Acquired progressive lymphangioma D18.10

Wilson Jones 1976

Acquired benign proliferation of superficial lymph vessels.

Mostly occurring on the trunk and/or the extremities; the genitals may also be involved.

First changes are observed as a reddish-brown, painless spot. Gradual but permanent growth in size to a slightly elevated plaque up to 30 cm in size. Excessively extensive, acquired cases with manifestation on abdomen, lower extremities as well as genitalia have been described (Larkin SC et al. 2018).

Kaposi's sarcoma (histology is diagnostic)

Targetoid hemosiderotic hemangioma (clinically typical cocordal stratification with hemosiderotic halo)

Apparently, systematically applied Sirolimus is able to stop the progression of the lymphatic malformation or to achieve partial remissions (Wiegand S et al. 2017).

Salman A et al. (2017) reported a cessation of size growth after application of a 5% Imiquimod cream.

1. Larkin SC et al (2018) A case of extensive acquired progressive lymphangioma. Pediatr Dermatol 35:486-489.
2. Salman A et al. (2017) Acquired progressive lymphangioma: case report with partial response to imiquimod 5% cream. Pediatr Dermatol 34:e302-e304.

3. Tronnier M et al (2021) Acquired progressive lymphangioma in a 13-year-old boy Dermatologist 72:610-614.

4. Wiegand S et al. (2017) Treatment of Lymphatic Malformations with the mTOR Inhibitor Sirolimus: A Systematic Review. Lymphat Res Biol 16:330-339.