This clinical entity is usually present at birth or appears very soon thereafter. Affected infants are otherwise healthy andskin lesions tend to involute spontaneously within weeks tomonths. [docslide.net]

Hashimoto-Pritzker syndrome (Hashimoto-Pritzker congenital self-healing reticulohistiocytosis) is a rare form of Langerhans cell histiocytosis that is usually present at birth or develops in the early neonatal period. [medeponyms.com]

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 [rarediseases.info.nih.gov]

A 2-month-old Japanese boy was diagnosed with "skin only Langerhans cell histiocytosis" after developing typical skin lesions positive for CD1a with a negative systemic workup. Complete regression of skin lesions occurred by age 9 months. [emedicine.medscape.com]

Treatment depends on the extent and the severity of the dis-ease. Patients with single-system bone or skin disease have agood prognosis and often require no or only limited treatment. [docslide.net]

Treatment - Hashimoto-Pritzker syndrome Not supplied. Resources - Hashimoto-Pritzker syndrome Not supplied. [checkorphan.org]

[…] 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 [rarediseases.info.nih.gov]

Prognosis - Hashimoto-Pritzker syndrome Not supplied. Treatment - Hashimoto-Pritzker syndrome Not supplied. Resources - Hashimoto-Pritzker syndrome Not supplied. [checkorphan.org]

[…] in cases with pulmonary hypertension Worse prognosis for those with baseline poor respiratory function and those with concurrent neoplasms Worse prognosis with extremes of age, prolonged constitutional symptoms, multiorgan involvement, extensive cysts [pathologyoutlines.com]

These syndromes represent a spectrum of severity and prognosis of the same underlying disorder which is usually sporadic. It has occurred in monozygotic twins and in a familial pattern. [database.pherobase.com]

The prognosis in older children and in those with very widespread disease tends to be poor. Figure 21–6 This is a spectrum of disease that encompasses Letterer-Siwe disease, Hand-Schüller-Christian disease, and eosinophilic granuloma. [accesspediatrics.mhmedical.com]

Patients with single-system bone or skin disease have agood prognosis and often require no or only limited treatment. In a recent study, McLelland et al showed that in 14 patientswith single-system disease, eight required no treatment. [docslide.net]

Etiology Etiology remains unknown. The documents contained in this web site are presented for information purposes only. [orpha.net]

A possible infectious etiology of LCH has been explored. [mussenhealth.us]

2007).The etiopathogenesis of reticulohistiocytosis is unknown, but the possibility of an immunological etiology has been sug- gested by the occurrence of a number of immunological abnor-malities in this disease. [docslide.net]

[…] syndrome Synonym(s): - Congenital Langerhans cell histiocytosis Classification (Orphanet): - Rare hematologic disease - Rare oncologic disease - Rare respiratory disease - Rare systemic or rheumatologic disease Classification (ICD10): - Neoplasms - Epidemiological [csbg.cnb.csic.es]

Summary Epidemiology Less than 50 cases reported so far. Clinical description Onset is at birth or during the neonatal period. The skin lesions may affect the entire body, or may manifest on palms and soles or head and scalp only. [orpha.net]

Epidemiologic study of Langerhans cell histiocytosis in children. J Pediatr 1997;130:774-84. 27. Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, et al. [content.sciendo.com]

Langerhans cell granulomatosis, histiocytosis X ("H-X") Multisystem diseases: Letterer-Siwi disease, Hand-Schüller-Christian disease, Hashimoto-Pritzker syndrome, where lung involvement is histologically indistinguishable from cellular phase of PLCH Epidemiology [pathologyoutlines.com]

Epidemiology LCH is rare in children. LCH affects 5–6 million children per year between the ages of 1 and 15. Incidence in children LCH is considered a rare disease. [obgynkey.com]

Although nearly a century has passed since histiocytic disorders were recognized, their pathophysiology has started to be elucidated with the application of molecular analyses. [disabled-world.com]

50 years More common in Caucasian than African Americans or Asians Sites 50% of cases only involve lung 20% of those with multicentric disease (i.e. bone, skin, lymph nodes, spleen, CNS, pituitary, rarely thyroid and thymus) have lung involvement Pathophysiology [pathologyoutlines.com]

The epidemiology of this disease, its pathophysiology and complications, and the roles of surgery and medical therapy are reviewed. [academic.oup.com]

Pathophysiology Langerhans cells arise from bone marrow precursors to populate the epidermis and act as antigen-presenting cells; they play a key role in immune surveillance and contact sensitivity. [emedicine.medscape.com]

Prevention - Hashimoto-Pritzker syndrome Not supplied. Diagnosis - Hashimoto-Pritzker syndrome signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of Hashimoto-Pritzker syndrome. [checkorphan.org]

Their ultimate goal is to promote medical research leading to the prevention and cure of histiocytosis. [rarediseases.org]

[…] hypothalamic pituitary axis hybridization protection assay HPC hematopoietic precursor cells hemangiopericytoma history of present condition HPD hearing protection device high-protein diet HP&D hemoprofile and differential HPDP health promotion and disease prevention [globalrph.com]

Centers for Disease Control and Prevention, more than twenty percent of male and female adults in the U.S. smoke cigarettes, while more than eighty percent of them light up a cigarette daily. Get My Free Ebook [mussenhealth.us]

Overall, patients with CSHR have an excellent prognosis, with a 100-percent survival with minimal or no treatment, given that it does not progress to multisystem disease.[22] Recognition of this entity at presentation is important as it will potentially prevent [jcadonline.com]