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Hereditary progressive mucinous histiocytosis.
D Wong1, M Killingsworth, G Crosland
1Skin and Cancer Foundation, Darlinghurst, Australia.
The British Journal of Dermatology
|December 22, 1999
Summary
Hereditary progressive mucinous histiocytosis is a rare skin condition causing multiple asymptomatic papules over decades. This case highlights its distinct clinical, histological, and ultrastructural features, aiding diagnosis.
Area of Science:
- Dermatology
- Histopathology
- Genetics
Background:
- Hereditary progressive mucinous histiocytosis (HPMH) is a rare genodermatosis.
- It is characterized by the development of multiple persistent papules with prominent mucinosis.
- This condition is a rare familial form of eruptive histiocytoma.
Observation:
- A 61-year-old woman presented with asymptomatic, symmetrical papules on her hands and forearms, present for 40 years.
- Physical examination revealed multiple small, firm, shiny, skin-colored papules.
- Laboratory results and systemic review were unremarkable.
Findings:
- Histology showed dermal nodules of epithelioid histiocytes and abundant mucin, separating collagen bundles.
- Immunohistochemistry revealed histiocyte positivity for vimentin, focal positivity for alpha1-antitrypsin and lysozyme, and interstitial positivity for tenascin.
- Electron microscopy identified histiocytes with myelin bodies and zebra bodies, suggesting lysosomal storage.
Implications:
- This case underscores the characteristic clinical, histological, and ultrastructural findings of HPMH.
- Accurate diagnosis relies on integrating these diverse findings.
- Understanding HPMH contributes to the broader knowledge of histiocytic disorders and mucinoses.