We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature. [ncbi.nlm.nih.gov]

Clinical presentation: Average age at presentation is about 50 years, predominantly in male. [histopathology-india.net]

• Generalized Lymphadenopathy

  Here in we describe a 52-year-old female who presented with symmetric polyarthritis and generalized stiffness for 7 months and papular lesions over extremities for 3 months duration. She in addition had generalized lymphadenopathy. [bioline.org.br]

• Fever

  We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. [ncbi.nlm.nih.gov]

  CASE REPORT A 33-yr-old female was hospitalised with a 3-month history of cough, dyspnoea, fever and skin rash. [erj.ersjournals.com]

• Weight Loss

  An 80-year-old woman presented with weight loss, fatigue, dizziness and a brain stem lesion. [ncbi.nlm.nih.gov]

  We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks’ duration. [mdedge.com]

  The commonest presenting picture includes fever, cough, rash/nodules, malaise, weight loss, neurologic abnormalities, dyspnoea, and chest pain (Katzenstein et al. 1979 ). [doi.org]

• Weakness

  An 87-year-old male had a week-long history of intermittent fever and general weakness. A chest radiograph showed multifocal patchy consolidations with nodular lesions. [ncbi.nlm.nih.gov]

  Immunophenotyping of peripheral blood was performed and showed positivity for: CD5, CD19, CD23, CD38, CD22, CD20 (weak), CD79b (weak) and CD11 (weak). Then, the diagnosis of CLL associated with ANCA vasculitis was confirmed. [scielo.br]

  EPOCH-R drugs can cause gastrointestinal problems, hair loss, and weakness. G-CSF can cause bone pain, body aches, and hair thinning. Chemotherapy can cause some patients to develop leukemia. [clinicaltrials.gov]

• Fatigue

  An 80-year-old woman presented with weight loss, fatigue, dizziness and a brain stem lesion. [ncbi.nlm.nih.gov]

  We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks’ duration. [mdedge.com]

  We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019 [patientslikeme.com]

  Constitutional symptoms, including weight loss, fatigue, sweating and chills, are typical ( 6 ). [spandidos-publications.com]

• Constitutional Symptom

  symptoms or multiple organ involvement (2/3 die within a year of pulmonary disease and infection) May progress to EBV+ diffuse large B cell lymphoma Gross description Lung nodules up to 10 cm with central necrosis and cavitation 15% of patients with [pathologyoutlines.com]

  What to be alert for in the history Lymphomatoid granulomatosis (LYG) should be considered when a patient presents with multiple papules, plaques or nodules, constitutional symptoms (fever, fatigue, night sweats, weight loss) and pulmonary symptoms (dyspnea [dermatologyadvisor.com]

  Classic clinical features are found in multiple organ systems: Nonspecific constitutional symptoms, such as fatigue, myalgias, weight loss, and fevers. [accessmedicine.mhmedical.com]

• Cough

  It most often affects the lungs, causing chest pains, cough, and shortness of breath. [medical-dictionary.thefreedictionary.com]

  We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. [ncbi.nlm.nih.gov]

  In addition to pulmonary involvement marked by cough, dyspnea, and occasionally hemoptysis, some patients exhibit nervous system and/or skin involvement. Clinically apparent involvement of other organ systems (e.g., kidney, liver) is less frequent. [clinicaladvisor.com]

• Dyspnea

  We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. [ncbi.nlm.nih.gov]

  In addition to pulmonary involvement marked by cough, dyspnea, and occasionally hemoptysis, some patients exhibit nervous system and/or skin involvement. Clinically apparent involvement of other organ systems (e.g., kidney, liver) is less frequent. [clinicaladvisor.com]

  He had been examined because of cough, dyspnea, and a skin rash 16 years previously and was subsequently diagnosed with LYG. [jamanetwork.com]

• Pleural Effusion

  Massive nodules, pleural effusions, pneumonitis, and cavitary nodules can be seen. Patients with hemoptysis are more likely to have cavitary nodules. [clinicaladvisor.com]

  As was the experience of others, the laboratory findings in our case were non-specific and although pleural effusion has been known to occur in LYG [12], commoner causes of pleural effusion in our environment like pneumonia, empyema and tuberculosis needed [nigeriamedj.com]

