Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report

Autor: Reem Diab, Mohammad Shahidi Dadras, Azadeh Rakhshan, Ali Kaddah, Fahimeh Abdollahimajd
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Journal of Dermatological Treatment, Vol 34, Iss 1 (2023)
Druh dokumentu: article
ISSN: 0954-6634
1471-1753
09546634
DOI: 10.1080/09546634.2022.2117538
Popis: 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.
Databáze: Directory of Open Access Journals
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