Congenital self-healing reticulohistiocytosis P83.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 24.10.2022

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Synonym(s)

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

History
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Hashimoto and Pritzker, 1973

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

Manifestation
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Mostly healthy children without systemic involution are affected.

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

Clinical features
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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).

Histology
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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").

Diagnosis
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Histology/electron microscopy are diagnostic.

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

External therapy
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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/forecast
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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.

Literature
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  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.

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Last updated on: 24.10.2022