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Introduction: Lupus erythematosus panniculitis (LEP) or lupus erythematosus profundus is a rare form of chronic cutaneous manifestation affecting both adults and pediatric patients. The prevalence of this manifestation was seldom reported in juvenile systemic lupus erythematosus (JSLE)., Case Reports: From January 1983 to December 2010, 5,506 patients were followed at the Pediatric Rheumatology Unit of our University Hospital and 278 (5%) of them met the American College of Rheumatology classification criteria for JSLE. Two (0.7%) of them had LEP at JSLE onset. These two cases had tender deep inflammatory subcutaneous nodules or plaques at the time of diagnosis, and the histopathologic pattern evidenced lobular or mixed panniculitis with lymphocytic inflammatory cells of the fat lobule. Treatments for LEP included mainly antimalarials, systemic corticosteroids and sunscreen protection. One male patient required thalidomide and immunosuppressive drugs, including mycophenolate mofetil, cyclosporin and intravenous cyclophosphamide. However, skin lesions improved only after rituximab treatment., Discussion: LEP was rarely observed in our cohort of JSLE patients as the first lupus manifestation. Anti-CD20 monoclonal antibody therapy may be an option for refractory LEP treatment in children.

SUMMARY Skin disease in patients with lupus erythematosus may be subdivided into two broad categories - those lesions that when biopsied demonstrate interface dermatitis and those that do not demonstrate interface dermatitis. The skin lesions that are represented by the interface dermatitis include discoid lupus erythematosus, subacute cutaneous lupus erythematosus and acute cutaneous lupus erythematosus. Patients with these 'specific' manifestations have varying degrees of systemic involvement from rare systemic disease in patients with localized discoid lupus erythematosus to common and often severe involvement in patients with acute cutaneous lupus erythematosus. Patients who do not demonstrate interface dermatitis also may have systemic disease and in some instances the skin manifestations are linked to some of the more severe systemic manifestations. Many patients with cutaneous lesions characterized by the interface dermatitis can be controlled with 'standard' therapies including sunscreens, protective clothing and behavioural alteration, and topical corticosteroids with or without an oral antimalarial agent. This review presents a brief summary of each common cutaneous manifestation of lupus erythematosus, its relationship to systemic involvement and treatment issues to effectively deal with the lupus erythematosus patient who has skin disease.

Background: Although subcutaneous T-cell lymphoma (SCTCL) is considered an aggressive form of lymphoma, some patients manifest a long waxing and waning phase unaccompanied by constitutional symptoms., Methods: Twelve patients were prospectively encountered, presenting with a lymphocytic panniculitis accompanied by lymphoid atypia, although not fulfilling criteria for SCTCL. Clinical, histologic, phenotypic, and genotypic analyses were conducted., Results: There were five men, one boy, and six women; none had symptoms compatible with lupus erythematosus or aggressive SCTCL. All but two had a waxing and waning course of years. Four patients had periodic cytopenias accompanied by fevers. While responding somewhat to prednisone, the lesions relapsed. In one patient, treatment with alemtuzumab (CAMPATH-1) led to complete lesional resolution with no recurrence. Light microscopy showed expansion of the interstices of the fat lobule by mildly atypical lymphocytes of the CD4 subset in 10 biopsies from eight patients; in the other four patients, there was an increase in CD8 lymphocytes. There was diminished expression of CD5 and/or CD7 in the majority of biopsies. Ten of 13 biopsies showed clonal T-cell receptor-gamma rearrangements., Conclusions: We apply the term atypical lymphocytic lobular panniculitis to this distinctive form of lymphocytic panniculitis manifesting this light microscopic, phenotypic, and genotypic profile.

Lupus profundus is an unusual clinical variant of cutaneous lupus erythematosus that has previously been described as a benign disease that follows a mild course. This report describes the extent of disease and associated comorbidities in patients with severe lupus profundus and systemic lupus erythematosus. Four cases of lupus profundus are reviewed and their associated systemic disease complications are highlighted. All four patients fulfilled at least four of the 11 criteria for systemic lupus erythematosus. One patient suffered from severe facial disfigurement and Parry-Romberg syndrome. Two patients developed nonhealing ulcers on the scalp. All four patients had scarring alopecia as well as depressed areas over large areas of their body surfaces. All patients were resistant to conservative therapy, and required long-term aggressive therapy. Clinical depression secondary to disfigurement was a major problem in three patients. Extensive lupus profundus may be associated with more serious systemic disease and warrants aggressive treatment early on to prevent permanent disfigurement and its resultant psychological consequences.

Objective: To review clinical and laboratory features of lupus panniculitis from a large group of patients., Methods: Retrospective chart review of patients diagnosed with lupus panniculitis at a tertiary medical center from 1976 to 1993., Results: Lupus panniculitis occurred most frequently in adult women. Skin lesions involved proximal extremities, trunk, face, and scalp. Only 4 of 40 patients fulfilled criteria for systemic lupus erythematosus (SLE), and, other than positive antinuclear antibodies, a paucity of other autoantibodies was seen. Average disease duration was 6 years (range 0-38). Treatment with antimalarial agents was undertaken in most cases. Disease related morbidity (disfigurement and disability) was relatively common, but death was rare., Conclusion: Lupus panniculitis is a chronic inflammatory disease of subcutaneous adipose tissue that can develop during the course of SLE, although most patients do not develop systemic lupus.

LE panniculitis is an uncommon but distinctive subset of LE. It may develop in patients with discoid LE or SLE or may occur as an isolated phenomenon. The typical clinical presentation is that of multiple indurated nodules or plaques (or both), often associated with lipoatrophy, there being a predilection for the proximal extremities and trunk. Because the clinical and histologic findings of LE panniculitis overlap with those of other connective tissue diseases, evaluation of patients suspected of having LE panniculitis should include a complete history and physical examination as well as serologic studies, determination of peripheral blood counts, and tests of renal function. A deep excisional biopsy rather than punch biopsy should be performed for diagnosis. The characteristic histologic pattern includes hyaline necrosis of fat; lymphoid nodules, often with germinal centers; and lymphocytic lobular panniculitis. Direct immunofluorescence testing of skin may help confirm the diagnosis in patients who have less than classic histologic features. LE panniculitis tends to have a chronic course marked by recurrent nodules or plaques (or both). Antimalarial agents, with or without courses of systemic steroids, are beneficial in most patients.