Larralde M, Gardner S S, Torrado M V, Fernhoff P M, Santos Muñoz A E, Spraker M K, Sybert V P
Department of Pediatric Dermatology, Ramos Mejía Hospital, Buenos Aires, Argentina.
Pediatr Dermatol. 1998 Jan-Feb;15(1):18-22. doi: 10.1046/j.1525-1470.1998.1998015018.x.
Unusual skin lesions were present at birth in four infants with Turner syndrome. The skin changes in these patients appear to have resulted either from in utero entrapment or pinching of edematous skin or from redundant skin remaining after in utero resolution of lymphedema. Distention by lymphedema is thought to cause several of the phenotypic characteristics seen in patients with Turner syndrome, including nuchal webbing and nail changes. In three of these patients the clinical appearance of the skin changes was similar to cutis verticis gyrata, marked by fixed thickened plaques in folds.
4例特纳综合征婴儿出生时即出现异常皮肤损害。这些患者的皮肤改变似乎是由于宫内水肿皮肤受压或被夹住,或者是宫内淋巴水肿消退后残留的多余皮肤所致。淋巴水肿引起的扩张被认为是特纳综合征患者出现几种表型特征的原因,包括颈部蹼状畸形和指甲改变。在其中3例患者中,皮肤改变的临床表现类似于回状头皮,其特征为褶皱处有固定增厚的斑块。