ABSTRACT
Necrolytic acral erythema [NAE] is unique in its acral location and strong association with hepatitis C virus [HCV] and altered immunological junctions. The aim of the present work was to evaluate HLA DRB1 alleles and association of some parameter of immune system functions [complements C3 and C4, antismooth muscle antibody [ASMA] and antinuclear antibody [ANA] in NAE. Response of cutaneous lesion to low dose interferon alpha [3 million unit [MU] / week] and hydroxychloroquine was also evaluated. This study included 22 patients with HCV- related NAE [group I], 45 chronic hepatitis C without NAE [group II as pathological controls] and 45 healthy subjects [group III, normal controls]. ANA was positive more in patients than normal controls [18.2% vs 0%, P <0.003], however no significant difference was seen between patients groups. ASMA was positive significantly more in patients with NAE than HCV patients, and in both patients groups than normal controls 59.1%, 17.3% and 0%; P<0.001 and 0.0001 respectively]. A significant decrease in C3 and C4 was found in NAE patients than HCV patients without NAE, [P<0.01] and in both patients groups than normal controls [P<0.001]. NAE was associated with HLA -DRB1 03, [77.7%, 16 of 22 vs 24.2%, 11 of 45 of normal controls, Pc <0.0009 and 35.6% 16 of 45 HCV patients without NAE, Pc=0.03. Clinical improvement and significant decrease in ALT [P<0.001] was observed in NAE patients after two months of interferon alpha and hydroxychloroquine therapy. Necrolytic acral erythema [NAE] appears to be an immune mediated response in chronic HCV patients, associated with, lower C3 and C4 and higher frequency of positive ASMA. The results suggest that the development of HCV related ANE is associated with HLA-DRB1 03 marker. And low dose interferon alpha [3 MU per week] and hydroxychloroquine are good treatment modalities for NAE