Hypertrophic discoid lupus erythematosus
Department of Dermatology, New York University, New York, New York
Hypertrophic discoid lupus erythematosus is a distinct form of chronic cutaneous (discoid) lupus, which is characterized by hyperkeratotic plaques that typically are observed over the face, arms, and upper trunk. We present the case of a 43-year-old man with verrucous plaques that were distributed symmetrically over the face, who initially was treated with oral antibiotics and topical glucocorticoids for acne vulgaris. A biopsy specimen confirmed the diagnosis of hypertrophic discoid lupus erythematosus. The clinical and histopathologic features of this clinical variant are reviewed.
A 43-year-old African American man presented to the Dermatology Clinic at Bellevue Hospital Center in January, 2008, for an initial evaluation of a two-year history of pruritic, crusted plaques on the face. He was initially treated for scarring acne vulgaris with oral doxycycline hyclate, clindamycin phosphate 1 percent lotion, tazarotene 0.05 percent cream, and benzoyl peroxide 5 percent gel and for an irritant dermatitis with hydrocortisone 2.5 percent cream and triamcinolone acetonide 0.1 percent ointment without improvement. In April, 2008, a punch biopsy was obtained from the right nasal bridge. A review of systems was negative for headaches, arthralgias, myalgias, photosensitivity, or Raynaud phenomenon. Past medical history included a schizoaffective disorder for which he takes olanzapine and sertraline. Social history is notable for chronic cigarette smoking.
Hyperpigmented and verrucous, hyperkeratotic plaques with a peripheral erythematous and slightly indurated rim were observed in the medial aspect of the eyebrows, forehead, nasal bridge, chin, and cheeks. Small erosions were noted on the upper and lower lips. Pitted scars were present on both cheeks.
A complete blood count and comprehensive metabolic panel were normal. Antinuclear, SS-A (anti-Ro), SS-B (anti-La), and antidouble-stranded DNA antibodies were absent. C3 and C4 were normal.
There is an interface dermatitis with irregular epidermal hyperplasia, hyperkeratosis, follicular plugs, and superficial and deep, perivascular and periadnexal, lymphocytic inflammation.
Hypertrophic discoid lupus erythematosus was described by Behçet as lupus erythematosus hypertrophicus et profundus in 1940 [1, 2]. This entity is characterized clinically by verrucous and hyperkeratotic plaques with indurated borders and is most frequently reported to occur on the extensor aspects of the forearms, face, and upper trunk . In contrast, discoid lupus erythematosus typically presents with atrophy, telangiectases, follicular plugs, and scale . Development of systemic lupus erythematosus may occur in 5 to 10 percent of patients with discoid lupus erythematosus that is confined to the head and neck. This percentage may be higher in patients with generalized cutaneous involvement .
In a retrospective study of 14 cases of hypertrophic discoid lupus erythematosus, all hypertrophic lesions occurred on sun-exposed sites and were accompanied in all patients with classic discoid lesions as well. Nine cases presented with facial involvement. The arms also were involved in seven cases. One patient developed a squamous-cell carcinoma on the nose 26 years after the onset of cutaneous disease . In contrast, all seven patients described in another case series presented with verrucous, papulo-nodular lesions that were located only on the upper extremities . In this study all patients demonstrated classical discoid lesions on the face and scalp.
Histopathological features reported in hypertrophic discoid lupus erythematosus include hyperkeratosis, follicular plugs, elongation of the rete ridges, irregular acanthosis, and papillomatosis [2, 4]. All 14 of the cases reported by Daldon, et al. demonstrated the presence of solar elastosis and atypia of basal keratinocytes. Characteristic features of cutaneous lupus erythematosus also are present, which include a thick basement membrane, dermal mucin deposition, vacuolar interface changes, and a superficial and deep, perivascular and periadnexal, lymphocytic inflammatory infiltrates . Transepidermal elimination of elastic fibers as well as features of both keratoacanthoma and hypertrophic lichen planus have been reported [2, 4].
An association between smoking and the development of lupus erythematosus has been reported. In addition to an increased incidence of lupus erythematosus observed in current smokers in several studies [5, 6], recent research has suggested a role in contributing to disease activity as well . Furthermore, smokers may be less likely to respond to antimalarial therapy .
Treatments reported to be efficaceous for hypertrophic discoid lupus erythematosus include intralesional triamcinolone acetonide, hydroxychloroquine, acitretin, thalidomide, and isotretinoin [2, 9, 10, 11].
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