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.

Figure 1

Squamous cell carcinomas (SCCs) in a 38-year-old HIV-seropositive Black albino woman. (a) SCC of the nose, cheeks and the lips, and (b) the labial mucosa and anterior part of the palate. The disease started as a small ulcer on the upper lip 18 months previously, progressing rapidly to involve the mouth and to destroy the lower face. (c) SCC of the left ear and temple started 8 months previously. (d) Panoramic radiograph showing destruction of the anterior maxilla. Histopathologically, the carcinoma was poorly differentiated. The patient died before investigation for metastases could be done. The extensive and striking sunlight-induced malignant facial damage is tragic evidence of the consequence of lack of sun-protection from an early age and of appropriate early medical care.

Figure 2

A 21-year-old HIV-seropositive Black albino woman with an exophytic crusted SCC of the upper lip, twelve months after she first noticed a small painless erosion. There was neither local lymph node involvement nor distant metastasis, and microscopically the carcinoma was moderately differentiated.