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      Lupus pernio (Besnier-Tenneson syndrome): A rare form of sarcoidosis

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          Abstract

          Lupus pernio (LP) is a chronic non-life threatining type of cutaneous sarcoidosis that can be related to chronic fibrotic sarcoidosis, hyperglobulinemia and hypercalcemia. The aim of this case report is to evaluate the clinical and demographic features of cutaneous sarcoidosis mainly presenting with a rare manifestation of LP. In this paper we report a case of systemic sarcoidosis presenting with LP and a review of the available literature. LP is a rare presentation with infiltrated erythematoviolaceous plaques affecting the nose. We address the main management approach, and possible association with an underlying systemic sarcoidosis. LP is a rare but chronic manifestation of systemic sarcoidosis that needs to be treated in order to prevent cosmetic defects and psychological effects. It is important to recognize such a condition early in order to avoid a delay in treatment and worsening of the condition, both physically and psychologically. Further research regarding the diagnostic approach and management is required to understand this condition thoroughly.

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          Most cited references16

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          Cutaneous sarcoidosis: differential diagnosis.

          Sarcoidosis is a multisystem disease with cutaneous lesions present in 20%-35% of patients. Given the wide variability of clinical manifestations, it is one of the "great imitators," making it necessary to consider clinical, epidemiological, radiographic, laboratory, and histopathological criteria to make the diagnosis. Cutaneous lesions have been classified as specific and nonspecific, depending on the presence of noncaseating granulomas on histologic studies. Specific lesions include maculopapules, plaques, nodules, lupus pernio, scar infiltration, alopecia, ulcerative lesions, and hypopigmentation among others. Nail, mucosal, and childhood sarcoidosis represent a distinct subset of the disease process. The most common nonspecific lesion is erythema nodosum. Others include calcifications, prurigo, erythema multiforme, nail clubbing, and Sweet syndrome. The importance of considering cutaneous sarcoidosis in the clinical differential diagnosis of a given skin lesion relies on the association with systemic involvement and the convenience of the skin as a tissue source for histologic analysis.
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            The Use of Tetracyclines for the Treatment of Sarcoidosis

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              The treatment of lupus pernio: results of 116 treatment courses in 54 patients.

              Lupus pernio is a disfiguring sarcoidosis skin lesion that is difficult to treat and often causes a major psychosocial impact that may adversely affect the patient's quality of life. We reviewed the treatment outcome of 54 patients with lupus pernio who received 116 individual courses of treatment in our sarcoidosis clinic. Lupus pernio patients were identified from an institution-approved database. All patients were assessed at each clinic visit with facial photographs. By examining the photographs, the percentage of face involved ( 25 to 50%, > 50%) was determined as was the effect of therapy (resolution, near resolution, improvement, no change, worsening). Medications included infliximab-containing regimens; systemic corticosteroids; noninfliximab, noncorticosteroid agents; and corticosteroids plus noncorticosteroid agents. In terms of achieving resolution or near resolution, infliximab regimens were superior to all others (infliximab, 77%; corticosteroids plus noncorticosteroids, 29%; corticosteroids, 20%; noncorticosteroids, 11%; infliximab vs other therapies: corticosteroids plus noncorticosteroids, p = 0.0015; corticosteroids, p = 0.0005; noncorticosteroids, p = 0.0002). The percentage of facial involvement also improved most with infliximab. Evaluating a secondary analysis of achieving resolution, near resolution, or improvement, infliximab (92%) was superior to noncorticosteroids (20%; p < 0.0001) and corticosteroids plus noncorticosteroids (56%; p = 0.0098), but not corticosteroids (72%; p = 0.2456); and noncorticosteroid agents were inferior to all other regimens. Infliximab appears superior to systemic corticosteroids with or without additional agents for the treatment of lupus pernio. Noninfliximab, noncorticosteroid-containing regimens are of limited use for this condition.
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                Author and article information

                Journal
                Dermatol Reports
                DR
                Dermatology Reports
                PAGEPress Publications, Pavia, Italy
                2036-7392
                2036-7406
                01 October 2018
                01 October 2018
                : 10
                : 2
                : 7696
                Affiliations
                Hospital of Skin and Venereal Disease Hospital named after VA Rakhmanov. I.M. Sechenov First Moscow State Medical University , Moscow, Russia
                Author notes
                Hospital of Skin and Venereal Disease Hospital names after VA Rakhmanov. I.M. Sechenov First Moscow State Medical University, Bolshaya Pirogovskaya street 4c1, Moscow, Russia. +79857598534. dr.amalhubail@ 123456gmail.com

                Contributions: the authors contributed equally.

                Conflict of interest: the authors declare no potential conflict of interest.

                Article
                10.4081/dr.2018.7696
                6250080
                26fdb582-781b-49c4-8d4e-00a3dfb98574
                ©Copyright A. Hubail et al., 2018

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 28 March 2018
                : 02 July 2018
                Page count
                Figures: 5, Tables: 1, Equations: 0, References: 19, Pages: 4
                Funding
                Funding: none.
                Categories
                Case Report

                sarcoidosis,cutaneous sarcoidosis,lupus pernio,nasal sarcoidosis

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