A 51–year–old female, with no significant past medical history, presented to the emergency room with dyspnea and swelling of the throat. The patient first began to experience shortness of breath two months prior to this encounter. At that time, the patient was evaluated in the emergency department of an outside hospital. Pulmonary embolus was ruled out via computed tomography (CT). The patient was then discharged, but she returned to the same hospital three weeks later with throat swelling and pain. A CT of the head and neck showed diffuse lymph node enlargement. The patient was admitted to the outside hospital and given a three day course of ceftriaxone. She was discharged without resolution of the swelling in the throat. Two weeks thereafter, the patient visited her primary care physician with persistence of the aforementioned symptoms and was given a ten day course of amoxicillin.
Over the ensuing three weeks, the patient became increasingly dyspneic. She also endorsed increased pain in her throat, intermittent xerostomia, subjective fevers, chills, and weight loss.
On admission, her vitals were stable and the laboratory values demonstrated a leukopenia of 4,200 white blood cells with an unremarkable cell differential. Pertinent physical exam findings included bilateral grossly enlarged, fixed and tender submandibular glands, and diffuse cervical lymphadenopathy. Direct flexible laryngoscopy showed no signs of airway compromise. The patient received morphine on admission, which alleviated her throat pain. The Oncology team was notified given concern for malignancy. A CT elucidated that the submandibular glands had increased in size from the prior study: now measuring 2.9cm x 3.0cm x 3.9cm on the right and 2.5cm x 2.5cm x 4.5cm on the left. A fine needle aspiration was done on these glands; the pathology of which showed a plethora of infiltrating lymphocytes, indicating chronic inflammation. An IgG Sublass 4 was elevated (797, Normal 1-123). These results suggested the diagnosis of Mikulicz’s Disease. Moreover, she was discharge and referred to a Rheumatologist for management and treatment with steroids.
Discussion:
Mikulicz’s Disease is a chronic condition characterized by the enlargement of glands in the head and neck. This occurs most frequently in the parotid, lacrimal, and salivary glands. It is generally benign, occurs mostly in females, and often is associated with an underlying disorder such as tuberculosis, leukemia, syphilis, Sjögren’s syndrome, or lupus – at which point it is termed Mikulicz’s Syndrome. Patents often experience recurrent fevers, diminished secretions of the affected glands, and pain. The exact cause of Mikulicz’s Disease and Syndrome is unknown.
This case demonstrates a presentation of Mikulicz’s Disease that was initially suspected to be lymphoma. It is often misdiagnosed as malignancy or an autoimmune inflammatory processes, such as Sjögren’s syndrome. Treatment of Mikulicz’s Disease mainly consists of administration of glucocorticoids like prednisonde. It is important to consider Mikulicz’s Disease in patients with glandular enlargement, as misdiagnosis may lead to unnecessary hospitalizations, surgeries, antibiotic exposure and a latent resolution to a treatable problem.
Conclusions:
Mikulicz’s Disease is an uncommon culprit of glandular enlargement and pain. Early recognition of Mikulicz’s Disease is pertinent both to minimize the morbidity of needless intervention, and to ensure proper treatment and symptom resolution.