  Effusion lymphoma. CT scan shows a large pleural effusion in the right lower lobe. Pyothorax-associated lymphoma A pleural-based mass may be seen on chest x-ray. [pulmonologyadvisor.com]

  Pleural effusions (25%) are usually small and present as costophrenic angle blunting. [spandidos-publications.com]

• Pulmonary Disorder

  disorders characterized by necrotizing, aseptic granulomas, usually with angiitis. [karger.com]

  […] lymphomatoid granulomatosis (PLG), which has been defined as a clinicopathological entity is a rare Epstein–Barr virus (EBV)-driven lymphoproliferative pulmonary disorder. [journals.lww.com]

  Pulmonary involvement is almost always present, while other common sites include central nervous system (CNS) and skin.[ 2 ] Currently, LYG is considered an EBV related B-lymphoproliferative disorder,[ 3 ] similar to other lymphoproliferative disorders [mjhid.org]

• Rales

  This was slowly tapered, but on August 22, 1995, she suffered an acute decompensation with hypoxemia, tachypnea, poor color, splenomegaly, diffuse rales, and cardiac gallop. [baillement.com]

  On lung auscultation, breath sounds were diminished over the lower left lung, and there were no crackles or rales on either side. A neurological examination revealed no focal defect. [spandidos-publications.com]

  General physical examination revealed bilateral crackles and rales on both lung fields. There was no evidence of cardiac murmur, lymphadenopathy, hepatosplenomegaly, or skin lesion. [journals.lww.com]

• Chest Pain

  It most often affects the lungs, causing chest pains, cough, and shortness of breath. [medical-dictionary.thefreedictionary.com]

  We describe a 54‐year‐old female patient with rheumatoid arthritis (RA) and Sjögren's syndrome (SS) who presented with right chest pain and a large mass visible in the upper right field of a chest X‐ray. [doi.org]

  We describe a 54-year-old female patient with rheumatoid arthritis (RA) and Sjögren's syndrome (SS) who presented with right chest pain and a large mass visible in the upper right field of a chest X-ray. [ncbi.nlm.nih.gov]

  Patients present with vague respiratory symptoms (cough, dyspnoea, haemoptysis and chest pain) and are found to have pulmonary nodules and masses which may cavitate. [histopathology-india.net]

• Hypotension

  Three months later she presented in hypotension with dyspnea, tachypnea and altered sensorium. Her hemogram, renal and liver functions were normal and cultures (blood, urine) were negative for infection. [bioline.org.br]

• Blurred Vision

  Neurological findings may include: • Cranial and peripheral nerve defects resulting in unsteadiness, blurred vision, weakness or numbness affecting facial muscles or hands and feet; • Seizures; • Altered cognition; • Focal motor and sensory complaints [secure.ssa.gov]

  While on therapy, he developed bilateral blurred vision and diplopia for which he was referred to the university hospital for further evaluation. Upon transfer he noted a 6 pound unintentional weight loss during the past month. [medcraveonline.com]

• Night Sweats

  We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks' duration. [ncbi.nlm.nih.gov]

  We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks’ duration. [mdedge.com]

  sweats, 10% weight loss A = absent B = present Extranodal sites are also designated M+ = marrow L+ = lung H+ = liver P+ = pleura O+ = bone D+ = skin and subcutaneous tissue Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also [surgpathcriteria.stanford.edu]

  After clinical improvement, the patient was discharged from hospital with planned repeat MR imaging in one to two months.Six weeks later, she developed progressive abdominal pain, night sweats, and non-positional presyncopal symptoms. [doi.org]

  What to be alert for in the history Lymphomatoid granulomatosis (LYG) should be considered when a patient presents with multiple papules, plaques or nodules, constitutional symptoms (fever, fatigue, night sweats, weight loss) and pulmonary symptoms (dyspnea [dermatologyadvisor.com]

• Cutaneous Manifestation

  […] of lymphomatoid granulomatosis cutaneous manifestations of lymphomatoid granulomatosis History and etymology It was Initially described by Leibow et al. in 1972 5 . [radiopaedia.org]

