-
JAAD Case Reports Aug 2022
PubMed: 35899146
DOI: 10.1016/j.jdcr.2022.06.022 -
The Lancet. Rheumatology May 2022Monoclonal proteins can provide important information on the diagnosis of several non-malignant systemic inflammatory disorders. At low concentration, they most commonly... (Review)
Review
Monoclonal proteins can provide important information on the diagnosis of several non-malignant systemic inflammatory disorders. At low concentration, they most commonly represent monoclonal gammopathy of undetermined significance (MGUS), whereas high concentrations often signify plasma cell myeloma or B-cell lymphoma. However, several rare inflammatory conditions associated with variable concentrations of monoclonal proteins, systemic symptoms, and organ dysfunction also exist. These conditions are termed monoclonal gammopathies of clinical significance (MGCS). Patients with MGCS might present to rheumatologists with undiagnosed systemic inflammatory disorders and the monoclonal protein provides an important, underappreciated clue for diagnosis. In this Review, we provide an approach to distinguishing MGCS from MGUS and lymphoid neoplasms, focusing on four rare MGCS that rheumatologists must recognise: scleromyxedema, Schnitzler's syndrome, idiopathic systemic capillary leak syndrome (also known as Clarkson's disease), and telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting (known as TEMPI) syndrome.
PubMed: 38294033
DOI: 10.1016/S2665-9913(21)00348-9 -
Journal of Cosmetic Dermatology Oct 2022Scleromyxedema is a rare primary cutaneous mucinosis characterized by numerous firm, waxy, confluent papules. Recently, intravenous immunoglobulin (IVIG) is accepted by...
INTRODUCTION
Scleromyxedema is a rare primary cutaneous mucinosis characterized by numerous firm, waxy, confluent papules. Recently, intravenous immunoglobulin (IVIG) is accepted by many authors as the first-line treatment option for severe cases. We report a 69-year-old male patient who has been suffering from scleromyxedema, with reduced mouth opening. He has been on a high-dose IVIG regime for 5 years.
METHODS
The patient stated that he had difficulty in wearing and removing his dentures because of reduced mouth opening lately. Before considering to add any other immunosuppressants to his regime, we injected 1500 IU of hyaluronidase in total in one session periorally. The patient has been told open his mouth maximum and photographs have been taken before injections and after one month. We used a photo measurement application when evaluating microstomia to increase accuracy. We also took punch biopsies in order to evaluate effect of hyaluronidase histopathologically before and one month after injections.
RESULTS
One month later, he was able to reattach and remove his dentures without adding any adjuvant immunosuppressants other than hyaluronidase. Mouth opening was increased in measurements and histopathologically, mucin deposition, fibroblastic proliferation, and perivascular lymphocytic infiltration were decreased.
CONCLUSIONS
We think hyaluronidase is a safe, easily accessible, and effective treatment option for microstomia caused by scleromyxedema.
Topics: Male; Humans; Aged; Scleromyxedema; Immunoglobulins, Intravenous; Hyaluronoglucosaminidase; Microstomia; Immunosuppressive Agents
PubMed: 35441782
DOI: 10.1111/jocd.15007 -
Blood Cancer Journal Apr 2022Monoclonal gammopathy associated with dermatological manifestations are a well-recognized complication. These skin disorders can be associated with infiltration and... (Review)
Review
Monoclonal gammopathy associated with dermatological manifestations are a well-recognized complication. These skin disorders can be associated with infiltration and proliferation of a malignant plasma cells or by a deposition of the monoclonal immunoglobulin in a nonmalignant monoclonal gammopathy. These disorders include POEMS syndrome, light chain amyloidosis, Schnitzler syndrome, scleromyxedema and TEMPI syndrome. This article provides a review of clinical manifestations, diagnostics criteria, natural evolution, pathogenesis, and treatment of these cutaneous manifestations.
Topics: Amyloidosis; Humans; Monoclonal Gammopathy of Undetermined Significance; Paraproteinemias; Plasma Cells; Skin Diseases
PubMed: 35411042
DOI: 10.1038/s41408-022-00661-1 -
Journal of Scleroderma and Related... Jun 2021Scleromyxedema is a mysterious cutaneous mucinosis of unknown etiology. Various types of scleromyxedema variant have been reported, which often give us a clue to...
