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systemic granulomatoses

Tuesday 10 June 2003

Etiology:

- infectious systemic granulomatoses

  • tuberculosis
  • atypical mycobacteriosis
    • Mycobacterium kansasii
    • Mycobacterium avium intracellualre (MAI)

- systemic tuberculoid granulomatoses

  • chronic familial granulomatosis
  • sarcoidosis
  • necrotizing sarcoid granulomatosis
  • Crohn disease
  • familial juvenile systemic granulomatosis (FJGS) (Blau syndrome) (CARD15 mutations)

- systemic necrotizing granulomatosis
- Wegener granulomatosis
- Churg-Strauss angiitis

- tumoral systemic granulomatoses

  • lymphomatoid granulomatosis
  • T-cell lymphomas

- systemic histiocytoses

  • langerhans histiocytosis
  • non-Langerhans cell histiocytoses
    • Erdheim-Chester disease
    • systemic juvenile xanthogranuloma
    • necrobiotic xanthogranuloma
    • adult disseminated xanthoma

- constitutional immune deficiencies

  • RAG1 and RAG2 germline mutations (#18463379#)

- miscellaneous

  • lipogranulomatoses
  • hyalinizing plasmacytic granulomatosis (#2451439#)
  • familial juvenile systemic granulomatosis (FJSG)

Localization

- tracheal granulomatosis
- pulmonary granulomatosis

  • bronchocentric granulomatosis
  • bronchiolocentric granulomatosis

- renal granulomatosis
- splenic granulomatosis
- digestive granulomatosis

Types

- necrotizing granulomatosis
- tuberculoid granulomatosis (sarcoid granulomatosis)
- familial systemic granulomatosis

See also

- Granulomas

  • tuberculoid granuloma
    • epithelioid granuloma

References

- Schuetz C, Huck K, Gudowius S, Megahed M, Feyen O, Hubner B, Schneider DT, Manfras B, Pannicke U, Willemze R, Knüchel R, Göbel U, Schulz A, Borkhardt A, Friedrich W, Schwarz K, Niehues T. An immunodeficiency disease with RAG mutations and granulomas. N Engl J Med. 2008 May 8;358(19):2030-8. PMID: #18463379#


- Atlas of Granulomatous Diseases by Yale Rosen, M.D.

Portfolio

  • Epitheloid granuloma in systemic granulomatosis (Case 12002)
  • Epitheloid granuloma in systemic granulomatosis (Case 12002)
  • Epitheloid granuloma in systemic granulomatosis (Case 12002)
  • Epitheloid granuloma in systemic granulomatosis (Case 12002)
  • Epitheloid granuloma in systemic granulomatosis (Case 12002)
  • Epitheloid granuloma in systemic granulomatosis (Case 12002)
  • Epitheloid granuloma in systemic granulomatosis (Case 12002)