammatory infiltrate. Conclusion; Histopathological features were conclusive in most of the cases of primary vesiculobullous lesions of the skin. The study also revealed additional histopathological findings which are not encountered normally. Analysis of the subtle light microscopic features apart from the classical diagnostic features assisted in the diagnosis of difficult cases. The immunofluorescence study done, helped in confirming the diagnosis where histopathology and clinical features alone were inconclusive.