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      Lupus profundus limited to a site of trauma: Case report and review of the literature

      case-report
      , MD a , b , , MA, BMBCh, MD, FAAD, FACD, FRCP (Edin) a , c , *
      International Journal of Women's Dermatology
      Elsevier
      lupus profundus, lupus panniculitis, cutaneous lupus erythematosus, trauma

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          Abstract

          Lupus erythematosus profundus (LEP) is a rare form of chronic cutaneous lupus erythematosus. We report on a case of a 56-year-old Caucasian woman who presented with a single, persistent, painful rash on the left hip and lateral aspect of the left upper thigh, which had been present for 2.5 years. The patient had a history of previous injury to this area before the rash started. Clinical findings showed an inflamed, hyperpigmented, and indurated plaque with a linear skin invagination and no associated systemic symptoms. A skin biopsy test result confirmed the diagnosis of LEP and the clinical and laboratory examinations ruled out systemic lupus erythematosus. After 2 months of treatment with methotrexate 20 mg weekly and 1 month of prednisolone 7.5 mg daily, the skin rash improved considerably. We also present a brief review of the epidemiology, etiology, clinical features, histopathology, laboratory findings, differential diagnosis, and treatment of LEP.

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          Most cited references37

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          Panniculitis. Part II. Mostly lobular panniculitis.

          The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and alpha(1)-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.
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            Cutaneous lupus erythematosus: issues in diagnosis and treatment.

            Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. Three recognized subtypes of cutaneous LE are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). ACLE may be localized (most often as a malar or 'butterfly' rash) or generalized. Multisystem involvement as a component of systemic LE (SLE) is common, with prominent musculoskeletal symptoms. SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-Ro antibodies and may be induced by a variety of medications. Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic scarring and pigmentary change. Less common forms of CCLE include hyperkeratotic LE, lupus tumidus, lupus profundus, and chilblain lupus. Common cutaneous disease associated with, but not specific for, LE includes vasculitis, livedo reticularis, alopecia, digital manifestations such as periungual telangiectasia and Raynaud phenomenon, photosensitivity, and bullous lesions. The clinical presentation of each of these forms, their diagnosis, and the inter-relationships between cutaneous LE and SLE are discussed. Common systemic findings in SLE are reviewed, as are diagnostic strategies, including histopathology, immunopathology, serology, and other laboratory findings. Treatments for cutaneous LE initially include preventive (e.g. photoprotective) strategies and topical therapies (corticosteroids and topical calcineurin inhibitors). For skin disease not controlled with these interventions, oral antimalarial agents (most commonly hydroxychloroquine) are often beneficial. Additional systemic therapies may be subdivided into conventional treatments (including corticosteroids, methotrexate, thalidomide, retinoids, dapsone, and azathioprine) and newer immunomodulatory therapies (including efalizumab, anti-tumor necrosis factor agents, intravenous immunoglobulin, and rituximab). We review evidence for the use of these medications in the treatment of cutaneous LE.
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              The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites.

              Systemic immunodeficiency is known to facilitate the onset of opportunistic infections, tumours and immune disorders in any district of the body. There are clinical events, such as chronic lymphoedema, herpetic infections, vaccinations and heterogeneous physical injuries which can selectively damage and immunologically mark the cutaneous district they act upon. After the causing event has disappeared, the affected district may appear clinically normal, but its immune behaviour is often compromised forever. An immunocompromised district becomes a site which is particularly susceptible to subsequent outbreaks of opportunistic infections, tumours and immune disorders confined to the district itself. In this review, there is an ample case-report collection of opportunistic disorders (infectious, neoplastic, immune) which appeared in immunocompromised districts. The cases have been grouped according to the clinical settings responsible for the local immune imbalance: regional chronic lymphoedema; herpes-infected sites, which feature the well-known Wolf's isotopic response; and otherwise damaged areas, comprising sites of vaccination, ionizing or UV radiation, thermal burns and traumas. Whatever the immunocompromising factor, a common denominator which facilitates the occurrence of tumours, infections and dysimmune reactions in an immunocompromised district may reside in locally hampered lymph drainage and/or locally altered neuromediator signalling. In fact, any obstacle to the normal trafficking of immunocompetent cells through lymphatic channels or any interference with the signals that the neuropeptides and neurotransmitters released by peripheral nerves send to cell membrane receptors of immunocompetent cells, can significantly alter the local immune response, thus paving the way for heterogeneous opportunistic disorders in the immunocompromised district.
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                Author and article information

                Contributors
                Journal
                Int J Womens Dermatol
                Int J Womens Dermatol
                International Journal of Women's Dermatology
                Elsevier
                2352-6475
                05 May 2017
                June 2017
                05 May 2017
                : 3
                : 2
                : 117-120
                Affiliations
                [a ]St. George Hospital, Sydney, Australia
                [b ]Clínica Alemana de Santiago, Facultad de Medicina Clínica Alemana-Universidad del Desarrollo, Santiago, Chile
                [c ]University of New South Wales, Sydney, Australia
                Author notes
                [* ]Corresponding Author. d.murrell@ 123456unsw.edu.au
                Article
                S2352-6475(17)30040-0
                10.1016/j.ijwd.2017.03.002
                5440450
                28560307
                e1b84e3a-d1e0-44b9-bbed-056754042d05
                © 2017 The Author(s)

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 5 December 2016
                : 27 March 2017
                : 27 March 2017
                Categories
                Case Report

                lupus profundus,lupus panniculitis,cutaneous lupus erythematosus,trauma

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