Lenalidomide and Dexamethasone in Scleromyxedema: A Case Report of Long-Term Efficacy and Challenges in a 54-Year-Old Patient. [PDF]
CureusPeshin S, Modi SK, Burwick N.
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Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema. [PDF]
J Eur Acad Dermatol VenereolIn severe forms of monoclonal gammapathy associated with scleromyxoedema with dermatoneuro syndrome and/or cardiac involvement, deep and durable responses allowing IVIG withdrawal could be achieved using anti‐plasma cell therapy, including Imids, anti‐CD38 or proteasome inhibitors. Abstract Background Scleromyxedema (SM) is a rare skin disorder related
Theves F +12 more
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Localized eruption of glabella and eyebrows [PDF]
JAAD Case ReportsCelestina Okoye, MD +2 more
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Current Opinion in Rheumatology, 2014 To synthesize the current known data on pathogenesis and treatment of scleromyxedema. This review will also highlight the clinical presentation, systemic features and outcomes and distinguishing features between scleromyxedema and scleroderma, as a common mimic.Most recent publications have focused on describing treatment responses with novel therapies,
Ryan R, Bogner +2 more
openaire +5 more sources
Eosinophilic fasciitis (Shulman's disease): review and comparative evaluation of seven patients. [PDF]
, 2019 OBJECTIVES: Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and
Cimbron, M +3 more
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Lichen myxedematosus: a rare group of cutaneous mucinosis [PDF]
Anais Brasileiros de Dermatologia: Cutaneous mucinoses are a heterogeneous group of dermatoses in which excess deposition of mucin in the dermis gives the skin a waxy appearance, with papules and plaques that can vary from self-healing mucinosis to even disrupting the normal shape of a ...
Ramiro Eugenio Cárdenas-Gonzalez +2 more
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Mechanism of NSF: New evidence challenging the prevailing theory [PDF]
, 2009 Nephrogenic systemic fibrosis (NSF) has been associated with the administration of gadolinium-based contrast agents in patients with severely impaired renal function (SIRF), endstage renal disease (ESRD), or acute renal failure (ARF).
Jimenez, MD, Sergio A. +1 more
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Absence of human herpes virus-8 (HHV8) in nephrogenic systemic fibrosis [PDF]
, 2008 Background Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder that exhibits CD34 expression in the majority of lesional spindle cells. Several features of NSF bear similarity to Kaposi sarcoma.
Pantanowitz Liron +2 more
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Scleromyxedema concurrent with dermatomyositis and paraproteinemia: A case report
Современная ревматология, 2015 The paper describes the case of a female patient who had at least three diseases: scleromyxedema, dermatomyositis/polymyositis (DM/PM), and paraproteinemia (monoclonal gammopathy).
M. N. Starovoitova +2 more
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Unilateral Extremity Swelling, a Rare Manifestation of Scleredema Adultorum of Buschke in a Child: Case Report. [PDF]
Clin Case RepABSTRACT Scleredema adultorum of Bushcke is a rare connective tissue disorder that is characterized by skin thickening that commonly starts from the neck and spreads to the face, shoulders, upper back, abdomen, and in some cases, thighs. The symptoms are generally seen after an infection associated with streptococcus.
Hajiani Ghotbabadi S +3 more
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