Scleromyxedema

Last update 08 May 2025

Basic Info

Synonyms

Arndt-Gottron disease, Arndt-Gottron syndrome, Generalised lichenoid papular eruption

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Introduction

A connective tissue disorder characterized by widespread thickening of SKIN with a cobblestone-like appearance. It is caused by proliferation of FIBROBLASTS and deposition of MUCIN in the DERMIS in the absence of thyroid disease. Most scleromyxedema cases are associated with a MONOCLONAL GAMMOPATHY, immunoglobulin IgG-lambda.

Clinical Trials associated with Scleromyxedema

NCT04656704

/ WithdrawnEarly Phase 1IIT

Investigating Hyaluronidase in Treating Oral Microstomia in Patients With Sclerosing Skin Disease

The principal objective is to measure the degree to which oral microstomia caused by sclerosing skin disease improves after treating patients with local hyaluronidase injections. Investigator will determine improvements in oral aperture by measuring the centimeters of the height of oral opening.
The secondary objectives are: Investigator will aim to assess changes in quality of life and functionality, by serial calculations of the Mouth Handicap in Systemic Sclerosis (MHISS). In addition, investigator would like to investigate how many treatments are required prior to treatment efficacy plateauing. Since there is minimal data on the use of hyaluronidase for oral microstomia, it is not yet clear how many treatments are ideally required for maximal effect.
Patients will be brought in monthly for photographs, examination, assessment, and treatment.
Our hypothesis is that hyaluronidase injections will significantly improve patients' ability to open their mouths and oral functionality. It remains unclear how many treatments will be required for maximal effect.

Start Date01 Jan 2020

Sponsor / Collaborator

The Brigham & Women's Hospital, Inc.

100 Clinical Results associated with Scleromyxedema

100 Translational Medicine associated with Scleromyxedema

0 Patents (Medical) associated with Scleromyxedema

939

Literatures (Medical) associated with Scleromyxedema

01 May 2025·Journal of the European Academy of Dermatology and Venereology

Plasma cell‐directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema

Article

Author: Royer, B. ; Arnulf, B. ; Theves, F. ; Parquet, N. ; Lahuna, C. ; Lemiale, V. ; Mahévas, T. ; Bouaziz, J.‐D. ; Elessa, D. ; Jachiet, M. ; Brignier, A. ; Talbot, A. ; Harel, S.

AbstractBackgroundScleromyxedema (SM) is a rare skin disorder related to monoclonal gammopathy. High dose intravenous immunoglobulins (HDIVIg) are usually used as a frontline therapy with initial efficacy. However, some patients evolve with relapse, refractory state or severe extra‐cutaneous complications such as dermato‐neuro syndrome (DNS) or cardiac involvement.The objective of the study is to evaluate the use of anti‐plasma cell treatment in these patients in order to obtain a deep and durable dermatological and haematological response.MethodsWe report here eight patients treated with HDIVIg together with anti‐plasma cell therapy including: lenalidomide and dexamethasone (n = 5); bortezomib, cyclophosphamide and dexamethasone (n = 1); daratumumab, lenalidomide and dexamethasone (n = 2).ResultsCombination of HDIVIg with a treatment targeting the monoclonal component led to a high level of haematological remission and drastically improved skin response with an acceptable safety profile in all patients. Moreover, HDIVIg was reduced and stopped in 4 of the 7 patients who achieved complete remission.ConclusionsThe association of lenalidomide and dexamethasone with HDIVIg could improve the treatment of relapsed or severe SM.

01 Mar 2025·Australasian Journal of Dermatology

Successful Treatment of a Case of Scleromyxedema With Intravenous Immunoglobulin and Thalidomide and a Two‐and‐a‐Half‐Year Follow‐Up

Article

Author: Sangwan, Priyanka ; Dash, Siddhartha ; Ayyanar, Pavithra ; Sahu, Deepak Kumar ; Behera, Biswanath

01 Mar 2025·BMJ Case Reports

Influenza-associated encephalopathy mimicking dermatoneuro syndrome

Article

Author: Schipani, Elke ; Rode, Matthew ; Rigby, Michael J ; Fugate, Jennifer

Dermatoneuro syndrome (DNS) is a rare diagnosis of exclusion characterised by fever, altered mental status and seizures in patients with scleromyxoedema. We present a complex case of a patient presenting with a fever, coma, seizures, rigidity and deep T2 fluid-attenuated inversion recovery hyperintensities in the context of both scleromyxoedema and influenza A with extensive, negative infectious workup. This case highlights the shared clinical features of DNS and influenza-associated encephalopathy, the latter being the eventual diagnosis for our patient. We review the DNS literature focusing on presentation, adequacy of investigation, treatment and prognosis. Finally, we provide an initial workup for patients with suspected DNS, including nasopharyngeal influenza PCR.

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Scleromyxedema

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