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    512 results match your criteria Lichen Myxedematosus

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    European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.
    J Eur Acad Dermatol Venereol 2017 Aug 8. Epub 2017 Aug 8.
    Department of Dermatology and Venereology, University Hospital of Cologne, Cologne, Germany.
    The term 'sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. Read More

    Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
    Clin Rev Allergy Immunol 2017 Jul 16. Epub 2017 Jul 16.
    Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
    Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. Read More

    Review of Primary Cutaneous Mucinoses in Nonlupus Connective Tissue Diseases.
    J Cutan Med Surg 2017 Jul 1:1203475417719053. Epub 2017 Jul 1.
    1 Division of Dermatology, Cumming School of Medicine, Calgary, AB, Canada.
    Lichen myxedematosus is an idiopathic, cutaneous mucinosis with 2 clinicopathologic subsets. There is the generalised papular and sclerodermoid form, more properly termed scleromyxedema, and the localised papular form. We report the first case, to our knowledge, of lichen myxedematosus in association with rheumatoid arthritis as well as a case in association with dermatomyositis. Read More

    [Granulomatous reaction pattern of the skin : Interstitial granulomatous dermatitis - lymphoma - vasculitis].
    Hautarzt 2017 Jul;68(7):553-559
    Konsultations- und Referenzzentrum für Vaskulitis-Diagnostik, MVZ am Marienkrankenhaus gGmbH, Hamburg, Deutschland.
    A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Read More

    Scleroderma-like Disorders.
    Curr Rheumatol Rev 2017 06 11. Epub 2017 Jun 11.
    Fortis Escorts Hospital, Jaipur, India
    Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. Read More

    Atypical Lichen Myxedematosus: A Case with Remarkable Response to Low Dose Melphalan.
    Indian Dermatol Online J 2017 May-Jun;8(3):198-200
    Department of Dermatology and Venereology, Government Medical College, Alappuzha, Kerala, India.
    A 41-year-old man was referred to our outpatient department with a diagnosis of urticaria with angioedema of 3 months duration. On examination, he had generalized coalescent waxy papules and diffuse periorbital swelling. Systemic examination was unremarkable except for limited finger flexion. Read More

    Scleroderma Mimickers.
    Curr Treatm Opt Rheumatol 2016 Mar 5;2(1):69-84. Epub 2016 Feb 5.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. Read More

    A Case of Autoimmune Polyglandular Syndrome .ype 2 Associated with Atypical Form of Scleromyxedema.
    Ethiop J Health Sci 2016 Sep;26(5):503-507
    Department of Clinical Immunology, Allergology and Endocrinology, Donetsk National Medical University, Ukraine.
    Background: Autoimmune polyglandular syndrome type 2 represents an uncommon endocrine disorder composed by Addison's disease with autoimmune thyroid disease (Schmidt's syndrome) and/or type 1 diabetes mellitus. Scleromyxedema is a rare progressive cutaneous mucinosis usually associated with systemic involvement and paraproteinemia. To the best of our knowledge, there is no case report of Schmidt's syndrome associated with scleromyxedema. Read More

    Cutaneous mucinosis in a patient taking ustekinumab for palmoplantar psoriasis.
    Dermatol Online J 2017 Mar 15;23(3). Epub 2017 Mar 15.
    Department of Dermatology, Tufts Medical Center, Boston, MA Tufts University School of Medicine, Boston, MA.
    Discrete papular lichen myxedematosus (DPLM), asubset of localized lichen myxedematosus, is a rarecutaneous mucinosis of unknown etiology. We reporta case of a 57-year-old woman with palmoplantarpsoriasis who developed DPLM 8 weeks after addingustekinumab to a long-term course of methotrexate.The patient had previously failed 2 prior tumor necrosisfactor (TNF) inhibitors, adalimumab and etanercept. Read More

    Acral papular mucinosis: a new case of this rare entity.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):111-113
    Complejo Universitario Hospitalario de Albacete - Castilla la Mancha, Spain.
    Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or laboratory manifestations. The lesions tend to persist and may increase slowly in number. Read More

    Scleromyxedema: clinical diagnosis and autopsy findings.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):48-50
    Private clinic - São Luís (MA) - Brazil.
    Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Read More

