Sarcoidosis as a Great Mimicker: Diagnostic Challenges in a Patient With Coccidioidomycosis.

Sarcoidosis presents as a systemic granulomatous disease of unknown etiology, characterized by the development of non-caseating granulomas that commonly affect the lungs, lymph nodes, skin, and eyes. Manifestations of various conditions such as infections, neoplasms, autoimmune, cardiovascular, and drug-induced diseases can bear resemblance to sarcoidosis. Coccidiosis, attributed to protozoan parasites of the Coccidia genus, primarily affects the intestinal tract but may also display systemic symptoms akin to those of sarcoidosis. In this particular case, we present a 46-year-old immunocompetent gentleman who had an extensive disease despite the patient's well-controlled diabetes and absence of residency in an endemic area with fungal infection, his only pertinent part of the history was his travel to endemic areas for short periods that raised the possibility of thinking about the disseminated fungal infection. The patient's symptoms initially attributed to and treated as sarcoidosis, which later did not respond to steroids, led us to consider other potential causes, including systemic fungal infection  Misdiagnosis of the sarcoidosis bears the risk of inappropriate treatment, potentially leading to exacerbated patient outcomes. Consequently, it is imperative for healthcare providers, particularly rheumatologists, to augment vigilance and conduct comprehensive diagnostic assessments encompassing microbiological testing and histopathological examination.

Sarcoidosis: a general overview.

Sarcoidosis is a systemic inflammatory disease of unknown origin, which consists of the formation of multiple sterile noncaseating granulomas. Inhaled antigens are believed to initiate disease in prone individuals, considering that almost all patients present pulmonary or mediastinal lymph node disease. Extrapulmonary manifestations are common and diverse: practically any organ system can be affected, and treatment can range from simple watchful waiting to intense immunosuppression. In this article, we review current concepts about sarcoidosis in an overview, focusing on recognition and treatment of its major clinical phenotypes.

Granulomatous Lymphocyte Interstitial Lung Disease: A Rare Complication of Common Variable Immunodeficiency Managed With Azathioprine and Rituximab.

Granulomatous lymphocytic interstitial lung disease (GL-ILD) is a rare, non-infectious pulmonary manifestation of common variable immunodeficiency (CVID). Diagnosing and managing GLILD remains challenging due to its poorly understood pathogenesis and high mortality. We present a complex case of a young female with CVID associated with lung and spinal cord involvement managed with azathioprine and rituximab.

Facial Granulomatous Rosacea: A Case Report.

Granulomatous rosacea is a chronic inflammatory skin disease. We present the case of a 30-year-old woman with a three-month history of erythematous monomorphic papules and nodules on the cheeks and forehead. Histopathological examinations revealed tuberculoid granulomas with multinucleated giant cells. Granulomatous rosacea should be differentiated from other similar granulomatous skin diseases such as cutaneous sarcoidosis and Lupus miliaris disseminates faciei.

Cutaneous Sarcoidosis: A Differential Diagnosis to Consider in Undiagnosed Skin Lesions.

The presentation of sarcoidosis varies depending on the organs involved. Cutaneous sarcoidosis usually presents with other organ involvement, but isolated presentation is possible. However, diagnosing isolated cutaneous sarcoidosis can be challenging in resource-poor countries, particularly where sarcoidosis is relatively uncommon, since cutaneous sarcoidosis usually does not cause troublesome symptoms. We present a case of cutaneous sarcoidosis in an elderly female who had been suffering from skin lesions for nine years. The diagnosis was made after the appearance of lung involvement, which raised the suspicion of sarcoidosis and prompted a skin biopsy. The patient was then treated with systemic steroids and methotrexate, and her lesions improved shortly thereafter. This case highlights the importance of considering sarcoidosis as a possible cause of undiagnosed, refractory cutaneous lesions.

Childhood sarcoidosis in the middle Anatolia of Turkey.

