Sclerosing Lipogranuloma with Multiple Skin Lesions and Pulmonary Involvement, Secondary to a Factitious Disorder

High HHV-8 viral load (VL) in Kaposi Sarcoma (KS) has been associated with severe Immune reconstitution inflammatory syndrome (S-IRIS-KS), which can occur after initiating cART, and is linked with high mortality particularly in patients with pulmonary involvement. We investigate if valganciclovir initiated before cART decreases HHV-8 VL and assess if it reduces the incidence of S-IRIS-KS and its attributable mortality. Methods: Open-label parallel-group randomized clinical trial in AIDS cART naive patients with disseminated KS (DKS) as defined by at least two of the following: pulmonary, lymph-node or gastrointestinal involvement, lymphedema, or equal or more 30 skin lesions. In the experimental group (EG), patients were randomized to valganciclovir 900 mg BID four weeks before cART and continued until week-48; in the control group (CG), cART was initiated on week-0. Non-severe-IRIS-KS was defined as: increase in the number of lesions plus equal or more than one log10 HIV-VL decrease or equal or more than 50 cells/mm3 increase or equal or more than 2-fold rise in baseline CD4+ cells. S-IRIS-KS was defined as abrupt clinical worsening of KS lesions and/or fever after ruling out another infection following cART initiation, and at least three of the following: thrombocytopenia, anemia, hyponatremia, or hypoalbuminemia. Results: 40 patients were randomized and 37 completed the study. In the ITT analysis, the overall mortality did not differ between groups. In the per-protocol analyses, the difference showed a trend for higher S-IRIS-KS mortality in the CG 3/19 (15.7%), compared to EG 0/18 (p=0.07). The incidence of S-IRIS KS was significantly lower in the EG; two patients, one each had S-IRIS-KS episode (0.038 per 100 patient-days) compared to CG group, four patients developed 12 S-IRIS-KS episodes (0.21 per 100 patient-days); incidence rate of 0.09 (95% CI 0.02-0.5 p=0.006). Mortality in patients with pulmonary KS was significantly lower in EG, 3/4 in CG vs 0/5 in EG. S-IRIS-KS was associated with higher HHV-8-VL; IL6 and CRP; valganciclovir was protective. Of survivors at week 48, 82% achieved more than 80% remission. No difference was found between groups in the number of non-S-IRIS-KS events. Conclusions: Valganciclovir significantly reduced the episodes of S-IRIS-KS although attributable KS mortality was lower in the EG the difference was not significant (p=0.07). Mortality was significantly lower in EG patients with pulmonary KS.

Download Full-text

Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature

Case Reports in Radiology ◽

10.1155/2018/2952084 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Rashid AL Umairi ◽

Danielle Blunt ◽

Wedad Hana ◽

Matthew Cheung ◽

Anastasia Oikonomou

Keyword(s):

Langerhans Cell Histiocytosis ◽

Corticosteroid Treatment ◽

Pulmonary Involvement ◽

Skin Lesions ◽

Langerhans Cell ◽

Sinus Histiocytosis ◽

Review Of The Literature ◽

Massive Lymphadenopathy ◽

Pulmonary Langerhans Cell Histiocytosis ◽

Rosai Dorfman Disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

Download Full-text

Severe but reversible pulmonary hypertension in scleromyxedema and multiple myeloma: a case report

BMC Pulmonary Medicine ◽

10.1186/s12890-019-1020-6 ◽

2020 ◽

Vol 20 (1) ◽

Cited By ~ 3

Author(s):

Mazen Kreidy ◽

Ali Al-Hilli ◽

Ralph Yachoui ◽

Jeffrey Resnick

Keyword(s):

