Update on management of connective tissue panniculitides

Abstract

In connective tissue diseases panniculitis can be the sole manifestation or occur along with the underlying disease process. The best described forms of connective tissue panniculitis are lupus erythematosus panniculitis (LEP) and lupus profundus, panniculitis associated with dermatomyositis, and morphea and scleroderma associated panniculitis. These processes cause significant morbidity, such as deep atrophic scars, cosmetic disfigurement and psychiatric sequelae. Due to the location of the inflammation in the subcutaneous adipose layer, topical therapies may not penetrate enough to be effective, and systemic agents are required. Despite the large number of reported cases and therapies, recommendations for treatment are based largely on case series and expert opinion due to a lack of controlled therapeutic trials. All treatments are off-label in the United States. The lack of validated clinical outcome measures makes systematic and controlled studies difficult. Nonetheless further investigation into the most effective therapies for these conditions are needed.

Lupus erythematosus panniculitis

“…Pathophysiology of LEP is not clear but hereditary C4 deficiency and trauma have been incriminated and the role of ultraviolet is questionable because LEP usually involves proximal extremities [3]. In our case, previous trauma could be a triggering factor for antigen presentation.…”

“…Clinically it manifests as indurated subcutaneous painful plaques or nodules usually with normal overlying skin and mainly involves face, trunk, breast and proximal extremities [3,5], although epidermal changes including erythema, hyperpigmentation, poikiloderma, ulceration, discoid lupus erythematosus (DLE)-like features have also been reported [1,5]. It mainly affects adults aged between 20 to 60 years and women are almost twice as likely to be affected as men.…”

“…Seventy percent of LEP cases have been associated with DLE but two to five percent of cases have been accompanied by SLE [6,7]. Most cases of LEP have mild and fluctuant courses but up to 15% of cases could be able to progress to SLE and 50% of them have evidences of SLE, so LEP could be a preceding feature of systemic involvement and all patients should be followed up meticulously especially in cases with positive ANA [1,3,[6][7][8]. Arai, et al reported positive ANA in 95.2% of LEP cases [8].…”

“…Lymphoid follicle formation, dermal mucin deposition, hyaline fat necrosis and fibrosclerosis are other characteristic features which have been reported [2,3]. LEP usually presents by subcutaneous nodules, indurated plaques or ulcerations which involves face, proximal extremities and breast [1,3,4].…”

“…LEP usually presents by subcutaneous nodules, indurated plaques or ulcerations which involves face, proximal extremities and breast [1,3,4].…”