Cutis marmorata telangiectatica congenita

Ahu Yorulmaz, Yildiz Hayran, Akin Aktas

Ankara City Hospital, Department of Dermatology, Pediatric Dermatology Unit, Ankara City Hospital, Ankara, Turkey

Corresponding author: Ahu Yorulmaz, MD


How to cite this article: Yorulmaz A, Hayran Y, Aktas A. Cutis marmorata telangiectatica congenita. Our Dermatol Online. 2021;12(e):e35.

Submission: 15.12.2020; Acceptance: 03.04.2021

DOI: 10.7241/ourd.2021e.35

Citation tools: 

     

Related Content

Copyright information
© Our Dermatology Online 2021. No commercial re-use. See rights and permissions. Published by Our Dermatology Online.


Sir,

A 24-day-old baby boy referred by the pediatrician to our pediatric dermatology outpatient clinic with bluish purple skin lesions all over his body. He was born out of a non-consanguineous marriage and was 3rd in birth order. He was born via normal vaginal delivery at 38 weeks of gestation, with a birth weight of 2500 gr. His personal and family history were unremarkable. His parents told that bluish purple skin lesions were present since birth, but faded gradually over time. Upon dermatological examination we observed generalized reticulated erythema with fixed reddish-blue to pale purple blotches over the extremities and trunk (Figs. 1 and 2). The face of the patient was reddish-purple with a lacy network (Fig. 3). When the patient awakened and began to cry the reticulated pattern of discoloration darkened. It was learnt from the medical database of the hospital that the routine examination, laboratory and radiological investigations of the patient were all normal. Based on history and clinical findings, we made a diagnosis of cutis marmorata telangiectatica congenita (CMTC) and the patient was taken under follow-up.

Figure 1: Generalized persistent reticulated erythema with fixed reddish-blue to pale purple patches.
Figure 2: Closer view of the patient. Note the fixed darker patches over the trunk. Face of the patient was reddish purple.
Figure 3: A lacy network of darker erythema appeared when the patient began to cry.

Cutis marmorata telangiectatica congenita (CMTC), which is also known as congenital livedo reticularis and van Lohuizen syndrome, is an uncommon, sporadic, congenital vascular anomaly, characterized by persistent reticulated erythema, which is present at or shortly after birth. A thorough literature review has revealed that the reported cases of CMTC is not more than a few hundred cases up to date [14]. However, CMTC might be subject to underreporting, given the benign nature of isolated CMTC. CMTC has typical clinical findings with a lacy pattern of vascular erythema and telangiectasia. The reticulated marbled erythema is persistent and it is either generalized, or segmental, or localized. The colour has a bluish hue with a deep purple appearance, when the patient is subject to changes in environmental temperature. In some patients skin atrophy and ulceration are also found [16].

CMTC differs from physiologic cutis marmorata, in that CMTC is unresponsiveness to local heating and associated with abnormalities such as congenital glaucoma, body asymmetry, limb hypoplasia/aplasia and central nervous system involvement. Recently, it has been documented that 42.5% of the reported patients with CMTC had associated abnormalities, the most common of which was body asymmetry, which was seen more than 30% of the patients. It has been suggested that CMTC has generally a good prognosis and the overall incidence of isolated cases of CMTC is high, owing to the fact that there is a tendency to only report complicated cases of CMTC [16]. This brief report describes a case of CMTC in an otherwise healthy infant. Further case reports would provide a better understanding of clinical findings of both isolated and complicated cases of CMTC.

Consent

The examination of the patient was conducted according to the principles of the Declaration of Helsinki.

The authors certify that they have obtained all appropriate patient consent forms, in which the patients gave their consent for images and other clinical information to be included in the journal. The patients understand that their names and initials will not be published and due effort will be made to conceal their identity, but that anonymity cannot be guaranteed.

REFERENCES

1. Bui TNPT, Corap A, Bygum A. Cutis marmorata telangiectatica congenita:a literature review. Orphanet J Rare Dis. 2019;14:283.

2. 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;34:319-23.

3. Gerritsen MJ, Steijlen PM, Brunner HG, Rieu P. Cutis marmorata telangiectatica congenita:report of 18 cases. Br J Dermatol. 2000;142:366-9.

4. Amitai DB, Fichman S, Merlob P, Morad Y, Lapidoth M, Metzker A. Cutis marmorata telangiectatica congenita:clinical findings in 85 patients. Pediatr Dermatol. 2000;17:100-4.

5. Sharma J. Cutis marmorata telangiectatica congenita. J Clin Neonatol. 2013;2:199.

6. Lagha IB, Zaara SY, Harbaoui S, Jaber K, Dhaoui MR, Doss N. Cutis marmorata telangiectatica congenital:two case reports. Our Dermatol Online. 2018;9:440-2.

Notes

Source of Support: Nil,

Conflict of Interest: None declared.

Request permissions
If you wish to reuse any or all of this article please use the e-mail (brzezoo77@yahoo.com) to contact with publisher.

Related Content:

Related Articles Search Authors in

      http://orcid.org/0000-0003-4687-0522
 

Comments are closed.