  Cutaneous lymphomatoid granulomatosis: correlation of clinical and biologic features. Am J Surg Pathol 2001;25:1111-20 James WD, Odom RB, Katzenstein A. Cutaneous manifestations of lymphomatoid granulomatosis. Arch Dermatol 1981;117:196-202. [bioline.org.br]

  Cutaneous manifestations of lymphomatoid granulomatosis: Report of 44 cases and a review of the literature. Arch Dermatol 1981 ; 117: 196 – 202. Google Scholar | Crossref | Medline 6. Drasga, RE, Williams, SD, Wills, ER, Roth, LM, Einhorn, LH. [journals.sagepub.com]

  Characteristic findings on physical examination Cutaneous lesions are the initial manifestation in up to one third of patients. [dermatologyadvisor.com]

• Skin Ulcer

  Skin findings may include: • Small red bumps; • Lumps under the skin; • Ulcers; • Thickened patches; or • Inflamed hair follicles. Skin lesions usually do not cause symptoms but they can be tender or itchy. [secure.ssa.gov]

  Other organs frequently affected by Granulomatosis with Polyangiitis granulomatosis include the eye (proptosis and double-vision from retro-orbital pseudotumor, scleritis), skin (ulcers, purpura). or peripheral nerve (mononeuritis multiplex). [hopkinsvasculitis.org]

  […] the lower legs with ulceration (sometimes). [dovemed.com]

• Pruritus

  Our findings suggest that pruritus, a younger age at diagnosis, and a more indolent course are defining clinical features. [ncbi.nlm.nih.gov]

  Particular instances of concern include a pigmented, ulcerative lesion, especially on the lower extremities in women (melanoma), pruritus presenting with a non-healing wound (tumor stage of mycosis fungoides), fever and/or profound weight loss (cutaneous [o-wm.com]

• Peripheral Neuropathy

  Abstract A case of lymphomatoid granulomatosis of the lung is described in which the presenting features were a skin eruption and peripheral neuropathy. [thorax.bmj.com]

  Patients may be asymptomatic at the time of diagnosis, but up to 60% can present non-specific symptoms such as cough, fever, rash, subcutaneous nodules, asthenia and anorexia, dyspnea, chest pain, ataxia or peripheral neuropathy. [archbronconeumol.org]

  A 48-year-old woman with peripheral neuropathy, hypercalcemia, and pulmonary infiltrates. Chest 1998 ; 114 : 1205 –1209. ↵ Lymphomatoid granulomatosis. In : Travis WD, Colby TV, Corrin B, Shimosato Y, Brambilla E. [erj.ersjournals.com]

• Dizziness

  An 80-year-old woman presented with weight loss, fatigue, dizziness and a brain stem lesion. [ncbi.nlm.nih.gov]

  Other deficiency signs for Manganese include poor growth in children, hearing problems, ear noises, dizziness and poor coordination. Insufficient Manganese is suspected if an individual has very low cholesterol readings. [indianjmedsci.org]

  […] rise to severe anemia The leukemic cells can overgrow and cause clogging of vessels resulting in stroke, or blurring of vision There may be complications related to chemotherapy used in treating the condition, which may include: Side effects such as dizziness [dovemed.com]

• Asthenia

  Patients may be asymptomatic at the time of diagnosis, but up to 60% can present non-specific symptoms such as cough, fever, rash, subcutaneous nodules, asthenia and anorexia, dyspnea, chest pain, ataxia or peripheral neuropathy. [archbronconeumol.org]

  However, patients with LYG often suffer from systemic symptoms including fever, asthenia, night sweats and weigh loss (11-16, 28-30). [shortnessofbreath.it]

Broad infectious workup was negative. Given the time course of presentation and anterior accentuation of the lung infiltrates, the greatest clinical concern was radiation pneumonitis followed by drug toxicity. [mdedge.com]

Extensive infectious disease workup included negative lyme serology, cryptococcal antigen; HSV, acid fast stain and rapid plasma regain (RPR). [medcraveonline.com]

Additional workup showed negative antinuclear antibodies and antineutrophil cytoplasmic antibodies (ANCA). Two skin biopsies were performed, showing a dense T-cell lymphoid infiltrate, EBV-ISH negative. [mjhid.org]

