scholarly journals A Rare Presentation of Sarcoidosis with Nasal Bone Involvement

2016 ◽  
Vol 7 (1) ◽  
pp. ar.2016.7.0152 ◽  
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.

scholarly journals Granulomatous nephritis: A rare presentation of juvenile-onset sarcoidosis

2021 ◽  
Author(s):  
Prashant Chotalia ◽  
Sapan Pandya ◽  
Puja Srivastava
Keyword(s):  
Renal Failure ◽  
Renal Involvement ◽  
Cervical Lymphadenopathy ◽  
Unknown Origin ◽  
Oral Prednisolone ◽  
Pulmonary Involvement ◽  
Juvenile Onset ◽  
Rare Presentation ◽  
Pyrexia Of Unknown Origin ◽  
Renal Functions

ABSTRACT Sarcoidosis is rare in children. Incidence and prevalence of sarcoidosis in India are not known. Renal involvement in childhood sarcoidosis is further rare with no clear data about prevalence. Here we report a case of a 13-year-old girl who presented with sarcoidosis with multi-system involvement including renal sarcoidosis. She initially presented with pyrexia of unknown origin and cervical lymphadenopathy – evaluation of which led to diagnosis of sarcoidosis. Later, after development of pulmonary involvement, she was treated with oral prednisolone and azathioprine. She again defaulted on medicines and later presented with renal failure and was diagnosed with a renal sarcoidosis. She was treated with oral prednisolone and mycophenolate mofetil with which she gradually improved with normal renal functions.

scholarly journals Impact of valganciclovir therapy on severe IRIS-Kaposi Sarcoma mortality: an open-label, parallel, randomized controlled trial.

2021 ◽  
Author(s):  
Patricia Volkow ◽  
Leslie Chavez-Galan ◽  
Lucero Ramon-Luing ◽  
Judith Cruz-Velazquez ◽  
Patricia Cornejo-Juarez ◽  
...  
Keyword(s):  
Controlled Trial ◽  
Kaposi Sarcoma ◽  
Pulmonary Involvement ◽  
Skin Lesions ◽  
Attributable Mortality ◽  
Control Group ◽  
Open Label ◽  
Parallel Group ◽  
Pulmonary Lymph Node ◽  
The Difference

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.

scholarly journals Rare Presentation of Self-Limiting Kikuchi–Fujimoto Disease in Relapsing Nature

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Karo Servatyari ◽  
Hero Yazdanpanah ◽  
Chamara Dalugama
Keyword(s):  
Lymph Nodes ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Cervical Lymphadenopathy ◽  
Excision Biopsy ◽  
Presentation Of Self ◽  
Rare Presentation ◽  
Cervical Lymph Nodes ◽  
One Year ◽  
Loss Of Appetite

Background. Kikuchi–Fujimoto disease (KFD) is a rare, benign, and self-limiting disease that is commonly associated with cervical lymphadenopathy and fever. The disease has a wide spectrum of clinical manifestations, and definitive diagnosis is based on the histological appearance in the excision biopsy of the lymph nodes. Recurrence of KFD is reported rarely. Case Presentation. A 56-year-old Iranian woman with a background history of thrombocytopenia presented with fever, malaise, loss of appetite, and weight loss with cervical lymphadenopathy. The excision biopsy of the cervical lymph nodes confirmed the diagnosis of KFD, and she made a full recovery with improvement of symptoms, regression of cervical lymph nodes, and normalization inflammatory markers. One year after remission, she presented with similar clinico-biochemical profile, and repeat biopsy confirmed KFD. Conclusion. Although the rate of recurrence of the disease is very low, the treating physician should consider the possibility and confirm it histologically.

scholarly journals Prevalence, clinical features and treatment pattern of patients with concurrent diagnoses of rheumatoid arthritis and psoriatic disease: results of a 14-year retrospective study in a tertiary referral center