Scleromyxedema is a mysterious cutaneous mucinosis of unknown etiology. Various types of scleromyxedema variant have been reported, which often give us a clue to understand the key aspects of this disease. Here, we describe a woman with highly unusual type of scleromyxedema. In addition to the rare manifestations of multiple subcutaneous nodules and IgM-λ paraproteinemia, our patient showed several characteristic symptoms of scleroderma such as shortened nails and fingertips, sclerodactyly, and bone resorption of fingertips and mandibles as a result of peripheral circulatory insufficiency, although this disease is known to be pathophysiologically different from scleroderma. A skin biopsy revealed cutaneous microvascular stenosis and occlusion due to intravascular mucin deposition and fibrotic changes, suggesting that scleromyxedema potentially develops peripheral circulatory disorders and other vascular involvement. The subcutaneous nodules were responsive to high-dose intravenous immunoglobulin. Scleromyxedema can represent a wide variety of systemic involvement, and therefore, we should pay attention to those symptoms as well as skin lesions.
PubMed: 35386736
DOI: 10.1177/2397198320974190 -
A Case of Scleromyxedema Development Following Intravesical Bacillus Calmette-Guérin Administration.The Journal of Rheumatology Jul 2022
Topics: Administration, Intravesical; BCG Vaccine; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Scleromyxedema; Urinary Bladder Neoplasms
PubMed: 35169063
DOI: 10.3899/jrheum.211010 -
Journal of the European Academy of... Jul 2022Cutaneous mucinoses are a heterogenous group of conditions, characterized by the deposition of glycosaminoglycans (mucin) in the dermis, follicles, or in the epidermis.... (Review)
Review
Cutaneous mucinoses are a heterogenous group of conditions, characterized by the deposition of glycosaminoglycans (mucin) in the dermis, follicles, or in the epidermis. Major cutaneous mucinoses include lichen myxedematosus, scleredema, mucinoses associated with thyroid disease, reticular erythematous mucinosis, papulonodular mucinosis associated with connective tissue diseases, and cutaneous focal mucinosis. The aim of this review is to provide an update of what has currently been reported in the last 30-year literature about several new or emerging conditions of acquired cutaneous mucinoses in adults. Two new clinico-pathologic entities have been described: (i) Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis; (OACM) (ii) Nodular mucinosis of the breast (NMB). Two relatively new disease categories encompassing cutaneous mucinoses with a common pathogenetic mechanism have been identified: (i) Cutaneous mucinoses associated with drug exposure including biologic therapy, anti-colony-stimulating factor 1 receptor (CSF1R) and subcutaneous intralesional interferons (toxic dermal mucinoses); (ii) Cutaneous mucinosis following physical agents including mechanical traumas and after knee replacement.
Topics: Adult; Breast; Connective Tissue Diseases; Humans; Mucinoses; Scleredema Adultorum; Scleromyxedema; Skin Diseases
PubMed: 35124832
DOI: 10.1111/jdv.17983 -
Cureus Oct 2021Solitary cutaneous focal mucinosis is a unique condition defined by the presence of mucin, a hyaluronic acid complex, in the dermis. The lesion typically presents as an...
Solitary cutaneous focal mucinosis is a unique condition defined by the presence of mucin, a hyaluronic acid complex, in the dermis. The lesion typically presents as an isolated, asymptomatic papule or nodule on the extremities or back and is not associated with any systemic condition. Conversely, multiple cutaneous focal mucinosis present with numerous skin lesions has been found to be associated with systemic diseases such as scleromyxedema, systemic lupus erythematous, and thyroid disease. Therefore, additional laboratory investigation should be considered when multiple cutaneous focal mucinosis is discovered. The case of a 37-year-old man with solitary cutaneous focal mucinosis is discussed. The skin lesion presented as an asymptomatic nodule on his right upper shoulder; microscopic evaluation established the diagnosis, and laboratory investigation was negative for any associated conditions. Similar to previous reports of solitary cutaneous focal mucinosis, our patient provides additional supporting evidence that laboratory studies for mucin-associated systemic disease are not required for individuals who present with cutaneous focal mucinosis consisting of only a solitary skin lesion.
PubMed: 34786224
DOI: 10.7759/cureus.18618 -
JAAD Case Reports Dec 2021
PubMed: 34778497
DOI: 10.1016/j.jdcr.2021.10.027 -
Rheumatology (Oxford, England) Mar 2022
Topics: Acro-Osteolysis; Adult; Antibodies, Antinuclear; Female; Humans; Scleromyxedema; Sjogren's Syndrome
PubMed: 34687298
DOI: 10.1093/rheumatology/keab792