DefinitionThis section has been translated automatically.
Microcystic, cutaneously or cutaneously/subcutaneously localized, circumscribed lymphatic vasectasia (see also Vascular Malformation, see also Lymphangioma cavernosum) with dilated lymphatic vessels. Lyphangioma circumscriptum is caused by a congenital (malformation) or acquired (usually postoperatively or after local radiotherapy) disturbance of the lymphatic flow, which leads to the clinically recognizable, mostly dense vesicles with water-clear (more rarely reddish or blue-reddish) content.
Occurrence/EpidemiologyThis section has been translated automatically.
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EtiopathogenesisThis section has been translated automatically.
- Primary lymphangioma circumscriptum: lymphatic malformation (dermal lymphectasia is often associated with subcutaneous cisterns which, as parts of the embryonic lymphatic vessel system, have no connection to the other lymphatic vessels). The increased intravascular pressure leads to an expansion of the papillary lymphatic vessels before birth or in the first years of life.
- Secondary (reactive) lymphangioma circumscriptum: Occurs after radiotherapy, postoperatively when lymph nodes have been cleared (up to decades of latency). Often associated with lymphedema.
ManifestationThis section has been translated automatically.
- Primary lymphangioma circumscriptum: Mostly congenital or occurring in the first years of life; less frequently in adults.
- Secondary lymphangioma circumscriptum: Mostly in older adults.
LocalizationThis section has been translated automatically.
Localizations in a larger collective (n=29): Anal/perianal region (25%), extremities (20%), tongue (15%), vulva (10%), scrotum (10%).
Clinical featuresThis section has been translated automatically.
HistologyThis section has been translated automatically.
- Numerous lymph vessels filling the papillary body with optically empty lumen or filled with single erythrocytes. The vessel walls are free of pericytes. No mitoses. Immunohistology: Endothelia pos. for CD31.
- Deep lymphangiomas have muscular wall segments.
Complication(s)This section has been translated automatically.
Operative therapieThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Cecchi R et al (1995) Lymphanioma circumscriptum of the vulva of late onset. Acta Derm Venerol 75: 79-93
- Chee JN et al (2014) Vesicles on the breast post-mastectomy: a quiz -- lymphangioma circumscriptum. Acta Derm Venereol 95:1037-1039.
- Eilers J et al (1988) Lymphangioma circumscriptum of the tongue. Acta Dermatol 14: 108-109
- Gupta S et al (2003) Lymphangioma circumscriptum of the penis mimicking venereal lesions. J Eur Acad Dermatol Venereol 17: 598-600
- Huilgol SC et al (2002 CO(2) laser therapy of vulval lymphangiectasia and lymphangioma circumscriptum. Dermatol Surgery 28: 575-577
- Kwon C et al (2014) Surgical resection of acquired vulvar lymphangioma circumscriptum. Arch Plast Surgery 41:183-186.
- Lai CH et al (2001) Lymphangioma circumscriptum treated with pulsed dye laser. Pediatric dermatol 18: 509-510
- Savas JA et al (2013) Carbon dioxide laser for the treatment of microcystic lymphatic malformations (lymphangioma circumscriptum): a systematic review. Dermatol Surge 39:1147-1157.
- Weyers W et al (1990) Lymphangioma circumscriptum cysticum after surgical and radiological therapy. Dermatologist 41: 102-114
Incoming links (13)Angiokeratoma circumscriptum; Hematolymphangioma; Lymphangiokeratoma; Lymphangioma; Lymphangioma; Lymphangioma circumscriptum cutis; Lymphangioma circumscriptum cysticum; Lymphangioma simplex; Lymphangiomatosis; Lymphangiomatosis; ... Show all
Outgoing links (12)Angiosarcoma lymphedema associated; Argon laser; Co2 laser; Cryosurgery; Erbium yag laser; Excision; Hematolymphangioma; Laser; Lymphangiokeratoma; Lymphangioma cavernosum; ... Show all
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