Multiple Myeloma ◽

Pulmonary Hypertension ◽

Connective Tissue ◽

Diuretic Therapy ◽

Pulmonary Involvement ◽

Skin Lesions ◽

Underlying Disease ◽

Connective Tissue Disorder ◽

Plasma Cell Dyscrasia ◽

Cell Counts

Abstract Background Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels. Pulmonary involvement occurs in a subset of patients, though the overall prevalence of pulmonary lesions in scleromyxedema is unknown. Since pulmonary hypertension presumably occurs in these patients due to disease progression and development of additional conditions, treatment of the underlying plasma cell dyscrasia and connective tissue disorder may improve pulmonary hypertension symptoms. Case presentation An elderly patient with scleromyxedema developed pulmonary hypertension refractory to vasodilator and diuretic therapy and subsequently multiple myeloma that responded to a combination therapy of bortezomib, cyclophosphamide, and dexamethasone treatment. Conclusions Treatment of the underlying disease(s) that contributed to pulmonary hypertension development with anti-neoplastic agents like bortezomib may improve cardiopulmonary symptoms secondary to reducing abnormal blood cell counts and paraprotein levels.

Download Full-text

Blurry Vision, Hearing Loss, and Constant Headache

Neuroimmunology ◽

10.1093/med/9780190693190.003.0009 ◽

2018 ◽

pp. 49-54

Author(s):

Aaron E. Miller ◽

Tracy M. DeAngelis ◽

Michelle Fabian ◽

Ilana Katz Sand

Keyword(s):

Pulmonary Involvement ◽

Giant Cells ◽

Skin Lesions ◽

Neurological Involvement ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Initial Symptoms ◽

Blurry Vision ◽

Systemic Manifestations ◽

Noncaseating Granulomas

Sarcoidosis is a multisystem inflammatory disorder of unknown etiology, characterized pathologically by the presence of noncaseating granulomas and multinucleated giant cells. For unknown reasons, the disorder tends to be more common among African Americans and Scandinavians. Among the most common systemic manifestations are pulmonary involvement, lymphadenopathy, and skin lesions. Nervous system involvement occurs in 5–10% of cases. Despite the rarity of neurological involvement, 50–70% of the time, the neurological symptoms are the initial symptoms. In most patients with neurosarcoid, there is evidence for involvement in other systems, either at presentation or during the course of the condition. The presentation in neurosarcoidosis is varied. Steroids are the first-line treatment, but some patients may also require steroid-sparing agents to control the condition.

Download Full-text

A Rare Presentation of Sarcoidosis with Nasal Bone Involvement

Allergy & Rhinology ◽

10.2500/ar.2016.7.0152 ◽

2016 ◽

Vol 7 (1) ◽

pp. ar.2016.7.0152 ◽

Cited By ~ 1

Author(s):

Mukadder Korkmaz ◽

Selen Uslu ◽

Hakan Korkmaz ◽

Yeliz Çetinkol

Keyword(s):

Medical Center ◽

Nasal Bone ◽

Cervical Lymphadenopathy ◽

Pulmonary Involvement ◽

Skin Lesions ◽

Dramatic Improvement ◽

Rare Presentation ◽

Susceptible Host ◽

Clinical Description ◽

Necrotizing Granulomas

Background Sarcoidosis is a multisystem granulomatous inflammatory disease that is induced by infectious or noninfectious environmental antigens in a genetically susceptible host. Tuberculosis and sarcoidosis are two diseases with similar clinical and pathologic findings. The link between these two diseases has been extensively studied. Objective Herein we describe a case of sarcoidosis associated with tuberculosis, treated for tuberculosis, and, 1 year, later presented with a nasal dorsal lump and skin lesions on the extremities. Methods Case report with clinical description. Results Our patient had a history of skin and cervical lymphadenopathy symptoms 1 year earlier and was treated with antituberculosis drugs in an outer medical center. Therapy had cured cervical lymphadenopathies, with no improvement in skin lesions. On appearance of the nasal dorsal lump, she presented to our outpatient clinic. We retrieved the previous specimens of the patient, which revealed coexistence of necrotizing granulomas with non-necrotizing granulomas, which was strongly indicative of the coexistence of tuberculosis and sarcoidosis. Radiologic, histopathologic, and microbiologic investigation revealed the diagnosis of sarcoidosis with nasal, cutaneous, and pulmonary involvement. Treatment with prednisolone and hydroxychloroquine resulted in dramatic improvement of nasal bone, pulmonary, and skin lesions within 2 weeks. Conclusion The clinical presentation of sarcoidosis can be complex, and the differential diagnosis from tuberculosis can be challenging. Atypical clinical pictures also can cause delays in diagnosis and proper management. In patients with granulomatous lesions that are unresponsive to antituberculosis therapy, physicians must be alerted to the possibility of coexistent sarcoidosis.

