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[Pseudotumoral lupus anetoderma. Child chorea. Development over 28 years]
Annales De Dermatologie Et De Venereologie
|January 1, 1988
Summary
This case highlights lupus anetoderma as the sole manifestation of systemic lupus erythematosus in a woman with a history of chorea. Antimalarial treatment improved skin lesions and reduced inflammation markers.
Area of Science:
- Dermatology
- Rheumatology
- Immunology
Background:
- Systemic lupus erythematosus (SLE) can present with diverse dermatological manifestations.
- Lupus anetoderma, characterized by the loss of dermal elastic fibers, is a rare cutaneous finding.
- A history of neurological disorders like chorea may precede or coincide with SLE development.
Observation:
- A 40-year-old woman presented with a 9-year history of a pseudotumoral left arm lesion.
- Histological examination revealed near-complete loss of dermal elastic fibers.
- Direct immunofluorescence showed a lupus band, supporting the diagnosis of lupus anetoderma.
Findings:
- The patient had a history of chorea at age 13, suggesting a potential link to SLE.
- Clinical and laboratory findings, including arthralgia, elevated ESR, leucopenia, hypocomplementemia, and positive antinuclear antibodies, supported an SLE diagnosis.
- Anticardiolipin antibodies, circulating anticoagulants, and VDRL tests were negative.
Implications:
- This case underscores the possibility of anetoderma being the sole presenting sign of SLE.
- The long interval between chorea and SLE diagnosis highlights the complex and sometimes delayed presentation of autoimmune diseases.
- Antimalarial therapy demonstrated efficacy in managing both cutaneous symptoms and systemic inflammation in this rare presentation.