Skin involvement is seen in approximately 40% of patients, and the rash consists of subcutaneous nodules (36%), dermal nodules (27%), maculopapular eruptions (15%), macular erythema (15%), and ulcers (7%). The rash is typically generalized but can be localized to the legs, trunk, face, or arms. The skin lesions can occur before, simultaneously, or after the lung lesions.
Central and peripheral nervous systems are involved in approximately one-third of patients. Signs of brain involvement include confusion, ataxia, hemiparesis, and seizures. Cranial nerve involvement can cause Bell palsy, diplopia, transient blindness, proptosis, deafness, and vertigo. Peripheral neuropathy is a common complaint and usually presents as symmetric paresthesias and weakness of the lower limbs.
LYG can also involve multiple organs: kidney, liver, brain, and spleen, and less commonly the adrenals, heart, pancreas, GI tract, and prostate.
Risk factors include a prior history of lymphoma or leukemia, especially acute lymphocytic or myeloid leukemia in remission, and various immunodeficiency states, including AIDS, variable immunodeficiency, X-linked agammaglobulinemia, hypogammaglobulinemia, and impaired T-cell function.
There have been patients with similar pathologies occurring after organ transplant or treatment with methotrexate for rheumatoid arthritis.
D47.Z9 – Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue
239940004 – Lymphomatoid granulomatosis
Differential Diagnosis & Pitfalls
- Posttransplant lymphoproliferative disorder – Very similar to LYG in a transplant patient.
- Granulomatosis with polyangiitis – Formation of true granulomas.
- Extranodal NK/T-cell lymphoma – T-cell lymphoma, while LYG is a disease of B cells.
- Hodgkin lymphoma
- Iatrogenic immunodeficiency-associated lymphoproliferative disorder
- Acute pulmonary histoplasmosis – Atypical lymphoid cells are absent; Gomori methenamine silver stain shows the organisms.
Drug Reaction Data