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Scleromyxedema in an adult following Sinopharm BBIBP-CorV vaccination: An extremely rare phenomenon.Journal of Scleroderma and Related... Jun 2023The Sinopharm BBIBP-CorV vaccine produces a variety of cutaneous adverse effects. Scleromyxedema is a mucinous connective tissue disorder that causes skin thickness and...
INTRODUCTION
The Sinopharm BBIBP-CorV vaccine produces a variety of cutaneous adverse effects. Scleromyxedema is a mucinous connective tissue disorder that causes skin thickness and sclerodermoid changes. According to our findings, this is the first case of scleromyxedema induced by the Sinopharm immunization.
CASE DESCRIPTION
We discuss the case of a 75-year-old woman who acquired progressive thickening of the skin in her limbs and trunk after getting the Sinopharm vaccination. Examination, laboratory testing, and a biopsy were used to verify scleromyxedema diagnosis. Intravenous immunoglobulins, mycophenolate mofetil, and prednisolone were used in the treatment of the patient. The outcomes from the 4-month follow-up were reassuring.
CONCLUSION
This study emphasizes the need of considering scleromyxedema as a connective tissue pathology in patients who have recently received Sinopharm vaccine and have similar cutaneous signs.
PubMed: 37287947
DOI: 10.1177/23971983221107321 -
BMJ Case Reports Dec 2020Altered mental status can have many causes ranging from emergent intracranial pathologies to more insidious, systemic toxic aetiologies. We report a rare case of...
Altered mental status can have many causes ranging from emergent intracranial pathologies to more insidious, systemic toxic aetiologies. We report a rare case of dermato-neuro syndrome in a 71-year-old man with a known history of scleromyxoedema. The patient initially presented with encephalopathy which quickly progressed to generalised tonic-clonic seizures and coma. While his presentation fits with other, although rare, cases of dermato-neuro syndrome, it is imperative to rule out lethal, more common causes of altered mentation. Due to the rarity and difficulty in diagnosis of dermato-neuro syndrome, there is a significant debate regarding the optimal management as there are no standardised treatment protocols. In our case, the patient was successfully treated with plasmapheresis resulting in improved neurologic function.
Topics: Aged; Brain Diseases; Clinical Deterioration; Coma; Diagnosis, Differential; Humans; Male; Neurologic Examination; Patient Care Management; Plasmapheresis; Respiration, Artificial; Scleromyxedema; Seizures; Syndrome; Treatment Outcome
PubMed: 33376089
DOI: 10.1136/bcr-2020-237170 -
Case Reports in Dermatological Medicine 2020Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous...
BACKGROUND
Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal manifestations. Generalized cutis laxa often has a cephalocaudal progression and is attributed to inflammatory cutaneous eruptions, medications, and infections. Cutis laxa is also associated with several other conditions including rheumatoid arthritis, systemic lupus erythematosus, and plasma-cell dyscrasias. . We report an unusual case of a 35-year-old male with progression of generalized acquired cutis laxa and vasculitis that occurred over a period of one year. No cutaneous inflammatory eruption preceded or accompanied his decreased skin elasticity, and a biopsy of the skin showed elastolysis. His cutaneous manifestation led to systemic evaluation and an eventual diagnosis of smoldering multiple myeloma accompanied by aortitis and anemia. His myeloma and vasculitis were successfully treated with cyclophosphamide, bortezomib, and dexamethasone and high-dose prednisone, respectively, with no improvement to his cutis laxa.
CONCLUSIONS
The presence of monoclonal gammopathy is strongly associated with several dermatological entities such as acquired cutis laxa. We propose a new term for the dermatological manifestations caused by paraproteinemia: monoclonal gammopathy of dermatological significance, or MGODS, and stress the evaluation of an underlying gammopathy in the setting of certain dermatologic conditions, including scleromyxedema and amyloidosis. We present a case of a newly acquired cutis laxa secondary to plasma-cell dyscrasias that exemplifies MGODS, alongside a brief literature review, and underscore the clinical relevance of monoclonal gammopathies of dermatological significance.
PubMed: 32099688
DOI: 10.1155/2020/7480607 -
International Medical Case Reports... 2016Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen....
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. We describe the case of a 56-year-old gentleman with features of scleromyxedema who had cutaneous and cardiac involvement, and significant mediastinal lymphadenopathy without monoclonal gammopathy.
PubMed: 27698568
DOI: 10.2147/IMCRJ.S115315 -
Frontiers in Immunology 2022Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have...
Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have praraproteinemia and extracutaneous, even lethal, manifestations. Yet the prognostic and therapeutic features of scleromyxedema are poorly documented. High-dose intravenous immunoglobulin (IVIG), used either alone or in conjunction with systemic steroids and/or thalidomide, has been suggested as a first-line treatment. We report the case of a 45-year-old woman diagnosed with scleromyxedema with paraproteinemia that initially did not respond to systemic steroids, retinoids, and thalidomide but greatly improvement in terms of systemic and cutaneous symptoms after treatment with IVIG.
Topics: Female; Humans; Middle Aged; Scleromyxedema; Immunoglobulins, Intravenous; Thalidomide; Exanthema; Rare Diseases; Paraproteinemias
PubMed: 36713453
DOI: 10.3389/fimmu.2022.1099918 -
Open Access Macedonian Journal of... Mar 2019Scleromyxedema, also referred to as the Arndt-Gottron (S-AG) syndrome or the systemic form of Lichen myxedematosus (LM), is a cutaneous mucinosis with a chronic course...
BACKGROUND
Scleromyxedema, also referred to as the Arndt-Gottron (S-AG) syndrome or the systemic form of Lichen myxedematosus (LM), is a cutaneous mucinosis with a chronic course and high lethality from systemic involvement of other organs and systems. Interesting in several aspects is the association between scleromyxedema and viral hepatitis about: 1) hepatitis virus infection as a possible etiological factor for the development of scleromyxedema, 2) antiretroviral therapy for the treatment of hepatitis as a method of reversing scleromyxedema and 3) antiviral drugs as inducers of scleromyxedema.
CASE REPORT
We present a 53-year old patient who for nine months had been on tenofovir disoproxil 245 mg (0-0-1) therapy for chronic hepatitis B. Three months after the start of antiviral therapy (i.e. for a period of 6 months), the patient observed swelling, itching and hardening of the skin on the face, ears and hands, which subsequently spread throughout the trunk. Subsequent histological study of a skin biopsy revealed changes of scleromyxedema at an advanced stage, though immunoelectrophoresis of serum and urine excluded the presence of paraproteinaemia or para proteinuria. Systemic antihistamine and topical corticosteroid therapy were instituted. Bone involvement with possible plasmacytoma was excluded, and a myelogram showed evidence of an erythroblastic reaction of bone marrow.
CONCLUSION
We believe that drug-induced scleromyxedema is a rare but possible phenomenon. We describe the first case of tenofovir-induced scleromyxedema within the framework of chronic hepatitis B treatment.
PubMed: 30962839
DOI: 10.3889/oamjms.2019.181 -
Journal of the European Academy of... Feb 2018
Letter on 'European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis'.
Topics: Dermatology; Fibrosis; Humans; Nephrogenic Fibrosing Dermopathy; Scleredema Adultorum; Scleroderma, Localized; Scleroderma, Systemic; Scleromyxedema; Skin Diseases; Undifferentiated Connective Tissue Diseases
PubMed: 29283443
DOI: 10.1111/jdv.14774 -
Case Reports in Dermatology 2016Scleromyxedema is a rare and distinctive variant of cutaneous mucinoses of unknown etiology. It is presenting with generalized papular eruption and sclerodermoid...
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. This study reports on a case of scleromyxedema without paraproteinemia in a subject who experienced a partial response to thalidomide and prednisolone.
PubMed: 27990110
DOI: 10.1159/000452319 -
JAAD Case Reports May 2019
PubMed: 31193003
DOI: 10.1016/j.jdcr.2019.03.005 -
Cureus Mar 2020Scleromyxedema is a rare disorder characterized by diffuse cutaneous and systemic mucinosis with paraproteinemia. Affected patients usually develop numerous waxy, firm...
Scleromyxedema is a rare disorder characterized by diffuse cutaneous and systemic mucinosis with paraproteinemia. Affected patients usually develop numerous waxy, firm papules and plaques as a result of subcutaneous mucin deposition and fibrosis. Systemic manifestations may involve the cardiovascular, gastrointestinal, pulmonary, musculoskeletal, renal, or nervous systems and are known to lead to significant morbidity and mortality if left untreated. As the skin of these patients can be heavily infiltrated with mucin and fibrosis, it is unknown if scleromyxedema affects wound healing. Additionally, owing to the rarity of the disorder, there is very little data regarding surgical outcomes in these patients and their optimal management in the pre- and post-surgical setting. Herein, we report a case of a patient who underwent elective coronary artery bypass grafting (CABG) while in active relapse of his scleromyxedema; he suffered no pre- or post-operative complications and his surgical incision site healed well without any intervention.
PubMed: 32257724
DOI: 10.7759/cureus.7185