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A man in his 50s presented with a 12-year history of itchy eruptions over the whole body. Multiple nodules appeared on the upper back 1 year previously and gradually increased in number and size. The patient was otherwise healthy and denied a family history of inherited ichthyosis or atopy. Physical examination revealed extensive, dry scaly patches on the trunk, arms, and legs. Several 0.5- to 1-cm, dome-shaped, red papules and nodules were found on the upper back (Figure, A and B). Mildly enlarged lymph nodes were observed on the cervical, axillary, and inguinal regions. Results of routine blood tests, biochemistry analyses, and peripheral blood flow cytometry tests were within normal limits. No atypical cells were found in the peripheral blood. A biopsy specimen was obtained from the nodules on the patient’s back (Figure, C and D).
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B. Ichthyosiform mycosis fungoides
Histopathologic examination revealed an atrophy of the epidermis with focal orthokeratosis and an extensive, dense infiltrate of lymphocytes in the entire dermis. Small to medium-sized, atypical lymphocytes with focal areas of epidermotropism could be observed (Figure, C). Immunohistochemical staining results were consistent with a T-helper phenotype of the infiltrating lymphocytes, which were positive for CD2, CD3, CD4 (Figure, D), and CD45RO. A few small reactive CD8+ T cells and CD7+ T cells were observed in the dermis. DNA was extracted from paraffin sections, and polymerase chain reaction detected a clonal amplification product for the γ-chain of the T-cell receptor.
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Corresponding Author: Zhimiao Lin, MD, Department of Dermatology, Peking University First Hospital, 8 Xishiku St, Beijing 100034, China (firstname.lastname@example.org).
Published Online: August 14, 2019. doi:10.1001/jamadermatol.2019.1987
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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