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Clinical Challenge

Generalized Nodules in an Older 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

Published Online: October 5, 2022

JAMA Dermatology Clinicopathological Challenge

Article Information and Disclosures

Jiang-Wei Cheng, MD^1,2;

Ling-Xi Liu, MD^1,3;

Jiu-Hong Li, MD, PhD^1,2

Author Affiliations

¹Department of Dermatology, The First Hospital of China Medical University, Shenyang, China
²National Joint Engineering Research Center for Theranostics of Immunological Skin Diseases, Shenyang, China
³Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, China

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An otherwise healthy man in his 60s presented to our department with an 8-month history of widespread pruritic papulonodular skin lesions on his extremities and trunk. The patient had been initially diagnosed with nodular prurigo at a local hospital and treated with various topical steroids and oral antihistamines for 2 months, without significant improvement. The skin lesions continued to progress and worsened after scratching. Physical examinations revealed disseminated erythematous papules, hypertrophic plaques approximately 1 to 2 cm in diameter, and dome-shaped nodules with excoriated whitish or pink centers on the scalp, chest, abdomen, back, waist, and extremities (Figure, A and B). Subsequent examinations of the mucosal membranes appeared normal. Other physical examinations as well as his medical and family histories were unremarkable. Peripheral eosinophils, serum total immunoglobulin E level, enzyme-linked immunosorbent assay (ELISA) test for HIV, and results of other routine laboratory tests were within normal ranges or negative. A biopsy specimen was taken from one of the skin lesions on his right forearm for histopathological examination and direct immunofluorescence (DIF) testing (Figure, C and D).

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D. Pemphigoid nodularis

Histopathological examination revealed marked hyperkeratosis, acanthosis with irregular elongation of the rete ridges, fibrotic dermis, and perivascular lymphocytic infiltrate (Figure, C). Results of DIF showed linear deposition of immunoglobulin G (IgG) (Figure, D) and C3 along the epidermal basement membrane zone (BMZ). Indirect immunofluorescence for anti–basement membrane antibody (IgG) was positive, with a titer of 1:256. Levels of autoantibodies for BP180 and BP230 detected via ELISA were elevated (116 and 101 [normal <9] U/mL, respectively). Based on these findings, we diagnosed pemphigoid nodularis (PN). The patient was treated with oral methylprednisolone, 28 mg/d (0.4 mg/kg per day), and topical halometasone cream twice a day. Eight weeks later, most of the papulonodular lesions had subsided, and no new lesions were observed. We gradually reduced oral methylprednisolone until a dosage of 7 mg/d (0.1 mg/kg per day) was reached. No recurrence was detected during a 1-year follow-up.

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Article Information

Corresponding Author: Jiu-Hong Li, MD, PhD, Department of Dermatology, The First Hospital of China Medical University, 155N Nanjing St, Shenyang 110001 PR China (pfkl2011@126.com).

Published Online: October 5, 2022. doi:10.1001/jamadermatol.2022.4297

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank Song Zheng, MD, PhD, and Wen-Jie Zheng, MM, The First Hospital of China Medical University, for their assistance in pathology. They were not compensated. We also thank the patient for granting permission to publish this information.

References

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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.

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