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JAAD Case Reports Aug 2022
PubMed: 35899146
DOI: 10.1016/j.jdcr.2022.06.022 -
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 -
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 -
Indian Journal of Dermatology,... 2021Stem cells are precursor cells present in many tissues with ability to differentiate into various types of cells. This interesting property of plasticity can have... (Review)
Review
Stem cells are precursor cells present in many tissues with ability to differentiate into various types of cells. This interesting property of plasticity can have therapeutic implications and there has been substantial research in this field in last few decades. As a result, stem cell therapy is now used as a therapeutic modality in many conditions, and has made its way in dermatology too. Stem cells can be classified on the basis of their source and differentiating capacity. In skin, they are present in the inter-follicular epidermis, hair follicle, dermis and adipose tissue, which help in maintaining normal skin homeostasis and repair and regeneration during injury. In view of their unique properties, they have been employed in treatment of several dermatoses including systemic sclerosis, systemic lupus erythematosus, scleromyxedema, alopecia, Merkel cell carcinoma, pemphigus vulgaris, psoriasis, wound healing, epidermolysis bullosa and even aesthetic medicine, with variable success. The advent of stem cell therapy has undoubtedly brought us closer to curative treatment of disorders previously considered untreatable. Nevertheless, there are multiple lacunae which need to be addressed including ideal patient selection, timing of intervention, appropriate conditioning regimens, post-intervention care and cost effectiveness. Further research in these aspects would help optimize the results of stem cell therapy.
Topics: Dermatology; Humans; Skin Diseases; Stem Cell Transplantation
PubMed: 34245532
DOI: 10.25259/IJDVL_19_20 -
JAAD Case Reports May 2021
PubMed: 33912636
DOI: 10.1016/j.jdcr.2021.03.023 -
Clinical Laboratory Mar 2021Scleromyxedema (SME) is a rare mucinosis associated with monoclonal gammopathy. Several biochemical peculiarities of monoclonal immunoglobulins (Ig) in SME patients were...
BACKGROUND
Scleromyxedema (SME) is a rare mucinosis associated with monoclonal gammopathy. Several biochemical peculiarities of monoclonal immunoglobulins (Ig) in SME patients were reported in case reports or short series, such as IgGλ over-representation, cationic migration, and partial deletion.
METHODS
Monoclonal immunoglobulins (Ig) from the serum of 12 consecutive patients diagnosed with scleromyxedema (SME) were analyzed using electrophoretic and immunoblotting techniques.
RESULTS
All monoclonal Ig from 12 SME were of IgG1 subclass, with an overrepresentation of the lambda-type light chain and a cationic mobility on standard zone electrophoresis, as compared with 21 cases of monoclonal gammopathy of undetermined significance (MGUS) of IgG1 subclass. Reactivity with specific monoclonal antibodies demonstrated no evident deletion of the heavy chain constant domains, which was also confirmed by analysis of Ig heavy chain molecular weight on a purified monoclonal component from one case.
CONCLUSIONS
Significant isotype restriction of both heavy and light chains, and peculiar biochemical properties suggest that monoclonal Ig might be involved in pathophysiological events of SME.
Topics: Antibodies, Monoclonal; Humans; Immunoglobulin G; Immunoglobulin lambda-Chains; Paraproteinemias; Scleromyxedema
PubMed: 33739037
DOI: 10.7754/Clin.Lab.2020.200605 -
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 -
Clinical Case Reports Dec 2020Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an...
Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an effective therapy for scleromyxedema, especially in patients with refractory or relapsed disease.
PubMed: 33363876
DOI: 10.1002/ccr3.3302