1

Introduction

Midline destructive lesions (MDLs) of the face were first reported in 1897 , but naming of MDLs started when Stewart reported 10 cases of a chronic destructive midfacial process in 1922. Later, Williams began to use the term lethal midline granuloma to describe a destructive process of the nasal cavity of unknown etiology. Since that time, a variety of terms have been used to describe MDLs involving the nose and paranasal sinuses, including idiopathic midline granuloma, idiopathic midline destructive disease (IMDD), midline nonhealing granuloma, lethal midline granuloma, polymorphic reticulosis, and Stewart syndrome, among others . Common to all is the ulcerative process that occurs in the nose characterized by epithelium and cartilage loss with crusting that may result in loss of nasal structure, support, and, ultimately, cosmetic and functional deformity.

The diagnostic approach to MDLs of the nose poses a difficult diagnostic dilemma. The variability of the underlying pathologic processes and nonspecific presenting symptoms makes the diagnosis complex. Although serologic testing, immunophenotyping, and genetic testing attribute most MDLs to Wegener’s granulomatosis (WG) or natural killer/T-cell–type non–Hodgkin’s lymphoma (NKTL) , the differential diagnosis at presentation remains extensive if systemic symptoms are absent. Herein, we discuss 8 patients who presented with an MDL, we review the current tools available for diagnosis, and we present a diagnostic algorithm.

2

Case series

Institutional review board approval was obtained for review of 8 consecutive patients who presented with various undiagnosed MDLs during 2007. Each received nasal endoscopy, computed tomography (CT) scan of the sinuses, laboratory workup, culture, and biopsy with flow cytometry. Laboratory testing included complete blood count, basic metabolic panel, erythrocyte sedimentation rate (ESR), angiotensin-converting enzyme (ACE), antineutrophil antibodies, rheumatoid factor, anti-Ro and anti-La antibodies, Epstein-Barr virus (EBV) antibodies, coccidiomycosis serology, HIV antibodies, rapid plasma reagin, fluorescent treponemal antibody absorption, classic antineutrophil cytoplasmic antibodies (cANCAs), perinuclear antineutrophil cytoplasmic antibodies (pANCAs), proteinase 3 (PR3), and myeloperoxidase (MPO). The spectrum of laboratory studies used was individualized for each patient. Cultures included aerobes, anaerobes, and fungi, and acid-fast bacilli. Table 1 shows pertinent patient history and physical, workup, and final diagnosis.

2

Case series

Institutional review board approval was obtained for review of 8 consecutive patients who presented with various undiagnosed MDLs during 2007. Each received nasal endoscopy, computed tomography (CT) scan of the sinuses, laboratory workup, culture, and biopsy with flow cytometry. Laboratory testing included complete blood count, basic metabolic panel, erythrocyte sedimentation rate (ESR), angiotensin-converting enzyme (ACE), antineutrophil antibodies, rheumatoid factor, anti-Ro and anti-La antibodies, Epstein-Barr virus (EBV) antibodies, coccidiomycosis serology, HIV antibodies, rapid plasma reagin, fluorescent treponemal antibody absorption, classic antineutrophil cytoplasmic antibodies (cANCAs), perinuclear antineutrophil cytoplasmic antibodies (pANCAs), proteinase 3 (PR3), and myeloperoxidase (MPO). The spectrum of laboratory studies used was individualized for each patient. Cultures included aerobes, anaerobes, and fungi, and acid-fast bacilli. Table 1 shows pertinent patient history and physical, workup, and final diagnosis.

Table 1

Eight patients presenting with a MDL

Age (y)/sex	History	Physical examination	Pertinent diagnostic tests	Diagnosis
1: 42/female	1-y history of nasal and maxillary pain and nasal crusting intermittent joint pain	Saddle nose deformity	CT : septal perforation	IMDD
		Gingivolabial-nasal fistula	Laboratory tests : negative
		Atrophic inferior turbinates	Culture : group C streptococci
	Periorbital edema	Cartilaginous septal perforation	Biopsy : focal areas of ulceration and necrosis with squamous metaplasia
	history of smoking	Significant crusting
2: 49/female	3 y history of facial swelling, congestion, drainage, pain, and pressure	Erythema of lower two thirds of nose ulceration of right nasal ala	CT : diffuse inflammation	WG
		Destruction of cartilaginous septum	CXR : negative
	history of arthritis and smoking	Granular-appearing mucopurulent mucosa	Laboratory tests : cANCA+, pANCA+, PR3-ANCA+
		Gross destruction endonasally	Culture : Staphylococcus aureus , Pseudomonas aeruginosa
			Biopsy : chronic inflammatory infiltrate
3: 63/male	2 y of recurrent severe epistaxis	Ulcerated mucosa with mucopurulent discharge	CT : septal perforation	IMDD
	Hyposmia	Septal perforation	CXR / CT chest : multiple calcified lung nodules bilaterally
	Chronic hearing discomfort	Gross destruction of endonasal structure	Laboratory tests : elevated ESR
	history of arthritis	Bilaterally dehiscent lamina papyracea	Culture : S aureus
			Biopsy : inflammation and granulation
4: 52/female	Long history of congestion, hyposmia, nasal drainage, right facial pain and headache, and episodic epistaxis	Anterior septal defect	CT : diffuse mucosal thickening	IMDD
		Truncated right inferior turbinate	Laboratory tests : ESR elevated, anticardiolipin+
		Granulation tissue	Biopsy : inflammation and granulation
	history of CRS, asthma, atopic dermatitis	Diffuse edema
5: 60/male	3 wk of left-sided facial erythema, swelling, and congestion	Necrotic friable debris between the septum and lateral nasal wall bilaterally	CT : ethmoidal soft tissue mass	NKTL
	history of CRS and asthma	Diffuse fibrinous exudates	Biopsy : necrosis, infiltrate of mostly medium to large transformed lymphocytes with irregular nuclear membranes visible nucleoli. Frequent mitoses.
			Flow cytometry : CD45−, CD34−, CD56+, TIA1+, CD33−, cytoplasmic CD3−, CD5−, CD8−
			PET : multiple increased uptake sites
6: 34/female	Postnasal drip/rhinorrhea	Polypoid disease	CT : moderate mucosal thickening and opacification of the sinuses	WG
	Hyposmia	Osteitic bone changes
	Headaches and maxillary facial pressure		Laboratory tests : antineutrophil antibodies+, cANCA+
	Green nasal drainage		Biopsy : necrotizing palisading granulomas
	Lacy rash on extremities and migratory arthralgias
	history of CRS status post FESS and asthma
7: 42/male	6 wk of progressive nasal obstruction and facial pain after septoplasty and FESS	Anterior septal perforation	CT : septal defect and bilateral nasal cavity opacification with erosion of the inferior turbinate bones and medial maxillary walls	NKTL
		Friable tissue overlying the bilateral inferior and middle turbinates	Biopsy : histopathology and flow cytometry confirmed angiocentric monomorphic T-cell infiltrate
		Erosion of the superior alveolus
8: 57/male	Epistaxis	Saddle nose deformity	CT : normal	IMDD
	Nasal crusting	Nodularity of the septum and inferior turbinates bilaterally	Laboratory tests : ESR elevated
	history of Crohn’s disease		Biopsy : chronic inflammation, fibrosis, focal necrosis

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