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Start free with EleplanRowell syndrome
ORPHA:658584Disease
What it is
A rare rheumatologic disease characterized by combination of systemic or cutaneous lupus erythematosus with erythema multiform-like skin lesions and characteristic immunologic findings including speckled pattern of antinuclear antibody, presence of anti-Ro/SSA or anti-La/SSB antibodies or rheumatoid factor. Chilblains may also be present. Patients may present with fever, hair loss, photosensitivity, polyarthralgia, morning stiffness, fatigue, weight loss and oral ulcers. Middle-aged women are predominantly affected.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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