Cutaneous Rosai–Dorfman disease: a challenging diagnosis

2021 ◽  
Vol 14 (2) ◽  
pp. e239244
Author(s):  
Rita Sampaio ◽  
Leandro Silva ◽  
Goreti Catorze ◽  
Isabel Viana
Keyword(s):  
Weight Loss ◽  
Differential Diagnosis ◽  
Physical Examination ◽  
Plasma Cells ◽  
Oral Prednisolone ◽  
Inflammatory Cells ◽  
Rosai Dorfman Disease ◽  
History Of ◽  
Nodular Lesions ◽  
Proliferative Disease

Rosai–Dorfman disease is a rare benign histiocytic proliferative disease of unknown cause that, in exceptional cases, presents with lesions confined to the skin. Clinically variable types of lesions such as papules, nodules and plaques have been reported. We present a case of a 27-year-old woman with a 1-year history of erythematous papular and nodular lesions on the malar and right axillary regions, previously misdiagnosed as acne. She reported no fever, malaise or weight loss, while physical examination and laboratory workup were normal. Bacteriological and mycobacteriological cultures were negative. Histopathological findings showed dense infiltration of inflammatory cells involving the entire dermis, consisting of large macrophages with emperipolesis, S100 and CD68 positive, neutrophils, eosinophils, lymphocytes and plasma cells. The patient was treated with oral prednisolone without improvement. Dapsone was subsequently initiated with favourable clinical response. The present article aimed to emphasise the clinical and histological differential diagnosis and share the treatment experience.

scholarly journals Cutaneous Rosai-Dorfman disease: A case report

2021 ◽  
Vol 12 (e) ◽  
pp. e84-e84
Author(s):  
Soukaina Maghfour ◽  
Sana Mokni ◽  
Marouane Ben Kahla ◽  
Rima Gammoudi ◽  
Amina Aounallah ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Plasma Cells ◽  
Histopathological Examination ◽  
Inflammatory Cells ◽  
Left Cheek ◽  
Skin Involvement ◽  
Rosai Dorfman Disease ◽  
History Of ◽  
Systemic Symptoms

Pure cutaneous Rosai-Dorfman is distinguished from classic Rosai-Dorfman disease by isolated skin involvement without lymphadenopathy or systemic symptoms. Herein, we report a case of a 30 year-old-man with 16 months history of a slowly enlarging, asymptomatic, purple plaque on her left cheek following cutaneous leishmaniasis successfully treated. The histopathological examination showed dense infiltration of inflammatory cells involving the entire dermis, consisting of large macrophages with emperipolesis, S100 and CD68 positive, lymphocytes and plasma cells. The patient was treated with intralesional triamcinolone with a significant improvement. The present article aimed to emphasise the clinical, histological differential diagnosis and to share the tumoral presentation of cutaneous Rosai-Dorfman disease.

Cutaneous Rosai-Dorfman Disease With Increased Number of Eosinophils: Coincidence or Histologic Variant?

2011 ◽  
Vol 135 (12) ◽  
pp. 1597-1600 ◽  
Author(s):  
John J Cangelosi ◽  
Victor G Prieto ◽  
Doina Ivan
Keyword(s):  
Differential Diagnosis ◽  
Pituitary Gland ◽  
Plasma Cells ◽  
S100 Protein ◽  
Clinical Context ◽  
Cutaneous Involvement ◽  
Eosinophilic Infiltrate ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Skin Biopsies

Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.

scholarly journals PC3 - 216 Presentation of Intradural Extramedullary Plasmacytoma with Intratumoral Hemorrhage: Case Report and Review of Literature

2016 ◽  
Vol 43 (S4) ◽  
pp. S22-S22
Author(s):  
A. Ghare ◽  
F. Haji ◽  
K. MacDougall
Keyword(s):  
Multiple Myeloma ◽  
Differential Diagnosis ◽  
Plasma Cells ◽  
Extramedullary Plasmacytoma ◽  
Left Frontal Lobe ◽  
Axial Mass ◽  
Dural Tail ◽  
History Of ◽  
Intradural Extramedullary ◽  
Contrast Ct

Plasmacytomas are solitary tumours characterized by neoplastic proliferation of plasma cells and can be found isolated or in associated with multiple myeloma. Plasmacytomas uncommonly occur intracranially, and dural plasmacytomas without involvement of the calvarium are exceedingly rare. Reported cases indicate durally-based plasmacytomas mimick the appearance of meningioma, lymphoma or sarcoma of the dura. The authors report a case of a 77-year-old male with known multiple myeloma who presented with a 3-week history of confusion, speech impediment, and right sided weakness. A non-contrast CT scan revealed a dense extra-axial mass in the left frontal lobe with initial concerns of an extra-axial hemorrhage. A subsequent MRI demonstrated a contrast enhancing mass with a broad-based dural tail and no underlying calvarial lesion. Differential diagnosis included meningioma or intracranial plasmacytoma. The patient underwent surgical resection and was found to have intratumoural hemorrhage, with pathology confirming plasmacytoma. In the published literature, there are only 20 prior reports of dural plasmacytomas (with and without primary calvarial infiltration), of which only five previous cases reported associated intratumoural hemorrhage. Our case, along with this literature, suggests that new onset of focal neurologic deficits in patients with a history of multiple myeloma merits careful investigation, and that intracranial plasmacytoma should be considered on the differential diagnosis even when imaging reveals masses consistent with hemorrhage or meningioma.