Download Full-text

CS-7 A case of Lymphomatoid granulomatosis with skin, lung, and intracranial lesions due to multicentric development

Neuro-Oncology Advances ◽

10.1093/noajnl/vdab159.109 ◽

2021 ◽

Vol 3 (Supplement_6) ◽

pp. vi28-vi28

Author(s):

Satoru Komaki ◽

Takuya Furuta ◽

Tetsuya Negoto ◽

Mayuko Moritsubo ◽

Hideo Nakamura ◽

...

Keyword(s):

Pulmonary Involvement ◽

Skin Lesions ◽

Lymphoproliferative Disease ◽

Host Immunity ◽

Surgical Removal ◽

Pathological Findings ◽

Lower Back ◽

Multiple Metastases ◽

Intracranial Lesions ◽

Pulmonary Masses

Abstract Introduction: LYG is very rare tumor and composed of large EB-positive B cells and reactive T cells. In this study, we experienced a case of LYG with multiple intracranial, cutaneous, and pulmonary masses. We report the pathogenesis and pathophysiology of LYG, including a discussion of the literature. case: A 69-year-old female presented with a growing lump in her lower back that had been present for several years. Six months later, she was found to have multiple masses in her lungs and intracranial region and underwent surgical removal for diagnostic purposes. Intraoperative findings: The tumor was substantial, reddish to grayish-white in color, and the margins of the tumor were whitish and hard, with some areas that could not be detached. Pathological findings: There were no atypical lymphocytes, and a small number of EBER-positive cells were observed. IgVH PCR: IgVH PCR was performed on the skin lesions and intracranial lesions, and bands of different sizes were detected, suggesting that the IgVH clone was present in the polyclonal region. Finally, we diagnosed LYG grade 1. discussion: EB-associated lymphoproliferative disease can lead to polyclonal reactive growth or monoclonal neoplastic growth depending on the balance between morphology and host immunity. The results of IgVH PCR suggest that the skin lesions did not cause multiple metastases, but rather that the enlargement of the skin lesions triggered intracranial and pulmonary lesions in an allo-centric manner. The results of IgVH PCR suggested that the skin lesions did not cause multiple metastases, but rather that the skin lesions grew to cause intracranial and pulmonary involvement in an other-centric manner.

Download Full-text

Sezary syndrome with pulmonary involvement: a case report

International Journal of Scientific Reports ◽

10.18203/issn.2454-2156.intjscirep20162168 ◽

2016 ◽

Vol 2 (7) ◽

pp. 168

Author(s):

Rohit Mukherjee ◽

Ashwin Songara ◽

Swapnil Shikha ◽

Awadhesh P.S. Solanki

Keyword(s):

T Cell ◽

Mycosis Fungoides ◽

Cell Lymphoma ◽

Pulmonary Involvement ◽

Skin Lesions ◽

Sézary Syndrome ◽

Sezary Syndrome ◽

Radiographic Findings ◽

Cell Clones ◽

End Stage

Mycosis fungoides and sezary syndrome are the type of cutaneous T cell lymphoma characterized by localization of malignant T-lymphocytes in the skin at presentation. Mycosis fungoides (MF) is characterized by an epidermotropic skin infiltrate of atypical CD4-positive helper T-cell clones, and sezary syndrome (SS), is characterized by erythroderma and leukemia. Here we are presenting a case of 50 year old male who presented with extensive skin lesions along with breathlessness since 25 days and fever, was misdiagnosed as end stage sarcoidosis on CT chest, but later proved to be pulmonary involvement of sezary syndrome. The objective of our study is to describe our experience identifying lung involvement by CTCL on the basis of clinical and radiographic findings.

