HEREDITARY PROGRESSIVE MUCINOUS HISTIOCYTOSIS

By Gary M. White, MD


Clinical

The onset is in early adolescence of multiple flesh-colored or red pea-sized tumors occurring all over the skin is characteristic. All reported patients so far have been women in this very rare disease. Inheritance is probably autosomal dominant. There is no internal involvement.

Histologically, one sees well-circumscribed aggregates of epithelioid and spindled histiocytes in the upper and mid dermis, with mucinous stroma, and dilated vessels.

Treatment

No treatment is needed. Individual lesion may be surgically removed for cosmesis.

References

  1. . AD 1988;124;12252.
  2. . AD 1994;130;1300(CP)

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