Course: Progressive Generalized Skin Laxity in a Young Woman
CME Credits: 1.00
Released: 2023-07-05
A Chinese woman in her late 20s presented with a 20-year history of progressive skin laxity. In early childhood, she had developed asymptomatic yellowish coalesced papules confined to flexural areas. Subsequently, severe skin sagging occurred on her abdomen with loss of elasticity, then spread extensively with developmental growth. The results of a physical examination showed thick and leathery skinfolds on the neck, axillae, inguinal regions, abdomen, and limbs (, A), with yellowish papules scattered on the dorsal neck. The patient had no extracutaneous involvement and was born to healthy nonconsanguineous parents. However, her younger sister had developed the same skin symptoms. Coagulation-related tests indicated a low clotting activity of factor X (52.2%; reference range, 77%-131%) and a prolonged prothrombin time (12.8 seconds; reference range, 10.4-12.6 seconds). The results of other laboratory investigations were unremarkable. Echocardiographic and fundoscopic examination results were normal. A skin biopsy specimen was obtained from her neck for histopathologic examination (, B).
Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
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