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Acquired progressive lymphangiomaD18.10

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 06.08.2021

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Synonym(s)

Acquired benign lymphangioendothelioma; benign lymphangioendothelioma; Benign lymphangioendothelioma; lymphatic malformation, lymphangioma, low-flow lymphatic malformation; progressive lymphangioma; Progressive lymphangioma

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HistoryThis section has been translated automatically.

Wilson Jones 1976

DefinitionThis section has been translated automatically.

Acquired benign proliferation of superficial lymph vessels.

Occurrence/EpidemiologyThis section has been translated automatically.

Rarely.

ManifestationThis section has been translated automatically.

Beginning in childhood or early adulthood.

LocalizationThis section has been translated automatically.

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

Clinical featuresThis section has been translated automatically.

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).

HistologyThis section has been translated automatically.

Emphasizes horizontally oriented growth of differently dilated lymph vessels in the upper and middle dermis. Infiltrating growth pattern, whereby in places adnexa or vessels are circulated (promontorium sign!). Absence of endothelial nuclear atypia, mitoses or multilayering. Focal endothelial hobnail formation is possible.

Differential diagnosisThis section has been translated automatically.

Kaposi's sarcoma (histology is diagnostic)

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

TherapyThis section has been translated automatically.

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

External therapyThis section has been translated automatically.

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

LiteratureThis section has been translated automatically.

  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.

Authors

Last updated on: 06.08.2021

Author: Prof. Dr. med. Peter Altmeyer