Lymphangioma circumscriptum D18.1

Fox and Fox, 1879




I don't want to make a specific amendment proposal here, but I was told after an email query that I should contact you via this route. There are two articles on lymphangioma circumscriptum. Could you please summarize them?

The other article is categorized under vascular medicine.

Benign, microcystic, cutaneous or cutaneous/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.

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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.

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

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

Densely standing, up to 1-3 mm large, thick-walled, sagocorn or frogspawn-like cysts that slowly increase in size (pseudovesicles). In longer populations wart-like aspect. Frequently bleeding, which is only very slowly absorbed, so that the impression of a haemangioma or haematolymphangioma can arise.

• 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.

Occasionally the congenital forms of Lymphangioma circumscriptum are associated with genetically determined syndromal diseases. A gain-of-function mutation of genes coding for proteins of the RAS/RAF signalling cascade, as detected in Noonan syndrome, leads to a typical dysmorphic clinical picture and developmental disorders of the cardiovascular and lymphatic vascular system (Roberts AE et al. 2013).

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. Fox T, Fox TC (1879) On a case of lymphangiectodes with an account of histology of the growth. Trans Path Soc Lond 30: 470
5. Gupta S et al (2003) Lymphangioma circumscriptum of the penis mimicking venereal lesions. J Eur Acad Dermatol Venereol 17: 598-600
6. Huilgol SC et al (2002 CO(2) laser therapy of vulval lymphangiectasia and lymphangioma circumscriptum. Dermatol Surgery 28: 575-577
7. Kwon C et al (2014) Surgical resection of acquired vulvar lymphangioma circumscriptum. Arch Plast Surgery 41:183-186.
8. Lai CH et al (2001) Lymphangioma circumscriptum treated with pulsed dye laser. Pediatric dermatol 18: 509-510
9. Roberts AE et al (2013) Noonan syndrome. Lancet 381:333-342
   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.
10. Schirra A et al (2018) Lymphangioma circumscriptum of the vulva in a patient with Noonan syndrome.
    J Dtsch Dermatol Ges 16:62-63.
11. Weyers W et al (1990) Lymphangioma circumscriptum cysticum after surgical and radiological therapy. Dermatologist 41: 102-114