1. CUTIS MARMORATA TELANGIECTATICA CONGENITA IN A TERM FEMALE NEWBORN
Kaïret K., Docx M., Van de Broek H.
ZNA Middelheim, Department of Neonatology
ABSTRACT
DISCUSSION
Cutis Marmorata Telangiectatica Congenita (CMTC) is an uncommon, benign vascular malformation, resembling physiologic cutis marmorata. It is characterized by a localized or generalized fixed reticulate erythematous rash that does not disappear with warming. When localized to a specific area of limb, the lesion remains unilateral without crossing the midline. CMTC is usually present at birth, but may develop later in life, from the age of 3 months till 2 years old. Associated congenital anomalies may be present. Prognosis is good with often spontaneous resolution. No specific therapy is needed.
CMTC was first described in 1922 by Van Lohuizen. We found in the literature the 300 reported cases.Local or generalized reticular erythema is characteristic of CMTC. Clinical features also include telangiectasia and phlebectasia. The local lesions do not cross the midline. The lower limbs are the predilection site for local lesions. Atrophy and ulceration of the affected skin is a possible complication. Most skin lesions are already present at birth, but they can appear also at the age of three months till two years of age. According to some observations, females are more affected with more severe generalized forms.
Diagnosis is made by clinical examination. Differential diagnosis with physiological cutis marmorata is made on clinical basis; CMTC does not disappear under local heat and becomes more prominent in cold temperature or during physical activity. Histopathology is nonspecific. Coexistence of other vascular birthmarks are possible. CMTC may be associated with other congenital anomalies, such as glaucoma. Appropriate complementary examinations should happen when we suspect other congenital anomalies.
The aetiology of CMTC is unknown and mostly multifactorial. Lethal gene hypothesis has been suggested for the patchy distribution and sporadic occurrence The prognosis in uncomplicated cases is favourable with spontaneous resolution in early childhood. Treatment is usually not required. Body asymmetry, either due to hypertrophy or hypoplasia, particularly of the limbs, is the most common complication. The results of laser therapy, in persistent cases, is variable and often disappointing Patients should be seen during their first year every 3 months and thereafter annually for minimum 3 years with correct monitoring of their limb girth and length measurements.
CASE REPORT
A. E. is newborn girl from a G1P0Ab0 young Serbian mother. The delivery was a spontaneous vaginal delivery. On day 1 we observed a fixed blue discolo-ration with reticular pattern on the right lower leg and hemi-abdomen up to the nipple line. Further clinical examination was normal. Ophthalmological and cardiac examination showed no abnormalities. Ultrasound of the abdomen showed no visceral organomegaly and the measurements of both kidneys were normal Colour Doppler examination of the right lower limb was normal. Transfontanellar ultrasound was normal.
The mother was diagnosed earlier with oligoarticular juvenile idiopathic arthritis. An association between her pathology and Cutis Marmorata Telangiectatica Congenita (CMTC) of her offspring has no yet been described in the literature. Both parents were Serbians with no consanguinity.
REFERENCES
Sharma J. Cutis Marmorata Telangiectatica Congenita. J Clin Neonatol Oct-Dec; 2 (4): 199
Ponnurangam VN, Paramasivam V. Cutis Marmorata Telangiectatica Congenita. Indian Dermatol Online J Jan-Mar; 5 (1): 80-82
De Maio C, Pomero G, Delogu A, Briatore E, Bertero M, Gancia P. Cutis Marmorata Telangiectatica Congenita in a Preterm Female Newborn: Case Report and Review of the Literature. Ped Med Chir. 2014, 36:
Levy R, Lam JM; Cutis Marmorata Telangiectatica Congenita: a Mimicker of a Common Disorder. CMAJ, March 8, 2011, 183 (4).
Figure 1 & 2: Purple reticular rash at the right lower limb, as well as partly right hemi-abdomen without crossing the midline consistent with CMTC