Hemangiomatosis

Classification according to ICD-10
Q27.8	Hemangiomatosis, affecting one localization
Q82.8	Other specified congenital skin malformations
D18.00	Hemangiomatosis, affecting one localization
ICD-10 online (WHO version 2019)

A hemangiomatosis referred to the presence of numerous haemangioma ( hemangiomas ) simultaneously. It is considered an embryonic malformation and can be part of a systemic disease.

A disturbance of the longitudinal growth can occur with an epiphyseal involvement.

Special forms

There are forms with special courses or properties:

• Benign neonatal hemangiomatosis
• Diffuse neonatal hemangiomatosis
• Disseminated hemangiomatosis of the skin with visceral involvement
• Disseminated form with visceral involvement, but without skin involvement

Visceral involvement can pose a vital threat to the affected child. Heart failure can occur as a result of the shunt effect, particularly if the liver is involved.

Benign neonatal hemangiomatosis

In this form, the changes are limited to the skin with no apparent involvement of internal organs. The manifestation occurs already in the newborn.

Diffuse neonatal hemangiomatosis

Compared to the benign form, diffuse neonatal hemangiomatosis has additional organ involvement. The disease is very rare and the cause is unknown. Clinically, there are numerous, rapidly growing hemangiomas of the skin and internal organs such as the liver, lungs, gastrointestinal tract or brain.

Capillary pulmonary hemangiomatosis

The capillary pulmonary hemangiomatosis is a rare dominant autosomal or autosomal - recessive hereditary form with infiltration of the lung resulting in a pulmonary hypertension .

Viscerocutaneous hemangiomatosis

Viscerocutaneous hemangiomatosis, also known as Bean syndrome or blue rubber bleb naevus syndrome , is a rare autosomal dominant inherited malformation with frequent acute bleeding.

Syndromes

Hemangiomatosis is also present in the following syndromes:

• Sturge Weber Syndrome
• Hippel-Lindau disease
• Klippel-Trenaunay Syndrome
• Maffucci-Kast syndrome
• Osler's disease
• Hemangiomatosis-Porencephaly Syndrome (Lubliner's Syndrome)

literature

• K. Frank: [Possible causes of hemangiomatosis with reference to its clinical picture]. In: Pediatrics and border areas. Volume 20, Number 4, 1981, pp. 231-234, ISSN  0030-932X . PMID 6289223 .

Individual evidence

1. ^ W. Pschyrembel: Clinical dictionary. 265th edition. Walter de Gruyter, 2014, ISBN 978-3-11-018534-8 , p. 634.
2. ^ 125th Congress of the German Society for Surgery from April 22 to 25, 2008 in Berlin
3. ↑ D. Bandyopadhyay: Benign neonatal hemangiomatosis. In: Indian pediatrics. Vol. 51, No. 7, July 2014, p. 591, ISSN  0974-7559 . PMID 25031151 .
4. ↑ hemangiomatosis. In: Orphanet (Rare Disease Database).
5. ↑ hemangiomatosis. In: Orphanet (Rare Disease Database).
6. ↑ hemangiomatosis. In: Orphanet (Rare Disease Database).
7. ↑ M. Betke, F. Eckert, W. Heldwein, R. Hein, M. Landthaler: Viscerocutaneous hemangiomatosis - the so-called blue rubber bleb nevus syndrome. In: The dermatologist ; Journal of Dermatology, Venereology, and Allied Fields. Vol. 42, No. 1, January 1991, pp. 23-27, ISSN  0017-8470 . PMID 2010283 .
8. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
9. ^ I. Modzelewska, E. Piorkowska: Porencephaly combined with generalized hemangiomatosis of the skin. In: Monthly for Pediatrics. Vol. 121, No. 9, September 1973, pp. 600-602, PMID 4767090 .

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 !