Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report
Hereditary progressive mucinous histiocytosis (HPMH) is an extremely rare progressive non-Langerhans cell histiocytic disorder presenting with only cutaneous manifestations. Patients typically present with multiple asymptomatic dome-shaped erythematous papules, usually involving the face and upper e...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2023-12-01
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| Series: | Journal of Dermatological Treatment |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/09546634.2022.2117538 |
| _version_ | 1827816508835758080 |
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| author | Reem Diab Mohammad Shahidi Dadras Azadeh Rakhshan Ali Kaddah Fahimeh Abdollahimajd |
| author_facet | Reem Diab Mohammad Shahidi Dadras Azadeh Rakhshan Ali Kaddah Fahimeh Abdollahimajd |
| author_sort | Reem Diab |
| collection | DOAJ |
| description | Hereditary progressive mucinous histiocytosis (HPMH) is an extremely rare progressive non-Langerhans cell histiocytic disorder presenting with only cutaneous manifestations. Patients typically present with multiple asymptomatic dome-shaped erythematous papules, usually involving the face and upper extremities. Twenty-six cases have been reported worldwide, with no spontaneous regression. Treatment with thalidomide stopped the progression of the disease in two cases. We report a case of progressive mucinous histiocytosis in a 31-year-old female patient with a history of tuberculosis who presented papular lesions on the face that later extended to the hands. She was treated with isoniazid for tuberculosis and isotretinoin for the skin lesions; the improvement was minimal during the next two months, with new lesions appearing on both hands. Thalidomide stopped the progression of the disease. The cause and pathogenesis of HPMH are undetermined. The pathogenesis of HPMH may be similar to that of lysosomal storage disease, considering the intra-cytoplasmic phospholipid deposition in both diseases, in addition to the likelihood of a role of macrophages in triggering the disease. In our patient, tuberculosis may have contributed. |
| first_indexed | 2024-03-12T00:15:19Z |
| format | Article |
| id | doaj.art-cc88fb93028e49858cb6c475884518f9 |
| institution | Directory Open Access Journal |
| issn | 0954-6634 1471-1753 |
| language | English |
| last_indexed | 2024-03-12T00:15:19Z |
| publishDate | 2023-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Journal of Dermatological Treatment |
| spelling | doaj.art-cc88fb93028e49858cb6c475884518f92023-09-15T14:28:52ZengTaylor & Francis GroupJournal of Dermatological Treatment0954-66341471-17532023-12-0134110.1080/09546634.2022.21175382117538Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case reportReem Diab0Mohammad Shahidi Dadras1Azadeh Rakhshan2Ali Kaddah3Fahimeh Abdollahimajd4Department of Dermatology, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical SciencesDepartment of Dermatology, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical SciencesDepartment of Pathology, Shohada-e Tajrish Hospital, School of Medicine, Shahid Beheshti University of Medical SciencesDepartment of Surgery, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical SciencesDepartment of Dermatology, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical SciencesHereditary progressive mucinous histiocytosis (HPMH) is an extremely rare progressive non-Langerhans cell histiocytic disorder presenting with only cutaneous manifestations. Patients typically present with multiple asymptomatic dome-shaped erythematous papules, usually involving the face and upper extremities. Twenty-six cases have been reported worldwide, with no spontaneous regression. Treatment with thalidomide stopped the progression of the disease in two cases. We report a case of progressive mucinous histiocytosis in a 31-year-old female patient with a history of tuberculosis who presented papular lesions on the face that later extended to the hands. She was treated with isoniazid for tuberculosis and isotretinoin for the skin lesions; the improvement was minimal during the next two months, with new lesions appearing on both hands. Thalidomide stopped the progression of the disease. The cause and pathogenesis of HPMH are undetermined. The pathogenesis of HPMH may be similar to that of lysosomal storage disease, considering the intra-cytoplasmic phospholipid deposition in both diseases, in addition to the likelihood of a role of macrophages in triggering the disease. In our patient, tuberculosis may have contributed.http://dx.doi.org/10.1080/09546634.2022.2117538hereditary progressive mucinous histiocytosis (hpmh)non-langerhans cell histiocytosis (non-lch)tuberculosis (tb) |
| spellingShingle | Reem Diab Mohammad Shahidi Dadras Azadeh Rakhshan Ali Kaddah Fahimeh Abdollahimajd Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report Journal of Dermatological Treatment hereditary progressive mucinous histiocytosis (hpmh) non-langerhans cell histiocytosis (non-lch) tuberculosis (tb) |
| title | Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report |
| title_full | Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report |
| title_fullStr | Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report |
| title_full_unstemmed | Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report |
| title_short | Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report |
| title_sort | progressive mucinous histiocytosis treated successfully with thalidomide a rare case report |
| topic | hereditary progressive mucinous histiocytosis (hpmh) non-langerhans cell histiocytosis (non-lch) tuberculosis (tb) |
| url | http://dx.doi.org/10.1080/09546634.2022.2117538 |
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