Histiocytoses - Overview

Histiocystoses is a category of skin disease that is composed of dysregulation of a type of white blood cell called a histiocyte which is either a macrophage or a Langerhans cell.  The category of Histocytoses is further sub divided into the Non-X Histiocytoses composed of macrophages and the X Histiocytoses composed of Langerhans cells.  The Non-X Histiocytoses include: Juvenile Xanthogranuloma, Benign Cephalic Histiocytosis, Generalized Eruptive Histiocytosis, Xanthoma Disseminatum, Progressive Nodular Histiocytosis, Papular Xanthoma, Hereditary Progressive Mucinous Histiocytosis in Women, Reticulohistiocytosis, Multicentric Reticulohistiocytosis, Indeterminate Cell Histiocytosis, and Sea Blue Histiocytosis.  The X Histiocytoses include: Congenital Self Healing Reticulohistiocytosis and Langerhans Cell Histiocytosis.

 Juvenile Xanthogranuloma Histology (http://dermaamin.com/site/images/histo-pic/t/touton-giant-cell-in-juvenile-xanthogranuloma/touton-giant-cell-in-juvenile-xanthogranuloma1.jpg)

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Sarcoidosis - Treatment of Cutaneous Disease

The treatment of cutaneous sarcoidosis is typically with topical corticosteroids or intralesional injection of 3mg/cc of Kenalog.  As the cutaneous lesions are usually asymptomatic the goal of treatment is to prevent the cosmetic disfigurement of sarcoidosis.  Systemic steroids are very effective but a high dose is generally needed, thus many side effects can result from the short term and long term use of this treatment modality.  Other treatment options are methotrexate and antimalarial medications.

Sarcoidosis (http://archderm.ama-assn.org/content/vol142/issue1/images/small/dce50005f1.gif)

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Sarcoidosis - Erythema Nodosum

Erythema Nodosum is a very common condition seen in sarcoidosis that I encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Eythema Nodosum is a non-specific cutaneous manifestation of sarcoidosis meaning that it is a reaction to a patient having the disease but when biopsied the skin does not have any sarcoidal granulomas on histology.  The lesions in Erythema Nodosum typically appear as indurated red/brown tender nodules on the anterior shins.  Treatment of Erythema Nodosum is bed rest and NSAIDs as well as treatments aimed at the sarcoidosis.  The finding of Erythema Nodosum is actually a good prognostic indicator as approximately 75% of sarcoidosis patients with these lesions will have spontaneous remission of their sarcoidosis within two years.  In some patients with sarcoidosis, they develop Lofgren's syndrome in which Erythema Nodosum is present as well as fever, uveitis, hilar adenopathy and fatigue.

 Erythema Nodosum (http://upload.wikimedia.org/wikipedia/commons/thumb/b/bb/ENlegs.JPG/230px-ENlegs.JPG)

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Sarcoidosis - Morpheaform Sarcoid

Morpheaform Sarcoidosis is a rare manifestation of cutaneous Sarcoid that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Morpheaform Sarcoidosis clinically appears like morphea in which the overlying skin is very indurated and scar like with a pink/red/purple hue.  The histology of this variant resembles Sarcoid and has no microscopic features of morphea.  Morpheafrom Sarcoidosis is most commonly seen in African American females.  Some patients respond to treatment with antimalarial drugs.

Morpheaform Sarcoidosis (http://www.medicaljournals.se/acta/content/files/web/2442-web-images/2442fig1_opt.jpeg)

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Sarcoidosis - Ichthyosiform Sarcoid

Ichthyosiform Sarcoid is a cutaneous variant of Sarcoidosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Ichthyosis is a condition in which scaly plate like coverings occur across the superficial portion of the skin. Ichthyosis can be acquired via medications or through the development of conditions such as Sarcoidosis, or inherited as a genetic condition present at birth.  Ichthyosiform Sarcoid almost never occurs in Caucasians and is much more commonly seen in African Americans.  Most patients with Ichthyosiform Sarcoid also have systemic Sarcoidosis.

 Ichthysosiform Sarcoidosis (http://dermatology.cdlib.org/114/NYU/NYUtexts/101204-1a.jpg)

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