Hashimoto-Pritzker Disease
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Congenital self-healing reticulohistiocytosis is a condition that is a self-limited form of
Langerhans cell histiocytosis Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Symptoms range from isolated bone lesions to multisystem dise ...
.


Symptoms

Non-specific
inflammatory response Inflammation (from la, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants, and is a protective response involving immune cells, blood vessels, and molecu ...
, which includes fever,
lethargy Lethargy is a state of tiredness, sleepiness, weariness, fatigue, sluggishness or lack of energy. It can be accompanied by depression, decreased motivation, or apathy. Lethargy can be a normal response to inadequate sleep, overexertion, overwo ...
, 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 In medicine, an intertriginous area is where two skin areas may touch or rub together. Examples of intertriginous areas are the axilla of the arm, the anogenital region, skin folds of the breasts and between digits. Intertriginous areas are known t ...
areas. Up to 80% of patients have extensive eruptions on the scalp. *Lymph node: Enlargement of the lymph nodes in 50% of Histiocytosis cases.


Diagnosis


Treatment


History

It was first described by Ken Hashimoto and M. S. Pritzkar in 1973.


See also

*
List of cutaneous conditions Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against t ...
*
X-type histiocytosis X-type histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of Langerhans cells, as opposed to Non-X histiocytosis in which the infiltrates contain monocytes/macrophages. Conditions included in this ...


References


External links

Monocyte- and macrophage-related cutaneous conditions {{Cutaneous-condition-stub