In 1963, Bovenmyer and Caplan1 described an 18-year-old woman who had generalized cutaneous normolipemic plane xanthoma with features of Ehlers-Danlos syndrome. In 1979, we had the opportunity to see this woman again and also to see her 9-year-old daughter, who showed the same clinical features as her mother. The long-range follow-up and the new genetic implications of our observations prompted this report.
Report of Cases
This 36-year-old woman was described in detail previously when she was 18 years old. She had the onset of yellow skin pigmentation at the age of 1½ years. The skin changes gradually progressed; a tendency to bruise easily and looseness and wrinkling of her skin had also developed. She showed sparseness of the hair in the eyelashes, eyebrows, and the axillary and pubic areas. The scalp hair was fine, dense, and grew normally. She noted brittle fingernails and a generally low level
Thomsen RJ, Caplan RM, Bartley JA. Generalized Normolipemic Plane XanthomaNew Observations and Long-range Follow-up. Arch Dermatol. 1981;117(8):521-522. doi:10.1001/archderm.1981.01650080075036