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Technique of Conjunctival Biopsy and Direct Immunofluorescence for Diagnosing Mucous Membrane Pemphigoid
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Plasminogen deficiency as a rare cause of conjunctivitis and lymphadenopathy

Sophia L Yohe1, Marcos Reyes, Daniel A Johnson

  • 1Department of Pathology, University of Texas Health Science Center, San Antonio, TX 78229, USA.

The American Journal of Surgical Pathology
|November 18, 2008
PubMed

Insights

Plasminogen deficiency can cause ligneous conjunctivitis and other mucosal plaques. This rare disorder can also affect lymph nodes, presenting unique diagnostic challenges for pathologists.

Area of Science:

  • Pathology
  • Rare Diseases
  • Immunology

Background:

  • Plasminogen deficiency is a rare genetic disorder.
  • It leads to the formation of firm, fibrinogen-rich plaques on mucous membranes, like ligneous conjunctivitis.
  • These plaques can be misdiagnosed by pathologists due to nonspecific findings.

Observation:

  • This study reports the first documented case of lymph node involvement in plasminogen deficiency.
  • The affected lymph nodes showed characteristic eosinophilic hyaline deposits.
  • These deposits stained positive with Periodic Acid Schiff and Masson trichrome, and contained fibrinogen.

Findings:

  • The patient's disease evolved over a 15-year period, highlighting the longitudinal nature of the condition.
  • Immunofluorescence confirmed the presence of fibrinogen within the hyaline deposits in the lymph nodes.
  • The study discusses the differential diagnosis for amorphous hyaline material found in lymph node biopsies.

Implications:

  • This case expands the known clinical manifestations of plasminogen deficiency.
  • Accurate identification of these hyaline deposits is crucial for timely diagnosis and management.
  • Understanding lymph node involvement aids in the differential diagnosis of similar conditions.

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