A Case of Behçet's Disease Initially Presenting With Acute Dyspnea Due to Subglottitis

Abstract

We report the case of a 17-year-old female patient who was referred to our department for acute dyspnea. She had noticed erythema all over her body and suddenly noticed severe hoarseness, cough, and wheezing three days before her presentation. Fiberscopy revealed subglottic swelling and aphthae. Subglottitis was diagnosed and intravenous corticosteroids and antibiotics were administered. Subsequently, oral ulcers, genital ulcers, and systemic papules were noted. Histologic examination of the skin suggested Behçet's disease. Behçet's disease is an inflammatory disorder that can affect multiple areas of the body, including the mucous membranes of the mouth and genital region, eyes, skin, and joints. To our knowledge, acute dyspnea due to subglottitis has never been reported in patients with Behçet's disease.

Keywords: apremilast; behçet’s disease; dyspnea; endoscopy; larynx; pseudocroup; subglottic stenosis.

Figure 1. Axial CT at the initial presentation.

The glottis appears intact (A). A slightly caudal section (B). Thickening of the anterior tracheal wall (arrow) suggests subglottic stenosis (B).

Figure 2. Laryngeal endoscopic findings.

A laryngeal image (A) shows bilateral subglottic swelling and aphthae (arrow). Subglottic swelling relieved after the administration of prednisolone (B). Eight months after the onset of symptoms, the patient developed mild dyspnea and showed recurrence of subglottic swelling and aphthae (arrow) (C). She was subsequently treated with apremilast 60 mg daily. A recent laryngeal image shows normal findings (D).

Figure 3. Skin lesions.

A red papule with a central pustule was observed on the left thigh (A), and erythematous nodules and plaques were present on the extensor aspect of the left lower leg (B). Histology of the erythema (hematoxylin and eosin, ×100) showed septal panniculitis with neutrophilic infiltration (C). The histopathological findings supported the diagnosis.