    Case for diagnosis. Lichen myxedematosus.
    An Bras Dermatol 2016 Nov-Dec;91(6):842-843
    Faculdade Evangélica do Paraná (Fepar) - Curitiba (PR), Brazil.
    Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. Read More

    Scleromyxedema without Paraproteinemia: Treatment with Thalidomide and Prednisolone.
    Case Rep Dermatol 2016 Sep-Dec;8(3):327-332. Epub 2016 Nov 21.
    Department of Dermatology, Tabriz University of Medical Science, Tabriz, Iran.
    Scleromyxedema is a rare and distinctive variant of cutaneous mucinoses of unknown etiology. It is presenting with generalized papular eruption and sclerodermoid induration. Numerous treatment modalities have been reported to produce partial or permanent responses. Read More

    [Therapeutic administration of immunoglobulins].
    Z Rheumatol 2016 Dec;75(10):956-963
    Klinik für Immunologie und Rheumatologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.
    Background: Intravenously administered immunoglobulins have multiple modes of action that are anti-inflammatory. They can therefore be beneficial in a number of autoimmune disorders.

    Objective: The aim of this article is to analyze and summarize studies on the administration of intravenous immunoglobulins in rheumatological diseases. Read More

    Dermato-neuro syndrome in a case of scleromyxedema.
    Eur J Rheumatol 2015 Dec 22;2(4):160-162. Epub 2015 Apr 22.
    Department of Dermatology, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey.
    Scleromyxedema is an uncommon connective tissue disease characterized by mucin deposits, fibrosis, and proliferation of fibroblasts in the dermis. Although it shares similar sclerodermoid features, it is a different clinical entity than scleroderma. A monoclonal gammopathy is almost always present; however, progression to multiple myeloma is rare. Read More

    Atypical scleromyxedema presenting with cutaneous and cardiovascular manifestations.
    Int Med Case Rep J 2016 19;9:295-299. Epub 2016 Sep 19.
    School of Psychiatry and Clinical Neurosciences, Faculty of Medicine, Dentistry and Health Sciences, University of Western Australia, Crawley, WA, Australia.
    Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen. This condition can be localized as discrete papular lichen myxedematous skin or as a systemic condition usually associated with paraproteinaemia. To date, there is no unifying treatment and is limited by rarity, small number of case reports, and the lack of randomized controlled trials. Read More

    Lichen myxedematosus: diagnostic criteria, classification, and severity grading.
    Int J Dermatol 2017 Mar 25;56(3):284-290. Epub 2016 Sep 25.
    Al-Haud Al-Marsoud Dermatology Hospital, Cairo, Egypt.
    Background: Lichen myxedematosus (LM) is a rare, chronic idiopathic disorder characterized clinically by waxy, closely set papules and histopathologically by diffuse dermal mucin deposition and fibroblast proliferation. The most recent classification of LM was proposed in 2001; however, it seems to be complex, confusing, and imprecise. Herein, we present seven cases of LM to evaluate the validity of the current classification, to propose new diagnostic criteria and classification, and to suggest a clinically relevant severity grading system for this rare disorder. Read More

    Mucin in the dermis: a case of tender tumors.
    Dermatol Online J 2016 Aug 15;22(8). Epub 2016 Aug 15.
    Department of Internal Medicine, Division of Dermatology, The Ohio State University Wexner Medical Center.
    We present an original case report of a 45-year-old woman with a five-month history of sporadic, tender, nodules present on the right upper abdomen, bilateral dorsal wrists, right upper arm, and left flank. Biopsy revealed a mild perivascular infiltrate, increased dermal mucin, and no significant increase in fibroblasts. Presentation and histology were most consistent with nodular lichen myxedematosus (NLM), a rare primary mucinosis. Read More

    Monoclonal gammopathy of cutaneous significance: review of a relevant concept.
    J Eur Acad Dermatol Venereol 2017 Jan 8;31(1):45-52. Epub 2016 Aug 8.
    Faculté de Médecine, Université de Strasbourg et Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
    Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided. Read More

    Atypical discrete papular lichen myxedematosus associated with monoclonal gammopathy: report of four cases and review of the literature.
    J Eur Acad Dermatol Venereol 2016 Dec 16;30(12):2048-2051. Epub 2016 Jul 16.
    Department of Medical Science, Unit of Dermatology, University of Cagliari, Cagliari, Italy.
    Background: Discrete papular lichen myxedematosus (DPLM) is a rare form of localized lichen myxedematosus that presents with skin involvement only and without systemic involvement.

