Overview of cutaneous lupus erythematosus
- Joseph F Merola, MD, MMSc, FAAD, FACR
Joseph F Merola, MD, MMSc, FAAD, FACR
- Assistant Professor, Harvard Medical school
- Department of Dermatology
- Department of Medicine, Division of Rheumatology
- Brigham and Women's Hospital
- Samuel L Moschella, MD, FAAD, FACP
Samuel L Moschella, MD, FAAD, FACP
- Lahey Clinic
- Section Editors
- David S Pisetsky, MD, PhD
David S Pisetsky, MD, PhD
- Section Editor — Lupus
- Professor of Medicine and Immunology
- Duke University Medical Center
- Jeffrey Callen, MD, FACP, FAAD
Jeffrey Callen, MD, FACP, FAAD
- Editor-in-Chief — Dermatology
- Section Editor — Skin and Systemic Disease
- Professor of Medicine
- University of Louisville School of Medicine
- Deputy Editors
- Abena O Ofori, MD
Abena O Ofori, MD
- Deputy Editor — Dermatology
- Assistant Professor
- Department of Medicine, Dermatology Division
- Medical College of Georgia
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine
- Harvard Medical School
Cutaneous lupus erythematosus (cutaneous LE) includes three subsets of LE-specific skin diseases: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE) (table 1). CCLE encompasses discoid lupus erythematosus (DLE), lupus erythematosus tumidus (LE tumidus), lupus profundus (also known as lupus panniculitis), chilblain lupus erythematosus (chilblain LE), and lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome).
Cutaneous LE can occur as a manifestation of systemic lupus erythematosus (SLE) or independent of SLE. The varying strengths of association between SLE and the individual subtypes of cutaneous LE are best illustrated in a graphic (figure 1). Patients with SLE may also develop a variety of LE-nonspecific skin diseases, cutaneous disorders that lack histologic features of LE, but occur with increased frequency in patients with SLE.
An overview of the various clinical manifestations of cutaneous LE and LE-nonspecific skin diseases is provided here. In-depth discussions of SLE and the treatment of DLE and SCLE are provided separately. (See "Overview of the clinical manifestations of systemic lupus erythematosus in adults" and "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults" and "Initial management of discoid lupus and subacute cutaneous lupus" and "Management of refractory discoid lupus and subacute cutaneous lupus".)
The modified Gilliam grouping system for cutaneous manifestations of LE provides a helpful organizational framework for the related but distinct clinical entities that comprise LE-specific and LE-nonspecific skin diseases. The three subcategories under LE-specific skin disease and their major clinical variants include:
●Acute cutaneous lupus erythematosus (ACLE)
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- ASSOCIATION WITH SLE
- LUPUS ERYTHEMATOSUS-SPECIFIC SKIN DISEASE
- Acute cutaneous lupus erythematosus
- Subacute cutaneous lupus erythematosus
- - Drug-induced SCLE
- - Neonatal lupus
- Chronic cutaneous lupus erythematosus
- - Discoid lupus erythematosus
- - Less common subtypes
- Lupus erythematosus tumidus
- Lupus profundus (lupus panniculitis)
- Chilblain lupus erythematosus
- Lupus erythematosus-lichen planus overlap syndrome
- - Direct immunofluorescence (lupus band test)
- LUPUS NONSPECIFIC SKIN DISEASE
- Vascular abnormalities
- Nonscarring alopecia
- OTHER MANIFESTATIONS OF LUPUS
- Mucosal manifestations
- Bullous cutaneous lupus erythematosus
- Neonatal lupus
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS