Abstract:

We report the case of a 37-year-old Caucasian male presenting with a 5-year history of progressive and mildly pruritic scaly erythematous plaques with epidermal atrophy and alopecia on the trunk and extremities. A biopsy from one of the plaques revealed changes consistent with cutaneous T-cell lymphoma. Interestingly, multiple soft papules overlying these plaques were also noted, raising the clinical differential of folliculotropic mycosis fungoides (MF), scleromyxedema and intradermal nevi. Subsequent biopsy of a polypoid lesion revealed an epidermal collarette with epidermotropism, folliculotropism, syringotropism and a dense underlying atypical lymphocytic infiltrate composed of CD3 (+), CD5 (+) T-cells with CD4 predominating over CD8 and loss of CD7 most consistent with mycosis fungoides. Given the clinical presentation and unusual architecture of the specimen, an elastic stain was performed revealing near-complete absence of elastic fibers surrounding the dense lymphocytic infiltrate raising the possibility of MF with secondary anetoderma. Although secondary anetoderma has been described in primary cutaneous B-cell lymphomas and generalized follicular mucinosis, anetoderma arising in classic MF is very rare, having only been described in two patients thus far. The exact mechanism underlying the destruction of elastic fibers, in addition to the prognostic significance of these lesions, remains unclear. Though it has been added to the list of clinicopathological variants of MF, it is likely underrecognized. We report this case in an effort to increase the awareness of this rare variant of MF in the dermatopathology community, and to further emphasize that mycosis fungoides can demonstrate multiple overlapping histologic patterns.

Financial Disclosure:
No current or relevant financial relationships exist.

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Neha Nagrani, MD