Mycosis Fungoides and Follicular Mucinosis

Vollmer, Robin T.

doi:10.1001/archderm.138.12.1614

Cited by 7 publications

(5 citation statements)

References 3 publications

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“…[18][19][20][21] Mucinous degeneration of the follicular epithelia has always been considered a very typical and defining feature of so-called ''MF-associated follicular mucinosis.'' [22][23][24][25][26][27] However, as previously reported, follicular mucinosis in folliculotropic MF is not a constant feature. 4,[28][29][30] In almost 50% of our cases, after sectioning, no follicular mucinosis could be identified.…”

Section: Discussionmentioning

confidence: 66%

Folliculotropic mycosis fungoides: Clinicopathological features and outcome in a series of 20 cases

Muniesa

Estrach

Pujol³

et al. 2010

Journal of the American Academy of Dermatology

102

121

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Section: Discussionmentioning

confidence: 66%

Folliculotropic mycosis fungoides: Clinicopathological features and outcome in a series of 20 cases

Muniesa

Estrach

Pujol³

et al. 2010

Journal of the American Academy of Dermatology

102

121

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“…3 There is scant information in the literature regarding its clinical manifestations. [4][5][6][7][8][9][10][11][12][13][14][15][16] Data regarding optimal therapies in FMF are similarly limited. In addition, only 2 other studies have provided any survival data for patients with FMF.…”

mentioning

confidence: 99%

Folliculotropic Mycosis Fungoides

Gerami

Rosen²,

Kuzel³

et al. 2008

Arch Dermatol

186

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To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group.Setting: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group.Patients: Forty-three patients with FMF were included in the study and compared with 43 age-and stagematched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time.Results: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (ՅIIA) had a 10-year survival of 82%, which took a steep drop off to 41% by 15 years. Patients with late-stage disease (ՆIIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage. Conclusions:The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skindirected therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.

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“…Criteria for the diagnosis ofFMF overlap considerably with follicular mucinosis (FM), a reactive process that also involves hair follicles. Whether follicular mucinosis represents a benign idiopathic condition or an early form of mycosis fungoides, however, remains controversial [48][49][50]. An essential component of this controversy is the presence of two distinct variants of FM that exhibit different clinical behavior: FM in young patients with limited head and neck involvement and an excellent prognosis (idiopathic FM) vs. FM in older patients (greater than 40 years old) with disseminated lesions and an increased risk of progression to MF [50,51].…”

Section: Cutaneous T Cell Lymphoma and Mycosis Fungoidesmentioning

confidence: 99%

Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children

et al. 2013

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Although more common with advanced age, the entire spectrum of benign and malignant cutaneous lymphoproliferative disorders can present in the pediatric population. Nevertheless, these conditions are often mistaken for other more common, benign dermatoses, resulting in delayed diagnosis. Standardized management of these conditions is also lacking. The purpose of this review is to provide an update on the most common benign and malignant cutaneous lymphoproliferative disorders in children: pityriasis lichenoides and mycosis fungoides. The presentation, evaluation and management of each of these conditions are discussed.

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Mycosis Fungoides and Follicular Mucinosis

Cited by 7 publications

References 3 publications

Folliculotropic mycosis fungoides: Clinicopathological features and outcome in a series of 20 cases

Folliculotropic mycosis fungoides: Clinicopathological features and outcome in a series of 20 cases

Folliculotropic Mycosis Fungoides

Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children

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