Hashimoto-Pritzker syndrome

Other Names: Hashimoto-Pritzker histiocytosis; Hashimoto-Pritzker disease; Congenital Langerhans cell histiocytosis;

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.

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.

Signs and symptoms

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. Development of lesions in adulthood, recurrence of the disease, as well as lung and eye involvement, are very rare.

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.

Treatment

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.

NIH genetic and rare disease info

• NIH info on Hashimoto-Pritzker syndrome

Hashimoto-Pritzker syndrome is a rare disease.

Hashimoto-Pritzker syndrome Resources
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