Hypopigmented Epitheliotropic T-Cell Dyscrasia as a Form of Prelymphomatous T-Cell Dyscrasia
Xuan Wang, Yen Chen Liu, Cynthia M Magro. Weill Medical College of Cornell University, New York, NY
Background: Hypopigmented lesions are a common presenting complaint in dermatology clinics and arise in various settings including hypopigmented variants of mycosis fungoides (MF). While the presence of atypical lymphocytes in these infiltrates raises the diagnostic possibility of hypopigmented MF, there are subsets of patients who fail to meet criteria for fully evolved MF.
Design: Twenty three cases of hypopigmented T-cell dyscrasia were identified in the data base of Weill Medical College of Cornell University. In each case, routine light microscopic analysis and phenotypic and molecular studies were conducted on paraffin embedded formal fixed tissue. The comprehensive phenotypic panel included antibodies to beta F1, CD2, CD3, CD4, CD5, CD7, CD8, and CD62L.
Results: Of the patients studied 16 were females and 7 were male with a mean age of 36 years of age. In children and adolescents, those of African American descendent were seen most commonly. All patients presented with hypopigmented lesions of variable size involving truncal photoprotected areas except in 3 cases where facial involvement with hair loss occurred. While the disease was persistent at times over years, in only one case was there disease progression to MF; the lesions responded to topical steroids and light therapy. From a morphologic perspective the cases exhibited reproducible light microscopic phenotypic features including the presence of a true vacuous cell poor interface associated with degenerative changes of keratinocytes and melanocytes, low grade cerebriform atypia, and paucity of other inflammatory cell elements. Phenotypically the cases showed a significant reduction in the expression of CD7 and CD62L corroborative of their categorization as a form of T cell dyscrasia. In 50% of the cases the implicated cell type was of the CD8 subset. Polyclonality was seen in almost all cases studied. Clonality was not identified.
Conclusions: We propose that this entity represents a form of indolent cutaneous lymphoid dyscrasia falling under the designation of the hypopigmented interface variant of cutaneous lymphoid dyscrasia, a term originally proposed by Guitart and Magro. It responds well to UVB therapy, like other forms of cutaneous lymphoid dyscrasia and rarely progresses to overt MF.
Wednesday, March 6, 2013 9:30 AM
Poster Session V # 73, Wednesday Morning