Cutis Marmorata

Authors:
Alexander K. C. Leung, MD, and Alex H. C. Wong, MD
University of Calgary

Benjamin Barankin, MD
Toronto Dermatology Centre

Citation:
Leung AKC, Wong AHC, Barankin B. Cutis marmorata. Consultant for Pediatricians. 2014;13(6):284-285.

During a routine physical examination, a 2-week-old infant was noted to have mottled skin on his limbs and trunk. According to his mother, the mottling became more intense after exposure to cold temperatures, and it disappeared on warming.

The infant was born at full term via normal vaginal delivery to a 25-year-old, gravida 2, para 1 mother following an uncomplicated pregnancy. The mother had consumed no tobacco, alcohol, or medications during the pregnancy. Apgar score was 7 at 1 minute and 9 at 5 minutes. Birth weight was 3.1 kg, and birth length was 51 cm. The neonatal course had been uneventful. Apart from the mottling of the skin, physical examination findings were otherwise normal.

A diagnosis of cutis marmorata was made. The parents were reassured of the benign nature of the condition and that the condition likely will resolve with time.

Cutis marmorata is characterized by a symmetrical, lacy, reticular, red-purple mottling of the skin after exposure to cold temperatures.¹ It usually is observed on the extremities and trunk. The evanescent, lacy network of small blood vessels is due to an exaggerated vasomotor response to cold temperatures that produces vasospasm, with subsequent hypoxia and vasodilatation of venules and capillaries. The mottling disappears when the skin is warmed.

Cutis marmorata is a common phenomenon in healthy infants. The condition often disappears with increasing age.¹ Cutis marmorata is more common in children with hypothyroidism, systemic lupus erythematosus, Down syndrome, trisomy 18, Menkes syndrome, familial dysautonomia, and de Lange syndrome.

Cutis marmorata must be distinguished from livedo reticularis. Livedo reticularis is characterized by the presence of a bluish purple, mottled or netlike pattern in unbroken circles on the skin.² Exposure to cold environments usually intensifies the vascular pattern. In contrast to cutis marmorata, the mottling does not resolve when the skin is warmed, and the discoloration is bluish purple rather than red. The lesion is more intense and is persistent.¹ Presumably, the condition results from slow or stagnant blood flow, vessel-wall pathology, and decreased oxygen tension.^3,4 Livedo reticularis occurs sporadically in most cases. The condition may be congenital or acquired.

Congenital livedo reticularis, also known as cutis marmorata telangiectatica congenita (CMTC), is noted at birth.⁴ CMTC typically affects one extremity, although the trunk and face also may be involved.⁵ Prominent blood vessels and telangiectasia often are seen.⁵ Body asymmetry is the most common anomaly associated with CMTC.⁶ Other associated anomalies include vascular malformation, cutaneous atrophy, and macrocephaly.⁵

Acquired livedo reticularis usually is an isolated finding. The lesion occurs predominantly on the lower extremities and less commonly on the upper extremities or the trunk.³ Acquired livedo reticularis may be drug-induced (eg, amantadine, rasagiline). Occasionally, the condition may be associated with coagulopathies, autoimmune disease, systemic vasculitis, and antiphospholipid syndrome.⁴ Children with livedo reticularis have a slightly increased risk for cerebrovascular accidents (Sneddon syndrome).²

Treatment of cutis marmorata should be directed at any known underlying cause, if possible. In healthy children, treatment usually is not necessary, because the tendency to mottle usually resolves by 6 to 12 months of age.

REFERENCES

1. Morelli JG. Diseases of the neonate. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Elsevier Saunders; 2011:2218-2220.
2. Mathias G, Cowley R, Morales A, Rogers RC. Congenital livedo reticularis and recurrent strokes in two unrelated young children. Clin Pediatr (Phila). 2006;45(4):367-372.
3. Kraemer M, Linden D, Berlit P. The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa: a literature review. J Neurol. 2005;252(10):1155-1166.
4. Rose AE, Saggar V, Boyd KP, Patel RR, McLellan B. Livedo reticularis. Dermatol Online J. 2013;19(12):20705.
5. Garzon MC, Schweiger E. Cutis marmorata telangiectatica congenita. Semin Cutan Med Surg. 2004;23(2):99-106.
6. Kienast AK, Hoeger PH. Cutis marmorata telangiectatica congenita: a prospective study of 27 cases and review of the literature with proposal of diagnostic criteria. Clin Exp Dermatol. 2009;34(3):319-323.