• Multiple Pulmonary Nodules

  Lymphomatoid granulomatosis (LYG) is a rare lung disorder diagnosed by radiological imaging of multiple pulmonary nodules and occasionally induced by methotrexate (MTX) use. To date, the treatment of LYG has not been standardized. [ncbi.nlm.nih.gov]

  Vahid B, Salerno DA, Marik PE: Lymphomatoid granulomatosis: a rare cause of multiple pulmonary nodules. Respir Care 2008;53:1227–1229. [karger.com]

  pulmonary nodules Skin involved in nearly half of cases Dermal and subcutaneous nodules show similar features to lung May show nonspecific plaque like dermal infiltrate CNS and kidney also frequently involved High grade lesions with preponderance of [surgpathcriteria.stanford.edu]

  A chest radiograph revealed bilateral nodules of variable sizes predominantly in the lower lung fields, and a computed tomography (CT) scan of the thorax showed multiple small excavated pulmonary nodules with poorly defined margins (fig. 1 ⇓ ). [erj.ersjournals.com]

• Pulmonary Infiltrate

  Given the persistence of the nodular pulmonary infiltrates, emperic itraconazole was started. Over the next several weeks, continued improvement and resolution of the pulmonary infiltrates was noted (Fig 2). [baillement.com]

  We report the case of a 23-year-old man clinically presented with fever, sweating, and physical intolerance, and bilateral pulmonary infiltrates of nodular type and destructive changes on the chest X-ray, previously treated with antituberculotics for [ncbi.nlm.nih.gov]

  […] histoplasmosis Acute form Flu symptoms, pulmonary infiltrates, and serologic evidence of Histoplasma infection Lymphohistiocytic infiltrate with parenchymal necrosis and vasculitis (differential diagnosis with LYG grade 1) Small necrotizing granulomas [basicmedicalkey.com]

  A 48-year-old woman with peripheral neuropathy, hypercalcemia, and pulmonary infiltrates. Chest 1998 ; 114 : 1205 –1209. ↵ Lymphomatoid granulomatosis. In : Travis WD, Colby TV, Corrin B, Shimosato Y, Brambilla E. [erj.ersjournals.com]

• Bilateral Pulmonary Infiltrates

  We report the case of a 23-year-old man clinically presented with fever, sweating, and physical intolerance, and bilateral pulmonary infiltrates of nodular type and destructive changes on the chest X-ray, previously treated with antituberculotics for [ncbi.nlm.nih.gov]

  A crucial factor contributing to the frequent delay in diagnosis of LYG is its ability to mimic various infectious diseases, vasculitides, and malignancies. [5] [10] Bilateral pulmonary infiltrates or nodules are usually seen. [ijcasereportsandimages.com]

• Granulomatous Tissue

  Kradin-RL; Mark-EJ; Belley-G; Norris-PJ; Ton-F A 48-year-old man with a cough and bloody sputum - Wegener's granulomatosis, with microabscesses, capillaritis, granulomatous vasculitis, diffuse granulomatous tissue, and palisading granuloma. [malattierare.regione.veneto.it]

• Pleural Effusion

  Massive nodules, pleural effusions, pneumonitis, and cavitary nodules can be seen. Patients with hemoptysis are more likely to have cavitary nodules. [clinicaladvisor.com]

  As was the experience of others, the laboratory findings in our case were non-specific and although pleural effusion has been known to occur in LYG [12], commoner causes of pleural effusion in our environment like pneumonia, empyema and tuberculosis needed [nigeriamedj.com]

  Effusion lymphoma. CT scan shows a large pleural effusion in the right lower lobe. Pyothorax-associated lymphoma A pleural-based mass may be seen on chest x-ray. [pulmonologyadvisor.com]

  Pleural effusions (25%) are usually small and present as costophrenic angle blunting. [spandidos-publications.com]

A review of the literature regarding aetiology, clinical features, diagnosis and treatment options is presented. [ncbi.nlm.nih.gov]

The clinical response to treatment was very good, and a post-treatment CT chest shows complete resolution of lung changes. [doi.org]

