Images (7)

Congenital self-healing reticulohistiocytosisP83.8

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 24.10.2022

Dieser Artikel auf Deutsch

Synonym(s)

Congenital self-healing Langerhans cell histiocytosis; Congenital self-healing reticulohistiocytosis; Reticulohistiocytosis congenital self-healing; self-healing Hashimoto-Pritzker histiocytosis

Requires free registration (medical professionals only)

Please login to access all articles, images, and functions.

Our content is available exclusively to medical professionals. If you have already registered, please login. If you haven't, you can register for free (medical professionals only).


Requires free registration (medical professionals only)

Please complete your registration to access all articles and images.

To gain access, you must complete your registration. You either haven't confirmed your e-mail address or we still need proof that you are a member of the medical profession.

Finish your registration now

HistoryThis section has been translated automatically.

Hashimoto and Pritzker, 1973

DefinitionThis section has been translated automatically.

Rare, congenital, benign Langerhans cell histiocytosis characterized by congenital nodular infiltration of the skin and histopathologic evidence of Langerhans cell infiltrates.

ManifestationThis section has been translated automatically.

Mostly healthy children without systemic involution are affected.

LocalizationThis section has been translated automatically.

Especially the face and the hairy head are affected. Occurrence on the whole integument is possible.

Clinical featuresThis section has been translated automatically.

In about 1/4 of newborns, only a single focus is found, usually a flat, asymptomatic, 0.2-1.0cm reddish plaque or plaque, with the surface usually intact. Ulceration is possible. The general condition of the patient remains undisturbed. The occurrence of multiple lesions is possible. In this constellation (see Fig.), Abt-Letterer-Siwe disease must be included in the differential diagnosis (progression or healing in the first months of life).

HistologyThis section has been translated automatically.

Dense dermal infiltrate of histiocytic cells (immunohistology: CD1a pos./S100 pos.) with irregular, large nuclei and partially foamy cytoplasm. Erythrocyte diapedesis.

Electron microscopy: Histiocytes with lamellar, false and true myelin figure-like inclusions. No Birbeck granules ("tennis rackets").

DiagnosisThis section has been translated automatically.

Histology/electron microscopy are diagnostic.

Differential diagnosisThis section has been translated automatically.

Abt-Letterer-Siwe disease; neonatal infant hemangiomatosis; juvenile xanthogranuloma; benign cephalic histiocytosis.

General therapyThis section has been translated automatically.

No causal therapy required. Spontaneous healing in 2-3 months.

External therapyThis section has been translated automatically.

To avoid superinfections and scars, it may be advisable to use antiseptic topicals such as 0.5-2% clioquinol cream/lotio(e.g. R049, R050, Linola-Sept) or disinfectant solutions with potassium permanganate (light pink), polihexanide (Serasept, Prontoderm) or quinolinol (e.g. Chinosol 1:1000 or R042 ).

Progression/forecastThis section has been translated automatically.

Spontaneous regression within the first years of life. Caution: Initially, differentiation from Abt-Letterer-Siwe disease may be difficult. Here, the clinical course must be awaited.

LiteratureThis section has been translated automatically.

  1. Brazzola P et al (2003) Congenital self-healing langerhans cell histiocytosis with atrophic recovery of the skin: clinical correlation of an immunologic phenomenon. J Pediatr Hematol Oncol 25: 270-273.
  2. Hashimoto K, Pritzker MS (1973) Electron microscopic study of reticulohistiocytoma. An unusual case of congenital, self-healing reticulohistiocytosis. Arch Dermatol 107: 263-270
  3. Inuzuka M et al (2000) Congenital self-healing reticulohistiocytosis presenting with hemorrhagic bullae. J Am Acad Dermatol 48: S75-77.
  4. Larralde M et al (2003) Congenital self-healing Langerhans cell histiocytosis: the need for a long term follow up. Int J Dermatol 42: 245-246
  5. Laugier P, Hunziker N et al (1975) Reticulohistiocytosis of benign evolution (Hashimoto-Pritzker type). Electron microscopy study. Ann Dermatol Syphiligr (Paris) 102: 21-31.

  6. Rizzoli A et al (2021) Congenital self-healing reticulohistiocytosis in a newborn: unusual oral and cutaneous manifestations. Ital J Pediatr 47:135.

Authors

Last updated on: 24.10.2022

Author: Prof. Dr. med. Peter Altmeyer