Association of lymphatic malformation and congenital nevus
Sabrina Oujdi, Hanane Baybay, Siham Boularbah, Sara Elloudi, Meryem Soughi, Zakia Douhi, Fatima Zahra Mernissi
Department of Dermatology, University Hospital Hassan II, Fes, Morocco
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Sir,
Congenital nevi (CN) are benign melanocytic proliferations originating from the neural crests, present at birth or appearing in the first weeks after birth.
The most commonly employed classification divides CN according to their largest diameter expressed in projected adult size (PAS) into three categories:
- Small-size CN: diameter < 1.5cm;
- Congenital CN (intermediate-size): 1.5 < diameter < 19.9 cm;
- Large-size CN: diameter > 20 cm, including giant CKDs larger than 40 cm.
It seems useful to perform MRI in any child with more than two CNs, regardless of the size of the largest CN and its location in order to detect an associated neurocutaneous melanosis [1].
Lymphatic malformations are rare, slow-flowing, benign congenital malformations composed of abnormal cystic dilatations, and although congenital, they are not always apparent at birth. They may involve the skin, mucous membranes, or underlying tissues. They are associated with an abnormality in the PIK3CA/mTOR signaling pathway [2]. Lymphatic malformations are classified as macrocystic (diameter > 1 cm), microcystic (diameter < 1 cm), or mixed [3].
Herein, we report a case of lymphatic malformation of the tongue associated with a large congenital nevus in a young boy.
A ten-year-old boy was sent by his pediatrician for a swelling on the dorsal surface of the tongue associated with a large pigmented lesion on the back. A general physical examination revealed a normal status, including weight, size, intelligence, speech, auditory function, and visual acuity. A dermatological examination revealed a swelling on the dorsal side of the tongue surmounted by vesicles with clear contents and a 20 cm, pigmented plaque on the back, which dates to birth according to his father (Figs. 1a and 1b). We explained to the patient and his parents that it was a vascular malformation that we were going to treat by sclerotherapy with bleomycin associated with sirolimus and, for the congenital nevus, biannual monitoring.
Figure 1: (a) Swelling on the dorsal side of the tongue surmounted by vesicles with clear contents. (b) A 20 cm, pigmented plaque on the back dating back to the boy’s birth. |
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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. Waelchli R, Aylett SE, Atherton D, Thompson DJ, Chong WK, Kinsler VA. Classification of neurological abnormalities in children with congenital melanocytic naevus syndrome identifies magnetic resonance imaging as the best predictor of clinical outcome. Br J Dermatol. 2015;173:739-50.
2. Canaud G, Hammill AM, Adams D, Vikkula M, Keppler-Noreuil KM. A review of mechanisms of disease across PIK3CA-related disorders with vascular manifestations. Orphanet J Rare Dis. 2021;16:306.
3. Walid N, El Fatoiki FZ, Chiheb S. Mixed cystic lymphatic malformations. Our Dermatol Online. 2023;14(Supp. 1):18-21.
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