CLINICAL AND HISTOLOGICAL CHARACTERIZATION OF PEMPHIGUS IN PATIENTS WITH DIRECT IMMUNOFLUORESCENCE.

Abstract

INTRODUCTION: Pemphigus is a group of life-threatening mucocutaneous autoimmune bullous disease with blister formation. Although the disease is rare but it is associated with high morbidity and mortality, if proper treatment is not taken. It is IgG-mediated autoimmune diseases of stratified squamous epithelium, like skin and oral mucosa, in which acantholysis causes blisters and erosions. The immune reaction breaks down the adhesion components and leads to epithelial cell detachment. There are several types of pemphigus; three types are associated with oral mucosa, pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus. The diagnosis of pemphigus should be promptly done as clinically it may be difficult to differentiate the different variants leading to delay or wrong treatment and therefore increasing the mortality and morbidity rates. Direct immunofluorescence (DIF) is a very sensitive test for the diagnosis of pemphigus.   

MATERIAL AND METHODS:  patients attending paediatric age group up to 12-15 years of age group were included in the study.28 clinically suspected cases of pemphigus were observed, screened and were included in the study. Histological and DIF investigations were carried out on all the patients. On the basis of histological, DIF and clinical diagnosis patients were confirmed and correlated for disease activity.

RESULTS:  26 cases were included in the study. Out of 26 cases 12 (46.2%) cases were male and 14 (53.8%) cases were female. Duration of the illness was 3.21 ± 12.14 months. Most of the cases started with mucosal involvement and then presented with oral and cutaneous presentation. 20 (76.9%) cases were diagnosedas Pemphigus vulgaris (PV), 5(19.2%) cases as pemphigus foliaceus (PF) and 1 (3.8%) was IgA pemphigus. In this study, 13 (50%) patients showed acantholytic cells on histopathological examination. In Pemphigus vulgaris (n=20) involvement of whole, lower and upper epidermis was 17 (85%), 2 (10%) and 1(5%) respectively. In Pemphigus foliaceus (n=5) whole epidermis involvement was in 1 (20%). In IgA Pemphigus only upper epidermis was involved. IgG was observed in 8 (60%) of PV cases while IgG + C3 was observed in 12 (40%) patients. In PF IgG was seen in 4 (80%) cases while IgG + C3 were observed in 1(20%) case. IgA was observed in one case.

CONCLUSION: Pemphigus even though a rare disease in children should be diagnosed promptly and reduce morbidity and mortality. DIF is a sensitive test and can be utilized for the diagnosis of pemphigus.

Key Wards: pemphix, PF, PV, DIF.