scholarly journals Cutaneous Polyarteritis Nodosa Presenting Atypically with Severe Pharyngeal Ulceration

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Bayanne Olabi ◽  
Justin C. Mason ◽  
Ziad Farah
Keyword(s):  
Weight Loss ◽  
Differential Diagnosis ◽  
Magnetic Resonance Angiography ◽  
Polyarteritis Nodosa ◽  
Rare Case ◽  
Significant Weight Loss ◽  
Histological Assessment ◽  
Cutaneous Polyarteritis Nodosa ◽  
Nodular Lesions ◽  
Appropriate Therapy

Polyarteritis nodosa (PAN) is a multisystem, necrotising vasculitis of small- and medium-sized arteries with a predilection for the visceral vessels. Cutaneous PAN is a rare variant with symptomatic vasculitis limited to the skin, typically presenting as nodular lesions on the extremities with a propensity to ulcerate. We describe a rare case of histologically confirmed cutaneous PAN presenting in a 55-year-old Ghanaian woman with severe oropharyngeal ulceration. This was associated with dysphagia and significant weight loss. Oesophagoduodenoscopy showed that the ulceration extended throughout the oropharynx. Systemic polyarteritis nodosa was ruled out with magnetic resonance angiography. Our patient was treated successfully with corticosteroids and methotrexate. This case suggests that cutaneous PAN should be considered in the differential diagnosis of patients with oropharyngeal ulceration and that histological assessment is pivotal in establishing the diagnosis early in order to instigate appropriate therapy.

Pneumatosis Coli in a Dog

2008 ◽  
Vol 44 (1) ◽  
pp. 32-35 ◽  
Author(s):  
Nicholas J. Russell ◽  
Dayle Tyrrell ◽  
Peter J. Irwin ◽  
Catherine Beck
Keyword(s):  
Weight Loss ◽  
Differential Diagnosis ◽  
Clinical Signs ◽  
Colorectal Disease ◽  
Abdominal Radiography ◽  
Pneumatosis Coli ◽  
History Of ◽  
Oral Metronidazole

A 17-year-old, castrated male Maltese was presented with chronic polyphagia and a 2-week history of tenesmus, diarrhea, hematochezia, weight loss, and ribbon-like feces. Pneumatosis coli was diagnosed by abdominal radiography. Concurrent hyperadrenocorticism was suspected. The clinical signs of colorectal disease resolved within 2 days of initiating a lowresidue diet and oral metronidazole. Pneumatosis coli should be considered as a differential diagnosis for colorectal disease in dogs.

scholarly journals Urachal Adenocarcinoma: A Case Report with Key Imaging Findings and Radiologic-Pathologic Correlation

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Willian Schmitt ◽  
Marta Baptista ◽  
Marco Ferreira ◽  
António Gomes ◽  
Ana Germano
Keyword(s):  
Weight Loss ◽  
Differential Diagnosis ◽  
Clinical History ◽  
Imaging Findings ◽  
Lesion Location ◽  
Patient Demographics ◽  
Pathologic Correlation ◽  
Associated Symptoms ◽  
History Of ◽  
Urachal Adenocarcinoma

Urachal pathologies are rare and can mimic numerous abdominal and pelvic diseases. Differential diagnosis of urachal anomalies can be narrowed down by proper assessment of lesion location, morphology, imaging findings, patient demographics, and clinical history. We report a case of a 60-year-old male, with a history of unintentional weight loss without associated symptoms, who was diagnosed with locally invasive urachal adenocarcinoma. With this article, we pretend to emphasize urachal adenocarcinoma clinical features along with its key imaging findings with radiologic-pathologic correlation.

scholarly journals Szaruhártya-lerakódások bizonytalan jelentőségű monoklonális gammopathiában. Irodalmi áttekintés és esetbemutatás

2018 ◽  
Vol 159 (39) ◽  
pp. 1575-1583 ◽  
Author(s):  
Orsolya Németh ◽  
Beáta Tapasztó ◽  
Sándor Tar ◽  
Viktória Szabó ◽  
Zoltán Zsolt Nagy ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Inflammatory Cells ◽  
Protein Deposition ◽  
Corrected Visual Acuity ◽  
History Of ◽  
Proper Diagnosis

