A 61-year-old woman presents concerned about a white discoloration on her left breast that she first noticed 6 months earlier. It itches at times, and she is worried it might be cancerous. She showed it to her gynecologist at her annual visit, and he referred her to the dermatology clinic. Physical examination of the left breast reveals a 10-cm white, smooth, porcelain-like plaque. There is no associated erythema and no noticeable scale.
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Lichen sclerosus (LS), also known as lichen sclerosus et atrophicus, is a chronic inflammatory dermatitis presenting with white patches, atrophy, and, sometimes, scarring. The condition predominantly involves the genital and perianal skin. Extragenital involvement, which is less common, mostly appears on the neck, shoulders, and trunk. A recent case study presented involvement on the plantar aspect of the foot.1
LS can occur in patients of all ages; girls and women are affected at a significantly higher rate, particularly during the prepubertal and postmenopausal stages.2 One author estimates that 10% to 15% of girls are affected before age 13 years.3 LS of the penis, also termed balanitis xerotica obliterans, is almost exclusive to uncircumcised or incompletely circumcised individuals.
The etiology of LS is unknown; however, several theories have been suggested, including hormonal activity, self-immunity phenomena, and genetic predisposition.4 Risk factors can include a history of trauma to the area or a family history of autoimmune disease.
Extragenital LS is usually asymptomatic, although patients may complain of mild itching. In contrast, patients with vulvar involvement often complain of intolerable pain and itching, and sometimes report dyspareunia, dysuria, or genital bleeding. Involvement of the anal region can cause painful defecation, diarrhea, or constipation. LS of the penis can cause phimosis and urinary obstruction resulting from stenosis of the urethral meatus.
Clinically, LS presents with macules or polygonal papules in a characteristic ivory or porcelain white color. Dermoscopy reveals comedo-like openings scattered in a white homogenous patch.5 As the condition progresses, the plugs disappear and the papules coalesce, leaving a large shiny, atrophic patch. With vulvar involvement, the whitening and atrophy often occur in an hourglass distribution around the vulva and anus, causing obliteration of the labia minora and stenosis of the introitus. In addition, fissuring and ecchymosis is sometimes present, particularly in pediatric cases.
The differential diagnoses to consider include all conditions that present with white patches, such as tinea versicolor, vitiligo, morphea, lichen planus, lichen nitidus, idiopathic guttate hypomelanosis, and discoid lupus. Genital involvement can mimic an irritant dermatitis; genital squamous cell carcinoma and sexual abuse in children must always be ruled out. LS of the penis can be confused with Bowen disease and balanitis.
Although vulvar LS can be diagnosed clinically, skin biopsy is often necessary for diagnosis of extragenital lesions. Histologic examination reveals epidermal atrophy, thickening of the papillary dermis by altered collagen bundles, and vacuolar interface dermatitis with a lichenoid inflammatory cell infiltrate.
First-line treatment of LS involves topical corticosteroids. Generally, a high-potency corticosteroid such as clobetasol 0.05% cream is the therapy of choice. Treatment starts with a once-daily application and tapers down to once- or twice-weekly application. Although topical immunomodulators, such as tacrolimus and pimecrolimus, are often considered, one study6 showed that topical clobetasol was significantly more effective in treating the signs and symptoms of LS. Intralesional corticosteroids should be considered in appropriate cases. Resistant cases may respond to topical tretinoin, oral isotretinoin, or photodynamic therapy. Surgical treatment is reserved for severe cases with scarring.
Asymptomatic extragenital cases may not require treatment, as the skin appearance frequently does not improve, even with topical steroid use. In addition, it is important to note that most cases of LS in the pediatric population resolve spontaneously during puberty.
Patients using potent topical steroids should be monitored closely for signs of skin atrophy. There is an increased risk (approximately 5% or less2) of malignant transformation of genital LS to genital squamous cell carcinoma; therefore, these patients should be carefully educated and examined annually.
The patient in our case was educated regarding the condition. She was treated with clobetasol cream, applied once daily for 4 weeks and twice weekly thereafter. At the 3-month follow-up visit, she reported resolution of the itching and her skin appeared moderately, although not completely, improved. As the condition was no longer bothersome, the patient chose to stop treatment and elected to monitor herself for any further progression.
Esther Stern, NP, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.
- Lopez RE, Troutman DL, Hossler EW. Plantar lichen sclerosus et atrophicus. J Am Acad Dermatol. 2013;68(Suppl 1):AB57.
- James WD, Berger TG, Elston DM. Epidermal nevi, neoplasms, and cysts. In: James WD, Berger TG, Elston DM, eds. Andrews’ Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, PA: Saunders-Elsevier; 2011.
- Paller AS, Mancini AJ. Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. 4th ed. Philadelphia, PA: Elsevier Saunders; 2011.
- Makaron T, Viscomi B, Matayoshi L, Michalany N, Petri V. Genital lichen sclerosus et atrophicus in childhood treated by tacrolimus. J Am Acad Dermatol. 2009;60(Suppl 1):AB151.
- Lacarrubba F, Pellacani G, Verzì AE, Pippione M, Micali G. Extragenital lichen sclerosus: clinical, dermoscopic, confocal microscopy, and histologic correlations. J Am Acad Dermatol. 2015;72(1 Suppl):S50-S52.
- Funaro D, Lovett A, Leroux N, Powell J. A double-blind, randomized prospective study evaluating topical clobetasol propionate 0.05% versus topical tacrolimus 0.1% in patients with vulvar lichen sclerosus. J Am Acad Dermatol. 2014;71:84-91.