Reticulohistiocytosis of the skin with benign course P83.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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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 histolopathological detection 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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Disseminated, blue to blackish, soft, prominent nodules and knots, with mostly intact surface. Extensive ulcerations are possible.

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 racket ).

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.

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

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Last updated on: 29.10.2020