The patient is a 38-year-old woman who has history of adrenal insufficiency and hypothyroidism. She initially developed scaly raised pink papules on her upper arms. Those lesions have progressed to her back, chest, and abdomen. The more recent physical examination revealed hypopigmented macules and scaly papules on her back (clinical figure), hypopigmented macules on her upper arm, and scaly papules on her anterior trunk and upper arms. A punch skin biopsy of one of the lesions on the right shoulder was performed.
Pathologic findings. Histologic sections revealed a single hair follicle infiltrated by small-sized lymphocytes associated with marked mucin deposition (follicular mucinosis). Occasional eosinophils were seen. Superficial epidermotropism was minimal. Although the lymphoid cells showed no obvious morphologic atypia, the vast majority of these lymphocytes were CD4+ T-cells with significant increase of CD4:CD8 ratio in the intra- and perifollicular infiltrate. An additional skin biopsy (not shown) from the left upper shoulder showed an atypical folliculotropic and perifollicular lymphoid infiltrate associated with mild follicular mucinosis. In this skin biopsy specimen, the lymphoid infiltrate was also positive for CD3 and CD4 with an increase of CD4:CD8 ratio. No significant loss of CD7 expression was identified.
Molecular findings. No monoclonal TCR- gene rearrangements were identified by PCR; however, low levels of a monoclonal TCR-beta gene rearrangement were detected.
Staging, treatment, and follow-up. The patient was diagnosed with folliculotropic MF, stage 1A. Flow cytometry immunophenotypic studies of a peripheral blood specimen failed to detect abnormal T-cells. The patient was treated with topical isotretinoin on her face and triamcinolone on her body, resulting in minimal improvement. She also received topical tazarotene and triamcinolone, which resolved some of lesions; but new pink papules appeared on the arms and trunk. In the last follow-up, the total body surface area involvement was 1.5% plaques, which improved from 2.5% plaques at the initial visit.