2019 ◽  
Vol 10 ◽  
pp. 204062231984790
Author(s):  
Kai-Lung Chen ◽  
Hsien-Yi Chiu ◽  
Jui-Hsiang Lin ◽  
Jian-De Ye ◽  
Yi-Hsuan Cho ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Clinical Features ◽  
Age Of Onset ◽  
Medical Center ◽  
Skin Lesions ◽  
Tertiary Referral ◽  
Female Ratio ◽  
Tertiary Referral Center ◽  
Referral Center ◽  
Psoriatic Disease

Background: Multiple comorbidities, including rheumatoid arthritis (RA), have been reported to be associated with psoriasis. Objective: This study aimed to determine the prevalence and the clinical features of RA among patients with psoriasis in a tertiary referral center. Methods: Between January 2000 and December 2013, all patients coded with psoriatic disease (ICD-9 CM 696.0 OR ICD-9 CM696.1) and RA (ICD-9 CM 714.0) in a tertiary medical center were enrolled. Results: There were 10,844 patients and 9073 patients with psoriatic disease and RA identified by diagnostic codes, respectively. Among patients with psoriasis, 111 patients had claim-based diagnosis of RA (1.02%). By reviewing medical records and telephone interview or clinic visits, 25 of the 111 patients (0.23%) was identified unequivocally as having concurrent RA. Among them, 17 (68%) were female and 16 (64%) patients developed arthritis prior to the onset of psoriasis with a mean lag of 6.3 years (1–19 years); 8 (32%) had psoriasis skin lesions prior to the onset of arthritis with a mean lag of 6.9 years (3–20 years); 1 (4%) had skin lesions and arthritis in the same time; 17 (68%) patients also fulfilled the CASPAR classification criteria for psoriatic arthritis. The mean age of onset for arthritis was 49.6 years old. Conclusions: The prevalence of RA in psoriasis might be overestimated in some previous studies using claimed database. Patients with concurrent RA and psoriasis showed a comparable age of onset and male to female ratio, but had more axial involvements compared to patients without psoriasis.

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

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
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.

scholarly journals A Comprehensive Clinical Description of Pediatric SARS-CoV-2 Infection in Western Pennsylvania

2020 ◽  
Author(s):  
Megan Culler Freeman ◽  
Kristina Gaietto ◽  
Leigh Anne DiCicco ◽  
Sherry Rauenswinter ◽  
Joseph R Squire ◽  
...  
Keyword(s):  
Primary Care ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Medical Center ◽  
Pediatric Primary Care ◽  
Mild Disease ◽  
Hispanic Patients ◽  
Western Pennsylvania ◽  
Clinical Description ◽  
Inflammatory Syndrome

Objective: We sought to characterize clinical presentation and healthcare utilization for pediatric COVID-19 in Western Pennsylvania (PA). Methods: We established and analyzed a registry of pediatric COVID-19 in Western PA that includes cases in patients <22 years of age cared for by the pediatric quaternary medical center in the area and its associated pediatric primary care network from March 11 through August 20, 2020. Results: Our cohort included 424 pediatric COVID-19 cases (mean age 12.5 years, 47.4% female); 65% reported exposure and 79% presented with symptoms. The most common initial healthcare contact was through telehealth (45%). Most cases were followed as outpatients, but twenty-two patients (4.5%) were hospitalized: 19 with acute COVID-19 disease, and three for multisystem inflammatory syndrome of children (MIS-C). Admitted patients were younger (p<0.001) and more likely to have pre-existing conditions (p<0.001). Black/Hispanic patients were 5.8 times more likely to be hospitalized than white patients (p=0.012). Five patients (1.2%) were admitted to the PICU, including all three MIS-C cases; two required BiPAP and one mechanical ventilation. All patients survived. Conclusions: We provide a comprehensive snapshot of pediatric COVID-19 disease in an area with low to moderate incidence. In this cohort, COVID-19 was generally a mild disease; however, ~5% of children were hospitalized. Pediatric patients can be critically ill with this infection, including those presenting with MIS-C.