Abstract: To summarize ophthalmological signs of monoclonal gammopathy of undetermined significance (MGUS) and to present a case report. Summary of the literature data and presentation of the history of a 46-year-old female patient. In MGUS, pathological, but non-malignant plasma cells produce abnormal monoclonal immunoglobulin. Its prevalence is 0.15%, but it increases with age. As yearly 1–2% of MGUS patients develop multiple myeloma, frequent hematological follow-up is necessary. Corneal opacifications in MGUS have been described in a few dozens of patients in the literature. These may be nummular or crystal-like, or even present with white or grey line-forming depositions in the stroma. They may be centrally or peripherally localized. In our patient, bilateral, branching, geographical corneal opacifications were detected predescemetally, that were progressing and reaching the optical centre during follow-up. With 0.15 best corrected visual acuity, penetrating keratoplasty was performed (postoperative best spectacle-corrected visual acuity 0.6). Masson trichrom staining of the explanted cornea verified protein deposition, immunhistochemistry identified kappa light chain immunglobulin deposition in the posterior stroma, surrounded with inflammatory cells. Serum electrophoresis and bone marrow biopsy of our patient proved MGUS, therefore, hematological follow-up is going on. In the case of progressive, atypical corneal opacification, the hematological diagnosis of monoclonal gammopathy must be excluded – monoclonal gammopathy of ocular significance –, as delay in proper diagnosis and treatment of the systemic disease may have devastating consequences. Orv Hetil. 2018; 159(39): 1575–1583.

Galactokinase deficiency: a treatable cause of bilateral cataracts

2021 ◽  
Vol 14 (6) ◽  
pp. e242227
Author(s):  
Catarina Cordeiro ◽  
Paula Garcia ◽  
Dalila Coelho ◽  
Mónica Oliva
Keyword(s):  
Differential Diagnosis ◽  
Family History ◽  
Physical Examination ◽  
Congenital Cataract ◽  
Genetic Variant ◽  
Metabolic Diseases ◽  
Systemic Diseases ◽  
Galactose Metabolism ◽  
Biochemical Studies ◽  
History Of

Congenital cataract can be caused by several systemic diseases and differential diagnosis should be done between infections, genetic or metabolic diseases. We present a case of a 12-month-old girl with bilateral nuclear cataracts that was referred for investigation. Since she did not present a family history of congenital cataracts or metabolic diseases, and her physical examination was normal, a systemic evaluation was performed. Biochemical studies disclosed abnormal galactose metabolism signs. The diagnosis of galactokinase (GALK1) deficiency was considered and the study of the GALK1 gene allowed identifying a pathogenic genetic variant and a predictably pathogenic missense mutation, previously not described. Dietary measures were imposed with a good evolution.

scholarly journals Induction of general anaesthesia by blowpipe darting in a fractious companion horse

2018 ◽  
Vol 6 (3) ◽  
pp. e000629
Author(s):  
Sabina Diez Bernal ◽  
Simone Lanz ◽  
Isabelle Schmutz ◽  
Tosso Leeb ◽  
Claudia Spadavecchia
Keyword(s):  
Weight Loss ◽  
Physical Examination ◽  
General Anaesthesia ◽  
Stress Level ◽  
Therapeutic Approach ◽  
Present Report ◽  
Genetic Mutations ◽  
History Of ◽  
Progressive Weight Loss ◽  
Hindlimb Lameness

A fractious nine-year-old, 520-kg, neutered Swiss Warmblood was presented with a history of anorexia, progressive weight loss and mild hindlimb lameness. Because of its temperament, standard physical examination was considered to be only feasible under general anaesthesia. For safety reasons, general anaesthesia was planned to be induced by blowpipe darting. Two attempts are described and discussed in the present report. The first attempt, using a combination of medetomidine and tiletamine-zolazepam, was unsuccessful. Conversely, detomidine combined with butorphanol, followed by a second dart of detomidine and tiletamine-zolazepam, proved to be adequate to induce anaesthesia. Factors that could have influenced the outcome, such as different therapeutic approach, drug protocol and dosages, stress level, or genetic mutations, are presented and discussed.

scholarly journals Whipple’s Disease: A Rare Cause of Diarreha

2021 ◽  
Vol 6 (6) ◽  
Keyword(s):  
Weight Loss ◽  
Fecal Incontinence ◽  
Physical Examination ◽  
Obese Patient ◽  
Personal History ◽  
Whipple’S Disease ◽  
Whipple's Disease ◽  
Associated Symptoms ◽  
History Of

We describe the case of a 55-year-old man, with a personal history of dyslipidemia, hyperuricemia and obesity, on atorvastatin and allopurinol. He went to a Gastroenterology consultation due to diarrhea with several years of evolution, from 3 daily spills, worsening in the last weeks to 10-12 daily spills, of liquid feces, without blood, mucus or pus, accompanied by defecatory urgency and fecal incontinence. He denied other associated symptoms, such as nausea, vomiting, weight loss or fever. Physical examination showed an obese patient, colored and hydrated skin and mucosa, without palpable adenomegalies, abdomen without changes.

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