Bardek, Ivana

Basta-Juzbašić, Aleksandra

Bukvić Mokos, Zrinka

Marinović, Branka

Milavec-Puretić, Višnja

Ivana Bardek

Aleksandra Basta-Juzbašić

Branka Marinović

Zrinka Bukvić Mokos

Višnja Milavec-Puretić

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134Acta Dermatovenerol Croat                    2009;17(2);134-138                        CASE REPORTHypertrophic Lupus Erythematosus: Case Report Ivana Bardek, Aleksandra Basta-Juzbašić, Branka Marinović, Zrinka Bukvić Mokos, Višnja Milavec-Puretić University Department of Dermatology and Venereology, Zagreb University Hospital Center and School of Medicine, Zagreb, CroatiaCorresponding author:Ivana Bardek, MDUniversity Department of Dermatology and  VenereologyZagreb University Hospital Center andSchool of MedicineŠalata 4HR-10000 ZagrebCroatiaivanabardek@yahoo.comReceived: October 20, 2008Accepted: May 11, 2009SUMMARY Discoid lupus erythematosus is the most common form of cutaneous lupus erythematosus. It is more common in women than in men, in individuals between 20 and 40 years of age. It is an inflammatory autoimmune disease in which genetically predis-posed individuals are stimulated by hormonal and a variety of ex-ogenous factors including UV radiation, stress, infections, and even temperature changes. Lesions are characterized by erythema, hy-perkeratosis and atrophy. Typical sites are light-exposed areas, i.e. forehead, nose, cheeks, upper part of the back, upper chest, and dorsal aspects of the hands and feet. A case of lupus erythemato-sus hypertrophicus with very good and rapid treatment results with antimalarials and topical corticosteroid is presented. KEY WORDS: discoid lupus erythematosus, clinical varieties, hy-pertrophic lupus erythematosus treatmentINTRODUCTION Discoid lupus erythematosus (DLE) is the most common form of cutaneous manifestation of lu-pus erythematosus, also known as lupus erythe-matosus chronicus or chronic cutaneous lupus erythematosus (CCLE) (1). It is most common in individuals between 20 and 40 years of age (1,2), with a female-male ratio of approximately 2:1 to 4:1 (1). Its incidence is estimated at more than 1:200 in black women (1). In children, even if the skin lesions are typical for DLE, one should sus-pect systemic lupus erythematosus. To make a diagnosis, histopathologic examination, immuno-fluorescent examination and laboratory examina-tion are needed. Mostly it is treated with topical corticosteroids and systemic antimalarial therapy. Prophylaxis (sun avoidance and sunscreens with high UVA and UVB protection factors) should be used religiously. Local mutilation and scarring alopecia can occur. Approximately 5% of patients with isolated localized DLE subsequently develop systemic lupus erythematosus (3). Lupus erythematosus hypertrophicus, also re-ferred to as hyperkeratotic or verrucosus DLE, is a rare variant of cutaneous lupus erythematosus in 135ACTA DERMATOVENEROLOGICA CROATICABardek et al.        Acta Dermato-venerol CroatHypertrophic lupus erythematosus      2009;17(2):which the hyperkeratosis normally found in classic DLE is greatly exaggerated. The extensor aspects of the arms, the upper back, and the face are the areas most frequently affected (4).CASE REPORT A 45-year-old Caucasian female, a smoker, presented to our Department complaining of skin changes on the left upper arm, upper part of the back, dorsal aspects of the hands, and lower lip. The changes had been present for five years. Family history was not significant. Five years be-fore, she had been diagnosed with hepatitis B infection. Skin changes manifested as erythema-tous and greatly hyperkeratotic lesions on the left upper arm, upper part of the back, dorsal aspects of the hands, and lower lip (Figs. 1, 2 and 3). Lesional biopsy was performed. Histopatho-logic examination showed acanthosis and hyper-keratosis of the epidermis with plugged follicles, vacuolar degeneration of the basilar keratino-cytes, more prominent and thickened basement membrane, perifollicular infiltrates of lymphocytes, and dilated blood vessels. The diagnosis of lupus erythematosus was confirmed. Immunofluores-cent examination showed IgM deposition at the dermoepidermal junction. In addition, the patient was positive for ANA and negative