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.

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Relapses frequently involve the sella turcica and the pituitary gland.

According to the clinical presentation and the hashkmoto internal organs involved, different names have been used in the past to describe this disease. Cutaneous involvement was considered frequent in this form. Nowadays, electron microscopy is hardly ever performed. Orthopedic disabilities are among the most common complications of LCH.

For all other comments, please send your remarks via contact us. Cutaneous lesions in the self-regressing forms usually disappear by 3 months. How to cite this article: Outline Masquer le plan.

Multifocal bone lesions are best treated with indomethacin or a short course of systemic oral steroids. Survival is better in children older than 2 years. Lesions are ill-defined, erythematous, scaly, and crusted eczematous patches and plaques -Napkin psoriasis: It may be tried in selected cases, when people cannot tolerate more aggressive treatment.

Congenital, Hashimoto-Pritzker syndrome, reticulohistiocytosis. Summary and hashimoro texts. 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.


The actual incidence remains unknown probably because of its benign and self-limiting nature. Congenital self-healing reticulohistiocytosis Hashimoto-Pritzker disease: Think of LCH in children with recurrent episodes of otitis media and mastoiditis. Biopsies can elucidate the diagnosis.

Congenital self-healing reticulohistiocytosis

However, the optimal conditioning regimens and the timing of such transplantation have not been rigorously evaluated. Moreover, some children with self-regressing diseases in early infancy have shown later recurrence of LCH with a more chronic evolution. Patients with multifocal bone disease or CNS risk should be treated with oral prednisone daily and intravenous vinblastine for 6 weeks. Disease definition Hashimoto-Pritzker histiocytosis HPH is a variant of Langerhans cell histiocytosis see this term characterized by multiple disseminated skin lesions firm, red-brown, painless papulo-nodules.

The epidermal Langerhans cell has been presumed to be the cell of origin of the disease. LCH also occurs in bone, skin, lymph node, lungs, central nervous system, other eg, thyroid, thymusspleen, liver, hematopoietic system, lungs.

One should also be alert for a history of recurrent episodes of otitis media and mastoiditis, suspect for bone involvement around the middle ear. Its infiltration can lead to unexplained diarrhea, failure to thrive and malabsorption. Access to the full text of this article requires a subscription. Pulmonary LCH is more common during the third and fourth decades of life. Less frequent sequelae include chronic pulmonary dysfunction and liver cirrhosis.


The most common are orthopedic disabilities, diabetes insipidus, hearing impairment, skin scarring.

Orphanet: Hashimoto Pritzker syndrome

Single cases with involvement of bone marrow, bone, lung and eye have been documented. The pathogenesis of LCH is still unknown. What to be alert for in the history LCH is mostly found in children younger than 15 years old.

Orthopedic treatments casting, bracing may be necessary in active disease or due to sequelae. Pediatr Blood Cancer ; Bone-marrow transplantation with reduced-intensity conditioning has shown promise as an effective salvage therapy.

Patients who continue to have active disease should repeat this regimen for another 6 weeks. Etiology Etiology remains unknown. Are You Confident of the Diagnosis? None, Conflict of Interest: There is exocytosis of Langerhans cells in the epidermis. Arguments supporting the reactive process are the occurrence of spontaneous remissions, the extensive elaboration of multiple cytokines by the LCH cells and by the T cells in the LCH lesions, and the good survival rate in the patients without organ dysfunction.

The real incidence of congenital self-healing reticulohistiocytosis CSHR may be underreported because of its high rate of spontaneous resolution and lack of clinical recognition. J Pediatr Hematol Oncol ; True hypersensitivity reactions to nitrogen mustard are rare and may be type I urticaria or type IV contact dermatitis.