OBJECTIVE: This retrospective study aimed to describe the clinical and radiological features, diagnostic methods, laboratory findings, organ involvement, and treatment strategies of 22 patients who followed up with late-onset childhood sarcoidosis and compare them with the literature. MATERIAL AND METHOD: This retrospective multicenter study reviewed the medical records of 22 children with sarcoidosis who applied to the Pediatric pulmonology department of Erciyes University Faculty of Medicine and Necmettin Erbakan Faculty of Medicine in 2012 and 2022. RESULTS: The mean age of the patients at the time of diagnosis was 13.1/year (interquartile range [IQR]1:6.3-[IQR]3:15.7). The most common first presenting symptom was cough 40.9% (n = 9), weight loss 31.8% (n = 7), and dyspnea 22.7% (n = 5). There were elevated levels of C-reactive protein (CRP; 59%), angiotensin-converting enzyme (ACE; 54.5%), erythrocyte sedimentation rate (ESR; 54.5%), and immunoglobulin G (IgG; 54.5%). Twenty patients (90%) received systemic steroid treatment. Eighteen (81.8%) of these patients responded positively to treatment. Two patients had a recurrence. CONCLUSION: The incidence of sarcoidosis in children in Turkey is currently unknown. However, a regional average of 2.2 cases per year has been documented for the first time. Contrary to previous studies, a significant prevalence of consanguineous marriage was observed in our study. While constitutional symptoms were most common in other studies, the cough was the most common symptom in our study. To our knowledge, this is one of the Turkish studies with the highest number of sarcoidosis in children and one of the few European studies on sarcoidosis in children.

Sarcoid-Like Reactive Lymphadenopathy in Metastatic Synovial Sarcoma.

A 56-year-old male underwent treatment for sarcoma with metastases to the lungs. Follow-up imaging revealed multiple pulmonary nodules and masses with a favorable response on positron emission tomography (PET) scanning showing enlarging mediastinal lymph nodes concerning for progression of the disease. To evaluate the lymphadenopathy, the patient underwent bronchoscopy with endobronchial ultrasound and transbronchial needle aspiration. The lymph nodes were negative for cytology but showed granulomatous inflammation. Granulomatous inflammation is a rare occurrence in patients with concurrent metastatic lesions and is exceedingly rare in cancers that have not originated in the thorax. This case report highlights the clinical significance of sarcoid-like reactions in the mediastinal lymph nodes and the need for further investigation.

Drug-induced sarcoidosis-like reaction three months after BNT162b2 mRNA COVID-19 vaccination: A case report and review of literature.

BACKGROUND: A 70-year-old man with hepatitis C virus-related recurrent hepatocellular carcinoma was admitted for further diagnosis of a 1 cm iso-hyperechoic nodule in segment (S) 5. CASE SUMMARY: Gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI) revealed the nodule in S5 with a defect at the hepatobiliary phase, hyperintensity on diffusion weighted imaging (DWI) and hypointensity on apparent diffusion coefficient (ADC) map. Contrast-enhanced computed tomography revealed hypervascularity at the early phase, and delayed contrast-enhancement was observed at the late phase. Contrast-enhanced ultrasound (US) revealed incomplete defect at the late vascular phase. Inflammatory liver tumor, lymphoproliferative disease, intrahepatic cholangiocarcinoma (small duct type) and bile duct adenoma were suspected through the imaging studies. US guided biopsy, however, showed a noncaseating hepatic sarcoid-like epithelioid granuloma (HSEG), and histopathological analysis disclosed spindle shaped epithelioid cells harboring Langhans-type multinucleated giant cells. One month after admission, EOB-MRI signaled the disappearance of the defect at the hepatobiliary phase, of hyperintensity on DWI, of hypointensity on ADC map, and no stain at the early phase. CONCLUSION: That the patient had received BNT162b2 messenger RNA (mRNA) coronavirus disease 2019 vaccination 3 mo before the occurrence of HSEG, and that its disappearance was confirmed 4 mo after mRNA vaccination suggested that the drug-induced sarcoidosis-like reaction (DISR) might be induced by the mRNA vaccination. Fortunately, rechallenge of drug-induced DISR with the third mRNA vaccination was not confirmed.

COVID-19 mRNA Vaccine-Associated Uveitis Leading to Diagnosis of Sarcoidosis: Case Report and Review of Literature.

