Histiocytoses – Hereditary Progressive Mucinous Histiocystosis in Women

April 7th, 2012

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