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Multiple Lip Papules and Nodules in an Adult Man

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his 60s presented with an approximately 4-month medical history of lip enlargement with nodular growths. Skin examination was significant for multiple erythematous and flesh-colored papules and nodules symmetrically affecting the upper and lower lips with extension onto the vermilion border (Figure 1A). There were no intraoral lesions, and all other findings of his examination were within normal limits. The patient denied fevers, chills, weight loss, night sweats, cough, lymphadenopathy, abdominal pain, change in bowel habits, or other systemic symptoms. A biopsy was obtained of the vermilion lip (Figure 1B).

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B. Leukemia labialis

Histopathologic examination demonstrated a nodular and diffuse dermal lymphoid infiltrate composed of small, mature lymphocytes with hyperchromatic and slightly convoluted nuclei (Figure 2A). Findings from CD20, CD79a, and LEF-1 immunostaining highlighted a neoplastic B-cell population occurring in sheets and clusters with aberrant coexpression of CD5 and CD23 (Figure 2B).

A subsequent complete blood cell count showed a white blood cell count of 10 600/µL with an absolute lymphocytosis of 4378 cells/µL. Flow cytometry on the peripheral blood demonstrated a monoclonal B-cell population with dim to moderate CD20 expression and aberrant expression of CD5 and CD23. The clinical, histologic, and flow cytometry findings were consistent with a diagnosis of chronic lymphocytic leukemia (CLL) presenting initially with only lip involvement.

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Article Information

Corresponding Author: Jonathan Leventhal, MD, Department of Dermatology, Yale University School of Medicine, 15 York St, LMP 5040, New Haven, CT 06510 (jonathan.leventhal@yale.edu).

Published Online: March 6, 2019. doi:10.1001/jamadermatol.2018.5240

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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