Severe cutaneous scleromyxedema treated by lenalidomide
Abstract Scleromyxedema is a rare clinical variant of primary cutaneous mucinosis. It is defined by a generalized papular and sclerodermoid eruption associated with monoclonal gammopathy. No specific definitive treatment is known and those tried have inconsistent results. The first‐line treatment is...
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2023-09-01
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| Series: | JEADV Clinical Practice |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/jvc2.159 |
| _version_ | 1797729129082126336 |
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| author | Théo Brochet Ali Dadban Quentin Beauvillain Clemence Boucher Fanny Dessirier Christophe Attencourt Catherine Lok Pierre Morel Aurélie Halle Guillaume Chaby |
| author_facet | Théo Brochet Ali Dadban Quentin Beauvillain Clemence Boucher Fanny Dessirier Christophe Attencourt Catherine Lok Pierre Morel Aurélie Halle Guillaume Chaby |
| author_sort | Théo Brochet |
| collection | DOAJ |
| description | Abstract Scleromyxedema is a rare clinical variant of primary cutaneous mucinosis. It is defined by a generalized papular and sclerodermoid eruption associated with monoclonal gammopathy. No specific definitive treatment is known and those tried have inconsistent results. The first‐line treatment is intravenous immunoglobulin infusions with variable response and frequent relapse. We describe, here, a patient with scleromyxedema with lambda monoclonal gammopathy treated effectively with low‐dose lenalidomide and dexamethasone. There was no clinical or biological relapse after 3 years of follow‐up. In our opinion, agents targeting the abnormal plasma cell clone like lenalidomide are the most promising in the treatment of scleromyxedema. |
| first_indexed | 2024-03-12T11:24:34Z |
| format | Article |
| id | doaj.art-d99ddc440b7c4775ac0026842f026d81 |
| institution | Directory Open Access Journal |
| issn | 2768-6566 |
| language | English |
| last_indexed | 2024-03-12T11:24:34Z |
| publishDate | 2023-09-01 |
| publisher | Wiley |
| record_format | Article |
| series | JEADV Clinical Practice |
| spelling | doaj.art-d99ddc440b7c4775ac0026842f026d812023-09-01T12:30:54ZengWileyJEADV Clinical Practice2768-65662023-09-012360861110.1002/jvc2.159Severe cutaneous scleromyxedema treated by lenalidomideThéo Brochet0Ali Dadban1Quentin Beauvillain2Clemence Boucher3Fanny Dessirier4Christophe Attencourt5Catherine Lok6Pierre Morel7Aurélie Halle8Guillaume Chaby9Department of Dermatology Amiens University Hospital Amiens FranceDepartment of Dermatology Amiens University Hospital Amiens FranceDepartment of Dermatology Amiens University Hospital Amiens FranceDepartment of Dermatology Amiens University Hospital Amiens FranceDepartment of Dermatology Amiens University Hospital Amiens FranceDepartment of Pathology Amiens University Hospital Amiens FranceDepartment of Dermatology Amiens University Hospital Amiens FranceDepartment of Hematology Amiens University Hospital Amiens FranceDepartment of Dermatology St Quentin Hospital St Quentin FranceDepartment of Dermatology Amiens University Hospital Amiens FranceAbstract Scleromyxedema is a rare clinical variant of primary cutaneous mucinosis. It is defined by a generalized papular and sclerodermoid eruption associated with monoclonal gammopathy. No specific definitive treatment is known and those tried have inconsistent results. The first‐line treatment is intravenous immunoglobulin infusions with variable response and frequent relapse. We describe, here, a patient with scleromyxedema with lambda monoclonal gammopathy treated effectively with low‐dose lenalidomide and dexamethasone. There was no clinical or biological relapse after 3 years of follow‐up. In our opinion, agents targeting the abnormal plasma cell clone like lenalidomide are the most promising in the treatment of scleromyxedema.https://doi.org/10.1002/jvc2.159lenalidomidemonoclonal gammopathymucinosisscleromyxedema |
| spellingShingle | Théo Brochet Ali Dadban Quentin Beauvillain Clemence Boucher Fanny Dessirier Christophe Attencourt Catherine Lok Pierre Morel Aurélie Halle Guillaume Chaby Severe cutaneous scleromyxedema treated by lenalidomide JEADV Clinical Practice lenalidomide monoclonal gammopathy mucinosis scleromyxedema |
| title | Severe cutaneous scleromyxedema treated by lenalidomide |
| title_full | Severe cutaneous scleromyxedema treated by lenalidomide |
| title_fullStr | Severe cutaneous scleromyxedema treated by lenalidomide |
| title_full_unstemmed | Severe cutaneous scleromyxedema treated by lenalidomide |
| title_short | Severe cutaneous scleromyxedema treated by lenalidomide |
| title_sort | severe cutaneous scleromyxedema treated by lenalidomide |
| topic | lenalidomide monoclonal gammopathy mucinosis scleromyxedema |
| url | https://doi.org/10.1002/jvc2.159 |
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