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Chronic cutaneous lupus erythematosus in vitiligo

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Chronic cutaneous lupus erythematosus in vitiligo
Hillary Johnson MD PhD, Nicole Bossenbroek MD, Karla Rosenman MD, Shane A Meehan MD, Mirin Robles MD, Miriam K Pomeranz MD
Dermatology Online Journal 14 (10): 10

Department of Dermatology, New York University


A 49-year-old woman presented with a seven-year history of pruritic, erythematous, scaling plaques on sun-exposed skin that localized only to pre-existing depigmented patches. Histopathologic examination showed changes consistent with cutaneous lupus erythematosus with lichenoid features and confirmed contiguous vitiligo. Diagnosis of chronic cutaneous lupus erythematosus localized to areas of vitiligo was determined by clinicopathologic correlation and may reflect an autoimmune diathesis. Consequently, hydroxychloroquine and topical glucocorticoids therapy were initiated with reported improvement in pruritus, erythema, and scale. Clinical monitoring for development of squamous-cell carcinoma in areas of chronic inflammation and sun-exposure is imperative.

Figure 1Figure 2


A 49-year-old Bangladeshi woman, who recently relocated to the United States, presented to the Dermatology Clinic at Bellevue Hospital Center in September, 2007, with a seven-year history of a pruritic, erythematous, scaling eruption localized to areas of pre-existing vitiligo of 15-year duration. The patient lacked systemic complaints. Family history of vitiligo and other cutaneous disorders was denied. The patient had a medical history of diabetes mellitus since 1999 that was treated with glicazide and pioglitazone. In 2002, the patient underwent electron-beam radiation therapy to the right extensor forearm for invasive squamous-cell carcinoma in the setting of a non-specific dermatitis according to a 2006 histopathology report. Additional surgical history included appendectomy and hysterectomy. She denied a history of tobacco use or chemical exposure. Review of medical records from India showed past treatment of unknown duration with hydroxychloroquine 200 mg twice daily, acitretin 10 mg once daily, tazarotene cream, topical glucocorticoids (clobetasol ointment, clobetasone butyrate ointment, or betamethasone cream), vitamin E cream, or collagen elastin cream for prior diagnoses of psoriasis and cutaneous lupus erythematosus. Fexofenadine and hydroxyzine had been provided for pruritus. The patient did not recall if the prior therapies resulted in clinical improvement.

After review of laboratory and histopathologic data obtained at the Bellevue Hospital Center, hydroxychloroquine 200 mg twice daily was initiated. In conjunction, affected areas were treated with clobetasol propionate 0.05 percent ointment twice daily on the trunk and extremities or fluocinolone acetonide 0.025 percent ointment twice daily on the face. In the subsequent two weeks, she reported reduction in pruritus, erythema, and scale.

Physical Examination

Generalized, well-demarcated, depigmented patches with enhancement under Wood's lamp examination were present on the face, postauricular neck, abdomen, back, buttocks, extensor apects of the arms and legs, and dorsal aspects of the hands. Many of the depigmented patches contained focal, round, normally-pigmented, brown macules within them. Within some of the depigmented patches on the face, forearms, and back were focal, brightly erythematous scaling plaques. Notably, the back was a sun-exposed area in this patient due to daily attire in Indian clothing (sari or saree). Similar erythematous, scaling plaques were observed in the conchal bowls and the mucosal lips. On the right extensor aspect of the forearm, one of the erythematous, scaling plaques was depressed at the site of past electron beam radiation for squamous-cell carcinoma. The patient exhibited frontal alopecia without evidence of scars. Examination of the nails was limited by the prior application of henna dye, but they did not appear abnormal.


A complete blood count, fasting lipid profile, and thyroid function panel were normal. Comprehensive metabolic panel showed an elevated serum glucose of 162 mg/dL, and hepatic function panel showed an elevated alkaline phosphatase of 113 IU/L. Erythrocyte sedimentation count and c-reactive protein were elevated at 55 mm/hr and 15.95 mg/L respectively. Anti-nuclear antibody, double-stranded DNA antibody, Smith antibody, ribonucleoprotein antibody, and rheumatoid factor were not detected. The Sjogren's single-stranded A index was elevated at 1.65 units (normal index ≤1.00) while Sjogren's single-stranded B antibody index was normal. Urinalysis and glucose-6-phosphate dehydrogenase level were normal.