for dsDNA and Sm antibodies. All test results were suggestive of the cutaneous form of lupus erythematosus. She was treated with systemic antimalarials, chloroquine 2x1 tbl à 250 mg, for two weeks, ta-pered to a single tablet daily. Before the introduc-tion of systemic treatment with antimalarials, the patient underwent ophthalmologic examination. She was also treated with topical high-potency corticosteroid ointment with occlusive dressing. As a prophylaxis she was recommended sunscreens with high UVA and UVB protection factors. The re-sults of treatment were rapid and very successful.DISCUSSION The cause of DLE is not fully understood. It is thought to be an immune and inflammatory me-diated disease in which genetically predisposed individuals are stimulated by hormonal and a va-riety of exogenous factors including UV radiation, stress, infections, and even temperature changes (1). It is often associated with HLA-B8 and HLA-DR3 haplotypes (4). These exogenous forces lead to epidermal cell death and the exposure of nor-mally intracellular antigens, which than trigger an autoimmune response characterized in the labora-tory by the presence of ANA. Our patient was also a long time smoker, and it is suggested in some Figure1. Nummular erythematosus and greatly hyperkeratotic lesions on the left upper arm.Figure2. Nummular erythematosus and hyper-keratotic lesions on the upper part of the back.Figure3. Erythematosus and hyperkeratotic le-sion on the lower lip. Bardek et al.      Acta Dermatovenerol CroatHypertrophic lupus erythematosus    2009;17(2):134-138136 ACTA DERMATOVENEROLOGICA CROATICAstudies that smokers have an increased frequency of DLE (2,5). The clinical expression of skin involvement in patients with lupus erythematosus shows a great variation, therefore, it has been difficult to issue the classification of cutaneous lupus erythema-tosus (Table 1) which is currently used (3). The lesions in our patient were consistent with hyper-trophic lupus erythematosus. The predilection sites of cutaneous lupus ery-thematosus are light-exposed areas, i.e. the fore-head, nose, cheeks, upper part of the back, upper chest, and dorsal aspects of the hands and feet (1,2,4,6). Other sites include the eyelids, the ears including the ear canal where sunlight normally does not reach, and the scalp (1). Our patient presented with DLE lesions on the left upper arm, upper part of the back, and dorsal aspects of the hands and lower lip, which are typical sites.  Discoid lupus erythematosus is characterized by inflammatory plaques with scaling, follicular plugging, atrophic scarring, central hypopigmen-tation, and central hyperpigmentation (3). These lesions can be quite destructive leading to de-struction of the nose, ears, cheeks, and scarring alopecia on the scalp (1,4). Histologically, the epidermis and dermis are affected in DLE. The characteristic microscopic features are hyperkeratosis with follicular plug-ging, thinning and flattening of the epithelium, and hydropic degeneration of the basal layer (liquefac-tion degeneration). In addition, there are scattered apoptotic keratinocytes (Civatte bodies) in the basal layer or in the epithelium. Particularly in old-er lesions, thickening of the basement membrane becomes obvious in the periodic acid-Schiff stain. In the dermis, there is a lichenoid or patchy lym-phocytic infiltrate with accentuation of the pilose-baceous follicles. There is interstitial mucin depo-sition and edema, and usually no eosinophils and neutrophils are present (3). Hypertrophic lesions as in our patient show acanthosis and hyperkera-tosis of the epidermis. Direct immunofluorescent examination of the affected skin shows deposition of IgG and C3, and to a lesser extent of IgM, IgA and C1 in a diffuse ir-regular band at the dermoepidermal junction (1,4). Our patient had IgM deposition at the dermoepi-dermal junction. About 25% of DLE patients have positive ANA, but dsDNA and Sm antibodies sug-gest systemic disease (1). Differential diagnosis includes systemic lupus erythematosus, lupus vulgaris, polymorphic light eruption, rosacea, seborrheic dermatitis, psoria-sis, tinea faciei and corporis, actinic keratoses and sarcoidosis (1,4). Treatment includes prophylaxis, and topical, surgical and systemic therapy. Sun avoidance and sunscreens with high UVA and UVB protection factors must be used religiously (1,4).  