- Lichen sclerosus
Lichen sclerosus Classification and external resources
Micrograph of lichen sclerosus showing the characteristic subepithelial sclerosus (right/bottom of image). H&E stain.
ICD-10 L90.0 ICD-9 701.0 eMedicine derm/234 MeSH D018459
Lichen sclerosus (LS) (also known as "Lichen sclerosus et atrophicus":227) is an uncommon disease of unknown cause that results in white patches on the skin, which may cause scarring on and around genital skin.
Synonyms and short history
Lichen sclerosus (LS) is also known as lichen sclerosus et atrophicus (LSA), balanitis xerotica obliterans (BXO), Csillag's disease, White Spot Disease and kraurosis vulvae. Typically it's called LSA or BXO when it affects men, LS when it affects women or in referring to the disease in general.
Lichen sclerosus et atrophicus was first described in 1887 by Dr. Hallopeau. Since not all cases of lichen sclerosus exhibit atrophic tissue, et atrophicus was dropped in 1976 by the International Society for the Study of Vulvovaginal Disease (ISSVD), officially proclaiming the name lichen sclerosus. Since then this is the official medical name for this disease.
Signs and symptoms
Women are more commonly affected than men (10 to 1 ratio), particularly around and after menopause, but younger women or girls may also develop the disease. The condition most commonly occurs on the vulva and around the anus with ivory-white elevations that may be flat and glistening. There may be marked itching or the condition may be without any symptoms. There may also be thinning and shrinkage of the genital area that may make coitus, urination and defecation painful.
In males, the disease may take the form of whitish thickening of the foreskin, which cannot be retracted easily (phimosis). In contrast to women, there is no perianal involvement. In men, this genital involvement has traditionally been known as balanitis xerotica obliterans (BXO).
On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs inside the orifices of hair follicles or sweat glands on the surface. Thinning of the skin may also occur.
Although it is not clear what causes LS, four theories have been postulated.
Autoimmunity is a process in which the body fails to recognize itself and therefore attacks its own cells and tissue. Specific antibodies have been found in LS. Furthermore, there seems to be a higher prevalence of other autoimmune diseases such as diabetes mellitus type 1, vitiligo and thyroid disease.
Both bacterial as well as viral pathogens have been implicated in the etiology of LS. A disease that is similar to LS, acrodermatitis chronica atrophicans is caused by the spirochete Borrelia burgdorferi. Viral involvement of HPV and hepatitis C are also suspected.
Since LS in females is primarily found in women with a low estrogen state, hormonal influences were postulated. To date though, very little evidence has been found to support this theory.
Local skin changes
The disease often goes undiagnosed for several years, as it is sometimes not recognized and misdiagnosed as thrush or other problems and not correctly diagnosed until the patient is referred to a specialist when the problem does not clear up.
It's rare for a biopsy of the affected skin to be taken, especially so in children. A biopsy is only performed if suspecting of cancer or VIN. Histologically there's hyperkeratosis, atrophic epidermis, sclerosis of dermis and lymphocyte activity in dermis. The biopsies are also checked for signs of dysplasia.
In females, recent studies indicate that the injection of PRP (Platelet-rich plasma) and stem cells in site may reduce symptoms and improve lesions. The usefulness of this treatment in males is under study. 
Distress due to the discomfort LS causes is normal. So are concerns with self-esteem and sex. Counseling can help.
The disease can last for a considerably long time. Occasionally, "spontaneous cure" may ensue, particularly in young girls.
Lichen sclerosus is associated with a higher risk of cancer. Skin that has been scarred as a result of lichen sclerosus is more likely to develop skin cancer. Women with lichen sclerosus may develop Vulvar carcinoma. Periodic consultation is therefore necessary.
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Cutaneous keratosis, ulcer, atrophy, and necrobiosis (L82–L94, 700–701.5) Epidermal thickening
keratoderma: Keratoderma climactericum • Paraneoplastic keratoderma (Acrokeratosis paraneoplastica of Bazex ) • Aquagenic keratoderma · Drug-induced keratoderma · Paraneoplastic keratoderma • psoriasis (Keratoderma blennorrhagica)
keratosis: Seborrheic keratosis (Clonal seborrheic keratosis · Common seborrheic keratosis · Irritated seborrheic keratosis · Seborrheic keratosis with squamous atypia · Reticulated seborrheic keratosis · Dermatosis papulosa nigra) • Keratosis punctata of the palmar creases
other hyperkeratosis: Acanthosis nigricans (Confluent and reticulated papillomatosis) · Callus • Ichthyosis acquisita · Arsenical keratosis · Chronic scar keratosis · Hyperkeratosis lenticularis perstans · Hydrocarbon keratosis · Hyperkeratosis of the nipple and areola · Inverted follicular keratosis · Lichenoid keratosis · Multiple minute digitate hyperkeratosis · PUVA keratosis · Reactional keratosis · Stucco keratosis · Thermal keratosis · Viral keratosis · Warty dyskeratoma · Waxy keratosis of childhoodother hypertrophy: Keloid · Hypertrophic scar • Cutis verticis gyrata ·
Necrobiosis/granulomaGranuloma annulare (Perforating, Generalized, Subcutaneous, Granuloma annulare in HIV disease, Localized granuloma annulare, Patch-type granuloma annulare) · Necrobiosis lipoidica · Annular elastolytic giant cell granuloma · Granuloma multiforme · Necrobiotic xanthogranuloma · Palisaded neutrophilic and granulomatous dermatitis · Rheumatoid nodulosis · Interstitial granulomatous dermatitis/Interstitial granulomatous drug reactionForeign body granulomaOther/ungroupedeosinophilic dermatosis (Granuloma faciale) Dermis/
localized CTDCutaneous lupus
atrophodermaKyrle disease · Reactive perforating collagenosis · Elastosis perforans serpiginosa · Perforating folliculitis · Acquired perforating dermatosisOther
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