Lymphangioma circumscriptum D18.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

lymphangioma circumscriptum cutis; lymphangioma circumscriptum cysticum; lymphangioma simplex

Definition
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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/Epidemiology
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Etiopathogenesis
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  • 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.

Manifestation
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  • Primary lymphangioma circumscriptum: Mostly congenital or occurring in the first years of life; less frequently in adults.
  • Secondary lymphangioma circumscriptum: Mostly in older adults.

Localization
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Localizations in a larger collective (n=29): Anal/perianal region (25%), extremities (20%), tongue (15%), vulva (10%), scrotum (10%).

Clinical features
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Densely standing, up to 1-3 mm large, thick-walled, sagocorn or frogspawn-like cysts, slowly increasing in size. With longer continuance wart-like aspect. Frequently bleeding, which is only very slowly absorbed, so that the impression of a haemangioma or haematolymphangioma can arise.

Histology
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  • 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)
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Often superficial lymphangiomas communicate with deeper parts.

Operative therapie
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Excision of smaller lesional areas if possible from the surface. Alternative: Diathermy (if necessary, pricking with diathermy needle). Experiments with ablating lasers (e.g. with Erbium-YAG-Laser, Argon-Laser, CO 2 -Laser) or cryosurgery (2-fold therapy cycle in open spray method) are promising. In view of the often far-reaching changes, success with superficial procedures is limited.

Progression/forecast
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Harmless, no transition to lymphangiosarcoma described.

Literature
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  1. Cecchi R et al (1995) Lymphanioma circumscriptum of the vulva of late onset. Acta Derm Venerol 75: 79-93
  2. Chee JN et al (2014) Vesicles on the breast post-mastectomy: a quiz -- lymphangioma circumscriptum. Acta Derm Venereol 95:1037-1039.
  3. Eilers J et al (1988) Lymphangioma circumscriptum of the tongue. Acta Dermatol 14: 108-109
  4. Gupta S et al (2003) Lymphangioma circumscriptum of the penis mimicking venereal lesions. J Eur Acad Dermatol Venereol 17: 598-600
  5. Huilgol SC et al (2002 CO(2) laser therapy of vulval lymphangiectasia and lymphangioma circumscriptum. Dermatol Surgery 28: 575-577
  6. Kwon C et al (2014) Surgical resection of acquired vulvar lymphangioma circumscriptum. Arch Plast Surgery 41:183-186.
  7. Lai CH et al (2001) Lymphangioma circumscriptum treated with pulsed dye laser. Pediatric dermatol 18: 509-510
  8. 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.
  9. Weyers W et al (1990) Lymphangioma circumscriptum cysticum after surgical and radiological therapy. Dermatologist 41: 102-114

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020