Hereditary progressive mucinous histiocytosis
Onset of lesions is typically in childhood or adolescence. The papules progress in size and number throughout life. They are usually asymptomatic. Sites of predilection include hands and forearms, thighs, and face. While spontaneous resolution of nodules has been reported in the first few years of life, lesions of older children and adults do not spontaneously resolve.
HPMH is presumed to be inherited in an autosomal dominant manner. The genes and mechanisms involved are not well understood; however, the histiocytes of HPMH are thought to be of the monocyte / macrophage subset.
For more information, see OMIM.
D76.3 – Other histiocytosis syndromes
402883006 – Non-Langerhans cell histiocytic dermatosis
- Progressive nodular histiocytoma – Benign histiocytosis that may result in plaques or painful ulcerations.
- Generalized eruptive histiocytoma – Lesions may be self-resolving.
- Molluscum contagiosum – Lesions will have central umbilication.
- Acral papular mucinosis (see lichen myxedematosus)
- Multicentric reticulohistiocytosis
- Granuloma annulare
Last Updated: 02/21/2019