Juvenile xanthogranuloma

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Classification according to ICD-10
D76.3 Other histiocytosis syndromes
ICD-10 online (WHO version 2019)

A juvenile xanthogranuloma (JXG) is occurring in infancy and early childhood benign form of a histiocytosis .

Synonyms are: nevoxanthoid endothelioma; Nevoxanthoma; Xanthoma juvenile; juvenile giant cell granuloma; Xanthelasma naeviforme

distribution

It is the most common form of juvenile non-Langerhans cell histiocytosis. Heaped come neurofibromatosis type 1 or juvenile chronic myelogenous leukemia before.

root cause

The cause has not yet been clarified.

Clinical manifestations

Clinical criteria are:

  • Skin changes in the first years of life, up to 20% already at birth.
  • Mostly head and trunk affected
  • Mostly single, sometimes multilocular or disseminated, soft papules or nodules 1–3 cm in size, initially red, then yellowish.

diagnosis

The diagnosis is based on clinical findings.

Differential diagnosis

Demarcate are histiocytosis X , a mastocytosis , tuberous xanthomas , Xanthelasmoide form of urticaria pigmentosa .

Therapy and prognosis

The disease usually resolves on its own over the course of a few years, with hyperpigmentation remaining.

history

The name was coined in 1954 by EB Helwig and VC Hackney .

The first description by NF Adamson is from 1905.

literature

  • MS Israel, R. Carlos, FR Pires: Oral Juvenile Xanthogranuloma: Report of Two Cases. In: Pediatric dentistry. Vol. 39, No. 3, May 2017, pp. 238-240, PMID 28583250 .
  • C. Martínez-Morán, B. Echeverría-García, JC Tardío, J. Borbujo: Ultrasound Appearance of Juvenile Xanthogranuloma. In: Actas dermo-sifiliograficas. [electronic publication before printing] March 2017, doi: 10.1016 / j.ad.2016.11.021 , PMID 28372783 .
  • D. Raffin, C. Gardair-Bouchy, M. Samimi, P. Guadagnin, A. Maruani: Xanthogranulome juvenile chez une enfant de 4 ans. In: Archives de Pédiatrie: organe officiel de la Societe francaise de pediatrie. Vol. 24, No. 3, March 2017, pp. 298-300, doi: 10.1016 / j.arcped.2016.11.022 , PMID 28161231 .

Individual evidence

  1. ↑ a b Entry on juvenile xanthogranuloma in Flexikon , a Wiki of the DocCheck company
  2. ↑ a b c d Encyclopedia Dermatology
  3. ^ EB Helwig and VC Hackney: Juvenile xanthogranuloma (nevoxanthoendothelioma). In: American Journal of Pathology , Vol. 30, 1954, pp. 625-6.
  4. ↑ NF Adamson: Congenital xanthoma multiplex in a child. In: British Journal of Dermatology , Vol. 17, 1905, pp. 222-3.

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