Folliculotropic mycosis fungoides: Clinicopathological features and outcome in a series of 20 cases
Accepted 12 March 2009. published online 18 January 2010.
Background
Folliculotropic mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma in which the neoplastic T lymphocytes display tropism for the follicular epithelium.
Objectives
To better categorize this rare form of cutaneous T-cell lymphoma we evaluated the clinical, pathological, and immunophenotypic findings, and the response to therapy and course of the disease.
Methods
Folliculotropic MF cases were selected from the registry of the Thematic Network of Cutaneous Lymphoma of Barcelona (Spain) from 1988 to 2007.
Results
Twenty patients (11 male, 9 female) with a mean age of 54 years were included. Mean follow-up time was 43 months. The most common sites of involvement were the head and neck (80%), upper extremities, and thorax. Infiltrated plaques (55%), acneiform lesions (comedo-like and epidermal cysts) (45%), and follicular keratosis-pilaris–like lesions (45%) were the more prominent features. Histopathological findings included selective infiltration of the follicular epithelium by atypical lymphocytes in all cases. Mucinous degeneration of the follicular epithelium occurred in 60% of cases. Psoralen plus ultraviolet A therapy was the treatment of choice in the majority of patients, but these patients did not respond as well as patients with classic MF. Radiotherapy (local or total skin electron beam) was found to be the most effective treatment. A good response to bexarotene was seen in some patients.
Limitation
This was a case series descriptive study.
Conclusions
Folliculotropic MF is a rare but well-defined clinicopathological variant of MF. Although refractory to standard therapies used in classic MF, most of our patients showed only slow disease progression.
aDepartment of Dermatology, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain
eDepartment of Pathology, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain
bDepartment of Dermatology at Hospital Clínic, IDIBAPS, Barcelona, Spain
cDepartment of Dermatology, Hospital del Mar, IMAS, Barcelona, Spain
dDepartment of Dermatology, Hospital de Sant Pau, Barcelona, Spain
Correspondence to: Octavio Servitje, MD, Department of Dermatology, Hospital Universitari de Bellvitge, c/Feixa Llarga s/n, ĹHospitalet de Llobregat, 08907-Barcelona, Spain.
Supported by Fondo de Investigación Sanitaria (FIS) grant PI050458.
ABSTRACT