ABSTRACT
A 55 years post menopausal lady presented with puffiness of face, and a pruritic urticarial rash over face and upper trunk of one week duration with accompanying dysphagia. Clinical examination revealed an urticarial rash over face and upper trunk, two small ulcers over floor of mouth and evidence of bilateral VIII, IX and Xth cranial nerve palsies. Hypocomplementemia, negative immune profile and evidence of vasculitis on skin biopsy suggested a diagnosis of hypocomplementemic urticarial vasculitis. The patient responded to a course of steroids.
Subject(s)
Complement C3/deficiency , Complement C4/deficiency , Cranial Nerve Diseases/diagnosis , Diagnosis, Differential , Female , Humans , Middle Aged , Urticaria/diagnosis , Vasculitis, Leukocytoclastic, Cutaneous/diagnosisABSTRACT
The distribution of HLA class-I (A, B and C), and class II (DR and DQ) antigens was studied in 50 randomly selected Kashmiri Muslim patients with established rheumatic heart disease and compared with that of 50 controls of similar ethnicity. A significant increase in the frequency of HLA-DR4 (P < 0.005, RR 3.27) and a significant decrease in the frequency of HLA-B5 (P < 0.001, RR 0.19) were found in the patient group. Though HLA-DQ3 showed a significant increase (P < 0.005, RR 2.52) and HLA-DR7 a significant decrease (P < 0.05, RR 0.42) in the patient group, the corrected P value was not significant. The findings suggest that susceptibility to RHD in the studied population is HLA-related, with HLA-DR4 influencing its occurrence and HLA-B5 conferring protection against the same.