R ecent study has shown a good response to treatment using interferon-alfa 2b, and there has also been a report of successful treatment with bone marrow transplantation. [histopathology-india.net]

The number of EBV(+) cells correlates with prognosis. This case was LYG grade 3. [basicmedicalkey.com]

Prognosis 64% mortality, median survival of 14 months; death is due to pulmonary destruction accompanied by sepsis; severe T-cell impairment may explain the malignant evolution of this condition. [medical-dictionary.thefreedictionary.com]

Advanced-stage disease does not tend to respond well to cytotoxic chemotherapy and is associated with a poor prognosis. We present a case of successful treatment of relapsed lymphomatoid granulomatosis with bexarotene, a novel retinoid agent. [ncbi.nlm.nih.gov]

No infectious etiology was identified. Following nondiagnostic lung and liver biopsies, the largest pulmonary mass was resected. The histopathologic findings were diagnostic of lymphomatoid granulomatosis. There was no residual B-ALL. [ncbi.nlm.nih.gov]

/ respiratory etiology (hemoptysis)/ progressive NHL References xxx [mdnxs.com]

TERMINOLOGY Abbreviations Wegener granulomatosis (WG) Definitions Systemic inflammatory process characterized by vasculitis and granulomatous reaction ETIOLOGY/PATHOGENESIS Etiology No definitive etiology is known for WG CLINICAL ISSUES Epidemiology Incidence [basicmedicalkey.com]

Etiology: - L ymphomatoid granulomatosis is closely associated with EBV. - P atients may have underlying defects in their cell-mediated immunity. [histopathology-india.net]

Epidemiology First described by Liebow in 1972 Mean age: 48 y/o (range: 7-85 y/o) Predisposed groups: M>F (>2:1) Associated with: Lymphoma, Sjogren’s, chronic viral hepatitis, RA, renal transplant Physiology Pulmonary Vasculitis Angiocentric and angio-destructive [mdnxs.com]

Her main area of interest is cancer epidemiology, with a particular focus on melanoma and other skin cancers. Nagwa M. Elwan is Professor of Dermatology at the Faculty of Medicine, Tanta University, Egypt. [books.google.es]

[…] immune surveillance Relationship between LYG and post-transplant lymphoproliferative disorders is unclear Clinical Associations Congenital and acquired immunodeficiencies Wiskott-Aldrich syndrome HIV infection High-dose chemotherapy CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Definition / general LyP is a chronic, self healing skin disease composed of large, atypical, anaplastic, immunoblastic or Hodgkin-like cells in a marked inflammatory background ( WHO 2008 ) Epidemiology Rare (0.1 - 0.2 cases per 100,000) Mostly adults [pathologyoutlines.com]

Its pathophysiology is unclear in numerous respects, thus making it difficult to diagnose and treat. We recently encountered a case of LYG that was followed clinically and histologically for 14 months. [ncbi.nlm.nih.gov]

Gain fresh perspectives and up-to-date insights from the world’s leading authorities on the pathophysiology, diagnosis, and management of stroke. [books.google.ro]

Etiology Pathophysiology LYG is an EBV-associated lymphoproliferative disorder. LYG may be more common in patients with immunodeficiency due to incomplete clearance of EBV and the resulting clonal proliferation of atypical EBV-infected B-cells. [dermatologyadvisor.com]

Prevention Little can be done to prevent this disease; however, avoiding the Epstein-Barr Virus (human herpesvirus 4) is one preventative measure. [knowcancer.com]

Stay up to date on hot topics such as mechanisms of action of commonly used drugs, neuronal angiogenesis and stem cells, basic mechanisms of spasm and hemorrhage, prevention of stroke, genetics/predisposing risk factors, and much more. [books.google.ro]

Since virtually all patients who did not have complete remission went on to have malignant lymphoma, early recognition and prompt treatment during the lymphomatoid-granulomatosis phase of disease may not only lead to complete remissions but also prevent [nejm.org]

Careful follow-up and monitoring of basic laboratory tests (eg, regularly obtaining complete blood cell counts) may prevent some adverse effects of treatment or minimize their impact. [accessmedicine.mhmedical.com]