    Object: To describe our experience with atypical cases of DPLM associated with monoclonal gammopathy.

    Methods: Data were collected from patients with clinicopathological evidence of DPLM associated with monoclonal gammopathy who presented to the Department of Dermatology of two tertiary university-affiliated medical centres from 2000 to 2015 and were followed prospectively. Read More

    Retin Cases Brief Rep 2016 Jun 24. Epub 2016 Jun 24.
    *Department of Medicine and Nutrition, University of Guanajuato, Leon Guanajuato, Mexico; and Departments of †Ophthalmology, and ‡Dermatology, Mayo Clinic, Jacksonville, Florida.
    Background/purpose: To describe the findings and clinical course of a patient with scleromyxedema complicated by retinal vasculitis and macular edema.

    Methods: Interventional case report.

    Results: A 64-year old Caucasian woman with recently diagnosed, biopsy proven scleromyxedema presented with decreased visual acuity (20/50 OD; 20/100 OS) due to retinal vasculitis and macular edema. Read More

    A rare association of scleromyxedema with cutis verticis gyrata.
    Indian Dermatol Online J 2016 May-Jun;7(3):186-9
    Department of Skin and STD, Al-Ameen Medical College and Hospital, Bijapur, Karnataka, India.
    Cutis verticis gyrata (CVG) is characterized by redundant skin on the scalp that exhibits deep furrows and convolutions. We report a 58-year-old male who presented with multiple raised asymptomatic skin-colored lesions over the chest and abdomen and features of CVG. Biopsy from the lesion confirmed the diagnosis of scleromyxedema. Read More

    Neurological Involvement in the Course of Scleromyxedema: A Case Report.
    J Stroke Cerebrovasc Dis 2016 Sep 6;25(9):e148-50. Epub 2016 Jun 6.
    Department of Neurology, A. Perrino's Hospital, Brindisi, Italy.
    Scleromyxedema is a rare disease of unknown etiology primarily affecting the skin, characterized by generalized papular eruption, dermal fibroblast proliferation with mucin deposition, and a monoclonal gammopathy. Neurological impairment is a rare but sometimes fatal complication of scleromyxedema that should be rapidly identified to prevent significant morbidity and mortality. A 63-year-old Caucasian man had a 2-year history of scleromyxedema, and was under immunosuppressive treatment with ciclosporine and methotrexate. Read More

    Scleromyxedema: long-term follow-up after high-dose melphalan with autologous stem cell transplantation.
    Int J Dermatol 2016 Oct 21;55(10):e539-43. Epub 2016 May 21.
    Department of Dermatology, MD Anderson Cancer Center, Houston, TX, USA.
    Background: Scleromyxedema is a cutaneous mucinosis that is often accompanied by severe systemic manifestations. New therapeutic options have been introduced for this condition, but data on the long-term efficacy of treatments are limited.

    Objectives: This study was designed to evaluate the long-term efficacy and safety of treatment with high-dose melphalan and autologous peripheral blood stem cell transplantation (PBSCT). Read More

    Cutaneous manifestations of multiple myeloma and other plasma cell proliferative disorders.
    Semin Oncol 2016 Jun 23;43(3):395-400. Epub 2016 Feb 23.
    Plasma Cell Disorders Program, Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute, Carolinas HealthCare System, Charlotte, NC. Electronic address:
    Plasma cell proliferative disorders cause rare but extremely varied dermatologic manifestations that may occur as an accompaniment to established diagnoses, or may be a first clue of an underlying neoplasm in the setting of clinical suspicion. In some instances skin lesions result from aggregation of misfolded monoclonal immunoglobulins or their fragments, as in light chain-related systemic amyloidosis. On other occasions the cutaneous lesions result from deposits of malignant plasma cells or monoclonal proteins. Read More

