 |
|
Images in Clinical Practices Indian Pediatrics 2002; 39:203 |
|
Cutis Marmorata Telangiectatica Congenita |
|
A 3½-month-old female child born of non-consanguineous parents was brought with dilated cutaneous blood vessels and mottled pattern of the skin since birth. There were no adverse antenatal or postnatal factors. Physical examination showed a persistent cutis marmorata pattern and dilated superficial cutaneous veins and capillaries over the trunk, extremities, face and scalp sparing palms and soles (Fig.1). There was a small hemangioma just above the outer canthus of right eye. There were two small superficial ulcers over the scalp and one similar ulcer over the extensor surface of each knee. Physical examination and investigations excluded any other co-existing systemic abnormality. Histopathological examination of the representative skin of anterior abdominal wall revealed normal epidermis. Dermis showed no dilated blood vessels but there were occasional thickned blood vessels and mild infiltration by mononuclears. This child died of broncho-pneumonia at the age of 4½ months. Cutis marmorata telangiectatica congenita (CMTC), also called Von Lohuizen syndrome or congenital generalized phlebectasia is a rare benign congenital vascular abnormality characterized by a persistent cutis marmorata pattern, spider nevus like telangiectasia and superficial ulceration. Rarely it may involve scalp skin as in the present case. Sometimes CMTC may be associated with PDA, Struge Weber syndrome, congenital glaucoma and limb abnormalities. CMTC is a clinical diagnosis and histopathological examination of skin is usually not diagnostic. Residual phase of neonatal lupus active during intrauterine life may simulate CMTC and should be ruled out by anti-Ro/SSA antibodies and anti-nuclear antibody (ANA) test in baby and mother. CMTC has a favorable prognosis with partial or complete resolution during adolescence and therefore needs no specific therapy. N.K. Anand,
|
