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

The rareness of LYG in conjunction with its nonspecific presentation contributes to delays in diagnosis in many situations. [doi.org]

• Inguinal Lymphadenopathy

  CT scan revealed mediastinal (complex confluent subcarinal lymph node mass measuring 5.6 cm), aortopulmonary, paratracheal, right tracheobronchial, axillary, upper abdominal (1.5 cm), external ileac and right inguinal lymphadenopathy (largest 10.7 mm) [doi.org]

• 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]

• 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]

• Chills

  Alpha interferon can have flu-like side effects of headache, fever, chills, and body aches. EPOCH-R drugs can cause gastrointestinal problems, hair loss, and weakness. G-CSF can cause bone pain, body aches, and hair thinning. [clinicaltrials.gov]

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

  Furthermore, the patient presented with serious systemic involvement characterized by fever, chills and rigors, hypoproteinemia, neutrophilia, and altered liver function findings. [docksci.com]

  He denied any fever, chills, sputum production, orthopnea or paroxysmal nocturnal dyspnea, anorexia, weight loss, recent or past exposure to tuberculosis, sick contacts, pets, recent travel or past exposure to cigarette smoke (active or passive), asbestos [jhoonline.biomedcentral.com]

• Axillary Mass

  We report on a patient of rheumatoid arthritis (RA) who sequentially developed an axillary mass and a fatal interstitial pneumonia during a 2-year course of methotrexate (MTX) therapy. [ncbi.nlm.nih.gov]

  Hideto Kameda Ayumi Okuyama Jun-ichi Tamaru Shinji Itoyama Atsushi Iizuka Tsutomu Takeuchi Case Report First Online: 03 January 2007 Abstract We report on a patient of rheumatoid arthritis (RA) who sequentially developed an axillary mass and a fatal interstitial [doi.org]

• Cough

  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]

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

  Pulmonary Hypertension (see Pulmonary Hypertension, [[Pulmonary Hypertension]]): due to pulmonary vasculitis Pneumonia-like illness: presents with dyspnea/ cough/ sputum Endobronchial lesion: obstructive symptoms/ hemoptysis (may be massive) Pneumothorax [mdnxs.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]

  […] have been reported in some cases (bronchioles>lobar bronchi), may produce obstruction CBC: usually normal Dysproteinemia: usually absent Clinical Upper Airway Manifestations Pulmonary Manifestations Epidemiology: usually present Clinical Chest pain Dyspnea [mdnxs.com]

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

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

• Pleural Effusion

  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]

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

• Right Upper Quadrant Pain

  A twenty-six year old Caucasian woman with colonic Crohn disease on maintenance azathioprine therapy presented with right upper quadrant pain and fever. Diagnostic imaging revealed extensive liver, pulmonary and cerebral lesions. [ncbi.nlm.nih.gov]

  Case presentation A 26-year-old Caucasian woman presented with three days of right upper quadrant pain, fever, and malaise. [doi.org]

• Abdominal Tenderness

  Physical examination revealed focal right upper quadrant abdominal tenderness but no palpable masses, organomegaly or signs of ascites. [doi.org]

• Gingival Ulceration

  A 65-year-old Caucasian man with a gingival ulceration underwent a biopsy. The histological pattern was compatible with a grade III lymphomatoid granulomatosis. The staging revealed a nodular lesion in the lower lobe of his right lung. [ncbi.nlm.nih.gov]

• Hemianopsia

  A 65-year-old man complained of visual disturbance and homonymous hemianopsia was designated. CT and MRI revealed an edematous, enhanced irregular and nodular lesion in the right occipital and parietal lobes. [ncbi.nlm.nih.gov]

• Arm Weakness

  A 70-year-old woman with rheumatoid arthritis treated with methotrexate (MTX) complained of right arm weakness. On CT and MRI, tumors were found in the right frontal lobe, bilateral lungs, and left renal parenchyma. [ncbi.nlm.nih.gov]

• 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]

• Sparse Hair

  Abstract Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity. [ncbi.nlm.nih.gov]

• Hair Hypoplasia

  Abstract Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity. [ncbi.nlm.nih.gov]

• Sparse Hair

  Abstract Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity. [ncbi.nlm.nih.gov]

• Swelling of the Scrotum

  A 55-year-old gentleman with confirmed lymphomatoid granulomatosis on lung biopsy was noted to have a swelling in his scrotum. [ncbi.nlm.nih.gov]

• Testicular Swelling

  This resulted in resolution of the testicular swelling and his other symptoms. Prognosis with lymphomatoid granulomatosis depends mainly on grade. [ncbi.nlm.nih.gov]

• 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. [ncbi.nlm.nih.gov]

  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]

• Aphasia

  Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. [ncbi.nlm.nih.gov]

• Homonymous Hemianopsia

  A 65-year-old man complained of visual disturbance and homonymous hemianopsia was designated. CT and MRI revealed an edematous, enhanced irregular and nodular lesion in the right occipital and parietal lobes. [ncbi.nlm.nih.gov]

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]

• Right Pleural Effusion

  A small right pleural effusion and little pleural thickening/pleural reaction was reported in dependent parts of the left lower lobe (Figure 1 ). Figure 1 CT scan chest. [doi.org]

  The unit's admitting diagnosis was that of right pleural effusion of uncertain aetiology to rule out mesothelioma. A diagnostic thoracentesis using a size 21G hypodermic needle was dry. [nigeriamedj.com]

  A chest radiograph and computed tomography (CT) scan showed multifocal patchy consolidations with nodular lesions of variable sizes and irregular margins combined with right pleural effusion, but there was no hilar or mediastinal lymphadenopathy (Fig. [journals.lww.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]

• Kerley A Lines

  Chest x-ray revealed increased coarse, linear shadowing in the mid- and lower zones, prominent hila, bilateral Kerley B lines, and a pleural reaction on the left. Nodular shadows were present bilaterally, the largest measuring 18 mm. [doi.org]

• 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

  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]

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

If the disease is localized, radiation treatment can be useful. Surgery and chemotherapy has helped some patients. Other Treatment Options Bone marrow transplants Interferon-alfa 2b treatments Antiviral therapy Rituximab [knowcancer.com]

The disease typically always relapse after successful treatment due to inability of the immune system and current viral drugs to eliminate an EBV-infection. [en.wikipedia.org]

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]

Lymph node/ spleen (rarely involved): involvement does not affect prognosis Nasopharynx and upper airways (rarely involved, in contrast to Wegener’s) Other: fever/ malaise Aggressive Lymphoma: occurs in 15-25% of cases Treatment Asymptomatic Disease [mdnxs.com]

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

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]

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]

Lymphomatoid granulomatosis (LYG) is a rare tumor with unknown etiology. [spandidos-publications.com]

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]

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]

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.es]

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]

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]

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.es]

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]