Lichen sclerosus

Lichen sclerosus is defined as a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy and scarring. Lichen sclerosus has both genital and extragenital presentations and also goes by the names lichen sclerosus at atrophicus.

lichen sclerosus

 

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Age/sex prevalence of Lichen sclerosus

Lichen sclerosus occurrence from male to female ratio is 1:6 with female genital cases make more of the cases.
Extragenital lichen sclerosus is very rare in children.

Races affected by Lichen sclerosus

Individuals of any race can be affected by lichen sclerosus.

Causes of Lichen sclerosus

The etiology and pathogenesis of lichen sclerosus is not known but there are many factors which act as predisposing factors to lichen sclerosus disease :-
• Genetic factors
• Infections
• Environmental factors
• Hormonal factors
Local irritation and trauma may play a role in some cases of lichen sclerosus most probably in genetically predisposed individuals. However the sequence of events that leads to altered fibroblast function, microvascular changes and hyaluronic acid accumulation in the upper dermis continues to be researched.

Lichen sclerosus sign and symptoms

Lichen sclerosus begins as white, polygonal papules that join into plaques.
Color of the skin is white,often with shiny porcelain appearance.
Female genital lesions may be confined to the labia majora but usually involve and eventually obliterate the labia minora and stenose the introitus. Often, an hourglass, butterfly, or figure-8 pattern involves the perivaginal and perianal areas, with minimal involvement of the perineum in between.
Male genital lesions usually are confined to the glans penis and the prepuce or foreskin remnants. Penile shaft involvement is much less common, and scrotal involvement is rare. The initial manifestation may be a sclerotic ring at the prepuce edge.

Investigations of Lichen sclerosus

Laboratory studies to be done are as follows
• Skin biopsy is the first test for diagnosis of lichen sclerosis
• Antinuclear antibody
• Vitamin B-12 levels
• Thyroid function tests
Imaging studies are not needed unless urinary obstruction secondary to severe stenosing genital lichen sclerosus is present.
Punch biopsy is also another test available to diagnose.

Treatment

Topical corticosteroids may help to treat symptoms of external skin.
Areas of vulvar lichen sclerosus are surgically excised or ablated with a laser as a prophylactic measure.
Circumcision may benefit male lichen sclerosus and the phimosis that may accompany it.

Medications to be given are as follows:-
• Topical testosterone
• Corticosteroids
• Retinoid like agents
• Topical skin medications

Complications

Complications in male genitalia are as follows :-
• Painful erections
• Urinary obstruction
• Inability to retaract the foreskin

In females complications occur are as follows:-
• Dyspareunia
• Urinary obstruction
• Secondary infection from chronic ulceration
• Squamous cell carcinoma

Prognosis

Prognosis is good for more acute genital cases, especially for those in pediatric age group that may resolve spontaneously. Prognosis for improvement is poor for extragenital cases and for chronic atrophic genital disease.

Differential diagnosis

• Albinism
• Anetoderma
• Atrophoderma of pasini and pierini
• Balantis circumscripta plasmacellularis
• Balantis xerotica obliterans
• Bowen disease
• Graft versus host disease
• Idiopathic guttate hypomelanosis
• Leukoplakia oral
• Morphea
• Tinea versicolor
• Vitiligo
• Squamous cell carcinoma

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