A Man with Painful Mouth Ulcers

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Posted by Carla Rothaus

Which cancers are most frequently associated with paraneoplastic pemphigus?

Evaluation of oral ulcers begins with determination of whether the patient has a single lesion or multiple lesions and whether the process is episodic or continuous. Read the NEJM Case Records of the Massachusetts General Hospital here.

Clinical Pearls

Q: Are the mucocutaneous ulcers caused by Epstein-Barr virus usually solitary or multiple?

A: Mucocutaneous ulcers caused by cytomegalovirus or Epstein–Barr virus are generally solitary and often occur in immunocompromised or immunosenescent persons.

Q: Do oral ulcers caused by pemphigus vulgaris have a predilection for any particular region of the oral cavity?

A: Oral ulcers caused by pemphigus vulgaris can occur at any site but have a predilection for the palatal mucosa, and they may be several centimeters in diameter. Many patients with pemphigus vulgaris initially present with oral ulcers, which can last for months before skin lesions develop.

Morning Report Questions

Q: Describe features of the oral ulcers associated with Behçet’s disease and herpes simplex virus (HSV) infection.

A: The presence of oral ulcers is one of the defining criteria of Behçet’s disease; the oral ulcers associated with this disease tend to wax and wane and often respond to colchicine and glucocorticoid therapy. Primary HSV infection is characterized by a prodrome of fever and malaise, and painful clustered ulcers (ruptured vesicles) develop on both the nonkeratinized and the keratinized tissue of the tongue dorsum, hard palatal mucosa, and gingiva. These lesions often coalesce to form irregularly shaped ulcers that resolve within several weeks. When HSV reactivation occurs in immunocompromised persons, ulcers may develop on any part of the oral mucosa, and multiple ulcers (similar to those seen in primary HSV infection) or a single ulcer may form.

Q: Which cancers are most frequently associated with paraneoplastic pemphigus?

A: Patients with paraneoplastic pemphigus, also known as paraneoplastic autoimmune multiorgan syndrome, usually have extensive skin involvement that mimics pemphigus vulgaris, pemphigoid, graft-versus-host disease, erythema multiforme, and lichen planus. The cancers most frequently associated with paraneoplastic pemphigus are non-Hodgkin’s lymphoma, chronic lymphocytic leukemia, B-cell lymphoma, and thymoma. Patients with paraneoplastic pemphigus may have extensive ulceration of the mouth, often with hemorrhagic crusts on the lips.

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