A 34-year-old Japanese person with male gender identity who had been taking intramuscular injection of methyltestosterone depot for 11 years after bilateral mastectomy noticed blurred vision 5 days after the second vaccination for COVID-19 (Tozinameran; Pfizer-BioNTech) in the interval of 3 weeks following the first vaccination. The patient was diagnosed as granulomatous iritis with mutton-fat keratic precipitates and small iris nodules at the pupillary margin in the right eye and began to have 0.1% betamethasone eye drops with good response. The patient, however, continued to have fever and malaise and showed a high level of serum soluble interleukin-2 receptor (sIL-2R) even 4 weeks after the second vaccination. Computed tomographic scan disclosed mediastinal and bilateral hilar small lymphadenopathy together with limited granular lesion in the right lung. Gallium-67 scintigraphy demonstrated high uptake not only in mediastinal and hilar lymph nodes but also in bilateral parotid glands. Right parotid gland biopsy revealed noncaseating granulomas and proved pathological diagnosis of sarcoidosis. The systemic symptoms were relieved by oral prednisolone 20 mg daily. Even though the causal relationship remains undetermined, this case is unique at the point that vaccine-associated uveitis led to the detection of pulmonary lesions and lymphadenopathy, resulting in clinical and pathological diagnosis of sarcoidosis. In literature review, 3 patients showed sarcoidosis-like diseases after COVID-19 vaccination: 2 patients were diagnosed clinically as Lofgren syndrome with acute onset of erythema nodosum and ankle swelling, with or without mediastinal and hilar lymphadenopathy, whereas 1 patient with mediastinal lymphadenopathy but no uveitis was diagnosed pathologically by biopsy as sarcoidosis.

Chorioretinal Biopsy-Proven Ocular Sarcoidosis in a Patient with a History of B-Cell Lymphoma.

The purpose of this report is to describe biopsy-proven ocular sarcoidosis (OS) in a 67-year-old patient with a history of sarcoidosis and diffuse large B-cell lymphoma (DLBCL). Nonspecific posterior chorioretinal lesions in a patient with prior malignancy necessitated chorioretinal biopsy to rule out metastatic lymphoma. The association between sarcoidosis and malignancy remains unclear and can complicate management of similar patients with nonspecific posterior segment findings. Chorioretinal biopsy may, therefore, be required to rule out malignancy in patients with a leading history.

Crohn's Disease of the Vulva: a Case Report.

Crohn's disease is a multi-systemic chronic inflammatory disease that can affect various organs besides the gastrointestinal tract such as joints, uvea, and the skin. Vulvar Crohn's disease is a rare entity occurring with vulvar lesions that show typical Crohn's disease granulomatous inflammation but are not contiguous with the gastrointestinal involvement. Vulvar Crohn's disease can be easily confused with other granulomatous diseases and awareness that such involvement may precede gastrointestinal symptoms must be raised. Few cases of vulvar Crohn's disease have been reported in the literature to date. Here, we report a case of a 43-year-old woman with a 6-month history of a vulvar lesion; the patient was diagnosed with Crohn's disease of the large bowel just over a year ago.

The Role of Propionibacterium acnes in the Pathogenesis of Sarcoidosis and Ulcerative Colitis: How This Connection May Inspire Novel Management of These Conditions.

A lesser-acknowledged role of Propionibacterium acnes is its effect on the development of sarcoidosis. This literature review not only further explores this association but also that of Propionibacterium acnes and other inflammatory conditions, such as ulcerative colitis and pyoderma gangrenosum, acne, ulcerative colitis syndrome (PAC syndrome). This article reviews the effect that isotretinoin, a commonly used treatment of acne, has on the pathogenesis of ulcerative colitis, and the immune dysregulation and genetic susceptibility of individuals prone to developing acne, sarcoidosis, and ulcerative colitis. Literature for this article review was obtained from PubMed by utilizing both regular keywords and medical subject heading (MeSH) subheadings for data gathering. Regular keywords were: Propionibacterium acnes, sarcoidosis, ulcerative colitis, and isotretinoin. MeSH subheadings used were: Propionibacterium acnes/immunology, Propionibacterium acnes/pathogenicity, Propionibacterium acnes/genetics, sarcoidosis/immunology, and sarcoidosis/genetics. Following the application of inclusion and exclusion criteria, a total of 5172 publications were obtained. A total of 5086 publications were removed due to a lack of relevancy to outcomes of interest. The remaining 86 publications from all the regular and MeSH keywords were selected due to relevancy to outcomes of interest. Following this, a refined manual search was done, with the removal of duplicates, and 33 publications from PubMed were selected for review. Following a review of these records, Propionibacterium acnes was repeatedly concluded to be a causative agent of sarcoidosis. Variable results for the association between Propionibacterium acnes and ulcerative colitis were found. Most studies showed no significant association between the use of isotretinoin and the development of ulcerative colitis. A strong overlapping role of genetic susceptibility and immune dysregulation in the pathogeneses of sarcoidosis, ulcerative colitis, and Propionibacterium acnes was found.

