Derm Dx: Wrinkled, depigmented skin on breasts, chest - Clinical Advisor

Derm Dx: Wrinkled, depigmented skin on breasts, chest

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A patient aged 37 years presents complaining of skin changes over the past several weeks. On exam, she has wrinkly and depigmented patches on her breasts and inframammary chest.

She has no other medical problems and takes no medications.

Lichen sclerosus (LS) is a chronic inflammatory disease of the skin and mucosa that most commonly affects the anogenital region, although extragenital disease may be present.   LS can present from childhood to old age, but the disease peaks during childhood...

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Lichen sclerosus (LS) is a chronic inflammatory disease of the skin and mucosa that most commonly affects the anogenital region, although extragenital disease may be present.  

LS can present from childhood to old age, but the disease peaks during childhood and after menopause. LS is more prevalent in female patients than in male patients. When present on the penis, lichen sclerosus is termed balanitis xerotica obliterans.

The pathogenesis of LS is unclear. Autoantibodies to extracellular matrix protein (ECM) 1 are found in 80% of LS patients and the titer of the ECM1 autoantibody correlates with the disease severity. However, the importance of this autoimmunity is unclear.

LS initially presents as white, polygonal, flat-topped papules, plaques, or atrophic patches, sometimes surrounded by an erythematous to violaceous halo. The atrophic lesions typically affect the vulvar and perianal areas, producing a “figure-eight” or “hourglass” distribution. In these cases, the vulvar skin is hypopigmented, thin, smooth, soft, and white. Areas of hyperkeratosis and scarring are often present, and can lead to architectural changes.

Burying of the clitoris due to fusion of the labia minora to the labia majora may occur, and in severe cases total loss of the labia minora may cause narrowing of the introitus with obliteration of the urethral meatus. Perianal erosions and fissures may produce constipation, stool holding, and rectorrhagia.

Other features such as bullae, purpura, and telangiectasias may be found within the patches. Patients usually have severe pruritus or soreness in affected areas, and dyspareunia due to narrowing of the introitus is a common complaint.

Children with LS are more likely to present with urinary symptoms, bowel symptoms (constipation), or hemorrhagic bullae, which may lead to the misdiagnosis of child abuse. Koebner phenomenon is often present, with local friction or rubbing inducing LS lesions.

Extragenital LS is rare and favors the submammary region, shoulders, neck, and wrist. Extragenital LS presents as asymptomatic, hypopigmented, wrinkled patches. The tongue and oral mucosa may also be involved.

The diagnosis of LS is established by a combination of clinical and characteristic histologic findings. Histology shows orthokeratosis (thickening of the stratum corneum), a thinned epidermis and a deep band-like lymphocytic infiltrate immediately beneath an altered papillary dermis.

LS of the genitalia, especially when squamous hyperplasia is present, may lead to an increased risk for genital squamous cell carcinoma (SCC). Therefore, longitudinal evaluation is essential in all afflicted patients and all non-healing fissures, ulcers, or nodules should be biopsied.

Treatment of choice for LS is a potent topical corticosteroid such as clobetasol. The steroid should be applied once daily for three months with subsequent tapering over two weeks. The initial dose alleviates symptoms in the majority of patients and clinical remission is maintained by the tapering application of a corticosteroid.

Complete resolution can be seen in some, but any scarring is irreversible. In long-standing disease where narrowing of the introitus has resulted in dyspareunia, surgical refashioning and dilators may be helpful.

Christopher Chu, BS, is a medical student at Baylor College of Medicine

Adam Rees, MD, a graduate of the David Geffen School of Medicine at UCLA, practices dermatology in Los Angeles.

References

  1. Bolognia J, Jorizzo J, Rapini J. “Chapter 73 – Anogenital (Non-venereal) Disease.” Dermatology. St. Louis, Mo.: Mosby/Elsevier, 2008. Print.
  2. James W, Berger T, Elston D, Odom R. “Chapter 12 – Lichen Planus and Related Conditions.” Andrews’ Diseases of the Skin: Clinical Dermatology. Philadelphia: Saunders Elsevier, 2006. Print.
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