Inherited Conditions Predisposing to Dermatological Malignancy Albinism
Oculocutaneous albinism is a group of autosomal recessively inherited disorders of melanin synthesis, whose clinical manifestations are pale skin, white hair, nystagmus, and photophobia. There is considerable genetic heterogeneity, many distinct types having been described. Actinic skin damage, squamous cell carcinoma, and basal cell carcinoma are well- recognized complications of oculocutaneous albinism; malignant melanoma is a much less common complication.
Any patient with albinism is at an increased risk of cutaneous malignancies. This susceptibility to cancer appears to be related to the lack of skin pigmentation and attests to the protective qualities of melanin. However, in patients with vitiligo or piebaldism, areas of skin where melanin (and melanocytes) is absent do not appear to be predisposed to malignancies in the same way individuals with albinism should avoid sunlight exposure and use sunscreen creams when they are exposed to it (Iannello et al. 2003; Karim et al. 2002).