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[Lymphomatoid granulomatosis vs. leprosy].

S J Adad1, R N Fleury, A Nunes

  • 1Depto. de Patologia, Faculdade de Medicina do Triâgulo Mineiro, Uberaba, MG.

Hansenologia Internationalis
|June 1, 1989
PubMed
Summary

Lymphomatoid granulomatosis (LYG) is a rare condition affecting lungs, skin, and other organs, often mistaken for leprosy. Early treatment with chemotherapy may improve outcomes for this T-cell lymphoma variant.

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Area of Science:

  • Pathology
  • Oncology
  • Dermatology

Background:

  • Lymphomatoid granulomatosis (LYG) is a rare lymphohistiocytic infiltrate characterized by granulomatous, polymorphous, pleiomorphic, angiocentric, and angiodestructive features.
  • LYG primarily affects the lungs, skin, kidneys, and central nervous system, sparing the spleen, bone marrow, and lymph nodes.
  • Currently, LYG is considered an angiocentric variant of T-cell peripheral lymphoma, histologically similar to polymorphic reticulosis of the upper airways.

Observation:

  • Skin lesions are the most common extrapulmonary manifestation, preceding pulmonary involvement in 13-34% of cases.
  • Cutaneous lesions present variably, often as erythematous or violaceous plaques or annular lesions with central clearing.
  • Differential diagnosis includes granuloma annulare, sarcoidosis, and Hansen's disease, particularly given LYG's potential for cutaneous nerve involvement and sensory disturbances.

Findings:

  • A case report details a 42-year-old male with cutaneous LYG and systemic manifestations, initially misdiagnosed as tuberculoid leprosy due to granulomatous and perineural infiltration.
  • Necropsy revealed polymorphous, pleiomorphic, and angiocentric infiltrates in the skin, CNS, heart, digestive tract, liver, prostate, testes, lungs, and kidneys.
  • Extensive nodules and necrotic areas were observed in the lungs and kidneys, consistent with advanced LYG.

Implications:

  • Accurate differential diagnosis is crucial, especially in regions where Hansen's disease is prevalent, due to overlapping clinical and histological features.
  • Prompt recognition and treatment with agents like cyclophosphamide and prednisone may lead to remission in early-stage LYG.
  • This case underscores the aggressive nature of LYG and highlights the importance of comprehensive histopathological examination in diagnosing systemic involvement.

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