Lymphomatoid granulomatosis differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Lymphamtoid granulomatosis must be differentiated from Bronchocentric granulomatosis and Churg-Strauss, Necrotizing sarcoid granulomatosis, Wegeners granulomatosis, Hodgkins disease, Non-hodgkin lymphoma, and Nasal angiocentric lymphoma

Differentiating Lymphamatoid granulomatosis from other Diseases

As Lymphamatoid granulomatosis manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtypes pulmonary being the most common. The sub types are the following:

• Pulmonary
• CNS
• Dermatologic

Pulmonary Lymphamatoid granulomatosis must be differentiated from other diseases that cause:

• Cough(non-productive, rarely hemoptysis can occur)
• Dyspnea
• Chest tightness

Other Symptoms that are asscociated with the pulmonary symptoms are:

• Malaise
• Weight loss
• Fatigue

From the symptoms listed above; Lymphamatoid granulomatosis is usually differtiated from the following diseases Bronchocentric granulomatosis and Churg-Strauss, Necrotizing sarcoid granulomatosis, Wegeners granulomatosis, Hodgkins disease, Non-hodgkin lymphoma, and Nasal angiocentric lymphoma.

In contrast, CNS lymphamatoid granulomatosis must be differentiated from other diseases that cause:

• Mental status changes
• Headaches
• Seizures
• Hemiparesis
• Ataxia

The differentials are the following CVA and Brain tumors or CNS lymphoma.

Finally Dermatologic Lymphamatoid granulomatosis must also be differtiated from other diseases that cause:

• Erythematous rash
• Macules
• Papules
• Plaques
• Subcutaneous nodules
• Larger ulcerated nodules

The differentials are the following Dermatomyositis, and Psoriasis

Differentiating Lymphamatoid Granulomatosis

On the basis of Cough, Dyspnea, and Chest tightness, Lymphamatoid granulomatosis must be differentiated from Bronchocentric granulomatosis and Churg-Strauss, Necrotizing sarcoid granulomatosis, Wegeners granulomatosis, Hodgkins disease, Non-hodgkin lymphoma, and Nasal angiocentric lymphoma.

Diseases	Clinical manifestations						Para-clinical findings							Gold standard	Additional findings
	Symptoms			Physical examination
							Lab Findings			Imaging			Histopathology
	Cough	Dyspnea	Chest tightness	Auscultation			Lab findings	Lab 2	Lab 3	X-ray	CT scan	Imaging 3
Bronchocentric granulomatosis	+	+	+	Wheezing Rales Rhonchi			Eosinophilia			Usually solitary lesions that appear on chest Xray as atelectasis or consolidation, not nodules			Large and medium bronchi infiltrated by neutrophils, eosinophils and necrotic debris surrounded by foreign body giant cells Fragmented elastic tissue (with elastic stain) Also bronchiolitis obliterans No fibrinoid necrosis of vessels		Granulomatous disease of lungs in which almost all granulomas are centered in bronchi or bronchioles causing their destruction A histologic pattern of disease, not a specific diagnosis Usually adults, often with asthma history and limited to lungs, may be asymptomatic
Churg-Strauss syndrome	+	+	+	Wheezing Rales Rhonchi			Greater than 50% have positive ANCA, often antimyeloperoxidase Eosinophilia			pulmonary infiltrates in at least half of the patients in the second phase of the disease and a greater percentage in the third phase of the disease. Typically, these are transient patchy alveolar infiltrates, usually without preferential, lobar, or segmental distribution. In some instances, a diffuse interstitial infiltrative pattern may be apparent.	Subpleural airspace consolidation is an additional feature in about half of cases, whereas more widespread consolidation is discerned occasionally. Enlarged hilar or mediastinal lymph nodes also are apparent on pulmonary thin-section CT scan		Lung and extrapulmonary sites with eosinophilic infiltrate, Granulomatous reaction around necrotic foci with radially arranged histiocytes and pallisading giant cells near small arteries or arterioles, eosinophilic vasculitis May have fibrin rich edema, lymphocytes, sarcoid-like granulomas, focal fibrosis and eosinophilic microabscesses		Very rare Systemic vasculitis that looks like polyarteritis nodosa or microscopic polyangiitis; associated with asthma, peripheral eosinophilia, pulmonary involvement and fever
Necrotizing sarcoid granulomatosis	+	+	-	Wheezing						Hilar adenopathy	bihilar lymphadenopathy bihilarlymphadenopathy and reticulonodular infiltrates bilateral pulmonary infiltrates fibrocystic sarcoidosis typically with upward hilar retraction, cystic and bullous changes		Inflammatory and granulomatous reactions with dense superficial and deep lymphocytes, eosinophils and plasma cells Variable parakeratosis, spongiosis, acanthosis and epidermal erosion May have lymphoid germinal centers resembling lymphoma with destruction of adnexae and atypia Dense, noncaseating granulomatous infiltrate in the dermis extends into subcutaneous fat; granulomas are discrete and uniform in size and shape; composed of epitheloid histiocytes with abundant eosinophilic cytoplasm and oval nuclei containing a small central nucleolus Variable Langhans giant cells, and scattered lymphocytes Discrete, small central foci of fibrinoid necrosis are present; also transepidermal elimination Also Schaumann bodies (calcium and protein inclusions inside of Langhans giant cells as part of a granuloma; basophilic laminated rounded conchoidal structures), asteroid bodies (small, intracytoplasmic, eosinophilic star shaped structure also present in tuberculoid leprosy, berylliosis and atypical facial necrobiotic xanthogranuloma), Hamazaki-Wesenberg bodies (peculiar PAS+ inclusions, may be large lysosomes containing hemolipofuscin)
Diseases	Symptom 1	Symptom 2	Symptom 3	Auscultation			Lab 1	Lab 2	Lab 3	Imaging 1	Imaging 2	Imaging 3	Histopathology	Gold standard	Additional findings
Wegeners granulomatosis
Hodgkin disease
Non-hodgkin lymphoma