Congenital self-healing reticulohistiocytosis (also known as “Hashimoto–Pritzker disease,” and “Hashimoto–Pritzker syndrome”) is a condition that is a. -Hashimoto-Pritzker disease, or congenital self-healing reticulohistiocytosis, was initially described in neonates, or during the first months of life, as a cutaneous. The diagnosis of congenital self-healing reticulohistiocytosis (Hashimoto–Pritzker syndrome) was considered based upon histopathogical findings along with.

Author: Tukinos Samugami
Country: Oman
Language: English (Spanish)
Genre: Relationship
Published (Last): 17 July 2005
Pages: 81
PDF File Size: 5.13 Mb
ePub File Size: 1.34 Mb
ISBN: 974-6-37317-572-3
Downloads: 62209
Price: Free* [*Free Regsitration Required]
Uploader: JoJogore

Reclassification Working Group of the Histiocyte Society”. Neonatal and early infantile cutaneous Langerhans cell histiocytosis: No consensus exists for the optimal therapy of LCH. Langerhans cell hyperplasia may be seen in scabies, insect bites, contact dermatitis ; non-Langerhans cell histiocytoses juvenile xanthogranuloma, Destombes-Rosai-Dorfman disease, Erdheim-Chester disease ; leukemia cutis with monocytic differentiation monocytic acute myeloid leukemia, chronic myelomonocytic leukemia.

Less common sequelae include chronic growth retardation, pulmonary dysfunction, liver cirrhosis, loose teeth, proptosis, and secondary malignancies due to chemotherapeutic treatments.

Congenital Langerhans cell histiocytosis Prevalence: LCH is mostly found in children younger than 15 years old. The lesional Langerhans cells have been shown to be clonal.

You can move this window by clicking on the headline. Single cases with involvement of bone marrow, bone, lung and eye have been documented. Langerhans cell histiocytosis LCH is a rare multi-organ disease due to accumulation of Langerhans cells in various tissues. Clonality is not sufficient to prove the neoplastic nature of the disease. According to the age of the lesion, the histopathologic aspect of cutaneous LCH may vary and the differential diagnosis includes the following entities: Patients with multisystem disease, craniofacial involvement, long-standing disease, or reactivation may be at increased risk of developing diabetes insipidus.

The Journal of Clinical and Aesthetic Dermatology. In adults, skin-only disease may also be treated with acitretin or thalidomide, which have been reportedly effective in some cases.


To date, no molecular or immunohistochemical criteria is able to predict self-regression of the lesions in infants with cutaneous LCH. Bisphosphonates can also be used to reverse bone destruction and alleviate the pain of bony lesions.

Material and methods Histologic evaluation. Check this box if you wish to receive a copy of your message. In France, estimated annual incidence is 4. Hematologic involvement portends a poorer prognosis and is the main cause of disease-related death.

Baseline clinical and biological evaluations should orientate the specific imaging studies and other tests to consider, according to suspected organ involvement. Views Read Edit View history. Approximately new cases per year are reported hsahimoto the United States.

Orphanet: Hashimoto Pritzker syndrome

None, Conflict of Interest: Histiocytic disorders of children and adults. However, liver cirrhosis is a very infrequent sequela. Additional hormonal hashimoro may be done in case of specific signs or presence of diabetes insipidus. Chest computed tomography CT will be performed in case of suspected lung involvement; brain and pituitary magnetic resonance imaging MRI in case of neurologic signs or signs of diabetes insipidus; ear CT scan in case of suspected ENT involvement; cholangio-MRI in case of suspected liver involvement.

How to cite this URL: Skeletal radiograph survey is mandatory. In case of CBC perturbation, a bone marrow aspirate is haahimoto. Fourteen percent of cases at diagnosis show involvement of an organ at risk.

Congenital self-healing reticulohistiocytosis

Disease severity evaluation is based on the stratification between single-system disease and multisystem disease, and on the involvement of risk organs spleen, liver, hematopoietic system, lungs. Down-regulation of E-cadherin in LCH cells may be associated with dissemination.

Middle ear involvement leads to recurring otitis media and pritzkwr secondary hearing loss. Congenital self-healing reticulohistiocytosis also known as “Hashimoto—Pritzker disease,” [1] and “Hashimoto—Pritzker syndrome” [2] is a condition that is a self-limited form of Langerhans cell histiocytosis.


You must be a registered member of Dermatology Advisor to post a comment. Sitemap What’s New Feedback Disclaimer. The disease seems to be due to accumulation rather than proliferation of the LCH cells. They should be especially aware of the poorer prognosis for refractory disease with hematologic dysfunction.

Unifocal bone, lymph node or cutaneous disease has an excellent prognosis.

There was a problem providing the content you requested

Cladribine 2-Cda pritzier be used as salvage monotherapy for patients with risk-organ involvement or patients with low-risk LCH hashimoro to initial therapy with steroids and vinblastine. Furthermore, no genetic abnormalities have been found in LCH via analysis of ploidy, karyotype, single-nucleotide polymorphism arrays, and array-based comparative genomic hybridization.

Otherwise, localized-to-moderate skin disease is best treated with moderate-to-potent topical steroids eg, mometasone furoate cream 0. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.

Access to the text HTML. Multisystem LCH is defined as an involvement of 2 or more organs or organ systems, irrespective of involvement of risk organ.

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. Additional information Further information on this disease Classification s 4 Gene s 1 Clinical signs and symptoms Other website s 3.