613 results match your criteria Lichen Myxedematosus


Clinical and histopathological improvement of scleromyxedema-induced microstomia after hyaluronidase injection.

J Cosmet Dermatol 2022 Apr 20. Epub 2022 Apr 20.

Dermatology Department, Faculty of Medicine, Bezmialem Vakif University, Istanbul, Turkey.

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. Read More

View Article and Full-Text PDF

Cutaneous manifestations of monoclonal gammopathy.

Blood Cancer J 2022 04 11;12(4):58. Epub 2022 Apr 11.

Division of Hematology, Mayo Clinic Rochester, Mayo Clinic, MN, USA.

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. Read More

View Article and Full-Text PDF

Unusual type of scleromyxedema with multiple subcutaneous nodules, IgM-λ paraproteinemia, and scleroderma-like microangiopathy.

J Scleroderma Relat Disord 2021 Jun 1;6(2):220-224. Epub 2020 Dec 1.

Department of Dermatology, University of Tokyo Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan.

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. Read More

View Article and Full-Text PDF

A Case of Scleromyxedema Development Following Intravesical Bacillus Calmette-Guérin Administration.

J Rheumatol 2022 Feb 15. Epub 2022 Feb 15.

Division of Rheumatology, Department of Internal Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey. The authors declare no conflicts of interest relevant to this article. Address correspondence to Dr. R. Yıldırım, Department of Rheumatology, School of Medicine, Eskişehir Osmangazi University Eskişehir, 26480, Turkey. Email:

We read with great interest the case report written by Boleto et al entitled, "Rapidly Progressive Systemic Sclerosis-associated Interstitial Lung Disease After Intravesical Bacillus Calmette-Guérin Therapy for Early-stage Bladder Cancer". In this article, the authors speculated on a possible association between intravesical bacillus Calmette-Guérin (BCG) administration and progression in systemic sclerosis (SSc)-associated interstitial lung disease. Read More

View Article and Full-Text PDF
February 2022

New and emerging conditions of acquired cutaneous mucinoses in adults.

Authors:
F Rongioletti

J Eur Acad Dermatol Venereol 2022 Jul 17;36(7):1016-1024. Epub 2022 Feb 17.

Dermatology Clinic, IRCCS San Raffaele Scientific Institute, Milan, Italy.

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. Read More

View Article and Full-Text PDF

Solitary Cutaneous Focal Mucinosis.

Cureus 2021 Oct 9;13(10):e18618. Epub 2021 Oct 9.

Dermatology, University of California, Davis Medical Center, Sacramento, USA.

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. Read More

View Article and Full-Text PDF
October 2021

Scleromyxedema.

Rheumatology (Oxford) 2021 Sep 23. Epub 2021 Sep 23.

Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Changhua Christian Hospital. Changhua County, Taiwan.

View Article and Full-Text PDF
September 2021

Widespread, waxy, coalescing cutaneous papules.

Clin Exp Dermatol 2021 Dec 7;46(8):1626-1628. Epub 2021 Sep 7.

Dermatology, Chelsfield Park Hospital, Kent, UK.

View Article and Full-Text PDF
December 2021

Scleromyxedema Without Monoclonal Gammopathy Treated with Intravenous Immunoglobulins.

Acta Dermatovenerol Croat 2021 Apr;291(1):51-53

Mathilde Letzelter, MD, 4 Rue Larrey, 49100 Angers,

Scleromyxedema is a generalized cutaneous mucinosis that may cause internal damage. This condition is frequently associated with monoclonal gammopathy. However, its physiopathological implications remains uncertain. Read More

View Article and Full-Text PDF

Lichen myxedematosus associated with pulmonary mucosa-associated lymphoid tissue lymphoma.

J Dermatol 2022 Feb 20;49(2):e46-e47. Epub 2021 Jul 20.

Department of Dermatology, Ehime University Graduate School of Medicine, Toon, Japan.

View Article and Full-Text PDF
February 2022

Acral persistent papular mucinosis.

Clin Dermatol 2021 Mar-Apr;39(2):211-214. Epub 2020 Oct 16.

