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Systemic Histiocytosis (SH) was originally recognized in closely related Bernese Mountain Dogs. SH is a generalized histiocytic proliferative disease with a marked tendency to involve skin, ocular and nasal mucosa, and peripheral lymph nodes.
The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells, sometimes called dendritic cell histiocytosis. These cells in combination with lymphocytes , eosinophils , and normal histiocytes form typical LCH lesions that can be found in almost any organ . [ 5 ]
A similar disease in humans, Hashimoto-Pritzker disease, is also a Langerhans cell histiocytosis. [1] Dog breeds that may be more at risk for this tumor include Bulldogs, American Pit Bull Terriers, American Staffordshire Terriers, Scottish Terriers, Greyhounds, Boxers, and Boston Terriers. They also rarely occur in goats and cattle. [2]
The mutations causes white blood cells (lymphocytes, macrophages, and eosinophils) to move towards dendritic cells, resulting in damage in any organ except the heart and kidneys. [ 1 ] The disease was once thought to be a lipid storage disease as the lesions have a high cholesterol content, but the blood cholesterol is usually normal.
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]
In the rare disease Langerhans cell histiocytosis (LCH), an excess of cells similar to these cells are produced. However LCH cells stain positive to CD14 which is a monocyte marker and shows a different, hematopoietic origin for the disorder. [12] LCH can cause damage to skin, bone and other organs. [citation needed]
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.
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