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Der Internist Aug 2019Various specific skin alterations can occur in patients with malignant diseases. If these skin diseases occur as associated symptoms of a malignant process, they are... (Review)
Review
Various specific skin alterations can occur in patients with malignant diseases. If these skin diseases occur as associated symptoms of a malignant process, they are called paraneoplastic. In this overview, obligate and frequent facultative paraneoplastic skin diseases are assigned according to the triggering type of malignancy. Some of the processes predominantly show a link with malignant diseases of the digestive tract, e.g. acanthosis nigricans, florid cutaneous papillomatosis, necrolytic migratory erythema, Leser-Trélat syndrome, palmoplantar keratoderma, panniculitis and pityriasis rubra pilaris. Others are predominantly associated with a hematolymphoid malignoma, e.g. acquired ichthyosis, exfoliative erythroderma, necrobiotic xanthogranuloma, paraneoplastic pemphigus, plane xanthoma, pyoderma gangrenosum, scleromyxedema, Sweet syndrome and leukocytoclastic vasculitis. In a third group paraneoplastic skin diseases are pooled in association with other malignancies, e.g. Trousseau's syndrome, dermatomyositis, erythema gyratum repens, hypertrichosis lanuginosa acquisita and papuloerythroderma of Ofuji. In order to initiate targeted diagnostics for detection of an underlying malignant disease, it is essential that accomplished physicians recognize the skin diseases that represent obligate or potential paraneoplasms as such.
Topics: Acanthosis Nigricans; Dermatomyositis; Erythema; Humans; Ichthyosis; Paraneoplastic Syndromes; Skin Diseases
PubMed: 31273399
DOI: 10.1007/s00108-019-0636-1 -
La Revue de Medecine Interne Sep 2016The finding of hardening and thickening of the skin is common and can be encountered in immune mediated, metabolic, neoplastic, toxic, genetic diseases, or associated... (Review)
Review
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.
Topics: Biopsy; Diagnosis, Differential; Female; Humans; Male; Scleroderma, Localized; Skin
PubMed: 26850934
DOI: 10.1016/j.revmed.2015.12.033 -
International Journal of Dermatology Oct 2020Scleromyxedema is a chronic disease with high morbidity and mortality and no definitive therapeutic guidelines. (Review)
Review
IMPORTANCE
Scleromyxedema is a chronic disease with high morbidity and mortality and no definitive therapeutic guidelines.
OBJECTIVE
To review all available data on the efficacy and the safety of the available treatments of scleromyxedema and suggest a possible therapeutic approach.
EVIDENCE REVIEW
We performed a systematic literature review in Pubmed/Medline, Embase, and Cochrane collaboration databases, searching for all articles since 1990 on the treatments of scleromyxedema, with no limits on participant age, gender, or nationality.
FINDINGS
Ninety-seven studies were included in this systematic review, of which one prospective, two retrospective, 70 case reports/case series, and 24 letters/correspondence/clinical image. Intravenous immunoglobulin (IVIG) was the most used first-line therapy based on its efficacy and its generally well-tolerated nature; most patients require continued treatment to remain in remission. Thalidomide and systemic glucocorticoids were mostly considered as second-line therapies and were given alone or in association with IVIG. Patients with severe or refractory disease were treated with autologous bone marrow transplantation, melphalan, or bortezomib with dexamethasone.
CONCLUSIONS AND RELEVANCE
Consideration of patient comorbidities, disease distribution, clinician experience, and treatment accessibility is mandatory in every therapeutic approach of scleromyxedema.
Topics: Bortezomib; Humans; Prospective Studies; Retrospective Studies; Scleromyxedema; Thalidomide
PubMed: 32358980
DOI: 10.1111/ijd.14888 -
Indian Journal of Dermatology,... 2016
Topics: Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Middle Aged; Scleromyxedema
PubMed: 26858056
DOI: 10.4103/0378-6323.174420 -
Medicina Clinica Jul 2014
Topics: Aged, 80 and over; Female; Humans; Scleromyxedema
PubMed: 24768199
DOI: 10.1016/j.medcli.2014.03.002 -
Zeitschrift Fur Rheumatologie Feb 2019Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases... (Review)
Review
BACKGROUND
Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult.
OBJECTIVE
The different disease entities that can lead to dermal fibrosis should be differentiated. An earlier diagnosis of SSc would therefore be facilitated.
METHODS
A literature search was carried out for clinical pictures that can be associated with skin fibrosis. The clinical picture, the etiology and the treatment of the individual diseases are described.
RESULTS
Diseases that can mimic the cutaneous symptoms of SSc include morphea, scleroderma, diabetic cheirarthritis, scleromyxedema, nephrogenic systemic fibrosis and eosinophilic fasciitis. The characteristic pronounced skin involvement, an accompanying Raynaud's phenomenon, capillary microscopy, histopathology and antinuclear antibodies help to enable a differentiation of SSc from its mimics.
