Histiocytoses - Indeterminate Cell Histiocytosis

Indeterminate Cell Histiocytosis is a rare type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Indeterminate cells are the precursors of Langerhans cells but lack the distinctive intracellular Birbeck granules.  Indeterminate Cell Histiocytosis is most common in males and occurs equally across all ages.  Clinically, Indeterminate Cell Histiocytosis appears as yellow/red/brown papules and nodules on the skin.  Rarely, does Indeterminate Cell Histiocytosis involve internal organs and the bones.  In severe cases, chemotherapy has been successfully used as a treatment.

      

Indeterminate Cell Histiocytosis (

http://archderm.ama-assn.org/content/vol147/issue8/images/small/dlv15006f1.gif)

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Histiocytoses - Multicentric Reticulohistiocytosis

Multicentric Reticulohistiocytosis is a rare Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Multicentric Reticulocytosis usually appears after age 50 and is most common in women.  Patients present with reddish brown papules typically on the fingers around the nailfold and the clinical appearance has been termed "coral beads."  The skin can also be involved on the extremities (especially over joints), face, ears, neck and trunk.  Multicentric Reticulohistiocytosis is a systemic disease and osteoarthritis is a hallmark finding.  This arthritis can be very destructive and disabling.  When the arthritis affects the fingers the term "opera glass shortening" has been used to describe the telescopic shortening of the phalange bones.  Other organ systems involved are: heart, lungs, liver, lymph nodes, liver, muscles and gastrointestinal tract.  Approximately, 1/4 of all patients with Multicentric Reticulohistiocytosis also have an internal malignancy.  Thus, the use of immunosuppresant therapy to treat this inflammatory disease must be weighed against lowering the body's defenses against fighting malignancy.

 Multicentric Reticulohistiocytosis (http://archderm.ama-assn.org/content/vol140/issue8/images/medium/dce30011f1.jpg)

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Histiocytoses - Hereditary Progressive Mucinous Histiocystosis in Women

Hereditary Progressive Mucinous Histiocytosis in Women is a rare type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  In patients with this disease it is genetically derived and is transmitted either in an autosomal dominant or X-linked fashion.  It is exclusively found in women. Clinically, the skin lesions appear as brown to red nodules and papules with a predilection for the face and extremities.  Typically, the lesions begin in the teenage years and continue to accumulate throughout life and do not usually resolve spontaneously.  Fortunately, this skin disease is strictly limited to the skin and does not have any systemic involvement.

 Hereditary Progressive Mucinous Histiocytosis in Women (http://www.medicaljournals.se/acta/content/files/web/3118-web-images/3118fig2_opt.jpeg)

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Histiocytoses - Papular Xanthoma

Papular Xanthoma is a type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands and Conroe dermatology offices.  Papular Xanthoma typically appears as multiple small yellow papules.  The lesions in Papular Xanthoma can be found anywhere on the body and have a predilection for the extensor surfaces.  In some instances, the lesions may resolve and leave scars.  Papular Xanthoma does not have any systemic involvement and the lipid levels are usually normal.

 Papular Xanthoma (http://archderm.ama-assn.org/content/vol143/issue5/images/medium/dlv60060f1.jpg)

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Histiocytoses - Progressive Nodular Histiocytosis

Progressive Nodular Histiocytosis is a type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Clinically, a patient develops a nodule that has a superficial portion near the surface of the skin and a deeper portion that can extend into the fat layer of the skin.  Multiple lesions can develop and are usually symmetrical.  In most patients, Progressive Nodular Histiocytosis is limited to the skin with no internal involvement.

 Progressive Nodular HIstiocytosis (http://binary-services.sciencedirect.com/content/image/1-s2.0-S1027811711000607-gr1.sml)

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