    Correspondence: The association between morphea profunda and monoclonal gammopathy: A case series.
    Dermatol Online J 2016 Mar 16;22(3). Epub 2016 Mar 16.
    University of Wisconsin.
    It is known that eosinophilic fasciitis can be associated with monoclonal gammopathy. There is clinical similarity between eosinophilic fasciitis and morphea profunda, but it is unclear whether morphea profunda might be associated with monoclonal gammopathy. The temporal quantification of gammopathy in morphea profunda has not been well characterized. Read More

    [Atypical scleromyxedema with a granulomatous histological pattern and delayed sclerosis].
    Ann Dermatol Venereol 2016 May 8;143(5):382-6. Epub 2016 Mar 8.
    Clinique dermatologique, hôpitaux universitaires, université de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France.
    Background: Papular mucinosis is characterised by primary mucin deposition in the dermis. The classification distinguishes between the localised form and the systemic form, which alone can result in complications, but this classification occasionally proves to be inadequate. Herein we report the progression of papular mucinosis, initially atypical due to the absence of cutaneous sclerosis and of misleading granulomatous histological features, which subsequently developed into characteristic scleromyxedema. Read More

    Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases.
    J Am Acad Dermatol 2016 Jun 18;74(6):1194-200. Epub 2016 Feb 18.
    Department of Dermatology, Ed. Herriot Hospital Group, Lyon, France.
    Background: Few histologic studies describe the histopathologic aspects of scleromyxedema.

    Objective: We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients.

    Methods: We studied all the cases with scleromyxedema diagnosed between 2000 and 2014 at participating centers. Read More

    [Scleroderma-like disorders].
    Rev Med Interne 2016 Sep 3;37(9):616-24. Epub 2016 Feb 3.
    Service de médecine interne, hôpital Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
    The finding of hardening and thickening of the skin is common and can be encountered in immune mediated, metabolic, neoplastic, toxic, genetic diseases, or associated with protein deposits. The lack of Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies should question the diagnosis of scleroderma and trigger the search for a scleroderma-like disorder, for which treatment and prognosis differ. This article gives a review of these disorders and their main characteristics. Read More

    [Monoclonal immunoglobulin (M-Ig) and skin diseases from the group of mucinoses--scleredema adultorum Buschke and scleromyxedema. Description of four cases and an overview of therapies].
    Vnitr Lek 2015 Dec;61(12):1072-87
    Introduction: The mucinoses of the type of scleredema and scleromyxedema are diseases marked by excessive production of mucin deposits in the skin and subcutaneous tissue, which causes skin hardening. The skin and subcutaneous deposits hamper the movement of limbs, the thorax as well as mouth. The same mechanism also damages other organs (the heart, lungs, oesophagus). Read More

    Fatal recurrent dermatoneuro syndrome associated with systemic AL amyloidosis.
    Clin Neuropathol 2016 Mar-Apr;35(2):72-7
    A male patient is presented with long-lasting paraproteinemia of monoclonal IgG λ, who suffered from recurrent, and until the last one, mostly reversible episodes of dermatoneuro syndrome, described exclusively in scleromyxedema. The skin biopsy revealed λ-light chain amyloid deposition instead of changes typical for scleromyxedema. Systemic AL amyloidosis was diagnosed post mortem since the patient had no clinical signs of any other organ impairment except skin and brain. Read More

    Frequency of cutis laxa-like clinical features and elastolysis in scleromyxedema: a retrospective clinicopathologic study of 19 patients with scleromyxedema.
    J Cutan Pathol 2016 Mar 4;43(3):246-51. Epub 2016 Feb 4.
    Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
    Background: Cutis laxa-like features were observed in a subset of patients with scleromyxedema. Given this observation, clinical and histopathologic features of scleromyxedema were reviewed in correlation with elastic tissue staining.

    Methods: We retrospectively reviewed clinical records and histopathologic features from patients with scleromyxedema seen at our institution from 1992 through 2013. Read More

    Scleromyxedema secondary to hepatitis C virus and successfully treated with antiviral therapy.
    Dermatol Online J 2015 Sep 17;21(9). Epub 2015 Sep 17.
    Oregon Health and Science University.
    Scleromyxedema (SM) is a chronic and progressive fibromucinous disease with no known etiology. We report a patient with scleromyxedema associated with hepatitis C virus, successfully treated with interferon and ribavirin therapy. Read More

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