Behçet disease (incomplete) and cutaneous polyarteritis nosa

Area of Science:

• Rheumatology
• Dermatology
• Ophthalmology

Background:

• Behçet disease is a multisystem inflammatory disorder characterized by oral ulcers, genital ulcers, eye lesions, skin lesions, and a positive pathergy test.
• Cutaneous polyarteritis nodosa (cPAN) is a vasculitis affecting medium-sized vessels, presenting as painful subcutaneous nodules.
• The co-occurrence of Behçet disease and cPAN is rare, prompting investigation into their potential relationship.

Observation:

• A 42-year-old male presented with a history of uveitis and oral aphthous ulcers, along with recurrent painful nodules on his lower extremities.
• Biopsy of the nodules revealed medium-vessel vasculitis with inflammatory infiltrates in subcutaneous adipose tissue, consistent with cPAN.

Findings:

• The patient's presentation included key features of Behçet disease (uveitis, oral ulcers) and findings consistent with cPAN.
• The histological findings of cPAN in a patient with established Behçet disease features are noteworthy.

Implications:

• This case suggests that cPAN-like lesions might serve as a cutaneous manifestation or marker of Behçet disease.
• Further research is warranted to elucidate the potential link between these two conditions and diagnostic implications.
• Recognizing this association may improve the diagnosis and management of patients presenting with overlapping symptoms.