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lichen sclerosus et atrophicus (LSA)
Chronic atrophic mucocutaneous disorder characterized by white, angular, well-delineated, indurated papules & plaques.
Etiology: unknown
Epidemiology:
1) adults & children 1-13 years of age
2) mean age of onset: 43 in males, 50 in females
3) female:male ratio 10:1
Pathology:
1) epidermis:
a) variably thickened, hyperkeratotic with follicular plugging (early)
b) atrophic (late)
2) dermis
a) band of homogeneous dermal collagen below epidermis
b) structureless & edematous
c) lymphocytic infiltrate
1] subepidermal (early)
2] below structureless & edematous dermis (later)
d) dilated capillaries & hemorrhage
* histopathology images [2]
Clinical manifestations:
1) lesions may be present for 1 year prior to detection
2) generally asymptomatic
3) in females vulvar lesions may be sensitive, especially while walking, or may be painful if erosions are present
4) in males, phimosis, recurrent balanitis
5) macules & papules, whitish in color, sharply demarcated
6) individual lesions may become confluent forming plaques
7) lesions may be elevated, flat or older lesions may be depressed
8) puncta in center
9) bullae & erosions may heal with fusion of the labia minora
10) purpura
11) telangiectasia
12) keratotic plaques on vulva may become macerated
13) vulva may become atrophic
14) distribution:
a) genital:
1] vulva in females
2] prepuce or glans penis in males
b) non-genital: trunk, esp upper back, periumbilical region, neck, axillae, flexor surface of wrists, rarely palms& soles
c) oral mucosa: blueish-white plaques on buccal or palatal mucosa &/or tongue
15) disease waxes & wanes
* images [2]
Differential diagnosis:
1) morphea (may coexist)
2) lichen simplex chronicus
3) lupus erythematosus
4) leukoplakia
5) lichen planus
6) intra-epithelial neoplasia (Bowen's disease)
7) extramammary Paget's disease
8) intertrigo
9) candidiasis
Complications:
- squamous cell carcinoma, predominantly in males (uncommon)
Management:
1) symptomatic: no curative therapy
2) topical corticosteroids
a) potent steroids effective for genital LSA
b) may be mixed with topical androgens
3) systemic therapy: hydroxychloroquine 250 mg PO QD
4) circumcision relieve phimosis in males
General
lichen sclerosus
References
- Color Atlas & Synopsis of Clinical Dermatology, Common
& Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY,
1997, pg 278-281
- Meffert J, James WD (images)
Medscape: Lichen Sclerosus et Atrophicus
http://emedicine.medscape.com/article/1123316-overview