Atrophic facial plaques and palatal erosions - Clinical Advisor

Atrophic facial plaques and palatal erosions

Slideshow

  • 1.2-cm brown oval atrophic plaque with a violaceous border on the nose with two more plaques on the right medial cheek.

    Figure 1

    1.2-cm brown oval atrophic plaque with a violaceous border on the nose with two more plaques on the right medial cheek.

  • Erosions were also found on the hard palate.

    Figure 2

    Erosions were also found on the hard palate.

Clinical description

A black woman, aged 31 years, developed multiple asymptomatic plaques on the central face over the course of one year. There was no improvement with application of vitamin E or cocoa butter. Although the patient had a history of smoking six to 10 cigarettes per day, she was presently abstaining because she was pregnant.

Exam revealed a 1.2-cm brown oval atrophic plaque with a violaceous border on the nose along with two additional plaques on the right medial cheek (Figure 1). Erosions were found on the hard palate (Figure 2). 
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The distribution on the head and neck, oval shape, violaceous color, atrophy, follicular plugging, scarring, and history of photosensitivity all suggested a diagnosis of discoid lupus erythematosus (DLE). Other inflammatory dermatoses considered were nodulocystic scarring acne, sarcoidosis, and psoriasis. The...

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The distribution on the head and neck, oval shape, violaceous color, atrophy, follicular plugging, scarring, and history of photosensitivity all suggested a diagnosis of discoid lupus erythematosus (DLE).

Other inflammatory dermatoses considered were nodulocystic scarring acne, sarcoidosis, and psoriasis. The patient’s review of systems was negative for fatigue, headache, pleural chest pain, and joint pain. She had no history of seizure disorder or coagulopathy. Serologic workup for anti-nuclear antibody (ANA) and Sjögren’s syndrome antigen (SSA) was negative. Urinalysis showed no proteinuria.

The skin biopsy demonstrated epidermal atrophy, follicular plugging, basal cell layer vacuolization, focal thickening of the basement membrane zone and a perivascular and periappendageal lymphocytic infiltrate.

DLE is a chronic photodistributed eruption categorized as a chronic cutaneous form of lupus erythematosus. Although rarely associated with systemic disease, DLE can cause significant scarring and disfigurement. The incidence of chronic DLE is estimated to be 3.56 cases per 100,000 persons.1 The frequency of DLE in women is two to three times that in men, and typical patients are aged 20 to 40 years.2 In addition, black patients are more commonly affected than whites or Asians, and there appears to be a genetic predisposition. The leading hypothesis regarding DLE pathogenesis is that UV light results in T-cell-mediated killing of keratinocytes.2 Along with UV radiation, other triggers for DLE include trauma, cold, infection, and burns.3

The typical DLE patient is a young woman with asymptomatic lesions on the head and neck, although patients may complain of itching or pain. In 5% of cases, DLE may be associated with SLE.4 Such patients are most likely to complain of arthralgias, but they may also have pleural chest pain, hematuria, or neurologic symptoms.2 If DLE is suspected, inquire about photosensitivity or a family history of the condition. Lesions of DLE are typically scaly papules and plaques with central atrophy, scarring, and depigmentation. Hair follicles in areas of involvement may demonstrate a keratinous plug and may be associated with alopecia.2 Lesions have a predilection for the conchal bowls of the ears, scalp, face, anterior neck, and extensor arms, but patients with generalized DLE are more likely to have systemic symptoms and a prolonged disease course.3 In oral DLE, there are mucosal ulcerations on the hard palate, lips, or within the nasal cavity, and such patients have a higher risk for systemic disease.

Diagnosis of DLE is confirmed via skin biopsy. Serologic testing for any patient with suspected DLE should include ANA and SSA assays (20% of those with DLE will test positive).2 If a patient has an anti-double-stranded DNA or anti-smooth-muscle antibody, SLE is more likely, and the clinician should ask about fever, oral ulcers, rash, nail changes, Raynaud’s disease, myalgias, serositis, dysphagia, chest pain, headaches, and hematuria. If the index of suspicion is high, more extensive labs may include complete blood count, erythrocyte sedimentation rate, rheumatoid factor, complement levels, and urinalysis.2 If SLE is suspected, refer the patient to rheumatology for further evaluation and treatment.

Treatment of DLE is aimed at preventing any disease progression, improving the patient’s appearance, and limiting scar formation. Such photosensitizing medications as hydrochlorothiazide, calcium channel blockers, and terbinafine should be ruled out.4 Smoking cessation is paramount, as those who smoke tobacco have worse DLE and less robust responses to treatments.5 Photoprotection is critical and should include behavior modification, incorporation of hats and sun-protective clothing into one’s wardrobe, and daily use of broad-spectrum sunscreens to affected areas.6

Pharmacotherapy for DLE begins with topical cortico­steroids. A randomized control trial showed that higher-potency topical steroids treat DLE lesions more effectively than the less potent versions.7 Although potent topical corticosteroids can cause atrophy, the disfiguring potential of DLE justifies their use. Topical calcineurin inhibitors may be used without a risk of atrophy but are more costly.2 If patients do not respond to topical therapies, start such oral antimalarials as hydroxychloroquine 400 mg/day. To enhance efficacy, quinacrine 100 mg/day may be added to hydroxychloroquine.6 Second-line treatment of DLE may include oral methotrexate, retinoids, dapsone, and mycophenolate mofetil.8 Alternative therapies include immunomodulators, thalidomide, and biologic agents, but these agents have only been reported useful in a small number of cases.8 Even when cutaneous disease is controlled, both dermatologists and primary-care providers should monitor these patients for signs and symptoms of SLE, most notably in the first one to three years following the diagnosis of DLE.4

Our patient began clobetasol 0.05% ointment daily on the facial plaques and reported improvement in crusting and redness after one month.

Jennifer Gloeckner Powers, MD, is a second-year resident in the department of dermatology, and Amit Garg, MD, is an associate professor and the director of the training program in the department of dermatology at Boston University School of Medicine. The authors have no relationships to disclose relating to the content of this article.


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References

1. Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Arch Dermatol. 2009;145:249-253. Available at archderm.ama-assn.org/cgi/content/full/145/3/249.

2. eMedicine. Lupus erythematosus, discoid. Available at emedicine.medscape.com/article/1065529-overview.

3. Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: issues in diagnosis and treatment. Am J Clin Dermatol. 2009;10:365-381.

4. Callen JP. Cutaneous lupus erythematosus: a personal approach to management. Australas J Dermatol. 2006;47:13-27.

5. Gallego H, Crutchfield CE 3rd, Lewis EJ, Gallego HJ. Report of an association between discoid lupus erythematosus and smoking. Cutis. 1999;63:231-234.

6. Kuhn A, Ruland V, Bonsmann G. Cutaneous lupus erythematosus: Update of therapeutic options Part I. J Am Acad Dermatol. 2010 Aug 23. Published online ahead of print.

7. Jessop S, Whitelaw DA, Delamere FM. Drugs for discoid lupus erythematosus. Cochrane Database Syst Rev. 2009;4:CD002954. Available at onlinelibrary.wiley.com/o/cochrane/clsysrev/articles/CD002954/frame.html.

8. Kuhn A, Ruland V, Bonsmann G. Cutaneous lupus erythematosus: Update of therapeutic options Part II. J Am Acad Dermatol. 2010 Aug 25. Published online ahead of print.

All electronic documents accessed January 15, 2011.

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