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Chronic cutaneous lupus erythematosus mimicking pseudoxanthoma elasticum

  • Author(s): Cassetty, Christopher T
  • et al.
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Chronic cutaneous lupus erythematosus mimicking pseudoxanthoma elasticum
Christopher T Cassetty MD
Dermatology Online Journal 11 (4): 26

Department of Dermatology, New York University School of Medicine

Abstract

54-year-old woman presented with an unusual presentation of chronic cutaneous lupus erythematosus that mimicked pseudoxanthoma elasticum. The lesions were asymptomatic, 2-3 mm, skin-colored, smooth papules without scale or erythema; they were located on the chin, medial malar cheeks, and upper lip. She previously failed traditional treatments, which included hydroxychloroquine and dapsone. The patient responded to thalidomide with a reduction in appearance and number of lesions.


A 54-year-old woman presented to the Connective Tissue Section of the Charles C. Harris Skin and Cancer Pavilion in 2002. She was referred for evaluation of the sudden appearance of asymptomatic papules on her chin, cheeks, and upper lip. Her evaluation by an outside dermatologist included a skin biopsy after the lesions failed to respond to Benzamycin, clindamycin, minocycline, and topical hydrocortisone cream. Other failed treatments included hydroxychloroquine (because of unwanted hyperpigmentation) and dapsone (because of decreased hematocrit). Thalidomide at a dose of 50 mg per day was started and provided a successful decrease in the appearance and number of lesions.

She denied arthralgias, fever, malaise, photosensitivity, or pleuritic chest pain. Her other medications include multivitamins and hormone replacement therapy.

Skin-colored, 2-3-mm, smooth papules without scale or erythema were located on the chin, medial aspects of the cheeks, and upper lip.


Figure 1 Figure 2

Figure 3

An erythrocyte sedimentation rate, antinuclear antibody, anti-cardiolipin, anti-Ro/SSA, anti-La/SSB, double-stranded deoxyribonucleic acid (dsDNA), C- reactive protein, glucose-6-phosphate dehydrogenase, Lyme serology, and angiotensin converting enzyme levels were negative. A complete blood count, electrolytes, chemistries, and urinalysis were normal except for a temporary decreased hematocrit attributed to dapsone.

Histopathology reveals a superficial and mid dermal perivascular infiltrate of lymphocytes, some of which extend to the dermoepidermal junction, in which there are focal vacuolar interface changes. There is epidermal atrophy, a thickened basement membrane, and an increase in the deposition of connective tissue mucin.


Comment

Cutaneous lupus erythematosus (LE) can be divided into chronic, acute, and subacute clinical variants. Chronic cutaneous lupus erythematosus (CCLE) includes discoid LE (DLE), hypertrophic LE, lichenoid LE, lupus panniculitis/profundus, mucosal DLE, palmoplantar erosive LE, and tumid LE [1, 2]. Discoid lesions are the most common manifestation of CCLE and are benign, localized, and affect women more than men. Generalized DLE may be seen in patients with systemic LE.

A classic lesion of DLE appears on the head and neck as a round patch of erythema centrally and hyperpigmentation peripherally. There is usually follicular hyperkeratosis. The advanced lesions usually appear as atrophic plaques with hypopigmentation centrally and hyperpigmentation peripherally [2]. A lesion of pseudoxanthoma elasticum typically appears as discrete yellow papules or plaques of coalesced yellow papules in flexural areas.

Histopathologic examination shows a superficial and deep, perivascular and periadnexal lymphocytic infiltrate, with a lichenoid pattern of vacuolar degeneration of the basal layer and follicular hyperkeratosis (plugs). Civatte bodies and colloid bodies also are present [2]. A direct immunofluorescence test shows IgG, IgM, C3, and C1q in a granular band-like pattern at the dermo-epidermal junction.

Treatment options for CCLE begin with sun avoidance and protection using products with high sun protection factors. Pharmacologic treatments include aminoquinoline antimalarials and dapsone as first-line agents. Gold, prednisone, oral retinoids, and thalidomide are considered second-line therapy. Third-line treatment options include cytotoxic immunosuppressive agents, such as azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, and cyclosporine. Other treatments include clofazamine, interferon-α-2b, and phased plasmapheresis plus cyclophosphamide [3, 4].

Cases of CCLE resistant to other therapies have responded to low dose thalidomide (50-200 mg/day) [3]. The proposed mechanism of action of thalidomide is through immunomodulation by inhibiting the production of tumor necrosis factor-α (TNF-α) and by inducing a T-helper2 (Th2) cell response [5]. TNF-α is thought to play a role in ultraviolet B susceptibility. A major side effect of thalidomide is the teratogenic effect called phocomelia. Other side effects include somnolence, drowsiness, parethesias, and proximal motor neuropathy [5].

References

1. Fabbri P, et al. Cutaneous lupus erythematosus diagnosis and management. Am J Clin Dermatol 2003;4:449

2. Pramatarov KD. Chronic cutaneous lupus erythematosus - clinical spectrum. Clin Dermatol 2004;22:113

3. Kyriakis KP, et al. Experience with low-dose thalidomide therapy in chronic discoid lupus erythematosus. Int J Dermatol 2000;39:218

4. Werth V. Current treatment of cutaneous lupus erythematosus. Dermatol Online J 2000;7:2

5. Knable Jr AL. Micellaneous systemic drugs. In: SE Wolverton, ed. Comprehensive Dermatologic Drug Therapy. Philadelphia: WB Saunders Company, 2001:454

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