Oculocutaneous Albinism and Squamous Cell Carcinoma of the Skin of the Head and Neck in Sub-Saharan Africa

Abstract

Oculocutaneous albinism which is characterised by impaired melanin biosynthesis is the most common inherited pigmentary disorder of the skin and it is common among Blacks in sub-Saharan Africa. All albinos are at great risk of developing squamous cell carcinoma of sun-exposed skin, and Black albinos in sub-Saharan Africa are at about a 1000-fold higher risk of developing squamous cell carcinoma of the skin than the general population. In Black albinos, skin carcinoma tends to run an aggressive course and is likely to recur after treatment, very probably because the aetiology and predisposing factors have not changed. Prevention or reduction of occurrence of squamous cell carcinoma of the skin in Black albinos might be achieved through educating the population to increase awareness of the harmful effects of exposure to sunlight and at the same time making available effective screening programs for early detection of premalignant and malignant skin lesions in schools and communities and for early treatment.

181 Global skin disease morbidity and mortality: An update from the Global Burden of Disease Study 2013

“…Global Burden of Disease 2013 demonstrates high incidence and burden of keratinocyte carcinoma in southern sub-Saharan Africa (including Botswana, Lesotho, Namibia, South Africa, Swaziland, and Zimbabwe), which is hypothesized to be particularly due to mortality from keratinocyte carcinoma reflecting poor access to health care and high prevalence of human immunodeficiency virus infection and albinism. 22, 23 The International Foundation for Dermatology has identified a particular vulnerable group in the tropics, persons with albinism (PWA), who are predisposed to premature skin cancer morbidity and mortality.…”

MITF and UV responses in skin: From pigmentation to addiction

“…OCA4, due to defects in membrane‐associated transporter protein, is found in one per 85,000 Japanese, but is very rare in Caucasians (Grønskov, Ek, & Brondum‐Nielsen, ). Among African albinos, the risk of developing squamous cell carcinoma is up to 1,000‐fold higher than in the general population (Lekalakala et al., ). Melanoma among albinos is rare (Kiprono, Chaula, & Beltraminelli, ; Mabula et al., ; Perry & Silverberg, ), although a slightly increased risk has been reported among individuals carrying the temperature‐sensitive TYR R402Q variant (Gudbjartsson et al., ).…”

Racial Differences in Cancer Susceptibility and Survival: More Than the Color of the Skin?

“…In fact, although African American have a much lower incidence of UV-induced cutaneous SCCs, they develop SCCs at sites of wound healing that are much more aggressive than in Caucasians [19]. In addition, African Albinos are disproportionately affected by skin SCCs compared to the general African population [20]. …”