UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Case Authors: Case Authors: Timothy Patton, DO; Grace Lee, MD, Drazen Jukic, MD
FEBRUARY 2004 CASE OF THE MONTH
DISCUSSION & DIAGNOSIS
Diagnosis:
Extragenital lichen sclerosus (LS)
DISCUSSION
Lichen sclerosus (also known as lichen sclerosus et atrophicus) is an inflammatory disorder of unknown etiology. It is more commonly described as occurring in the genital region, although extragenital cases also exist. While LS occurring in the genital skin is associated with pruritis, pain, dystrophy, and a progression to squamous cell carcinoma in some cases, extragenital LS is more commonly described as asymptomatic without a higher risk of SCC(1). No universally agreed upon pathogenesis exists; however, infectious (specifically Borrelia infection)(2), autoimmune(3), environmental(4,5), and hormonal(6) etiologies have all been proposed. In one series, histologic differences were reported between extragenital and genital LS, suggesting different etiologies for the two diseases(7).
The histologic findings in LS include epidermal atrophy with basal layer vacuolization,
edema and homogenous sclerosis of the papillary dermis, and a lichenoid infiltrate
below the edema and sclerosis. Histologic differentials include morphea and
chronic radiodermatitis. Indeed, some consider LS and morphea to be similar
diseases along the same continuum, while other consider them distinct entities.
First line treatment involves the use of ultra-potent or potent topical glucocorticoids.
In men, circumcision can be curative. Other symptomatic therapies including
avoidance of irritation and systemic antihistamines can be helpful. Topical
hormone therapy (testosterone, progesterone, and estrogen), topical retinoids,
topical cyclosporine have all been reported as effective. Destructive modalities
including surgical excision photodynamic therapy, and CO2 laser ablation can
also be considered in recalcitrant or severe cases.
The patient denied the existence of any genital lesions, and was treated with
clobetasol proprionate ointment twice daily.
REFERENCES:
1. Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol. 1995 Mar;32(3):393-416.
2. Ozkan S, Atabey N, Fetil E, Erkizan V, Gunes AT. Evidence for Borrelia burgdorferi in morphea and lichen sclerosus. Int J Dermatol. 2000 Apr;39(4):278-83.
3. Farrell AM, Marren PM, Wojnarowska F. Genital lichen sclerosus associated with morphoea or systemic sclerosis: clinical and HLA characteristics. Br J Dermatol. 2000 Sep;143(3):598-603.
4. Pock L. Koebner phenomenon in lichen sclerosus et atrophicus. Dermatologica. 1990;181(1):76-7.
5. Yates VM, King CM, Dave VK. Lichen sclerosus et atrophicus following radiation therapy. Arch Dermatol. 1985 Aug;121(8):1044-7.
6. Wines N, Willsteed E. Menopause and the skin. Australas J Dermatol. 2001 Aug;42(3):149-8.
7. Carlson JA, Lamb P, Malfetano
J, Ambros RA, Mihm MC Jr. Clinicopathologic comparison of vulvar and extragenital
lichen sclerosus: histologic variants, evolving lesions, and etiology of 141
cases. Mod Pathol. 1998 Sep;11(9):844-54.
