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Letterer–Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. Oral cavity and gastrointestinal involvement may also be seen.
The disease has gone by several names, including Hand–Schüller–Christian disease, Abt-Letterer-Siwe disease, Hashimoto-Pritzker disease (a very rare self-limiting variant seen at birth) and histiocytosis X, until it was renamed in 1985 by the Histiocyte Society.
Before the Histiocyte Society classified histiocytoses in the 1980s, the condition was also known as "Histiocytosis X", where "X" denoted the then unknown cause. [9] [10] [11] It is now known as chronic multifocal Langerhans cell histiocytosis, a subtype of LCH. [3] The disease is rare. [12] Most present between the ages of two and six. [2]
According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year. [2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. The disease usually occurs from birth to age 15. [3]
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.
Indeterminate cell histiocytosis (LCH) is an uncommon proliferative illness where the predominant cells have characteristics from both non-Langerhans cell histiocytosis (NLCH) and Langerhans cell histiocytosis (LCH) in terms of morphology and immunophenotypic characteristics. [1]
The disease also tends to appear mainly in dogs aged under 3 years. [ 13 ] Eosinophilic granuloma lesions in dogs primarily consists of eosinophils with addition of various cell subtypes such macrophages , neutrophils , plasmocytes , lymphocytes , mast cells and many others.
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. [1]: 720 Conditions included in this group are: [1]: 720–4 Congenital self-healing reticulohistiocytosis