Lichen sclerosus is a chronic skin condition of unknown origin that mostly affects genitals. This condition is 10 times more common in middle aged females than in males; however, it does not have any racial or ethnic predilection. It is also known by the name lichen sclerosus et atrophicus.
What is Extragenital Lichen Sclerosus?
Extragenital lichen sclerosus is the occurrence of lichen sclerosus in areas other than genitals such as skin of thighs, abdomen, buttocks, lower back, under breasts, neck, shoulders and/or armpits.
Causes and Risk Factors for Extragenital Lichen Sclerosus
The chances of getting extragenital lichen sclerosus increases with autoimmune diseases and certain bacterial infections. The disease is extremely rare in circumcised men, which is suggestive of chronic damage due to urine clogging under the foreskin.
The cause of lichen sclerosus is still unclear and studies point toward genetic, hormonal, environmental irritants, trauma and infection. It is considered an autoimmune disease and extracellular matrix protein-1 (ECM-1) antibodies have been identified in about 60 to 80% of women with vulval lichen sclerosus. Since lichen sclerosus commonly affects postmenopausal women, lack of estrogen is also considered an important factor.
Symptoms of Extragenital Lichen Sclerosus
The symptoms of extragenital lichen sclerosus depend on the severity of the case, on occasions, mild cases are found to be non-symptomatic. When symptoms are present, they can range from mild to severe pruritis (itching) with wrinkled (parched) white plaques on the affected skin. Itching is the most common symptom of these lesions. The affected area may cause discomfort or pain with easy bruising and chafing. Severe cases might lead to blistering, ulcerations and bleeding too. They have a tendency to appear on their own and subside with subsequent relapse.
Diagnosis of Extragenital Lichen Sclerosus
The diagnosis of extragenital lichen sclerosus is based on careful clinical examination along with signs and symptoms and medical history. In doubtful cases, biopsy may be needed and sent to laboratory for microscopic examination that might confirm the diagnosis and help with differential diagnosis as the lesions signs and symptoms may resemble few other lesions.
Treatment of Extragenital Lichen Sclerosus
The treatment of extragenital lichen sclerosus varies from individual to individual and in most of the individuals it subsides on its own without any treatment. In mild cases without any symptoms, it limits on its own and no treatment is required. Although, there is no definitive cure for the disease, there are some measures that may be adopted to limit the disease. Topical steroids are the mainstay for lichen sclerosus such as clobetasol, mometasone, beclomethasone. Systemic steroids may also be used.
Additional management includes oral reinoids (isotretinoin, acitretin), methotrexate, ciclosporin and immune suppressing drugs (tacrolimus, pimecrolimus). Lichen sclerosus may also be treated with ultraviolet light therapy, photodynamic therapy, CO2 ablation laser, fat injections and stem cell and platelet rich plasma injections along with surgery in severe cases.
Complications of Extragenital Lichen Sclerosus
The complications of extragenital lichen sclerosus include intense itching that may lead to bruising and ultimately secondary bacterial or fungal infections. There is also a greater probability that severe lichen sclerosus may lead to invasive squamous cell carcinomas. Recurrent and longstanding lesions may lead to self image issues in some individuals causing emotional as well as psychological stress leading to depression.
Prevention and Prognosis of Extragenital Lichen Sclerosus
Unfortunately, there are no efficient ways to prevent extragenital lichen sclerosus since the cause of this disease is still unknown. However, some measures will help prevent progression and worsening of the disease. These include incorporating good hygiene and care and refraining from scratching the area, no matter how severe the itch. Treatment of other autoimmune disorders can be undertaken too.
The prognosis of extragenital lichen sclerosus depends on the severity of the condition. The disease is usually not life threatening and mild cases have very good prognosis. Lichen sclerosus has a propensity for recurrence and severe cases may become chronic and pose difficulty with their management. However, most of the cases are asymptomatic and mild, which subside on their own, having good prognosis.
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