Congenital self-healing reticulohistiocytosis

Congenital self-healing reticulohistiocytosis
Other names: Hashimoto–Pritzker disease,[1] and Hashimoto–Pritzker syndrome[2]
SpecialtyDermatology

Congenital self-healing reticulohistiocytosis is a condition that is a self-limited form of Langerhans cell histiocytosis.[2]: 720 

Symptoms and signs

Non-specific inflammatory response, which includes fever, lethargy, and weight loss. This is suspected of being a genetic disorder, and as the name implies, is self healing.

  • Skin: Commonly seen are a rash which varies from scaly erythematous lesions to red papules pronounced in intertriginous areas. Up to 80% of patients have extensive eruptions on the scalp.
  • Lymph node: Enlargement of the lymph nodes in 50% of Histiocytosis cases.[3]

Diagnosis

a)Round histiocyticcells with dense eosinophilic cytoplasm b,c)positive for S100 and CD1a
See also: Langerhans cell histiocytosis § Diagnosis

In terms of the diagnosis of Congenital self-healing reticulohistiocytosiss depends on histopathologic features of the disease. Additionally, it is a diagnosis of exclusion.[4]

Treatment

The management of Hashimoto-Pritzker disease is usually done via topical corticoids. Though it is a self-limited disorder, long term follow-up is recommended.[5]

History

It was first described by Ken Hashimoto and M. S. Pritzkar in 1973.[6][7]

See also

References

  1. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  2. 1 2 James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
  3. ↑ "Langerhans Cell Histiocytosis - Patient UK". Archived from the original on 2012-02-12. Retrieved 2007-05-10.
  4. ↑ "Congenital Self-Healing Reticulohistiocytosis: Background, Pathophysiology, Etiology". 8 November 2021. Archived from the original on 11 September 2021. Retrieved 30 December 2021.
  5. ↑ "Hashimoto-Pritzker syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Archived from the original on 23 November 2021. Retrieved 29 December 2021.
  6. ↑ John Thorne Crissey; Lawrence C. Parish; Karl Holubar (2013). Historical Atlas of Dermatology and Dermatologists. CRC Press. p. 179. ISBN 978-1-84214-100-7. Archived from the original on 2022-01-11. Retrieved 2020-12-16.
  7. ↑ Lee, Young H.; Talekar, Mala K.; Chung, Catherine G.; Bell, Moshe D.; Zaenglein, Andrea L. (February 2014). "Congenital Self-Healing Reticulohistiocytosis". The Journal of Clinical and Aesthetic Dermatology. 7 (2): 49–53. ISSN 1941-2789. PMC 3935651. PMID 24578781.
External resources


This article is issued from Offline. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.