Familial amyloidosis cutis dyschromica: six cases from three families

Amyloidosis cutis dyschromica, a rare form of primary cutaneous amyloidosis requiring histopathological confirmation, is characterized by generalized, asymptomatic hyperpigmentation intermingled with several hypopigmented spots without papulation, atrophy, and telangiectasia. Its onset usually begins before puberty. We describe six patients from three families, four male and two female. The mean age at onset was 10.2 years. Although the skin eruptions had developed extensively since childhood, systemic involvement was not evident even after long-term follow-up. Due to its unique and characteristic features, this condition should be considered as a separate entity and differentiated from other variants of primary cutaneous amyloidosis. The familial occurrence in our report suggests a genetic causal factor in this disease.