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Nayla Idriss, BS, MS III Dark brown scaly plaques George Washington University, Washington, DC

on face and ears Shahbaz A. Janjua, MD Consultant Dermatologist, Ayza Skin and Research Center, Lalamusa, Pakistan 27-year-old woman, otherwise FIGURE 1 healthy, presented for evaluation Amor Khachemoune, MD, Facial plaques A of a mildly pruritic eruption of CWS plaques on her face and ears. The erup- Clinical Instructor, Dermatology, Dermatologic Surgery, SUNY tion started as a few small, scaly red Downstate Medical Center, papules on her cheeks and nose about Brooklyn, NY 3 months earlier. The papules slowly expanded to form well-demarcated,® Dowden Health Media rounded, dark brown plaques covered with superficial scale. Similar lesions appeared on the conchaCopyright of both ears.For personal use only The older lesions started to regress after 2 months. Complete regression was followed by residual scarring and hyper- pigmentation. Physical examination revealed multi- ple well-defined, erythematous, hyper- pigmented, round plaques covered with adherent scale affecting her nose, cheeks, and the concha of both ears (FIGURE 1). The mucosae and the rest of her skin and adnexa were unaffected. She had no personal or family history of similar skin findings or autoimmune disorders. She also had no history of any drug intake prior to the eruption. Her routine Well-demarcated dark brown-colored scaly blood tests and urinalysis results were erythematous plaques. Hyperpigmented unremarkable, and the serological resulting from the spontaneous healing of older lesions are also visible. analysis for antinuclear antibodies had negative results. A punch biopsy was taken from one infiltrate of the subepidermal and of the lesions for histopathology. The perivascular dermal areas.

epidermal histopathological findings CORRESPONDENCE were remarkable for , fol- ❚ What is your diagnosis? Amor Khachemoune, MD, licular plugging, pigment incontinence, CWS, Department of ❚ Dermatology, 450 Clarkson and vacuolization of the basal cell layer. How would you treat this Avenue Box 46, Brooklyn, NY There was a predominantly lymphocytic patient? 11203. E-mail: [email protected]

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❚ Diagnosis: Discoid lupus centrally depressed scars.3 Scalp involve- ROUNDS erythematosus ment in DLE results in more sclerotic and Discoid (DLE) is an depressed scars with subsequent scarring autoimmune inflammatory disorder of the alopecia.4 PHOTO skin that often leads to scarring and alope- Differential diagnosis is wide. The dif- cia. It may be localized to sun-exposed ferential diagnosis of DLE is extensive and areas such as the face, ears, and scalp but includes actinic , dermatomyositis, occasionally is much more extensive, annulare, granuloma faciale, involving the trunk and extremities. Most keratoacanthoma, , subacute patients are otherwise healthy, and DLE cutaneous lupus erythematosus, , may be the only clinical finding. rosacea, , squamous cell carci- Although its prevalence is not known, noma, syphilis, and nongenital . DLE is not uncommon. It affects females twice as often as males, and patients fall between the ages of 25 to 45 years, with no ❚ Histopathology racial predilection. While about 15% to Histopathological findings include hyper- 20% of patients with systemic lupus ery- keratosis, parakeratosis, follicular plug- thematosus (SLE) manifest DLE lesions, ging, telangiectasias, and of the only about 5% to 10% of patients with . Liquefaction or hydropic DLE go on to develop SLE.1 degeneration of the basal layer leads to Like SLE, DLE is believed to be an pigmentary incontinence. A perivascular autoimmune disorder. Unlike SLE, howev- and perifollicular mononuclear inflam- er, DLE patients do not have similar sero- matory cell infiltrate is present in the logic abnormalities. Skin trauma and ultra- superficial and deep dermis.5,6 violet light exposure have been reported to Direct immunofluorescence demon- induce or exacerbate the lesions of DLE. strates immunoglobulins and comple- Sex hormones may also play a role: exac- ment deposits at the dermoepidermal erbation may occur during pregnancy, junction.5 This test was not recommend- FAST TRACK during menstrual or premenstrual periods, ed for this patient, as the diagnosis of The primary or while taking oral contraceptives. Drugs DLE had already been confirmed on such as procainamide, hydralazine, isoni- clinical and histopathologic grounds. therapeutic azid, diphenylhydantoin, methyldopa, approach: penicillamine, guanides, and lithium may educate patients also precipitate DLE lesions.2 ❚ Workup: Exam, biopsy DLE usually begins as dull red macules In addition to a routine history and phys- regarding with adherent scales on sun-exposed areas. ical examination, the workup for DLE exposure If the overlying scales are removed, an should include a complete blood count, to sunlight undersurface of horny plugs is revealed. antinuclear antibody levels, anti-Ro, These plugs fill the follicles and resemble anti-La, hepatic and renal function tests, carpet tacks or a cat’s tongue (langue au and urinalysis. Consider a diagnosis of chat). The macules slowly expand to form SLE by using the American College of large plaques. Rheumatology criteria. A biopsy for Usually only a mild pruritus and ten- histopathology of a fresh lesion or a derness is seen with DLE lesions. As the biopsy for immunofluorescence of an old lesions progress, the scale may thicken and lesion can confirm the diagnosis. pigmentary changes become evident. The lesions heal with atrophy, scarring, telang- iectasias, and changes in pigmentation. ❚ Therapeutic options Morphological appearance of older lesions are varied may vary from erythematous plaques to Effective early therapies for DLE are hyperkeratotic, dark gray plaques with available, but patients who do not

