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Hematology

Nonhealing Genital Ulcers as Clue to a Multisystem Disease

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

1 Credit CME

JAMA Dermatology

Published Online: January 11, 2023

JAMA Dermatology Clinicopathological Challenge

Article Information and Disclosures

Anubha Dev, MD, DNB¹;

Debajyoti Chatterjee, MD, DM²;

Keshavamurthy Vinay, MD, DNB¹

Author Affiliations

¹Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
²Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

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A female patient in her late 40s presented with a 3-year history of nonhealing ulcers in her groin and axillae. These lesions started as itchy, weepy, reddish papules and plaques, which gradually progressed to form ulcers and were associated with pain and pus discharge. They did not heal completely after multiple courses of topical as well as systemic antibiotics, antifungals, and steroids. She frequently experienced scaling and greasiness on her scalp, which was treated with shampoos and topical steroids. She also complained of polyuria and polydipsia for the past 4 years.

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C. Langerhans cell histiocytosis

Histopathological examination revealed a dense infiltration of lower epidermis and upper dermis by atypical Langerhans cells. These cells were large and had a characteristic coffee bean–shaped grooved vesicular nuclei and moderate amphophilic cytoplasm. They also stained positive for CD1a and Langerin on immunohistochemistry. Results of routine blood investigations were normal, along with negative results for HLA-B51 and enzyme-linked immunosorbent assay for Epstein-Barr virus IgM and IgG. Positron emission tomography showed fluorodeoxyglucose-avid involvement of bone marrow, skeletal system (in the form of lytic lesions), lungs, bilateral salivary glands, mucosae, and skin of flexural areas and scalp. Pituitary involvement in the form of diabetes insipidus was confirmed on contrast-enhanced magnetic resonance imaging of sella, which revealed absence of posterior pituitary bright spot. Thus, a final diagnosis of multisystem Langerhans cell histiocytosis (LCH) with organ dysfunction of low-risk type was made.

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Article Information

Corresponding Author: Keshavamurthy Vinay, MD, DNB, Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India (vinay.keshavmurthy@gmail.com).

Published Online: January 11, 2023. doi:10.1001/jamadermatol.2022.5863

Conflict of Interest Disclosures: None reported.

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

References

Mu EW , Khurram NA , Pei Z , et al. 55-year-old man with ulcers in inguinal fold and intergluteal cleft found to have systemic Langerhans cell histiocytosis. JAAD Case Rep. 2018;4(8):837-840. doi:10.1016/j.jdcr.2018.05.016 PubMed Google Scholar Crossref

Singh A , Prieto VG , Czelusta A , McClain KL , Duvic M . Adult Langerhans cell histiocytosis limited to the skin. Dermatology. 2003;207(2):157-161. doi:10.1159/000071786 PubMed Google Scholar Crossref

Kurtzman DJB , Jones T , Lian F , Peng LS . Metastatic Crohn’s disease: a review and approach to therapy. J Am Acad Dermatol. 2014;71(4):804-813. doi:10.1016/j.jaad.2014.04.002 PubMed Google Scholar Crossref

Nakamura K , Tsunemi Y , Kaneko F , Alpsoy E . Mucocutaneous manifestations of Behçet’s disease. Front Med (Lausanne). 2021;7:613432. doi:10.3389/fmed.2020.613432 PubMed Google Scholar Crossref

Charli-Joseph Y , Saeb-Lima M , Hernández-Salazar A , Domínguez-Cherit J . Nasal-type extranodal natural killer/T-cell lymphoma presenting as genital ulcers. J Am Acad Dermatol. 2012;67(4):e157-e159. doi:10.1016/j.jaad.2011.12.031 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.