To the Editor:
The term “lupus erythematosus panniculitis” (LEP) was first proposed by Irgang in 1940 to describe a rare condition which was first recognized by Kaposi in 1883.1 Currently, LEP is believed to represent a distinct clinicopathological variant of lupus erythematosus that involves the subcutaneous fat. It manifests as recurrent deep tender subcutaneous nodules or plaques over the proximal extremities, buttocks, trunk, breast, face, scalp, and rarely the legs.2 The overlying skin may be normal, or may portray the typical features of discoid lupus erythematosus (DLE). The lesions have a chronic relapsing course and frequently heal with depressed lipoatrophic scars. Most patients are women ranging in age from 20 to 60 years.3-5
LEP can present as an isolated phenomenon or in association with DLE or systemic lupus erythematosus (SLE).6 Regarding its frequency of association with SLE, LEP tends to develop in about 2%-5% of patients with SLE and confers a milder disease course.3 The risk of development of SLE and DLE in a patient with LEP varies with the different clinical series and ranges from 10% to 40% and 20% to 60%, respectively.1-4
So far, there is no definitive histopathologic finding that is predictive of systemic disease development. However, 3 reports highlighted the coexistence of extensive membranocystic (lipomembranous) changes and calcification on histology with systemic disease, suggesting that such histologic changes may correlate with SLE development. The first case was that of a 46-year-old Japanese woman with long standing SLE and subsequent LEP lesions.7 The second case depicted a 56-year-old woman with long-standing SLE and thrombophilia with secondary thrombosis of femoral and iliac veins, and with subsequent subcutaneous induration of the lower leg.8 The third case illustrated the previously mentioned histologic features and additional features of occlusive vasculopathy (endarteritis obliterans) in an 84-year-old woman with a 30-year history of SLE.9
We, herein, report the case of a woman with LEP that remained subcutis limited over a follow-up period of 8 years, despite exhibiting on histology extensive subcutaneous necrosis with lipomembranous changes and calcification. The patient was a 41-year-old Lebanese woman and she presented with a 5-year history of an occasionally painful lesion over the left arm which was consistent histologically with lupus panniculitis. She was prescribed hydroxychloroquine 200 mg twice daily by an outside physician. The lesion responded well to the treatment and healed with a depression. Two years later, and after hydroxychloroquine was tapered to one tablet per day, she developed a recurrence. The patient was otherwise healthy and she denied trauma or injection at the site. Her system review was nonrevealing. In particular, she reported no constitutional symptoms, fever, malar rash or photosensitivity, arthralgia/arthritis, abdominal or pleuritic pain, or any neurologic symptoms. Family history was positive for DLE in her brother and SLE in her sister. Physical examination demonstrated evidence of a skin depression over the upper lateral aspect of the left arm. In addition, she had a firm, circumscribed erythematous subcutaneous nodule which was slightly tender over the depressed area. The rest of the physical examination was unremarkable. A skin biopsy from the subcutaneous nodule showed an unremarkable epidermis, a dense superficial and deep perivascular and periadnexal lymphocytic infiltrate with occasional plasma cells, deep dermal fibrosis, and extensive liqueficative necrosis involving the subcutaneous septae and lobules with lipomembranous changes and scattered foci of calcification (Fig. 1). Von Kossa stain highlighted large calcium aggregates along with multiple small foci of dystrophic calcification amidst necrotic adipocytes. PAS stain did not reveal basement membrane thickening. Colloidal iron stain highlighted increased interstitial mucin deposition. The lymphocytic infiltrate was predominantly composed of CD-4 and CD-8 and to a lesser extent CD-20-positive cells. The T cells also stained positive with CD-3 and CD-45RO. CD-56 stain was negative. Gram, and Acid Fast stains were nonrevealing. Direct immunofluorescence studies were not performed. Initial laboratory investigation consisted of routine hematological, full biochemical profile (including kidney and liver function tests, and amylase and lipase levels), erythrocyte sedimentation rate, and urinalysis which were normal. Antinuclear antibody (ANA) was weakly positive showing a speckled pattern at 1:40 dilution (ANA measured using the NOVA Lite HEp-2 kit and the NOVA Lite Titratable ANA pattern control). Anti-Ds DNA, anti-SSA (Ro), anti-SSB (La), anti-cardiolipin antibodies (immunoglobulin G and immunoglobulin M) were negative. C3 and C4 complement levels were on the lower side of the normal range [C3 = 0.89 g/L (0.9-1.8 g/L) and C4 = 0.11 g/L (0.1-0.4 g/L)]. Based on the above findings, the diagnosis of LEP was made. The patient was followed-up periodically for over 8 years during which the LEP lesions remained inactive with no evidence of systemic involvement.
The histologic findings in the current case were unusual and raised the differential of pancreatic panniculitis, traumatic panniculitis, and subcutaneous lymphoma; all of them were excluded by proper history taking, laboratory studies including immunohistochemical stains, and clinical follow-up. In addition, the current report offers an 8-year follow-up period over which there was no progression to SLE. This suggests that the above histologic features are not necessarily specific to or predictive of systemic disease development, and may be rather attributed to underlying ischemic and/or local circulatory disturbances.
Tatiana Khoury, MD,
Thuraya Arayssi, MD
Department of Internal Medicine, American University of Beirut, Medical Center, Beirut, Lebanon
Abdul-Ghani Kibbi, MD,
Samer Ghosn, MD
Department of Dermatology, American University of Beirut, Medical Center, Beirut, Lebanon
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