Sporadic progressive mucinous histiocytosis [Histiocytose mucineuse progressive sporadique]

Progressive mucinous histiocytosis is a very rare, benign, non-Langerhans' cell histiocytosis limited to the skin. This disorder has been observed solely in women, with the exception of three cases in male patients, and most cases are hereditary. The hereditary forms begin in childhood, with sporadic cases occurring later, and it is characterized by numerous papules of slow progression. The aetiology and mode of genetic transmission remain unclear. We report one sporadic case of progressive mucinous histiocytosis.