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IgG4-related midline destructive lesion
  1. Emanuel Della-Torre1,2,
  2. Hamid Mattoo1,
  3. Vinay S Mahajan1,
  4. Vikram Deshpande3,
  5. Donald Krause4,
  6. Philip Song5,
  7. Shiv Pillai1,
  8. John H Stone6
  1. 1 Massachusetts General Hospital Cancer Center, Massachusetts General Hospital, Boston, Massachusetts, USA
  2. 2 Unit of Medicine and Clinical Immunology, Università Vita-Salute San Raffaele, Milan, Italy
  3. 3 Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
  4. 4 Rheumatology Service, St. Joseph Healthcare, Bangor, Maine, USA
  5. 5 Department of Otolaryngology, Massachusetts Eye & Ear Infirmary, Massachusetts General Hospital, Boston, Massachusetts, USA
  6. 6 Division of Rheumatology, Allergy, & Immunology, Massachusetts General Hospital, Boston, Massachusetts, USA.
  1. Correspondence to Dr John H Stone, Rheumatology Clinic/Yawkey 2, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA; jhstone{at}mgh.harvard.edu

https://doi.org/10.1136/annrheumdis-2014-205187

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    Midline destructive lesion (MDL), also known as ‘lethal midline granuloma’, represents a fibroinflammatory condition characterised by relentless erosion of the nose, paranasal sinuses and palate.1 ,2 Disease attribution remains elusive for many cases, which continue to be labelled ‘idiopathic’.2

    Four patients, two males and two females, were referred because of progressive erosion of midline facial structures that had occurred over 2–3 years. Their median age was 40 years (range 27–54). Presenting symptoms included chronic sore throat, difficulty swallowing (nasal regurgitation) and progressive nasalisation of speech. Tissue destruction developed insidiously in all four patients, initially as small ulcerative lesions of the soft palate and nasal septum that enlarged gradually into perforations of palatal and nasal structures. Spontaneous loss of the uvula occurred in three patients. Three had palatal prostheses assembled in order to prevent the passage of food into the nasopharynx (figure 1A,B). All four developed saddlenose deformities.

    Figure 1

    Clinical and radiological features of IgG4-related midline destructive lesion in four patients. (A) Soft palate erosion with uvula reabsorption. (B) Nasal septum perforations (patients 2 and 4) and the palatal prostheses used by patients 1 and 3. (C) Computed tomography scan of the head and neck showing destruction of the hard palate with communication …

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    Footnotes

    • Contributors ED-T and JHS conceived of and conducted the clinical studies. HM and VSM conducted laboratory studies with guidance from SP. DK and PS helped with clinical diagnosis and management of patients. VD helped with pathological diagnosis of patients. ED-T and JHS drafted the manuscript. All authors read and approved the final version.

    • Funding This study was funded by grants AI 064930 and AI 076505 from the NIH and a pilot grant from the Harvard Institute of Translational Immunology supported by the Helmsley Foundation.

    • Competing interests None.

    • Ethics approval This study involves human subjects and was approved by the Ethics Committee/Institutional Review Board of the Massachusetts General Hospital (Partners).