Skin and mucosal manifestations in patients with Thalassemia major

Abstract

Objective: β-Thalassemia major is one of the common genetic disorders in Iran. In Isfahan, the prevalence of gene carriers is about 8%. As yet, no much information about skin manifestations of these patients has been published. This study was done to evaluate skin manifestations in thalassemia major patients and compared them with laboratory and pathologic findings. Methods: 367 cases of β-thalassemia major who referred to this center, were clinically examined during (1998-99). Routine and special lab tests were done on all cases and skin biopsy was done on selected cases. The findings were analyzed by statistical methods and compared with the sex, age, serum ferritin, thyroid hormone level, liver enzymes and amount of injected desferal. Findings: Skin dryness (53%), freckling (33%), lentigo (28%), coarse hair (24%), scar and hyperpigmentation at the desferal injection site (28% and 30% respectively), distal nail brittleness (23%), nail plate roughness (21%) and hard palate pigmentation (30%) were the most common findings. Some skin lesions related to sage, sex, serum ferritin level, thyroid hormone and hemoglobin. All lesions, except hair and skin dryness, were severe in older patients. Conclusion: Skin and mucosal manifestations are common in thalassemia major cases. Most manifestations are related to iron deposition in skin and other organs and incomplete treatment by present methods.

Keywords

• thalassemia
• skin manifestations
• mucosal manifestations