Congenital fascial dystrophy: the stiff skin syndrome.
Congenital fascial dystrophy is an uncommon disorder that may mimic scleroderma. The skin is bound down and feels "wood hard" on palpation of the trunk and extremities. Sclerodactyly, telangiectases, and nail fold changes are not present. Unlike scleroderma, the onset is at birth. Flexion contractures result and other associated abnormalities may include developmental delay and mild hirsutism. We report a typical case to illustrate the clinical and pathologic findings of patients with this disorder.
Helm
T N
TN
Department of Dermatology, Buffalo Medical Group, New York, USA.
Wirth
P B
PB
Helm
K F
KF
D000328:Adult; D001707:Biopsy, Needle; D003286:Contracture / Q000209:etiology*; D003937:Diagnosis, Differential; D005145:Face; D006801:Humans; D008297:Male; D016059:Range of Motion, Articular; D012594:Scleroderma, Localized / Q000175:diagnosis; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000151:congenital* / Q000175:diagnosis*; D013577:Syndrome; D014820:Vitiligo / Q000209:etiology