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[Sporadic progressive mucinous histiocytosis]
M Cascarino1, Y Caron2, C Butnaru3
1Service d'anatomie et cytologie pathologiques, hôpital Necker-Enfants-Malades, AP-HP, 75015 Paris, France.
Annales De Dermatologie Et De Venereologie
|October 22, 2016
Summary
Progressive mucinous histiocytosis is a rare skin condition. Histopathology reveals dermal histiocytes with mucin, but its cause and treatment remain unknown.
Area of Science:
- Dermatology
- Histopathology
- Rare diseases
Background:
- Progressive mucinous histiocytosis is a rare, benign skin condition.
- Primarily affects women, with most cases being hereditary.
- Aetiology and genetic transmission are unclear.
Observation:
- A 33-year-old woman presented with a 15-year history of asymptomatic purplish papules on her legs.
- Initial diagnosis was suspected to be lichen planus.
- Punch biopsy revealed dermal histiocyte accumulation with mucin.
Findings:
- Histopathology shows dermal accumulation of histiocytes with abundant mucin deposition.
- Ultrastructural findings suggest similarities to lysosomal diseases.
- The exact cause, possibly macrophage proliferation, is undetermined.
Implications:
- Currently, no effective treatment exists for progressive mucinous histiocytosis.
- Further research is needed to understand the aetiology and genetic transmission.
- Distinguishing it from other histiocytic disorders is crucial for diagnosis.
Keywords:
Histiocytose mucineuse progressiveHistiocytose non langerhansienneMucinMucineNon Langerhans’ cell histiocytosisProgressive mucinous histiocytosisMore Related Videos
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