Synonym(s)
Congenital self-healing Langerhans cell histiocytosis; Congenital self-healing reticulohistiocytosis; Reticulohistiocytosis congenital self-healing; self-healing Hashimoto-Pritzker histiocytosis
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 histolopathological detection of Langerhans cell infiltrates.
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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.
Disseminated, blue to blackish, soft, prominent nodules and knots, with mostly intact surface. Extensive ulcerations are possible.
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 racket ).
Differential diagnosisThis section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- 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
- Hashimoto K, Pritzker MS (1973) Electron microscopic study of reticulohistiocytoma. An unusual case of congenital, self-healing reticulohistiocytosis. Arch Dermatol 107: 263-270
- Inuzuka M et al (2000) Congenital self-healing reticulohistiocytosis presenting with hemorrhagic bullae. J Am Acad Dermatol 48: S75-77
- 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
- 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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Incoming links (6)
Clioquinol cream 0.5-2% (o/w); Clioquinol lotio 0.5-5%; Quinolinol sulphate monohydrate solution 0,1 % (nrf 11.127.); Reticulohistiocytosis, congenital, self-healing; Reticulohistiocytosis multicenter; Reticulohistiocytosis(s);Outgoing links (10)
Abt-letterer-siwe disease; Clioquinol; Clioquinol cream 0.5-2% (o/w); Clioquinol lotio 0.5-5%; Hemangiomatosis, neonatal; Histiocytosis benign cephalic; Juvenile xanthogranuloma; Langerhans cell histiocytosis (overview); Polihexanide; Potassium permanganate;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer
