Lichen sclerosus (LS) (et atrophicus) (λειχήν/leichen = lichen on trees or bodies; σκληρός/sklēros = dry, hard, firm, brittle; synonyms: Lichen albus; Lichen atrophicus; Lichen sclerosus; Lichen sclerosus et atrophicans; Lichen sclerosus et atrophicus LSA); Morphoeid scleroderma; White spot disease; White spot disease; ICD-10-GM L90. 0: Lichen sclerosus et atrophicus) is a rare, chronic inflammatory disease of the connective tissue, which probably belongs to the autoimmune diseases. Lichen describes a thickening of the epidermis and sclerosis a proliferation of connective tissue with hardening.
Lichen sclerosus (LS) belongs to the atrophic dermatoses (skin diseases). These are skin diseases associated with tissue atrophy. Lichen sclerosus et atrophicus (LSA) affects the genital region in over > 80% of cases.
The disease is not contagious.
Sex ratio: males to females is 1: 6-10.
Peak incidence: In children, the disease occurs predominantly between the ages of 5 and 11 years. Females and males are usually affected in the 5th and 6th decades of life; uncircumcised males are usually affected. Women preferentially after menopause (female menopause).
The prevalence (disease incidence) is reported to be 1.4-2.1/100,000 in cross-sectional analyses of larger non-preselected male US collectives.The prevalence of LS in prepubertal girls is estimated to be 1: 900.
The incidence (frequency of new cases) is approximately 14 cases per 100,000 population per year (in Germany).
Course and prognosis: Lichen sclerosus is a chronic inflammatory connective tissue disease with a relapsing course that may last for decades. In boys/men, the penis (glans and prepuce) is usually affected. This is referred to as balanitis xerotica obliterans (a form of glans inflammation), which leads to phimosis (narrowing of the foreskin) requiring surgery. In female infants, the disease can destroy the hymen (hymen). In females, the genitoanal area is affected in approximately 90% of cases. In the late stages, the disease shows varying degrees of severity of atrophy (regression) of the vulva (entirety of the external primary sex organs). In infantile lichen sclerosus, there is a chance of cure.
Comorbidities: There is a frequent comorbidity with autoimmune diseases such as diabetes mellitus type 1, Hashimoto’s thyroiditis (autoimmune disease leading to chronic inflammation of the thyroid gland) and vitiligo (white spot disease). Other common conditions include inflammatory bowel disease, alopecia areata (circular hair loss), pernicious anemia (a form of anemia), rheumatoid arthritis (a chronic inflammatory multisystem disease that usually manifests as synovitis (inflammation of the synovial membrane); it predominantly affects the joints, but less commonly other organs such as the eyes and skin), and psoriasis (psoriasis). The prognosis is quoad vitam (“in terms of life/survival”) good, quoad sanationem (“in terms of cure”) doubtful.
