Histiocytosis, hereditary, progressive, mucinousD76.0
HistoryThis section has been translated automatically.
Bork and Hoede, 1988
DefinitionThis section has been translated automatically.
Rare, probably autosomal-dominantly inherited systemic disease from the group of cutaneous non-Langerhans cell histiocytoses.
ManifestationThis section has been translated automatically.
In his first decade of life.
LocalizationThis section has been translated automatically.
Nose, hands, forearms.
Clinical featuresThis section has been translated automatically.
Red to brown firm papules with smooth surface. Slow progression in the course of life. No visceral involvement known.
HistologyThis section has been translated automatically.
Collection of irregular, spindle-shaped histiocytes mixed with lymphocytes. No giant cells or mitotic figures. Distinct accumulations of mucin (see mucinoses below).
Immunohistology: Stabilin-1 weakly positive on lesional histiocytes/macrophages; CD68 clearly positive.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
No causal therapy known. Possibly CO2 laser therapy.
LiteratureThis section has been translated automatically.
- Bork K (1994) Hereditary progressive mucinous histiocytosis. Immunohistochemical and ultrastructural studies in an additional family. Arch Dermatol 130: 1300-1304
- Bork K, Hoede N (1988) Hereditary progressive mucinous histiocytosis in women. Report of three members in a family. Arch Dermatol 124: 1225-1229
- Mizushima J (1997) Hereditary progressive mucinous histiocytosis. Int J Dermatol 36: 958-8960
- Schroder K et al (1996) Hereditary progressive mucinous histiocytosis. J Am Acad Dermatol 35: 298-303
- Wong D et al (1999) Hereditary progressive mucinous histiocytosis. Br J Dermatol 141: 1101-1105