Dermatology Clinic, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy. Electronic address:

Acral persistent papular mucinosis (APPM) is a subtype of localized lichen myxedematosus (LM) characterized by the chronic development of white to skin-colored papules and limited to the extensor surfaces of the hands and distal part of forearms, in the absence of systemic or laboratory manifestations. There is a strong predominance in women. Histopathology shows focal accumulation of mucin in the upper portion of the dermis sparing the grenz zone. Read More

View Article and Full-Text PDF
November 2021

Stem cell therapy in dermatology.

Indian J Dermatol Venereol Leprol 2021 Nov-Dec;87(6):753-767

Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.

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. Read More

View Article and Full-Text PDF
February 2022

First report of histopathological and ultrasonographic correlation of localized lichen myxoedematosus.

Clin Exp Dermatol 2021 Dec 18;46(8):1614-1615. Epub 2021 Aug 18.

Department of Dermatology, Chang Gung Memorial Hospital, Taoyuan, Taiwan.

View Article and Full-Text PDF
December 2021

Acral persistent papular mucinosis: A case report and summary of 24 Japanese cases.

J Dermatol 2021 Oct 25;48(10):1574-1578. Epub 2021 Jun 25.

Department of Dermatology, Nara Medical University School of Medicine, Kashihara, Japan.

Acral persistent papular mucinosis is a rare subtype of localized lichen myxedematosus. We report a case of a 41-year-old woman with acral persistent papular mucinosis. She had a 5-year history of multiple, scattered, whitish papules, on the back of both hands, of 2-5 mm in diameter. Read More

View Article and Full-Text PDF
October 2021

Versican deposits in the sclerotic skin of a patient with localized lichen myxedematosus accompanied by joint involvement.

J Dermatol 2021 Aug 2;48(8):1299-1301. Epub 2021 Jun 2.

Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

View Article and Full-Text PDF

Coexistence of scleromyxedema and Sneddon syndrome.

JAAD Case Rep 2021 May 20;11:47-50. Epub 2021 Mar 20.

Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy.

View Article and Full-Text PDF

Lichen Myxedematosus Presenting as a Solitary Nodule.

Ann Dermatol 2020 Apr 11;32(2):168-169. Epub 2020 Mar 11.

Department of Dermatology, Chungbuk National University College of Medicine, Cheongju, Korea.

View Article and Full-Text PDF

IgG1 Subclass Restriction and Biochemical Peculiarities of Monoclonal Immunoglobulins in Scleromyxedema.

Clin Lab 2021 Mar;67(3)

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. Read More

View Article and Full-Text PDF

Acral lichen myxedematosus showing acro-osteolysis in a patient with Sjögren syndrome.

J Dermatol 2021 Apr 9;48(4):e173-e174. Epub 2021 Feb 9.

Department of Dermatology, Fukushima Medical University School of Medicine, Fukushima, Japan.

View Article and Full-Text PDF

Successful treatment of dermato-neuro syndrome with plasmapheresis.

BMJ Case Rep 2020 Dec 29;13(12). Epub 2020 Dec 29.

Hematology and Oncology, Providence Park Hospital, Novi, Michigan, USA.

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. Read More

View Article and Full-Text PDF
December 2020

Scleromyxedema.

J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467

Department of Dermatology, University of Heidelberg, Germany.

Scleromyxedema is a rare, cutaneous deposition disorder from the group of mucinoses, which can affect multiple organs and is virtually always associated with a monoclonal gammopathy. Cutaneous manifestations are usually generalized, 2 to 3 mm sized, dome-shaped or flat-topped, waxy, slightly red to skin-colored papules and sclerodermoid indurations. Neurological, rheumatological, cardiovascular, gastrointestinal, respiratory tract, renal and ophthalmologic manifestations can occur, with decreasing frequency. Read More

View Article and Full-Text PDF
December 2020

Treatment of scleromyxedema with lenalidomide, bortezomib and dexamethasone: A case report and review of the literature.

Clin Case Rep 2020 Dec 17;8(12):3043-3049. Epub 2020 Sep 17.

Department of Hematology and Oncology University of Arizona Tucson Arizona.

