Cutis marmorata telangiectatica congenita—a report of two cases occurring in male children

Summary We report on two cases of male children with cutaneous changes consistent with the diagnosis of cutis marmorata telangiectatica congenita (CMTC) present from birth. One case was associated with an unexplained hepatosplenomegaly, which resolved spontaneously and there has been some improvement in the appearance of the skin. This case has severe late‐onset atopic eczema. In the second case there is a reduced circumference of an affected arm compared with the unaffected side. This child also had eczema of a seborrhoeic pattern. CMTC is a rare disorder, 1 more common in females, which may he associated with congenital abnormalities in up to 50% of cases. 2 The typical cutaneous changes are usually present at birth but they have been reported to appear within the first 2 years of life. 2 When the changes occur after birth the condition must be distinguished from‘genuine diffuse phlebectasia, 3,4 a progressive disorder that usually starts in late childhood. Most cases of CMTC show a persistent pattern of cutis marmorata with a tendency to improve in some cases, although ulceratiun is occasionally a problem. 2 There may be associated telangiectasia and phlebectasia and in some cases there is marked atrophy of the subcutaneous tissue overlying the livedo. 2 The area of involvement is typically patchy often with a tendency to mid‐line delineation (Fig. 2) and the limbs are frequently involved but there may be generalized skin changes. Although some familial cases have been reported 2,5 a hereditary transmission remains unproven.