Disturbed iron homeostasis characterizes β-thalassemia and increases its morbidity. Our aim was to retrospectively associate β-thalassemia disease characteristics with treatment-requiring skin conditions. The files of adult β-thalassemia (including sickle β-thalassemia) patients were screened over a 10-year period for treatment-requiring skin disease episodes and their correlation with hematologic diagnoses and epidemiological and serological characteristics. Seventy-eight patients were identified, and 7 (9%) developed at least one relevant episode including cutaneous small-vessel vasculitis (CSVV), urticaria, and leg ulcers. Average ferritin serum level correlated significantly with development of a dermatosis (2,034 ± 799 μg/l in cases vs. 920 ± 907 μg/l in the overall population; p = 0.001, ANOVA). This difference relied exclusively on the high ferritin levels observed in patients with ‘generalized' dermatoses (urticaria and CSVV: 3,860 ± 1,220 μg/l) as opposed to values within the normal range in the case of ‘localized' ones (leg ulcers: 662 ± 167 μg/l). The employed iron chelation treatment influenced ferritin levels (p = 0.002, Kruskal-Wallis test) since chelation with a single agent seems to increase the risk of a skin disease (p = 0.013, likelihood ratio method). Conclusively, serum ferritin can be evaluated as risk factor for generalized dermatoses, but not for leg ulcers, in patients with the β-thalassemia genotype. This risk can be efficiently controlled with adequate chelation.

Keywords:
β-Thalassemia, Chelation, Cutaneous small-vessel vasculitis, Deferasirox, Deferiprone, Deferoxamine, Leg ulcers, Serum ferritin, Skin diseases, Urticaria
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