scholarly journals Pyoderma Gangrenosum-like ulceration as a presenting feature of pediatric Granulomatosis with Polyangiitis

2020 ◽  
Author(s):  
Rotem Semo Oz ◽  
Oluwakemi Onajin ◽  
Liora Harel ◽  
Rotem Tal ◽  
Tomas Dallos ◽  
...  
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Newly Diagnosed ◽  
Rare Presentation ◽  
Case Presentation ◽  
Appropriate Treatment ◽  
The Mean ◽  
Systemic Symptoms

Abstract Background: Granulomatosis with polyangiitis (GPA) is an anti-neutrophilic cytoplasmic antibody-associated vasculitis affecting small to medium-sized vessels and involves most commonly the kidneys and the respiratory tract. Skin involvement can be seen in up to 50% of children with GPA and is the initial presenting symptom in 7.7%. Pyoderma gangrenosum (PG)-like ulcers are rarely described as a skin manifestation in GPA and very few cases have been reported previously in children. Case presentation: We describe 3 new pediatric cases of GPA with PG-like ulcerations. The mean age at first symptom was 15 years. Two patients had PG-like ulceration as their initial presentation; additional symptoms eventually led to the diagnosis of GPA 2-24 months later. In 1 case, proteinase 3 (PR3) was negative when first tested, but converted to positive when systemic symptoms emerged, in the other 2 cases PR3 was positive at presentation. All 3 patients had prominent facial lesions. None of the patients responded to treatment with antibiotics or medications commonly used to manage PG, including corticosteroids and cyclosporine. All patients had excellent responses to rituximab. An electronic database literature review was performed and 4 previously reported cases were identified. We assessed the clinical characteristics, serology, and response to treatment of 4 previously reported and newly diagnosed cases. Conclusion: PG-like ulceration is a rare presentation of pediatric GPA which may precede classic systemic GPA symptoms. The predominance of facial ulcer, granulomatous and neutrophilic inflammation on skin biopsy and lack of response to PG treatments are characteristic of GPA-associated PG-like ulcers. Our review suggests that treatment with rituximab may be needed to improve the skin lesions. Recognizing that PG-like ulcerations can occur in pediatric GPA may result in timely diagnosis, appropriate treatment and improved prognosis.

scholarly journals Acrokeratosis paraneoplastica Bazex syndrome associated with esophageal squamocellular carcinoma

2008 ◽  
Vol 65 (6) ◽  
pp. 485-487 ◽  
Author(s):  
Ljiljana Medenica ◽  
Mirjana Gajic-Veljic ◽  
Dusan Skiljevic ◽  
Predrag Pesko
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Cervical Lymphadenopathy ◽  
Skin Lesions ◽  
Specific Marker ◽  
Upper Aerodigestive Tract ◽  
Skin Changes ◽  
Underlying Malignancy ◽  
Acrokeratosis Paraneoplastica Bazex

Background. Acrokeratosis paraneoplastica Bazex (APB) is a very rare disease in the group of obligate paraneoplastic dermatoses, associated mostly with squamous cell carcinoma of the upper aerodigestive tract and metastatic cervical lymphadenopathy. The disease is characterized by violaceous erythemosquamous changes on the acral regions. This entity was first reported by Bazex in 1965. About 160 cases have been presented so far. Case report. We presented a patient with a three-month history of violaceous erythema, edema, erosions and scaling on the acral regions, elbows and knees and severe nail dystrophy. When the diagnosis was established, he did not have any symptom of internal malignancy. Esophagogastroscopy revealed ulcerovegetant lesion of the esophagus, while histology showed squamocellular invasive carcinoma. Surgical tumor removal resulted in significant improvement of skin changes in 15 days. Unfortunately, four months later, extensive skin lesions pointed to metastasis of squamous cell carcinoma. Conclusion. Skin changes can precede a few years the first manifestations of neoplasia. The course of the disease in our patient proved that APB is a specific marker of underlying malignancy.

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