Atrophic cutis marmorata telangiectatica congenita

Dear Sir,

Cutis marmorata telangiectatica congenita (CMTC) is a rare, reticular, and congenital vascular anomaly reported in fewer than 500 patients.^[1] It is usually widespread or segmental. We report a case of atrophic CMTC.

A 10-day-old healthy female neonate presented with asymptomatic, non-progressive, nonblanchable, and persistent vascular lesions with reticulate extensions over the right side of the abdomen since birth [Figure 1]. In addition to a sharp midline cutoff, the lesions had a depressed and atrophic center without any ulcerations. The lesions did not resolve with warming or repositioning. She did not have any limb asymmetry, macrocephaly, or extracutaneous vascular or pigmentary abnormalities. There was no family history of vascular malformations or bleeding disorders. The neonate was born out of a non-consanguineous marriage without any history of trauma, infections, or medication use during pregnancy. Her mother had an uneventful vaginal delivery without any complications. Based on the characteristic history and examination findings, we made a diagnosis of atrophic CMTC. We reassured the patient regarding the benign nature of the disease and asked them to review with us regularly.

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Figure 1:

Fixed, non-blanchable vascular lesions with reticulate extensions and an atrophic center over the right side of the abdomen.

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CMTC occurs sporadically and usually presents at birth. While CMTC lightens due to the normal thickening and maturation of the skin over the first 2 years of life, most lesions do not resolve completely. CMTC can uncommonly occur on a small body area with a sharp midline demarcation.^[2] The most common sites for localized CMTC are the lower limbs.

CMTC lesions often present with ulceration. In infants, they heal without any complications. However, adults with persistent ulcerative CMTC can experience significant distress. Up to half of the patients can have associated anomalies, including hypoplasia, neurological defects, ophthalmological complications, cardiovascular defects, Mongolian spots, and genitourinary defects.^[3,4] Asymmetry of the limbs is a common association. The affected limb may get either atrophic or hypertrophic. Children with facial CMTC also tend to have glaucoma.

CMTC may be confused with the physiological appearance of cutis marmorata, which resolves with rewarming. It should also be differentiated from angioma serpiginosum, unilateral dermatomal superficial telangiectasia, capillary malformation, and abortive hemangioma [Table 1]. Angioma serpiginosum is a vascular nevus presenting as a small, partially compressible, red-to-purple spot. The congenital variant of unilateral dermatomal superficial telangiectasia has a dermatomal distribution and does not have a reticulated pattern. While capillary malformations and abortive hemangiomas can have a reticulated appearance, both do not have atrophy.

There have been conflicting reports with regard to the efficacy of laser therapy in CMTC.^[3] Combination of sympathetic nerve blockade with vasodilator therapy has been shown to reduce pain.^[5] The reticulated pattern and atrophic center lead us to the diagnosis in our patient. Dermatologists should be aware of this entity to make an early diagnosis.