Download Full-text

Skin Lesions and Lower Extremity Edema in a newly diagnosed HIV patient

Journal of Advances in Internal Medicine ◽

10.3126/jaim.v1i1.5833 ◽

1970 ◽

Vol 1 (1) ◽

pp. 12-15

Author(s):

Andrew B Trotter ◽

Debra D Poutsiaka

Keyword(s):

Differential Diagnosis ◽

Lower Extremity ◽

Highly Active Antiretroviral Therapy ◽

Kaposi Sarcoma ◽

Pulmonary Involvement ◽

The United States ◽

Skin Lesions ◽

Newly Diagnosed ◽

Mortality And Morbidity ◽

Highly Active

The differential diagnosis of skin lesions in human immunodeficiency virus patients is diverse and includes both infectious and non infectious causes. In this case presentation, a 37 year old male newly diagnosed with HIV presented to a hospital in the United States with skin lesions and worsening lower extremity swelling. After presentation, he developed respiratory distress and was found to have disseminated Kaposi sarcoma with cutaneous and pulmonary involvement. Kaposi sarcoma has decreased in incidence in the era of highly active antiretroviral therapy. However, it remains an important acquired immunodeficiency syndrome defining illness which may be the presenting complaint in a patient with occult HIV infection. Kaposi sarcoma has substantial mortality and morbidity and should be included in the differential diagnosis of skin lesions in a HIV positive patient. DOI: http://dx.doi.org/10.3126/jaim.v1i1.5833 Journal of Advances in Internal Medicine. 2012; 1(1): 12-15

Download Full-text

Case Report: Pulmonary Kaposi Sarcoma in a non-HIV patient

F1000Research ◽

10.12688/f1000research.7137.1 ◽

2015 ◽

Vol 4 ◽

pp. 1013 ◽

Cited By ~ 3

Author(s):

Arber Kodra ◽

Maciej Walczyszyn ◽

Craig Grossman ◽

Daniel Zapata ◽

Tarak Rambhatla ◽

...

Keyword(s):

Bronchoalveolar Lavage ◽

Skin Diseases ◽

Case Reports ◽

Kaposi Sarcoma ◽

B Cell Lymphoma ◽

Pulmonary Involvement ◽

Skin Lesions ◽

Human Herpes Virus 8 ◽

Bronchoscopic Biopsy ◽

Immunodeficiency Syndrome

Kaposi Sarcoma (KS) is an angioproliferative tumor associated with human herpes virus 8 (HHV-8). Often known as one of the acquired immunodeficiency syndrome (AIDS)-defining skin diseases, pulmonary involvement in KS has only been discussed in a handful of case reports, rarely in a non-HIV patient. Herein we report the case of a 77 year-old- male who presented with a 6-week history of progressive dyspnea on exertion accompanied by productive cough of yellow sputum and intermittent hemoptysis. His past medical history was significant for Non-Hodgkin’s Follicular B-Cell Lymphoma (NHL). Patient also had biopsy-confirmed cutaneous KS. His physical exam was notable for a 2cm firm, non-tender, mobile right submandibular lymph node. Lungs were clear to auscultation. He had multiple violet non-tender skin lesions localized to the lower extremities. CT scan of the chest showed numerous nodular opacities and small pleural effusions in both lungs. A thoracenthesis was performed, showing sero-sanguineous exudative effusions. Histopathology failed to demonstrate malignant cells or lymphoma. A subsequent bronchoscopy revealed diffusely hyperemic, swollen mucosa of the lower airways with mucopurulent secretions. Bronchoalveolar lavage PCR for HHV-8 showed 5800 DNA copies/mL. It was believed that his pulmonary symptoms were likely due to disseminated KS. This case illustrates the potential for significant lung injury from KS. It also demonstrates the use of PCR for HHV-8 to diagnose KS in a bronchoalveolar lavage sample in a case when bronchoscopic biopsy was not safe. Furthermore, this case is unique in that the patient did not match the typical KS subgroups as HIV infection and other immune disorders were ruled out. Recognition of this syndrome is critical to the institution of appropriate therapy. As such, this case should be of interest to a broad readership across internal medicine including the specialties of Pulmonology and Critical Care.

Download Full-text