An idiopathic inflammatory disease affecting the skin, hair, nails, and mucous membranes, usually self-limiting in nature.
Characteristic eruption consists of itchy, shiny, flat-topped violaceous papules and plaques favoring the extremities.
White net-like patches or ulceration of mucous membranes, nail deformity, scarring alopecia, and other types of skin lesions may also occur.
Most patients develop the disease between ages 30 and 60 years.
Hepatitis C infection may increase susceptibility, especially to oral disease.
Treatment may involve corticosteroids, retinoids, calcineurin inhibitors, immunosuppressants, and phototherapy.
History and exam
Key diagnostic factors
- violaceous, flat-topped papules or plaques
- Wickham striae
- mucosal erosions and lacy white network
- scarring alopecia
- nail involvement
Other diagnostic factors
- dorsal pterygium
- history of hepatitis C (HCV)
- hepatitis C (in some patient populations)
- -308 G/A polymorphism in TNF-alpha gene
- hepatitis B vaccination
- influenza vaccination
- psychosocial stress
- oral allergens
1st investigations to order
- clinical diagnosis
Investigations to consider
- hepatitis C screen
Susan Cooper, MBChB (Bristol), MRCGP, FRCP
Oxford University Hospitals Foundation Trust
Honorary Senior Clinical Lecturer
University of Oxford
SC is chair of the British Society for the Study of Vulval Disease and receives royalties for contributions to UpToDate.
Deepani Munidasa, MBBS, MD
Department of Dermatology
Anuradhapura Teaching Hospital
DM declares that she has no competing interests.
Dr Susan Cooper and Dr Deepani Munidasa would like to gratefully acknowledge Dr Rebecca Dunn, Dr Julia S. Lehman, Dr Megha M. Tollefson, and Dr Lawrence E. Gibson, previous contributors to this topic. RD, JSL, MMT, and LEG declare that they have no competing interests.
Brian Swick, MD
Clinical Assistant Professor
University of Iowa College of Medicine
BS declares that he has no competing interests.
David Cassarino, MD, PhD
Department of Pathology and Laboratory Medicine
University of California
DC declares that he has no competing interests.
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