Mycosis fungoides with follicular mucinosis displaying aggressive tumor-stage transformation: Successful treatment using radiation therapy plus oral bexarotene combination therapy

Narin Apisarnthanarax, Chul S Ha, Madeleine Duvic

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Follicular mucinosis is a tissue reaction pattern characterized by mucin deposition with follicular sebaceous units and is found as an idiopathic, primary, benign process (alopecia mucinosa), or as a secondary process due to inflammatory and neoplastic disorders (mycosis fungoides). When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis. We present a case of mycosis fungoides with follicular mucinosis that was treated with incomplete courses of interferon, isotretinoin, and polychemotherapy with subsequent rapid progression to tumor-stage mycosis fungoides with large cell transformation and nodal and bone marrow involvement. In this setting, the patient was treated with local radiation therapy, total-skin electron beam therapy, and therapy and maintenance with the oral retinoid-X-receptor retinoid bexarotene, and achieved a durable complete remission.

Original languageEnglish (US)
Pages (from-to)429-433
Number of pages5
JournalAmerican Journal of Clinical Dermatology
Volume4
Issue number6
DOIs
StatePublished - 2003
Externally publishedYes

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Follicular Mucinosis
Mycosis Fungoides
Radiotherapy
Neoplasms
Retinoid X Receptors
Isotretinoin
Retinoids
Mucins
Therapeutics
Combination Drug Therapy
Interferons
Bone Marrow
Maintenance
Electrons
Skin
bexarotene

ASJC Scopus subject areas

  • Dermatology

Cite this

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title = "Mycosis fungoides with follicular mucinosis displaying aggressive tumor-stage transformation: Successful treatment using radiation therapy plus oral bexarotene combination therapy",
abstract = "Follicular mucinosis is a tissue reaction pattern characterized by mucin deposition with follicular sebaceous units and is found as an idiopathic, primary, benign process (alopecia mucinosa), or as a secondary process due to inflammatory and neoplastic disorders (mycosis fungoides). When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis. We present a case of mycosis fungoides with follicular mucinosis that was treated with incomplete courses of interferon, isotretinoin, and polychemotherapy with subsequent rapid progression to tumor-stage mycosis fungoides with large cell transformation and nodal and bone marrow involvement. In this setting, the patient was treated with local radiation therapy, total-skin electron beam therapy, and therapy and maintenance with the oral retinoid-X-receptor retinoid bexarotene, and achieved a durable complete remission.",
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AU - Ha, Chul S

AU - Duvic, Madeleine

PY - 2003

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N2 - Follicular mucinosis is a tissue reaction pattern characterized by mucin deposition with follicular sebaceous units and is found as an idiopathic, primary, benign process (alopecia mucinosa), or as a secondary process due to inflammatory and neoplastic disorders (mycosis fungoides). When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis. We present a case of mycosis fungoides with follicular mucinosis that was treated with incomplete courses of interferon, isotretinoin, and polychemotherapy with subsequent rapid progression to tumor-stage mycosis fungoides with large cell transformation and nodal and bone marrow involvement. In this setting, the patient was treated with local radiation therapy, total-skin electron beam therapy, and therapy and maintenance with the oral retinoid-X-receptor retinoid bexarotene, and achieved a durable complete remission.

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