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Cutis laxa with ultrastructural abnormalities of elastic fiber

1989; Elsevier BV; Volume: 21; Issue: 2 Linguagem: Inglês

10.1016/s0190-9622(89)80039-8

1097-6787

Yukio Kitano, Kenju Nishida, Natsuko Okada, Takashi Mimaki, Hyakuji Yabuuchi,

Dermatological and Skeletal Disorders

A case of a congenital, autosomal recessive form of generalized cutis laxa is reported. The patient was a 27-month-old boy with generalized flaccid skin and short stature. Radiologic examination revealed that the age of the bones of the wrist was compatible with a chronologic age of only 1 year. Elastic fibers were diminished throughout the dermis, and results of electron microscopic study showed globular and unstained elastin and relatively large amounts of the microfibrillar components of elastic fibers.