Histiocytosis, hereditary, progressive, mucinous D76.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.10.2022

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History
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Bork and Hoede, 1988

Definition
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Rare, probably autosomal-dominantly inherited systemic disease from the group of cutaneous non-Langerhans cell histiocytoses.

Manifestation
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In his first decade of life.

Localization
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Nose, hands, forearms.

Clinical features
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Red to brown firm papules with smooth surface. Slow progression in the course of life. No visceral involvement known.

Histology
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Collection of irregular, spindle-shaped histiocytes mixed with lymphocytes. No giant cells or mitotic figures. Distinct accumulations of mucin.

Immunohistology: Stabilin-1 weakly positive on lesional histiocytes/macrophages; CD68 clearly positive.

Differential diagnosis
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Therapy
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No causal therapy known. Possibly CO2 laser therapy.

Literature
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  1. Bork K (1994) Hereditary progressive mucinous histiocytosis. Immunohistochemical and ultrastructural studies in an additional family. Arch Dermatol 130: 1300-1304
  2. Bork K, Hoede N (1988) Hereditary progressive mucinous histiocytosis in women. Report of three members in a family. Arch Dermatol 124: 1225-1229
  3. Mizushima J (1997) Hereditary progressive mucinous histiocytosis. Int J Dermatol 36: 958-8960
  4. Schroder K et al (1996) Hereditary progressive mucinous histiocytosis. J Am Acad Dermatol 35: 298-303
  5. Wong D et al (1999) Hereditary progressive mucinous histiocytosis. Br J Dermatol 141: 1101-1105

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Last updated on: 19.10.2022