Short-term high-potency topical corticosteroids and intralesional corticosteroids are usually effec-tive for early lesions before scarring has devel-oped (1,4). Cryotherapy is effective especially for hyperkeratotic lesions (1,4).  Several other topical agents might be of use in individual patients with cutaneous lupus erythe-matosus. Retinoids, specifically tretinoin, might be effective and have been primarily used in patients with DLE and hypertrophic lupus erythemato-sus. Tazarotene, a topical retinoid, might also be used. Topical application of calcipotriene, a topi-cal vitamin D derivative, may be tried (3). Some recent studies suggest the treatment with topical pimecrolimus and tacrolimus to be effective, but large controlled studies are needed for further evaluation (7-11). Finally, because it is known that systemically administered interferon is effective for CLE, it might be helpful to apply imiquimod to individual lesions (3). When the existing lesions are not controlled with topical agents or intralesional corticosteroids, systemic therapy is often indicated. The standard systemic therapy includes antimalarials, usually chloroquine 250 mg daily. The usual initial dose is 250 mg twice daily for 10-14 days, tapered to a single dose daily (1,4). The major concern with antimalarials is retinal toxicity, so ophthalmologic Table 1. Düsseldorf classification of cutaneous lu-pus erythematosus 2003 (3)Acute cutaneous lupus erythematosus (ACLE)Subacute cutaneous lupus erythematosus (SCLE)Chronic cutaneous lupus erythematosus (CCLE)   Discoid lupus erythematosus (DLE)      Hypertrophic/verrucosus variant      Teleangiectoid variant   Lupus erythematosus profundus (LEP)   Chilblain lupus erythematosus (CHLE)Intermittent cutaneous lupus erythematosus (ICLE)   Lupus erythematosus tumidus (LET)Bullous lesions in lupus erythematosus (BLE)   LE – specific bullous skin lesions   LE – nonspecific bullous skin lesions   Primarily bullous skin disorders associated with LEBardek et al.      Acta Dermatovenerol CroatHypertrophic lupus erythematosus    2009;17(2):134-138137ACTA DERMATOVENEROLOGICA CROATICARadoš et al.       Acta Dermatovenerol CroatHalo phenomenon      2009;17(2):examination is necessary. One should wait for at least 3 months before deciding if the antimalarials are not helping. Several authors have shown that patients with DLE who smoke are less responsive to antimalarial treatment (2,12,13). In difficult cases, multiple other approaches have been advocated for the treatment of cutane-ous lupus erythematosus. Dapson may be useful in bullous lupus erythematosus and in oral ulcer-ations. Auranofin, an oral form of gold, has been used for cutaneous lupus erythematosus, and the results were encouraging (3). Also, low-dose tha-lidomide is an effective treatment option for DLE in cases resistant to other treatments, especially for mucosal ulcers and scalp involvement (1,14,15). Oral retinoids, isotretinoin and acitretin, are helpful in patients with hypertrophic lesions or those with lesions on the palms or soles. Several cytotoxic agents, azathioprine, methotrexate and mycophe-nolate mofetil, have also been reported to be ben-eficial for the control of cutaneous lupus erythe-matosus lesions. High-dose intravenous globulin and cytokine therapy with interferon-α have been used successfully, but long-term remission is rare-ly achieved. In contrast, chimeric CD4 monoclonal antibody infusions showed long-lasting improve-ment (3).  Ointments are more effective than creams for more hypertrophic and hyperkeratotic lesions as in our patient. Occlusive therapy with corticoste-roids with plastic wrap can potentiate the benefi-cial effects of topical steroids.  Our patient showed a very good and rapid ther-apeutic result after one month of treatment with synthetic antimalarials and occlusive therapy with topical corticosteroids.  