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[Lucio phenomenon. Vasculitis or occlusive vasculopathy?]
L Azulay-Abulafia1, L Pereira Spinelli, D Hardmann
1Institut für Dermatologie Prof. R.D. Azulay, Santa Casa da Misericórdia, Rio de Janeiro.
Summary
Lucio's phenomenon (LP), a complication of Lucio leprosy (LuL), shares clinical and histopathological similarities with antiphospholipid syndrome (APS). This study suggests LP may be a form of APS secondary to LuL, responding well to multidrug treatment.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Lucio's phenomenon (LP) in Lucio leprosy (LuL) presents with cutaneous lesions whose histopathology is debated, with theories including thrombotic occlusion or leukocytoclastic vasculitis.
- Similarities between LP and antiphospholipid syndrome (APS) manifestations prompted an investigation into a potential relationship.
Observation:
- A case of LuL with LP was studied, including clinical, laboratory, and histopathological analyses, comparing findings to APS.
- Antiphospholipid antibodies, anticardiolipin (aCL) and lupus anticoagulant (LAC), were positive.
- Histopathology revealed LuL features, thrombi, endothelial proliferation, and vessel wall thickening, but not leukocytoclastic vasculitis.
Findings:
- The clinical presentation of LuL and LP in the patient mirrored established descriptions.
- Necrotic lesions in LP were clinically similar to those seen in APS.
- Histopathological findings supported a thrombotic/occlusive process rather than vasculitis.
Implications:
- The findings suggest that Lucio's phenomenon could be interpreted as antiphospholipid syndrome secondary to Lucio leprosy.
- Successful treatment with multidrug therapy (MDT-MP) for multibacillary leprosy indicates that specific treatments for APS may not always be necessary.
- This research highlights a potential link between infectious and autoimmune-like conditions, impacting diagnostic and therapeutic approaches.