The Diagnostic Dilemma of Sarcoidosis: A Case of Acute Hypercalcemia.

Hypercalcemia is a common clinical laboratory abnormality with a majority of cases attributed to malignancy or hyperparathyroidism. Although hypercalcemia is a common manifestation of sarcoidosis, it is rarely the initial presentation. Here we present a case of acute hypercalcemia in a 60-year-old gentleman, which was diagnosed as sarcoidosis following an elaborate workup, including radiological assessment and multiple organ biopsies. This case highlights the diagnostic dilemma of sarcoidosis due to varying clinical presentation that can mimic multiple conditions, including malignancy. Biopsy showing noncaseating granulomas is pathognomic of sarcoidosis. Due to its multisystemic and nonspecific presentation, selecting an appropriate biopsy site is key to diagnosis.

Isolated Pancreatic Sarcoidosis Diagnosed by Endoscopic Ultrasound-guided Fine-needle Aspiration.

We herein report a 52-year-old man with multiple hypoechoic lesions in the body and tail of the pancreas detected during a screening ultrasound. Computed tomography (CT) showed no lesions other than those in the pancreas and peripheral lymph nodes. Contrast-enhanced CT identified hypovascular tumors in the pancreas. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) demonstrated partial fibrosis and noncaseating granulomas with Langhans giant cells. To our knowledge, this is the first report of isolated pancreatic sarcoidosis diagnosed by EUS-FNA. Although pancreatic sarcoidosis is very rare, clinicians should be aware of this possibility in patients presenting with multiple hypovascular pancreatic tumors.

Micropapular Cutaneous Sarcoidosis Reviewed.

BACKGROUND: Micropapular cutaneous sarcoidosis (MPCS) is a rare variant of sarcoidosis. Herein we review the literature and include a recent case of MPCS discussing pathogenesis, diagnosis, treatment, and prognosis. METHOD: A review was conducted using the terms "micropapular sarcoidosis" and "micropapular sarcoid." A recent case of a 50-year-old male patient with biopsy-identified MPCS was also included in the review. RESULTS: In total, 12 cases with an aggregate of 18 patients were included in the review. Presentation among all patients was consistent, with scattered, occasionally pruritic, faintly erythematous shiny white papules. Skin biopsy demonstrated noncaseating granulomas. Systemic prednisone, oxytetracycline, and hydroxychloroquine, as well as topical betamethasone, were used for therapy. CONCLUSION: In our review there does not seem to be a clear link as to the definite cause of the MPCS. While the relationships to tuberculosis and autoimmunity seem to be often emphasized, there was no clear association with either etiology.

A Case of Severe Cardiac Sarcoidosis with Minimal Pulmonary Involvement: A Case Report with Literature Review.

Sarcoidosis is a granulomatous disease of unknown etiology. Although sarcoidosis is a systemic disease, there appears to be a predilection for involvement of certain organs. The pulmonary system is the most commonly affected system among all racial groups. Cardiac and respiratory complications are the leading causes of death due to sarcoidosis and in certain patient populations about half of these deaths are attributed to cardiac sarcoidosis. There are few autopsy case reports of cardiac sarcoidosis with minimal respiratory involvement making this case report relevant to the importance of the recognition and awareness of this entity. Acad Forensic Pathol. 2018 8(2): 407-415.

Nodular Sarcoidosis Masquerading as Cancer.

