Management Challenges in an Unusual Case of Malignant Otitis Externa and CLL Associated with Multiple Head and Neck Cutaneous SCCS

Background Patients with chronic lymphocytic leukemia (CLL) are at a higher risk for developing cutaneous malignancies. A study conducted in the United States reported that patients with CLL were eight times more likely to have skin cancer than patients without CLL, particularly squamous cell carcinoma (SCC). Many reports suggest that in CLL, the dysfunctional lymphocytes are unable to elicit an antitumor response, thereby contributing to the increased incidence and clinical characteristics of SCC and other forms of skin cancers in these patients. Studies have shown data suggesting that the immunosuppressive effects of chemotherapeutic agents and irradiation used to treat CLL increase the risk of skin cancer in these patients, while other studies suggest genetic abnormalities to be the cause of association between CLL and skin cancers. Cutaneous SCC in CLL tends to be multiple and aggressive, with a higher incidence of posttreatment recurrence and spread.

Malignant otitis externa (MOE) also referred to as skull base osteomyelitis occurs mainly in elderly patients with diabetes or in immunocompromised patients. It is often initiated by Pseudomonas, while methicillin-resistant Staphylococcus aureus has also been identified as a cause. It is characterized by persistent and severe, deep-seated otalgia, foul-smelling purulent otorrhea, and granulation tissue or exposed bone in the ear canal. Varying degrees of conductive hearing loss may occur. In severe cases, facial nerve paralysis, and even lower cranial nerve (IX, X, or XI) paralysis, may ensue as this erosive, potentially life-threatening infection spreads along the skull base. Treatment is with systemic and topical antibiotics, regular aural toilet/microsuction, meticulous control of diabetes, and in more extensive cases, surgical debridement may be required.

Objectives Presentation of an unusual case of an elderly diabetic patient with CLL associated with multiple head and neck cutaneous SCCs, MOE, and facial nerve paralysis.

We discuss the challenges/dilemmas in managing this complex case including diagnosis, decision for antibiotics and surgical intervention, and timings for chemotherapy and radiotherapy.

To the best of our knowledge, this is the first case of its kind in English literature.

Methods Case note review.

Results A multidisciplinary team consisting of ENT surgeons, the skull base team, an oncologist, hematologist, radiologist, microbiologist, and pathologist were involved in the management of this patient. Patient had chemotherapy for CLL and long-term antibiotics and surgical debridement for MOE. His facial nerve paralysis had not improved. He subsequently had a relapse of CLL and treatment for his cutaneous SCCs had to be deferred. The patient has unfortunate now passed away.

Conclusion It is always a challenge to manage such complex cases. Radiotherapy for the CLL would have worsened the MOE, whereas treating the MOE would have meant withholding/delaying radiotherapy for the cutaneous SCC. Therefore, a multidisciplinary approach is vital for the treatment of such cases. One of the main differential diagnoses of MOE is malignancy. Patient education, frequent assessment, and low threshold for biopsy of new lesions along with assessment of acute problems alongside existing disease allow the most effective and appropriate treatment regimen to be devised.