Four children with cutis laxa (generalized elastolysis) are reported. The first three cases were siblings from a Canadian Indian family and the fourth case was the only affected child in an American Black family. Loose and sagging skin folded over the face, neck and trunk, gave a premature senile appearance. Post-mortem examination was performed on the first three cases. The most common and serious visceral involvement was development of pulmonary emphysema. This was present in two autopsied cases and was demonstrated by chest X-ray in the fourth case. Other abnormalities included large inguinal and perineal hernia, rectal diverticulum and multiple diverticulae of the urinary bladder.

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To diagnose ALMM, one has to be cognizant of this condition and has to consider a constellation of findings that are unusual with melanocytic lesions. Questionable lesions must be biopsied for definitive histopathologic diagnosis.

A child with Winchester syndrome, studied in Iran, is the sixth reported case in the literature. The cutaneous manifestations of this syndrome include leathery thickening of the skin, hyperpigmentation and hypertrichosis. The lips and gingiva are hypertrophic. There are also arthritic changes involving the small joints.

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To diagnose ALMM, one has to be cognizant of this condition and has to consider a constellation of findings that are unusual with melanocytic lesions. Questionable lesions must be biopsied for definitive histopathologic diagnosis.

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