Mucocutaneous Manifestations of Behçet's Disease

Abstract

Behçet's disease (BD) is a chronic, relapsing, systemic inflammatory disease with clinical features showing mucocutaneous lesions involving the ocular, articular, and further miscellaneous organs. Mucocutaneous manifestations, one of the most characteristic signs of BD, have been most commonly observed upon onset or at any disease stage and are exceptionally important in its diagnosis. Given the lack of specific diagnostic laboratory tests for BD, diagnosis has been based on clinical findings. All diagnostic criteria published have thus far relied heavily on mucocutaneous manifestations, particularly oral ulcers (OU), genital ulcers (GU), cutaneous lesions, and pathergy test positivity. Worldwide, OU, GU, cutaneous lesions, and ocular and articular manifestations have been the most common symptoms, with erythema nodosum (EN)-like lesions and papulopustular lesions being the most prevalent cutaneous manifestations. While majority of the patients worldwide have reported OU as the most frequent symptom upon disease onset, GU, and EN-like lesions have also been identified upon onset. Considering that mucocutaneous symptoms precede severe organ involvement in most patients, familiarity with such symptoms is imperative for early diagnosis and prevention of potentially serious organ involvement through appropriate management.

Keywords: Behçet's disease; erythema nodosum-like lesion; genital ulcer; oral ulcer; papulopustular lesion.

Figure 1

(A) Oral ulcer on the buccal mucosa with rolled borders and grayish necrotic base. (B) Genital ulcer with yellowish necrotic tissue on the scrotum. The genital ulcer is deep and surrounded by erythema. The scrotum is the site most commonly involved in the male. (C) Erythema nodosum-like lesions on the lower leg. A painful, reddish erytemanous nodule is detected in the pretibial lesion. (D) Papulopustular folliculitis are sterile pustules on the erythematous base of the trunk. (E) Superficial thrombophlebitis is erythematous tender subcutaneous nodules arranged in a linear fashion on the leg. (F) Histological feature of erythema nodosum-like lesion includes a dense neutrophil infiltrate around the vessels in the subcutaneous tissue. (G) Histological feature of papulopustular lesion includes a leukocytoclastic vasculitis with neutrophil infiltrate in the dermis. (H) Histology of thrombophlebitis shows vascular thrombus and lymphocytic infiltrate in a subcutaneous vein. (I) Skin pathergy reaction is a pustule formation at the needle site after 24 h on the forearm. (A,F,H,I) are derived from Nakamura et al. (3, 4).