[Cutis marmorata telangiectatica congenita. 4 new cases and review of the literature].
Cutis marmorata telangiectatica congenita (CMTC) is characterized by a persistent vascular mottling of the skin, usually on the limbs. Four cases (2 males and 2 females) followed up for varying lengths of time are reported. In the first patient, whose vascular lesions were mild, careful re-examination after 10 years revealed some scars. In the second patient the lesions were located on the right side of the body, notably on the leg; 6 years later atrophic scars and a brownish plaque were visible on that leg. In the third patient the lesions occupied almost the entire body, although one side was more affected than the other and hypotrophic; the child's face was asymmetrical because of hypoplasia of the jaw and curvature of the nose; after 1 year the patient's general condition was satisfactory and the skin lesions were less evident but still present. In the fourth patient hypoplasia of a lumbar vertebra was discovered; the skin lesions were similar to those observed in the third patient. Examination of the deep vessels, performed whenever possible, did not show any abnormality, but the possibility of future vascular defects, such as varicosities, must be considered. While some authors emphasize the functional character of the disease, in our opinion the high frequency of multiple associated congenital abnormalities makes CMTC not only a vascular disorder but also a syndrome including other neuroectodermal and mesodermal defects. Therefore, any patient presenting with the cutaneous changes typical of CMTC should be examined with this in mind.
Gelmetti
C
C
Institut de Clinique Dermatologique I et Dermatologie Pédiatrique, l'Université de Milan, Italia.
Schianchi
R
R
Ermacora
E
E
D001808:Blood Vessels / Q000002:abnormalities* / Q000473:pathology; D003490:Cyanosis / Q000209:etiology*; D004108:Dilatation, Pathologic; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013684:Telangiectasis / Q000151:congenital*
Cutis marmorata telangiectatica congenita. Quatre nouveaux cas et revue de la littérature.