Nodular lung disease is a rare pulmonary manifestation of sarcoidosis and resembles metastatic neoplasm disease. Nodular sarcoidosis is rare, varying from 1.6% to 4% of patients with sarcoidosis. Radiographic nodules measure from 1 to 5 cm in diameter that typically consist of coalescent granulomas. There is limited data on this form of sarcoidosis and its presentation can mimic primary or metastatic pulmonary neoplasms. Nodular sarcoidosis has a favorable prognosis, and resolution can be seen with oral corticosteroids. Herein, we present such a case of nodular pulmonary sarcoidosis with a lung nodule measured up to 6 cm.

Colonic sarcoidosis: unusual onset of a systemic disease.

Sarcoidosis is a multisystem chronic inflammatory condition of unknown etiology that has the potential to involve every tissue in the body. Sarcoidosis in the gastrointestinal system, and particularly the colon, is very rare. Here, we report the case of a 57-year-old man with no previous diagnosis of sarcoidosis who presented with new onset of abdominal pain and constipation. A colonoscopy revealed that the abdominal pain was caused by an obstructing lesion in the cecum-ascending colon and lacked a clear histologic diagnosis. Radiologic investigation revealed concentric wall thickening of the cecum-ascending colon with multiple satellite lymphadenopathies, highly suggestive of a malignancy. The patient underwent a laparotomy and a right hemicolectomy was performed. A diagnosis of colonic sarcoidosis was made after the resected specimen was examined. Additionally, a chest computed tomography scan revealed lung involvement with atypical radiologic features in the absence of respiratory symptoms. Only histologic examination of the surgical specimen can yield a diagnosis of gastrointestinal sarcoidosis due to the non-specificity of endoscopic and radiologic findings.

Association between I/D polymorphism in the ACE gene and sarcoidosis in Turkish patients.

Sarcoidosis is a chronic inflammatory disease with a complex pathogenesis and unknown etiology characterized by noncaseating granulomas that invade the lungs, eyes, liver and other organs. Insertion (I)/deletion (D) polymorphism in the gene encoding the angiotensin-converting enzyme (ACE) has been studied to examine the genetic predisposition to sarcoidosis in different populations, but the results have been inconsistent and inconclusive. This study aimed to determine the frequencies of the genotypes and alleles of I/D polymorphism in the ACE gene in Turkish patients as a distinct ethnic group and to investigate whether such polymorphism is associated with predisposition to sarcoidosis. Genomic DNA samples obtained from 154 individuals (70 patients with sarcoidosis and 84 healthy controls) were used in the study. The DNA was amplified using polymerase chain reactions using allele-specific primers. The amplified products were analyzed by 2 % agarose gel electrophoresis followed by UV transillumination. The allele frequencies and genotype distribution of the groups were analyzed using the Chi square test. There were no significant differences between the controls and sarcoidosis cases with respect to genotype distribution (χ(2) = 4.202, p = 0.122) and allele frequencies (χ(2) = 1.358, p = 0.244). Our results suggest that I/D polymorphism in the ACE gene does not cause a genetic predisposition to sarcoidosis in Turkish patients.

A Rare Variant of Pancreatic Sarcoidosis: Diagnostic Challenge.

Pancreatic sarcoidosis is one of the variant of systemic sarcoidosis which is extremely rare in literature and opined as an enigma. Hence, its diagnosis is very challenging as its presentation mimics pancreatic carcinoma. In view of a better understanding, we are presenting a case of pancreatic sarcoidosis to emphasize the above. To clearly distinguish sarcoidosis from malignancy, repeated computed tomography scans, Positron emission tomography scans, fine needle aspiration cytology, endoscopic retrograde cholangio-pancreatography supplemented with histology and relevant blood tests proved it to be a pancreatic sarcoidosis then a malignancy. Over the course, concrete evidence was divulged from lymph node biopsy and histology in clinching the diagnosis which later transformed into a malignancy. Therefore, we concluded it is a rare variant of pancreatic sarcoidosis and careful serial investigations include repeated imaging, histology and blood tests are essential to establish and most importantly differentiate the diagnosis. HOW TO CITE THIS ARTICLE: Khangura T, Uddin G, Davies A, Keating J. A Rare Variant of Pancreatic Sarcoidosis: Diagnostic Challenge. Euroasian J Hepato-Gastroenterol 2015;5(2):118-121.