Lymphangiectasia

Classification according to ICD-10
I89.0	Lymphedema, not elsewhere classified, including: lymphangiectasia
ICD-10 online (WHO version 2019)

The lymphangiectasia (from latin lympha , clear water '+ gr. Ἀγγεῖον angeion , vascular' + ἔκτασις éktasis , widening ') is a rare abnormal enlargement of lymphatic vessels .

root cause

The cause of the enlargement of the lymph vessels lies in the formation of scars due to various processes. Postoperative obstruction of the lymphatic system is often the cause , an abnormal skin structure, scar keloids and scleroderma , aging due to exposure to light (photoaging) or steroid- induced atrophy can be the causes.

distribution

The frequency is not known. Acquired forms usually occur in adults as a long-term consequence after mastectomy with or without radiation therapy as chronic lymphedema .

Classification

Different forms can be distinguished depending on the organs affected . The most common are:

• Intestinal lymphangiectasia of the small intestine
  • Waldmann disease , synonyms: lymphangiectasia, primary intestinal
  • Secondary intestinal lymphangiectasia
• Cutaneous lymphangiectasia of the skin
• Penile lymphangiectasia , synonym: coronary furrows - non- venereal lymphangitis of the penis
• Conjunctival lymphangiectasia of the conjunctiva
• Pulmonary lymphangiectasia of the lungs (Njolstad syndrome)

In principle, lymphangiectasia can occur anywhere.

In the context of syndromes

Lymphangiectasia can be a feature of some syndromes :

• Bronspiegel-Zelnick syndrome , synonym: Aplasia cutis congenita, autosomal recessive
• Hennekam syndrome
• Müller-Gang derivatives - lymphangiectasia - polydactyly

Clinical manifestations

Clinically, lymphangiectasia consists of a collection of fluid-filled vessels 2 to 10 mm in diameter with normal tissue in between.

diagnosis

The diagnosis arises from the clinic and localization.

See also

• Lundehund Syndrome

Individual evidence

1. ↑ Willibald Pschyrembel : Clinical Dictionary , 266th, updated edition, de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0 , keyword lymphangiectasia
2. ↑ ^{a b c d e} emedicine
3. ↑ Lymphangiectasia, primary intestinal. In: Orphanet (Rare Disease Database).
4. ↑ Encyclopedia Dermatology Intestinal Lymphangiectasia
5. ↑ Lymphangiectasia, intestinal secondary. In: Orphanet (Rare Disease Database).
6. ↑ Encyclopedia Dermatology Cutaneous Lymphangiectasia
7. ↑ Encyclopedia Dermatology Lymphangiectasia of the penis
8. ↑ Lymphangiectasia, pulmonary, congenital. In: Orphanet (Rare Disease Database).
9. ↑ K. Bansal, B. Sureka, S. Pargewar, A. Arora: Renal lymphangiectasia: One disease, many names! In: Indian journal of nephrology. Vol. 26, No. 1, 2016 Jan-Feb, pp. 57-58, doi: 10.4103 / 0971-4065.155730 , PMID 26937083 , PMC 4753746 (free full text).
10. ↑ E. Errichetti, E. Pegolo, V. De Francesco: Acquired lymphangiectasia of the vulva. In: Journal of the German Dermatological Society = Journal of the German Society of Dermatology: JDDG. Vol. 13, No. 3, March 2015, pp. 237-239, doi: 10.1111 / ddg.12505 , PMID 25706522 .
11. ↑ Aplasia cutis congenita with intestinal lymphangiectasia. In: Orphanet (Rare Disease Database).
12. ↑ Müller-Gang derivatives - lymphangiectasia - polydactyly. In: Orphanet (Rare Disease Database).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !