Lymphangioma circumscriptum D18.1

Microcystic, cutaneous or cutaneous/subcutaneous localized circumskriptal lymphatic vascular ectasia (see also Vascular Malformation, see also Lymphangioma cavernosum) with dilated lymphatic vessels. The cause of the lyphangioma circumskriptum is

• a congenital (malformation)
• or
• acquired (mostly postoperatively or after local radiotherapy)

disorders of the lymphatic drainage, which leads to the clinically recognizable, usually dense, solid vesicles with water-clear (rarely reddish or blue-reddish) contents.

m:w=6:4;

Primary lymphangioma circumscriptum: lymphatic malformation (the dermal lymphectasias are often associated with subcutaneously located cisterns which, as parts of the embryonic lymphatic vascular system, did not receive a connection to the other lymphatic vessels). The increased intravascular pressure leads to dilatation of papillary lymphatic vessels before birth or in the first years of life.

Secondary (reactive) lymphangioma circumscriptum: Occurrence after radiotherapy, postoperatively during lymph node evacuation (latency up to decades). Frequently associated with lymphedema.

Primary lymphangioma circumscriptum: Mostly congenital or occurring in the first years of life; rarely 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%).

Dense, up to 1-3 mm large, thick-walled, sagokorn or frogspawn-like cysts, slowly increasing in size. If prolonged, wart-like aspect.

Frequent hemorrhages into the lymphatic cavities, which are very slowly reabsorbed, so that the impression of a hemangioma or hematolymphangioma may be produced.

Numerous lymphatic 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 muscularly built wall segments.

Excision of smaller lesional areas if possible in terms of surface area.

Alternative: diathermy (if necessary, pricking with diathermy needle).

Alternative: Attempts with ablative lasers (e.g. with erbium-YAG-laser, argon laser, CO ₂ -laser) or cryosurgery (2-fold therapy cycle in open spray procedure) are promising.

Note: Considering the often deep-reaching changes, success with superficial procedures is limited.

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. Degheili JA et al (2021) Acquired lymphangioma circumscriptum post-neonatal circumcision. Turk Arch Pediatr 56:539-540.

4. Eilers J et al (1988) Lymphangioma circumscriptum of the tongue. Akt Dermatol 14: 108-109
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 Surg 28: 575-577
7. Kwon C et al (2014) Surgical resection of acquired vulvar lymphangioma circumscriptum. Arch Plast Surg 41:183-186.
8. Lai CH et al (2001) Lymphangioma circumscriptum treated with pulsed dye laser. Pediatr Dermatol 18: 509-510.
9. Savas JA et al (2013) Carbon dioxide laser for the treatment of microcystic lymphatic malformations (lymphangioma circumscriptum): a systematic review. Dermatol Surg 39:1147-1157.
10. Weyers W et al (1990) Lymphangioma circumscriptum cysticum after surgical and radiologic therapy. Dermatol 41: 102-114