Net-like superficial lymphatic malformation D18.1

Noguera-Morel, 2016

Rare (<10 cases published internationally to date) superficial, reticular lymphoid malformation (LM) characterized by characteristic clinical, dermoscopic and histological findings.

Early childhood-o adolescence

Red to purple spots interspersed with a fine net-like pattern of thin vascular structures. There are also smaller red papules.

Dermoscopy shows arborizing telangiectatic vessels.

The histology is characterized by vascular proliferation from thin-walled vessels in the upper dermis, which stains positive with podoplanin (D2-40) (Iznardo H et al. 2021).

Regression?

Noguera-Morel L et al. (2016): In one boy and two girls, first skin lesions developed at the age of 5 years, 6 years and 18 months, respectively. In all patients, the lesions progressed over the following years. Regressions were also observed. Overall, however, surface growth occurred. The lesions consisted of large, irregularly shaped, angled, bright red to red/brown to purple patches with a fine reticular pattern of reticular or branched vascular structures. Back areas with shoulder region, buttocks and thighs were affected.

Some older lesional areas lost their branched appearance and developed a more cohesive rust-red to purple color. Finally, red or purple sessile papules also developed on the surface of the lesions. The remaining physical examination revealed no other abnormalities in the three children.

Dermoscopy showed deep red, reticular or branched telangiectatic vessels in all cases, without erythema between the dilated vessels. The deep red color of the vessels on dermoscopy was in contrast to the bright red color typically seen on dermoscopy of capillary malformations.

Magnetic resonance imaging and ultrasound showed no dermal, subcutaneous or deep vascular abnormalities in all cases.

1. Iznardo H et al. (2021) Net-like superficial lymphatic malformation. Pediatr Dermatol 38:516-517.
2. Noguera-Morel L et al. (2016) Net-like superficial vascular malformation: clinical description and evidence for lymphatic origin. Br J Dermatol 175:191-193.
3. Trindade F et al. (2012) Hobnail hemangioma reclassified as superficial lymphatic malformation: a study of 52 cases. J Am Acad Dermatol 66:112-115.
4. Vide J et al. (2018) Net-like superficial lymphatic malformation: a new entity? Clin Exp Dermatol 43:732-734.
5. Wassef M et al. (2015) Vascular anomalies classification: recommendations from the International society for the study of vascular anomalies. Pediatrics 136:e203-e214.