CONCLUSION
An early differential diagnostic distinction between SSc and other sclerosing diseases is important due to SSc-associated and potentially life-threatening systemic organ involvement. If a diagnosis of SSc has been made, a critical and organ-specific evaluation with respect to pulmonary, gastrointestinal, renal and cardiac involvement is mandatory and should be repeated at regular intervals.
Topics: Connective Tissue Diseases; Diagnosis, Differential; Eosinophilia; Fasciitis; Humans; Scleredema Adultorum; Scleroderma, Localized; Scleroderma, Systemic; Scleromyxedema; Skin; Synovitis
PubMed: 30255410
DOI: 10.1007/s00393-018-0538-y -
Frontiers in Immunology 2023Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected... (Review)
Review
Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.
Topics: Scleroderma, Systemic; Humans; Diagnosis, Differential
PubMed: 37600771
DOI: 10.3389/fimmu.2023.1180221 -
Postepy Higieny I Medycyny... Jan 2015Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is... (Review)
Review
Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is characterized by fibrosis of the skin and subcutaneous tissues with significant thickening of fascia. Visceral involvement is rare. Characteristic feature in laboratory tests is peripheral blood eosinophilia. Differential diagnosis should be performed, including ruling out systemic sclerosis, nephrogenic systemic fibrosis, eosinophilia-myalgia syndrome, scleromyxedema, hypereosinophilic syndrome or Churg-Strauss syndrome. Final diagnosis is confirmed by histopathological examination. In treatment of the disease corticosteroids and/or immunosuppressive drugs are used. Some other drugs showed activity in this disease e.g. dapsone, infiximab or rituximab. Prognosis is rather good but sometimes a long-term treatment is necessary. In this paper we summarized the current knowledge on eosinophilic fasciitis.
Topics: Diagnosis, Differential; Eosinophilia; Fasciitis; Glucocorticoids; Humans; Immunosuppressive Agents; Prognosis
PubMed: 25897110
DOI: 10.5604/17322693.1149872 -
International Journal of Dermatology Aug 2014Scleromyxedema is a rare generalized form of lichen myxedematosus, a chronic cutaneous mucinosis of unknown etiology usually associated with a monoclonal gammopathy and...
BACKGROUND
Scleromyxedema is a rare generalized form of lichen myxedematosus, a chronic cutaneous mucinosis of unknown etiology usually associated with a monoclonal gammopathy and underlying systemic disorders. It is characterized by the presence of lichenoid papules and diffuse indurations of the skin. Histologically, mucin deposits are observed in the dermis as variable degrees of fibrosis. Numerous treatment modalities have been reported as producing partial or inconsistent responses associated with significant adverse effects.
METHODS
We report an unusual case of scleromyxedema not associated with monoclonal gammopathy in a young patient who was treated with thalidomide.
RESULTS
Patient remained stable with maintenance of injuries despite treatment with thalidomide.
CONCLUSIONS
Scleromyxedema is a rare presentation for which a defined therapeutic regimen remains to be established. Treatment with thalidomide has proved to be effective in the management of these patients. We suggest that these patients should be followed up with periodic protein electrophoresis with immunofixation for a monoclonal component in blood and urine. As the therapeutic approach to scleromyxedema remains challenging and treatment is based on symptomatic presentation, further clinical studies to substantiate an effective therapeutic regimen with a positive long-term safety and risk profile are required.
Topics: Adolescent; Humans; Immunosuppressive Agents; Male; Paraproteinemias; Scleromyxedema; Thalidomide
PubMed: 24527753
DOI: 10.1111/ijd.12124 -
Zeitschrift Fur Rheumatologie Dec 2016Intravenously administered immunoglobulins have multiple modes of action that are anti-inflammatory. They can therefore be beneficial in a number of autoimmune disorders. (Review)
Review
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.
METHODS
A selective search and analysis of the literature was carried out related to the mode of action and efficacy of intravenous immunoglobulins in rheumatological diseases.
RESULTS AND CONCLUSION
Intravenous immunoglobulins have a broad mode of action and can therefore be beneficial in almost all autoimmune diseases. Conditions in which they are of special benefit include immunothrombopenia (ITP), Kawasaki disease and idiopathic inflammatory myopathies. In rare situations, they may also be indicated in systemic lupus erythematosus (SLE), Sjögren's syndrome and neuropathies, catastrophic antiphospholipid syndrome (APS), scleroderma, antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, pyoderma gangrenosum and scleromyxedema. Severe adverse events are rare. In view of the high costs of the therapy, intravenous immunoglobulins are mostly applied in emergency situations, as salvage therapy when other standard therapies have failed or when severe infections are a contraindication to the administration of immunosuppressants.
Topics: Anti-Inflammatory Agents; Autoimmune Diseases; Critical Care; Dose-Response Relationship, Drug; Evidence-Based Medicine; Humans; Immunoglobulins; Rheumatic Diseases; Salvage Therapy; Treatment Outcome
PubMed: 27796482
DOI: 10.1007/s00393-016-0217-9