There is a band-like lymphocytic infiltrate, with a vacuolar change and necrotic keratinocyte at the dermoepidermal junction. There is epidermal acanthosis, hypergranulosis, and hyperkeratosis. Thickening of the basement membrane and hyalinization of dilated superficial blood vessels are present. A S100 stain shows a loss of epidermal melanocytes. Acid-fast bacillus and Steiner stains for mycobacteria and spirochetes are negative.


Chronic cutaneous lupus erythematosus (CCLE) is an inflammatory and photosensitive dermatosis that likely stems from a poorly understood immune dysregulation with an autoimmune reaction in genetically predisposed individuals. While patients with systemic lupus erythematosus (SLE) may have cutaneous involvement, studies estimate CCLE progression to SLE in less than 5 percent to 10 percent. CCLE occurs more frequently in women and is often characterized by erythematous, scaling plaques but may exhibit clinical variability, such as hypertophic or lichenoid features. Serologic abnormalities are not common; however, some patients with CCLE exhibit positive antinuclear antibody, double-stranded DNA antibody, or Smith antibody. Sjogren's single-stranded A (SS-A or Ro) autoantibody may be found in a small percentage of patients with CCLE. Typical treatment includes photoprotection, topical or intralesional glucocorticoids, or antimalarial medication. Alternative therapies include retinoids or immunosuppressive agents, such as thalidomide, methotrexate, mycophenolate mofetil, or azathioprine [1-9].

Coexistence of cutaneous lupus erythematosus (CLE) and vitiligo has been infrequently reported [10-18]. Vitiligo is a part of systemic autoimmune dysregulatory process with underlying genetic susceptibility since approximately 30 percent with generalized vitiligo have associated autoimmune disorders, such as thyroid disease, diabetes mellitus, and alopecia areata. Unlike CLE, there is no sex-related predilection [19, 20, 21]. Autoimmune endocrinopathy and cutaneous disease have been linked to syndromes of systemic immune disorder. Diabetes mellitus has been associated with vitiligo, and a number of connective-tissue disorders that include SLE [22, 23]. In murine models, antibodies obtained from the sera of non-obese diabetic mice exhibit nuclear and cytoplasmic staining similar to the pattern observed in autoimmune connective-tissue diseases [24].

In the case presented, an autoimmune diathesis with sequential development of generalized vitiligo in 1992, diabetes mellitus in 1999, and CLE in 2002 was notable. In published literature, the majority of patients with concurrent CLE and vitiligo resided in regions with potential chronic sun exposure, such as India and southern Europe [10-18]. Thus, photosensitivity and chronic sun exposure may serve as a triggering factor although it is unclear if formation of CLE in vitiligo represents a transformation or collision of two cutaneous disorders. In lupus erythematosus, ultraviolet B-induced apoptotic and necrotic keratinocytes are putative targets for autoantibodies and mediators of inflammatory cascades. A reported association between autoantibody specificities for Sjogren's single-stranded antibodies (SS-A/Ro and SS-B/La) and CLE is controversial due to poor reproducibility [25].

Ultravioletlight exposure and chronic inflammation play roles in development of squamous-cell carcinoma (SCC), a known complication of CCLE. While SCC formation in long-standing vitiligo is rare, it has been associated with PUVA photochemotherapy and with intense sun exposure [26, 27, 28, 29, 30]. Squamous-cell carcinomas, sometimes multiple, have been described as arising in lesions of CCLE [15, 31]. In addition, KA and SCC may be difficult to distinguish from hypertrophic LE [32].The appearance of keratoacanthoma has been reported in a 65-year-old Indian woman with generalized vitiligo, in whom the depigmented patches on sun-exposed areas contained erythematous, scaling, sclerotic plaques considered to be secondary actinic change or chronic discoid lupus erythematosus. The keratoacanthoma erupted rapidly in an existing depigmented and sclerotic plaque that had been noted for several years [17]. Our patient presented with a history of invasive squamous cell carcinoma arising from a similar appearing depigmented and inflammatory lesion on the forearm. Thus, exposure to ultraviolet light may potentiate the appearance of CCLE and complication of SCC in patients with vitiligo.


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