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 many 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 -thalassemiamajor 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% & 30% respectively), distal nail brittlleness (23%), nail plate roughness (21%) and hard palate pigmentation (30%) were the most common findings. Some skin lesions related to age, sex, serum ferritin level, thyroid hormone and hemoglobin. A11lesions, 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.