The skin in Behçet's disease: Mucocutaneous findings and differential diagnosis

Abstract

Behçet's disease (BD) is a chronic multisystemic inflammatory disease with vasculitis and skin findings. Attacks and remissions characterize the course of the disease. In general, the disease activity decreases with advanced age. Skin symptoms are characteristic of BD and they are the most common findings in the course of the disease. The earliest sign of the disease is almost always recurrent oral aphthous ulcers. Genital ulcers are very specific for BD and they are the equivalent of oral ulcers in the genital area. The most common location is labium majus in females and scrotum in males. The papulopustular lesions are mainly observed on the lower extremities in BD patients. Erythema nodosum-like lesions and superficial thrombophlebitis are relatively common skin findings. Extragenital ulcerations presenting on the intertriginous area are rare but specific for the disease. The clinical skin manifestations are crucial for the diagnosis of BD, as there is no pathognomonic confirmatory laboratory test.

Definition of activity cut-off point in patients with Behçet’s disease using the Arabic version of the Behcet’s Syndrome Activity Scale.

“…Throughout the course of the disease, Behçet's disease has a varied pattern of organ involvement that takes place in recurrent bouts of acute inflammation in the form of remission and exacerbation [13,14].…”

Imaging More than Skin-Deep: Radiologic and Dermatologic Presentations of Systemic Disorders

“…Multiple, bilateral pulmonary artery aneurysms are a rare but characteristic feature of BD ( Figure 7 E), which may present as parahilar nodular opacities on chest radiograph. CTA/MRA delineate vessels and collaterals, the presence of thrombus, and evidence of mediastinal involvement [ 40 , 43 ]. CNS disease manifests in 10–50% of patients.…”

“…Acute attacks initially involve the basal ganglia or brainstem, with extension to the diencephalic structures, and show contrast enhancement and scattered areas of T2 hyperintensity ( Figure 7 F). Months later, small scattered hyperintense lesions present in the periventricular white matter [ 40 , 43 ].…”

“…MRI is commonly used for the evaluation of neoplastic lesions [ 50 ]. Optic gliomas (pilocytic astrocytoma) are the most common tumor in NF-1 (18%) and can occur bilaterally [ 15 , 43 , 44 ]. On MRI, gliomas are seen as T1-hypointense and T2-hyperintense with variable contrast enhancement ( Figure 9 C).…”

Behçet’s Disease: A Comprehensive Review on the Role of HLA-B*51, Antigen Presentation, and Inflammatory Cascade

“…According to The International Study Group criteria published in 1990 for BD, oral lesions plus two of uveitis, genital lesions, other skin lesions, and a positive skin pathergy test (SPT) are required for BD diagnosis [4]. Although mucocutaneous symptoms are the most common presenting symptoms [5], patients may exhibit vasculitis and multisystemic involvement resulting in significant morbidity [3,6].…”