Each patient with newly a diagnosed DLE should be evaluated for the signs of systemic lupus erythematosus. Chronic discoid lesions may be an initial manifestation of systemic lupus erythema-tosus in approximately 10% of patients (3). Dis-coid lupus erythematosus should also be viewed as a precancerous condition because squamous cell carcinoma may develop in chronic lesions of DLE (1,16). Sometimes mutilation can occur, typi-cally seen on the nose and ears, and also scarring alopecia that is permanent. Approximately 5% of patients with isolated localized DLE subsequently develop systemic lupus erythematosus (3).CONCLUSION Discoid lupus erythematosus is a chronic in-flammatory autoimmune cutaneous disease. It affects genetically predisposed individuals stimu-lated by a variety of factors, UV radiation being the most important one. Remission can be suc-cessfully achieved by using local and intralesional corticosteroids and systemic antimalarial treat-ment. Calcipotriene and retinoids may be effective in some patients. Recently, local immunomodula-tors and systemic thalidomide therapy have been shown to be very effective. Local mutilation and scarring alopecia can occur. In approximately 5% of patients the disease evolves to a systemic form.References1.   Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Disease of connective tissue. In: Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC, eds. Dermatology. 2nd Completely Revised Edition. Berlin: Springer; 2000. pp. 797-802.2.   Costner MI, Sontheimer RD. Lupus erythe-matosus. In: Freedberg IM, Eisen AZ, Wolf K, Austen KF, Goldsmith LA, Katz SI, eds. Fitzpatrick’s Dermatology in General Medici-ne. 6th Edition. New York: McGraw-Hill; 2003. pp. 1677-93.3.   Kuhn A, Lehmann P, Ruzicka T. Cutaneous lupus erythematosus. Berlin: Springer-Verlag, 2005.4.   Dobrić I, Marinović B, Stipić T. Bolesti vezivnog tkiva. In: Dobrić I et al., eds. Dermatovenerolo-gija. Zagreb. Grafoplast; 2005. pp. 222-4.5.   Miota HA, Miota LDB, Haddadb GR. Associa-tion between discoid lupus erythematosus and cigarette smoking. J Dermatol 2005;211:118-22.6.   Štulhofer-Buzina D. Bolesti vezivnog tkiva. In: Lipozenčić J et al., eds. Dermatovenerologija. Zagreb: Medicinska naklada. 2004: pp. 262-4.7.   Tlacuilo-Parra A, Guevara-Gutierrez E, Gu-tierrez-Murillo F, Soto-Ortiz A, Barba-Gomez F, Hernandez-Torres M, et al. Pimecrolimus 1% cream for the treatment of discoid lupus erythematosus. J Rheumatol 2005;44:1564-8.8.   Sugano M, Shintani Y, Kobayashi K, Sakaki-bara N, Isomura I, Morita A. Successful tre-atment with topical tacrolimus in four cases of discoid lupus erythematosus. J Dermatol 2006;33:887-91.9.   Yoshimasu T, Ohtani T, Sakamoto T, Oshima A, Furukawa F. Topical FK506 (tacrolimus) th-Bardek et al.      Acta Dermatovenerol CroatHypertrophic lupus erythematosus    2009;17(2):134-138138erapy for facial erythematosus lesion of cuta-neous lupus erythematosus and dermatomyo-sitis. Eur J Dermatol 2002;12:50-2.10. Lampropoulos CE, Sangle S, Harrison P, Hu-ges GR, D’Cruz DP. Topical tacrolimus thera-py of resistant cutaneous lupus erythemato-sus: a possible alternative. Rheumatology 2004;43:1383-5.11. Heffernan MP, Nelson MM, Smith DI, Chung JH. 0.1% tacrolimus ointment in the treatment of discoid lupus erythematosus. Arch Dermatol 2005;141:1170-1.12. Rahman P, Gladman DD, Urowitz MB. Smo-king interferes with efficacy of antimalarial therapy in cutaneous lupus. J Rheumatol 1998;25:1716-9.13. Jewell ML, McCauliffe DP. Patients with cuta-neous lupus erythematosus who smoke are less responsive to antimalarial treatment. J Am Acad Dermatol 2000;42:983.14. Lyakhovisky A, Baum S, Shpiro D, Salomon M, Trau H. Thalidomide therapy for discoid lu-pus erythematosus. Harefuah 2006;145:489-92, 551.15. Holm AL, Bowers KE, McMeekin TO, Gas-pari AA. Chronic cutaneous lupus erythemato-sus treated with thalidomide. Arch Dermatol 1993;129:1548-50.16. Grover WCS, Murthy GCPS, Rajagopal WCR, Jalpota (Retd) GCYP, Sudha KV. Discoid lu-pus erythematosus leading to squamous cell carcinoma. MJAFI 2007;63:184-5.Bardek et al.      Acta Dermatovenerol CroatHypertrophic lupus erythematosus    2009;17(2):134-138How to solve the problem of hypertrichosis - according opinion of Dolly sister - take Taky cream; year 1930.(from the collection of Mr. Zlatko Puntijar)ACTA DERMATOVENEROLOGICA CROATICA

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Hypertrophic Lupus Erythematosus: Case Report

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