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Dark brown scaly plaques on the face and ears ▲

respond appropriately may end up with FIGURE 2 deep scars, alopecia, and pigmentary After treatment changes that are considerably disfiguring, especially for dark-skinned people. Therefore, the goal of treatment is not only to improve the appearance of the skin by minimizing the scarring and preventing further lesions, but also to prevent future complications. Avoiding sunlight. The primary thera- peutic approach is to educate patients regarding exposure to sunlight. Sun- protective measures include the use of high-SPF sunscreen lotions and protec- tive clothing, such as baseball caps with- out vent holes and wide-brimmed hats.7 Medication options. Current treatment options for DLE include antimalarial agents such as chloroquine, topical and intralesional glucocorticoids, and thalido- mide. The use of potent topical steroids may prevent significant scarring and Perceptible regression of the lesions after one month of therapy with topical steroid cream and deformity, especially of the face. Common oral hydroxychloroquine. side effects include steroid withdrawal syndrome, perioral dermatitis, steroid acne, and rosacea. All of these side effects other treatments for DLE include azathio- can be treated and result in less long-term prine, retinoids, and dapsone. Recent deformity than untreated DLE (FIGURE 2). reports confirm the efficacy of thalido- Systemic agents. Widespread disease mide for cutaneous lupus erythematosus FAST TRACK may require you use systemic agents, such in dosages ranging from 50 to 200 mg/d.10 Widespread as antimalarials. Chloroquine has long Twice-daily application of tacrolimus been considered the gold standard in the 0.01% has also been shown to be effective disease may treatment of DLE.8 Due to the frequency in a few clinical trials.11 require systemic of ocular side effects with chloroquine, Surgery. Surgical intervention is useful agents (eg, anti- hydroxychloroquine is by far the most to remove scarred lesions, and laser thera- widely used agent. Hydroxychloroquine py has also been effective, especially for malarials); surgery at a dose of 6.5 mg/kg/d for 3 months lesions with prominent telangiectasias.12 is useful to remove may lead to resolution of lesions for Patient education. Patient education scarred lesions many patients.7 In resistant cases, higher plays an important role in the treatment dosages (eg, 400 mg/d) or combinations of DLE. Advising patients on how to (eg, hydroxychloroquine 200 mg/d plus avoid the sun and instructing them in the quinacrine 50 to 100 mg/d) may be proper use of sunscreens is extremely required for months or even years. An important. Smoking cessation has also ophthalmological evaluation is advisable been shown to be beneficial.13 before starting antimalarial treatment, Patients with DLE generally have a and you should repeat it at 4- to 6-month favorable prognosis with regards to mor- intervals during treatment.9 Systemic bidity and mortality. Because DLE is usu- corticosteroids may be needed to obtain ally self-limited, the course is most often timely initial control, especially for wide- benign; therefore, early recognition and spread and disfiguring lesions. adequate therapy may prevent clinical When this therapy is inadequate, complications. Many patients with DLE

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go on to develop destructive or deforming 3. Callen JP, Fowler JF, Kulick KB. Serologic and clinical fea-

ROUNDS tures of patients with discoid lupus erythematosus: relation- 14 scarring or pigmentary disturbances, ship of antibodies to single-stranded deoxyribonucleic acid and of other antinuclear antibody subsets to clinical mani- especially in the spring and summer festations. J Am Acad Dermatol 1985; 13(5 Pt 1):748–755. months when the sun is the strongest. 4. Wilson CL, Burge SM, Dean D, Dawber RP. Scarring alope- PHOTO cia in discoid lupus erythematosus. Br J Dermatol 1992; 126:307–314. 5. Patel P, Werth V. Cutaneous lupus erythematosus: a review. ❚ This patient’s outcome Dermatol Clin 2002; 20:373–385, v. Our patient was treated with topical flu- 6. Biesla I, et al. Histopathologic findings in cutaneous lupus erythematosus. Arch Dermatol 1994; 130:54–58. ocinolone acetonide 0.025% ointment 7. C a l l e n J P. Treatment of cutaneous lesions in patients with and oral hydroxychloroquine 200 mg/d lupus erythematosus. Dermatol Clin 1994; 12:201–206. 8. Goldman L, Cole DP, Preston RH. Chloroquine diphosphate for 1 month. The patient responded well in treatment of discoid lupus erythematosus. J Am Med to the treatment and the skin lesion Assoc 1953; 152:1428–1429. regressed perceptibly (FIGURE 2). The 9. Rynes RI. Ophthalmologic safety of long-term hydroxy- chloroquine sulfate treatment. Am J Med 1983; 75:35–39. treatment was nevertheless continued for 10. Kyriakis KP, Kontochristopoulos GJ, Panteleos DN. another 5 months, and resulted in Experience with low-dose thalidomide therapy in chronic discoid lupus erythematosus. Int J Dermatol 2000; complete regression of the lesions, with 39:218–222. minimal residual scarring. 11. Heffernan MP, Nelson MM, Smith DI, Chung JH. 0.1% tacrolimus ointment in the treatment of discoid lupus ery- thematosus. Arch Dermatol 20 05 ; 141 : 1170 – 1171. 12. Nunez M, Boixeda P, Miralles ES, et al. Pulsed dye laser treat- REFERENCES ment of telangiectatic chronic erythema of cutaneous lupus erythematosus. Arch Dermatol 1996; 132:354–355. 1. Ca l l e n J P. Systemic lupus erythematosus in patients with 13. Miot HA, Bartoli Miot LD, Haddad GR. Association between chronic cutaneous (discoid) lupus erythematosus. Clinical discoid lupus erythematosus and cigarette smoking. and laboratory findings in seventeen patients. J Am Acad Dermatology 2005; 211:118–122. Dermatol 1985; 12(2 Pt 1):278–288. 14. de Berker D, Dissaneyeka M, Burge S. The sequelae of 2. Hess E. Drug-related lupus. N Engl J Med 1988; chronic cutaneous lupus erythematosus. Lupus 1992; 318:1460–1462. 1:181–186.