ALBINISM

Albinism is an inherited disorder of melanocytes, which do not
synthesise melanin (pigment). This results in absence of pigmentation of
skin, hair and eyes, combined with photophobia and nystagmus from
birth. The skin is white, the hair white or yellow and the iris light blue.
These patients are very light sensitive because they have no UVabsorbing
melanin, which usually protects people from solar damage.
After short term sun exposure sunburn, freckling, and early ageing of the
skin already occurs and actinic keratoses with a tendency towards
malignant transformation appear. Squamous cell carcinoma is seen at an
early age, even in children.
Management of albinism
Sunprotection to prevent solar damage:
- Wear protective clothing (long sleeves, long skirts and trousers), a sunhat with a
wide rim, providing protection for face, ears and neck, and sunglasses.
- Stay indoors as much as possible during the hot hours of the day.
- Children with visual impairment should be seated in front rows in classrooms.
- Use sunscreens with a high sun protection factor (SPF), e.g. PABA (para amino
benzoic acid) which has SPF 15. Total sunblockers have even higher SPF’s. Zinc oxide
cream/paste/ointment blocks out sunlight and can be used for the lips. Apply
sunscreen whenever going outdoors.
Regular skin check-ups for early detection and treatment of pre-cancerous
keratoses and skin cancer. Single keratoses can be treated with liquid nitrogen,
curettage and electro-dissecation, shave excision followed by electrosurgery.
Multiple and/or extensive lesions can be treated with topical 5%
5-fluoro-uracil. The extract from the fruit of the sausage tree (Kigelia Africana)
is an effective alternative (ready-made cream at many pharmacies). The rough
skin can be softened with urea 10% ointment or salicylic acid 2-5% ointment.