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Cutaneous manifestations of proteus syndrome: correlations with general clinical severity Nguyen D; Turner JT; Olsen C; Biesecker LG; Darling TNArch Dermatol 2004[Aug]; 140 (8): 947-53BACKGROUND: Proteus syndrome is a rare congenital disorder with progressive asymetric overgrowth of multiple tissues. OBJECTIVES: To determine the range of cutaneous findings in Proteus syndrome and to correlate cutaneous findings with overall disease severity. DESIGN: A prospective cohort study was performed at the National Institutes of Health, a tertiary referral center. PATIENTS: Twenty-four consecutive children and adults with Proteus syndrome meeting recent diagnostic criteria. INTERVENTIONS: Physical examination, including complete skin examination, and review of medical records. MAIN OUTCOME MEASURES: Frequency of skin findings; correlation of skin findings with extracutaneous findings; cluster analysis of findings. RESULTS: The 24 patients had skin abnormalities: 22 (92%) had lipomas, 21 (88%) had vascular malformations, 20 (83%) had cerebriform connective tissue nevi on the soles of the feet, 16 (67%) had epidermal nevi, 9 (38%) had partial lipohypoplasia, and 5 (21%) had patchy dermal hypoplasia. Some patients had localized alterations in skin pigmentation and hair or nail growth. Patients with a greater number of skin abnormalities tended to have a greater number of extracutaneous abnormalities. The number of abnormalities tended to increase with age up to 8 years. CONCLUSIONS: Patients with Proteus syndrome exhibit a variable but defined assortment of cutaneous findings. The correlation between numbers of cutaneous and extracutaneous is consistent with the postulated mosaic basis for this syndrome.|Adolescent[MESH]|Adult[MESH]|Child[MESH]|Child, Preschool[MESH]|Cohort Studies[MESH]|Female[MESH]|Humans[MESH]|Infant[MESH]|Male[MESH]|Medical Records[MESH]|Prospective Studies[MESH]|Proteus Syndrome/*pathology[MESH]|Severity of Illness Index[MESH] |