Case
A36-year-old otherwise healthy white woman with a history of chronic sinusitis presented with progressive left facial pain and swelling of 5 months’ duration. Associated symptoms included epistaxis and left-sided epiphora. Prior to presentation, she had been treated as an outpatient with multiple rounds of oral antibiotics and steroids for presumed sinusitis without improvement. Social history included routine cocaine use 10 years ago. Physical examination demonstrated substantial erythema and edema of the left infraorbital region and left nasal sidewall and purulent nasal secretions (Figure 1A). Endoscopic nasal examination revealed substantial swelling of the left nasal vestibule completely obstructing the left naris, swelling of the left nasolacrimal duct orifice, nearly total septal perforation with areas of necrotic bone, and no identifiable intranasal landmarks except for a remnant of the left middle turbinate (Figure 1B). There was no cervical lymphadenopathy. Laboratory evaluation revealed a white blood cell count of 12 300/μL with normal neutrophil count; erythrocyte sedimentation rate of 83 mm/h; and a C-reactive protein level of 134 mg/L. (To convert white blood cells to ×109/L, multiply by 0.001; C-reactive protein to nanomoles per liter, multiply by 9.524.) A maxillofacial computed tomographic (CT) scan demonstrated cartilaginous and anterior osseous nasal septal perforation and left nasal and preseptal soft-tissue thickening with associated periosteal reaction of the nasal process of the maxilla.
Figure 1.
A, Periorbital and nasal cellulitis. B, Nasal endoscopic examination demonstrating significant soft-tissue necrosis.
What Is Your Diagnosis?
- Granulomatosis with polyangiitis
- Cocaine-induced midline destructive lesion
- Extranodal natural killer/T-cell lymphoma, nasal type
- Chronic invasive fungal sinusitis
Discussion
Diagnosis
B. Cocaine-induced midline destructive lesion
The patient began treatment with broad-spectrum intravenous antibiotics, which was deescalated to oral trimethoprim-sulfamethoxazole and rifampin treatment for 6 weeks after nasal swab culture grew methicillin-sensitive Staphylococcus aureus. A repeated nasal culture at follow-up grew Pseudomonas aeruginosa, so she was then treated with a 6-week regimen of levofloxacin and rifampin. After completion of the antibiotics regimen, her condition showed only mild improvement, so she underwent biopsy and surgical debridement of necrotic tissue, which exposed an underlying nasocutaneous fistula over the left nasal sidewall (Figure 2). Biopsy specimens demonstrated necroinflammatory debris and acute and chronic osteomyelitis, which together with her history were suggestive of cocaine-induced midline destructive lesion (CIMDL). No pathognomonic lesions suggestive of granulomatosis with polyangiitis (GPA) were seen.
Figure 2.
Axial noncontrast computed tomographic maxillofacial scan after antibiotic treatment demonstrates destruction of the left frontal process of the maxilla and cartilaginous and osseous nasal septum.
Cocaine acts as a local anesthetic, vasoconstrictive, and euphoric agent. It blocks sodium channels and thereby halts electrical propagation of neurons to cause local anesthesia.1 It also inhibits the reuptake of norepinephrine and dopamine at the presynaptic nerve endings, leading to an accumulation of these neurotransmitters at nerve synapses.2 In the peripheral nervous system, norepinephrine primarily accumulates, causing an increase in sympathetic tone.3 In the central nervous system, dopamine accumulation is responsible for cocaine’s euphoric and addictive properties.3,4 It is reported that close to 30 million people in the United States have used cocaine and that 6 million of them are compulsive users; however, CIMDL is only seen in 4.8% of users.5,6
Nasal septal perforation, nasal cavity wall erosion, pharyngeal wall ulceration, and hard palate perforation are all manifestations of CIMDL. The damage to the nasal architecture is multifactorial, primarily the result of intense vasoconstriction with repeated cocaine use, leading to local tissue necrosis. However, the chemical irritation from adulterants, such as lactose and inositol, and mechanical irritation from self-instrumentation and high-velocity cocaine crystal inhalation have also been implicated in mucosal and perichondrial erosion.7
The clinical presentation of CIMDL also mimics certain systemic diseases, most notably GPA. Clinical differentiation can be made based on more severe destruction of nasal architecture, such as loss of inferior and middle turbinates, and lack of systemic manifestations in CIMDL.8Histopathological findings are very similar in CIMDL and GPA unless discriminatory findings of GPA are seen, such as necrotizing granulomas and multinucleated giant cells.8 Finally, positive findings for antineutrophil cytoplasmic antibodies (ANCAs) are common in both patient populations, but reaction against human neutrophil elastase (HNE) in CIMDL is its most distinguishing feature.9 The presence of HNE-ANCA is what is believed to predispose select cocaine users to CIMDL because it enhances the local inflammatory response to injury—highlighting a possible autoimmune component to the disease.9 It is also reported that there is a time and cocaine-dose dependence to developing the disease, suggesting a chronicity, rather than fulminant stage, to the disease entity.6
It is important to remember that patients tend to understate cocaine use, as did this patient, who only reported recent use after her pain progressed. Treatment includes complete abstinence from cocaine, debridement of necrotic tissue, and treatment of any superinfection. The most common surgical procedures are closure of septal and palatal perforation, with most surgeons requiring 6 to 12 months of cocaine abstinence prior to intervention.9 Of note, immunosuppression is not indicated; therefore, correct identification of the underlying disease process is critical.6 The present case demonstrates an uncommonly severe presentation of CIMDL and illustrates the importance of correct and timely diagnosis to guide subsequent treatment.
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Article Information
Published Online: April 18, 2019. doi:10.1001/jamaoto.2019.0333
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
References
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