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      Sneddon-Wilkinson Disease and Monoclonal Gammopathy of Undetermined Significance in the Elderly: Case Report

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          Abstract

          Sneddon-Wilkinson disease (SWD) or subcorneal pustular dermatosis is considered a rare pustular skin disease with chronic relapsing course. An association between SWD and other chronic conditions, such as IgA or IgG monoclonal gammopathy of undetermined significance (MGUS), IgA myeloma, pyoderma gangrenosum, thyroid gland disorders, and neoplastic diseases other than MGUS/myeloma, is known. We describe the case of a 92-year-old male patient with SWD and a concurrent IgG MGUS who had been treated with systemic betamethasone, topical mometasone furoate, and methylprednisolone aceponate, with a complete and durable resolution of symptoms and skin lesions without side effects. Systemic and topical steroids were very effective and well tolerated in our patient. This is the second case reported in the literature on the efficacy of a corticosteroid regimen in SWD in a fragile patient. This therapeutic approach (instead of dapsone therapy) has been used due to its relatively good safety profile.

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          Subcorneal pustular dermatosis: 50 years on.

          Alex M C Yu, Khaled M. Ben-Elhaj, Adrian Cheng … (2008)
          We review the key developments in our understanding of subcorneal pustular dermatosis (SCPD, also known as Sneddon-Wilkinson disease) over the past 50 years. SCPD is a rare, chronic, sterile pustular eruption that was first described by Sneddon and Wilkinson in 1956. The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid fluid blisters. Histologically the salient feature is a subcorneal accumulation of neutrophils, suggesting the presence of chemoattractants such as tumour necrosis factor (TNF)alpha in the uppermost epidermis. However, to date its exact pathophysiology is unknown. Cases in association with pyoderma gangrenosum, benign monoclonal IgA gammopathy and multiple myeloma are well documented. There are anecdotal reports of SCPD associated with other internal malignancies such as chronic lymphocytic leukaemia, thymoma, apudoma and epidermoid carcinoma of the lung. The treatment of choice is dapsone. Therapeutic alternatives include retinoids, phototreatment with psoralen ultraviolet (UV) A, broadband or narrow band UVB and corticosteroids. Anecdotal uses of tacalcitol, ketoconazole, azithromycin, tetracycline, minocycline, vitamin E, ciclosporin, colchicine, mizoribine, mebhydrolin, infliximab and adalimumab with mycophenolate mofetil have all been reported.
          Article 0 comments Cited 24 times – based on 0 reviews     Review now
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            Homocysteine plasma levels in psoriasis patients: our experience and review of the literature.

            M. Giannoni, V Consales, A Campanati … (2015)
            People with psoriasis are at higher cardiovascular risk. Plasma levels of homocysteine over the normal range have been recognized as marker of cardiovascular risk. Psoriasis patients express higher levels of plasma homocysteine than healthy people.
            Article 0 comments Cited 22 times – based on 0 reviews     Review now
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              Characterization and profiling of immunomodulatory genes in resident mesenchymal stem cells reflect the Th1-Th17/Th2 imbalance of psoriasis.

              A Campanati, M Orciani, V Consales … (2014)
              The expression of genes encoding for Th1, Th2 and Th17 cytokines has been extensively evaluated in differentiated skin cells of psoriatic patients. The microenvironment exerts a control on the phenotype of resident mesenchymal stem cells (MSCs) into the skin of psoriasis patients. Aim of the study was to extensively evaluate the relative expression of 43 genes encoding for Th1, Th2 and Th17 cytokines in MSCs isolated from skin of psoriasis patients. MSCs resident into psoriatic skin were isolated, characterized and profiled by PCR array for the relative expression of genes encoding for cytokines involved in Th1, Th2 and Th17 pathways. MSCs isolated from the skin of healthy subjects were used as control. The MSCs isolated from skin of psoriasis patients showed a greater relative expression of the most part of the analyzed genes encoding for Th1 and Th17 cytokines: INF-γ, CCR5, CXCL9, CXCL10, IL6, IL8, TNF-α, IL23A, CCL2, CCL20, CXCL2, CXCL5, IL17C, IL17F, IL17RA, IL21, TLR2 than healthy subjects. On the contrary, the relative expression of genes encoding for Th2 cytokines: CCL1, CCL22, CXCL12, IL2, IL3, IL4, IL13B, IL 22, IL 27, TGF-β1, was similar between the MSCs isolated from psoriasis and healthy subjects. In conclusion, the MSCs isolated from psoriasis show an imbalance between the Th1-Th17 and Th2 pathways, which reflects the well-known abnormal balance observed in differentiated skin cells. This evidence could strengthen the hypothesis of an early involvement of resident MSCs in the pathogenesis of psoriasis.
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                Author and article information

                Journal
                Journal ID (publisher-id): CDE
                Journal ID (pmc): CDE
                Journal ID (doi): 10.1159/issn.1662-6567
                Title: Case Reports in Dermatology
                Publisher: S. Karger AG
                ISSN (Electronic): 1662-6567
                Publication date Subscription-year: 2019
                Publication date Collection: May -August 2019
                Publication date (Electronic): 10 July 2019
                Volume: 11
                Issue: 2
                Pages: 209-214
                Affiliations
                [_a] aDermatology Unit, Department of Clinical and Molecular Sciences, United Hospital of Ancona, Polytechnic Marche University, Ancona, Italy
                [_b] bPathology Department, Polytechnic Marche University, Ancona, Italy
                Author notes
                *Anna Campanati, Dermatological Unit, Polytechnic Marche University, Via Conca 71, IT–60200 Ancona (Italy), E-Mail anna.campanati@gmail.com
                Article
                Publisher ID: 487003 Pmcid ID: PMC6696771 Other ID: Case Rep Dermatol 2019;11:209–214
                DOI: 10.1159/000487003
                PMC ID: PMC6696771
                PubMed ID: 31427943
                SO-VID: aa96146a-724b-4239-bca5-d5933df34f02
                Copyright © © 2019 The Author(s). Published by S. Karger AG, Basel
                License:

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                Date received : 08 January 2018
                Date accepted : 19 January 2018
                Page count
                Figures: 3, Pages: 6
                Categories
                Subject: Single Case

                ScienceOpen disciplines: Oncology & Radiotherapy,Pathology,Surgery,Dermatology,Pharmacology & Pharmaceutical medicine
                Keywords: Sneddon-Wilkinson disease,Monoclonal gammopathy of undetermined significance,Corticosteroids,Subcorneal pustular dermatosis
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy, Pathology, Surgery, Dermatology, Pharmacology & Pharmaceutical medicine
                Keywords: Sneddon-Wilkinson disease, Monoclonal gammopathy of undetermined significance, Corticosteroids, Subcorneal pustular dermatosis

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