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Hashimoto-Pritzker syndrome


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Other Names:
Hashimoto-Pritzker histiocytosis; Hashimoto-Pritzker disease; Congenital Langerhans cell histiocytosis; Hashimoto-Pritzker histiocytosis; Hashimoto-Pritzker disease; Congenital Langerhans cell histiocytosis; OBSOLETE: Hashimoto-Pritzker syndrome See More
Categories:
Blood Diseases; Lung Diseases
This disease is grouped under:
Langerhans cell histiocytosis

Summary Summary


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Hashimoto-Pritzker disease, also known as congenital self-healing reticulo-histiocytosis, is a very rare disease characterized by single or multiple red-purple or brown pimples (papules) and lumps (nodules) present at birth (congenital) or soon thereafter. Classic signs and symptoms include congenital or early development of painless papules, nodules or plaques with spontaneous regression in 2-3 months, and increase of a type of immune cells known as Langerhans cell histiocytes. Langerhans cells help regulate the immune system, and are normally found throughout the body. An excess of immature Langerhans cells usually form tumors called granulomas.  Most patients have multiple lesions, but in about 25% of cases there is only one lesion.[1][2][3] Development of lesions in adulthood, recurrence of the disease, as well as lung and eye involvement, are very rare.[1][3] Because the lesions often cure by themselves, treatment is usually not necessary, although topical corticoids may be used for persistent lesions. It is considered as a benign, self-limited disorder, but long-term follow-up and a thorough evaluation for internal organ abnormalities is recommended.[3][4]

Hashimoto-Pritzker disease is one form (congenital self-healing variant) of Langerhans cell histiocytosis (LCH). The other forms include a severe, acute and disseminate form known as Letterer-Siwe disease, an intermediate chronic form with multiple lesions known as Hand-Schüller-Christian disease (characterized by diabetes insipidus, bulging of the eye and localized lesions in the bone) and a less severe disease known as eosinophilic granuloma, characterized by
solitary or few, and chronic lesions of bone or other organs. Because all the variants have many common symptoms it is though that they may be manifestations of Langerhans cell histiocytosis and not separate syndromes.[1][2]  
Last updated: 6/7/2017

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • Histio UK
    PO Box 159
    Liskeard, PL14 9DQ United Kingdom
    Telephone: 07850 740241
    E-mail: Histio@HistioUK.org
    Website: https://www.histiouk.org/
  • Histiocytosis Association
    332 North Broadway
    Pitman, NJ 08071
    Toll-free: 1-800-548-2758 (from US and Canada)
    Telephone: +1-856-589-6606
    Fax: +1-856-589-6614
    E-mail: info@histio.org
    Website: https://www.histio.org/
  • Histiocytosis Association of Canada
    41 Milverton Close
    Waterdown, ON
    L8B 0A9
    Canada
    E-mail: info@histio.ca
    Website: https://histiocytosis.ca/
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Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hashimoto-Pritzker syndrome. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers


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References References


  1. Shea CR. Langerhans Cell Histiocytosis. Medscape Reference. March 7, 2017; http://emedicine.medscape.com/article/1100579-overview.
  2. Lipton JM. Langerhans Cell Histiocytosis. Merck Manuals. June, 2013; http://www.merckmanuals.com/professional/hematology-and-oncology/histiocytic-syndromes/langerhans-cell-histiocytosis.
  3. Butler DF. Congenital Self-Healing Reticulohistiocytosis. Medscape Reference. March 08, 2016; http://emedicine.medscape.com/article/1336764-overview.
  4. Zanuncio VV, de Carvalho LR, Guedes ACM, Silva CMR, Gontijo B. Case for diagnosis. Anais Brasileiros de Dermatologia. 2013; 88(6):1001-1003. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3900360/.
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