ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (18)
Lymphomatoid granulomatosis - Skin
Print
Other Resources UpToDate PubMed

Lymphomatoid granulomatosis - Skin

Print Images (18)
Contributors: Jason Birnbaum, Lowell A. Goldsmith MD, MPH, Art Papier MD, William Van Stoecker MD
Other Resources UpToDate PubMed

Synopsis

Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-related B-cell lymphoproliferative disorder of the lungs with variable involvement of skin, central nervous system, liver, and kidneys. It mainly affects middle-aged adults with a mean age of 48 years and a male to female ratio of 2:1; rarely, children are affected. The most common presenting symptoms are fever (60%), cough (56%), malaise (35%), weight loss (35%), dyspnea (29%), and chest pain (13%). Less common symptoms include arthralgias, myalgias, and gastrointestinal (GI) complaints. LYG causes greater than 50% mortality, with most deaths occurring within 2 years of diagnosis. Cause of death is usually extensive destruction of the pulmonary parenchyma.

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 rheumatoid arthritis treated with methotrexate.

Codes

ICD10CM:
D47.Z9 – Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue

SNOMEDCT:
239940004 – Lymphomatoid granulomatosis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required

References

Subscription Required

Last Updated: 11/03/2017
Copyright © 2018 VisualDx®. All rights reserved.
Lymphomatoid granulomatosis - Skin
Print 18 Images Filter Images
View all Images (18)
(with subscription)
 Reset
Lymphomatoid granulomatosis : Cough, Fever, Rash, Hypoxemia, Malaise, Mental status alteration, Respiratory alkalosis, Dyspnea
Clinical image of Lymphomatoid granulomatosis
Copyright © 2018 VisualDx®. All rights reserved.