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. Read More

View Article and Full-Text PDF
December 2020

Scleromyxedema in a 21 year old female patient with acute lymphoblastic leukemia: a case report.

BMC Dermatol 2020 12 4;20(1):18. Epub 2020 Dec 4.

Department of Critical Care , An-Najah National University Hospital , Nablus, Palestine.

Background: Scleromyxedema is a rare, para-neoplastic, chronic, progressive condition of the Lichen myxedematosus (LM) family. The clinical picture consists of generalized confluent papular eruptions with possible systemic manifestations, which may be fatal as it still constitutes a therapeutic dilemma. Histologically, it is characterized by dermal mucin deposition, fibroblast proliferation with fibrosis, with monoclonal gammopathy in the absence of thyroid disease. Read More

View Article and Full-Text PDF
December 2020

An atypical presentation of discrete papular mucinosis in the genitalia mimicking a molluscum contagiosum infection.

Dermatol Online J 2020 Sep 15;26(9). Epub 2020 Sep 15.

Dermatology Department. Araba University Hospital. Vitoria-Gasteiz.

Discrete papular mucinosis is a rare variant of primary cutaneous mucinosis. Involvement of genitalia is extremely rare and can mimic molluscum contagiosum. We report the second case of a papular mucinosis with an exclusive genital involvement. Read More

View Article and Full-Text PDF
September 2020

Mycophenolate mofetil treatment of an H syndrome patient with a SLC29A3 mutation.

Dermatol Ther 2020 11 26;33(6):e14375. Epub 2020 Oct 26.

Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

H syndrome is a complex multi-organ disorder with autosomal recessive inheritance. The skin manifestations include early onset hyperpigmentation and hypertrichosis, followed by skin induration often diagnosed as scleromyxedema and morphea. There is no effective treatment. Read More

View Article and Full-Text PDF
November 2020

A Rare Case of Tumoral Scleromyxedema.

Indian J Dermatol 2020 Jul-Aug;65(4):310-312

Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Scleromyxedema is an uncommon disease, affecting the skin mainly and other internal organs sometimes, characterized by fibroblasts proliferation, fibrosis, and mucous deposition in the absence of thyroid disorder. It is associated with monoclonal gammopathy in most cases. We are reporting a case with a rare presentation of tumoral scleromyxedema in the neck, with a mass mimicking other tumoral lesions, highlighting the importance of diagnosis and histopathologic correlation. Read More

View Article and Full-Text PDF

Long-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema.

Acta Dermatovenerol Croat 2020 Jul;28(1):24-28

Katarina Trčko, MD, PhD, Department of Dermatology and Venereal Diseases, University Medical Centre Maribor, Ljubljanska ulica 5, 2000 Maribo,r Slovenia;

Scleromyxedema or generalized lichen myxedematosus is a rare depositional disorder. Diagnostic criteria encompass a generalized papular and sclerodermoid eruption, monoclonal gammopathy (paraproteinemia), most often with G-lambda type immunoglobulin, a characteristic microscopic triad (mucin deposition, fibroblast proliferation, fibrosis), and absence of thyroid disease. Many internal manifestations of scleromyxedema have been described to date, leading to high mortality and morbidity. Read More

View Article and Full-Text PDF

Characterization of circulating myeloma tumor cells by next generation flowcytometry in scleromyxedema patient: a case report.

Medicine (Baltimore) 2020 Jul;99(27):e20726

Hamad Medical Corporation, Hematology and Medical Oncology Department.

Introduction: Scleromyxedema (rare cutaneous mucinosis), is characterized by the formation of lichenoid papules and presence of Serum monoclonal IgG in most cases, or all; after repeated testing.

Patient Concerns: The patient is a 51-year-old male presented with thick, disfiguring elephant-like erythematous skin folds over the forehead, papular shiny eruptions over ears and trunk and waxy erythematous papules over arms and hands without dysphagia or respiratory or neurologic symptoms DIAGNOSIS: : Skin biopsy from right arm was consistent with scleromyxedema. Serum cryoglobulin was reported negative. Read More

View Article and Full-Text PDF