Primarily cutaneous disorder due to proliferation of typical Touton giant cells, histiocytes, lymphocytes, plasma cells and eosinophiles. About 80% of patients present in their first year of life. Single orange colored papule in 80% of cases. Size range from 5 to 20 mm. Predilection for head, face, trunk and extensor surface of extremities. Annual eye examinations should be performed. Ocular involvement occurs in 10% of cases. Iris is the commonest site of involvement with potentially sight threatening complications: spontaneous hyphema and secondary glaucoma.
Aik Kah, Tan, M.D., Universiti Malaysia Sarawak¦2, Kuching, Malaysia