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Pathological Case of the Month
Christof Dame, MD;
Ingrid Hausser, ScD;
Janique Geukens, MD;
Rolf E. Brenner, MD
From the Children's Hospital (Drs Dame and Brenner) and the Department of Dermatology (Dr Geukens), University of Bonn, Bonn, Germany; and the Institute for Ultrastructure Research of the Skin, Department of Dermatology, Ruprecht-Karls University, Heidelberg, Germany (Dr Hausser). Dr Brenner is now with the Division for Biochemistry of Joint and Connective Tissue Diseases, Department of Orthopaedics, University of Ulm, Ulm, Germany.
Arch Pediatr Adolesc Med. 2001;155:1275-1276.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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SKIN HYPEREXTENSIBILITY was present in a 10-year-old girl (Figure 1). After minor injuries, connective tissue fragility manifested as easy bruising; the wounds were widely gaping, and more than 20 surgical procedures were performed. Thin, brownish, discolored, atrophic scars with a papyraceous appearance were found on typical pressure points such as the forehead, elbows, and knees. On the elbows, molluscoid pseudotumors were obvious. Joint hypermobility was shown by passive and active dorsiflexion of the fingers, passive opposition of the thumbs to the flexor aspect of the forearm, and hypermobility of the elbows and knees. Kyphoscoliosis and multiple hematomas were obvious. Within the patient's family, similar clinical features were reported for her father and her great grandmother. Electron micrographs of the patient's skin showed numerous "composite" collagen fibrils, described as "cauliflower" deformity within the whole dermal connective . . . [Full Text of this Article]
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