Quiz

Dermatology

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

JAMA Dermatology

April 1, 2019

JAMA Dermatology Clinicopathological Challenge

Article Information and Disclosures

Nathaniel Smith, MD¹;

Earl Glusac, MD¹;

Jonathan Leventhal, MD¹

Author Affiliations

¹Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut

Read article on JAMA Network

Read Case Take Quiz Answer

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).

Take Quiz

Please finish quiz first before checking answer.

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.

Buy This Activity

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

Obi C, Holler P, Pugliese D, et al. Leukemia labialis: a rare presentation of leukemia cutis limited to the lips. J Am Acad Dermatol. 2012;67(4):e146-e147. doi:10.1016/j.jaad.2011.12.003 PubMed Google Scholar Crossref

Kakagia D, Tamiolakis D, Lambropoulou M, Kakagia A, Grekou A, Papadopoulos N. Systemic B-cell chronic lymphocytic leukemia first presenting as a cutaneous infiltrate arising at the site of a herpes simplex scar. Minerva Stomatol. 2005;54(3):161-163.PubMed Google Scholar

Cerroni L, Zenahlik P, Höfler G, Kaddu S, Smolle J, Kerl H. Specific cutaneous infiltrates of B-cell chronic lymphocytic leukemia: a clinicopathologic and prognostic study of 42 patients. Am J Surg Pathol. 1996;20(8):1000-1010. doi:10.1097/00000478-199608000-00009 PubMed Google Scholar Crossref

Robak E, Robak T. Skin lesions in chronic lymphocytic leukemia. Leuk Lymphoma. 2007;48(5):855-865. doi:10.1080/10428190601137336 PubMed Google Scholar Crossref

Plaza JA, Comfere NI, Gibson LE, et al. Unusual cutaneous manifestations of B-cell chronic lymphocytic leukemia. J Am Acad Dermatol. 2009;60(5):772-780. doi:10.1016/j.jaad.2008.12.047 PubMed Google Scholar Crossref

Menter T, Dirnhofer S, Tzankov A. LEF1: a highly specific marker for the diagnosis of chronic lymphocytic B cell leukaemia/small lymphocytic B cell lymphoma. J Clin Pathol. 2015;68(6):473-478. doi:10.1136/jclinpath-2015-202862 PubMed Google Scholar Crossref

Colburn DE, Welch M-A, Giles FJ. Skin infiltration with chronic lymphocytic leukemia is consistent with a good prognosis. Hematology. 2002;7(3):187-188. doi:10.1080/1024533021000008164 PubMed Google Scholar Crossref

Critchlow WA, Chang D. Cheilitis granulomatosa: a review. Head Neck Pathol. 2014;8(2):209-213. doi:10.1007/s12105-013-0488-2 PubMed Google Scholar Crossref

Femiano F, Lanza A, Buonaiuto C, Perillo L, Dell’Ermo A, Cirillo N. Pyostomatitis vegetans: a review of the literature. Med Oral Patol Oral Cir Bucal. 2009;14(3):E114-E117.PubMed Google Scholar

10.

Clark LG, Tolkachjov SN, Bridges AG, Camilleri MJ. Pyostomatitis vegetans (PSV)-pyodermatitis vegetans (PDV): a clinicopathologic study of 7 cases at a tertiary referral center. J Am Acad Dermatol. 2016;75(3):578-584. doi:10.1016/j.jaad.2016.03.047 PubMed Google Scholar Crossref

Multiple Lip Papules and Nodules in an Adult Man

You answered correctly!

You answered incorrectly.

Multiple Lip Papules and Nodules in an Adult Man

You answered correctly!

You answered incorrectly.

See More About

If you are not a JN Learning subscriber, you can either:

A subscription to JN Learning (CME from JAMA Network) provides:

Sign in to access

If you are not a JN Learning subscriber, you can either:

Sign in to customize your interests

With a personal account, you can:

Name Your Search

Sign in to save your search

With a personal account, you can:

Lookup An Activity

My Saved Searches

Sign in to customize your interests

With a personal account, you can:

Purchase access