scholarly journals A Sinister Disease Hides Behind Recalcitrant Mucocutaneous Disorder

2021 ◽  
Vol 19 (2) ◽  
pp. 49-53
Author(s):  
Harihar Adhikari ◽  
Deeptara Pathak Thapa ◽  
Priya Bhusal
Keyword(s):  
Histopathological Examination ◽  
Signs And Symptoms ◽  
Autoimmune Disorder ◽  
Castleman Disease ◽  
Surgical Team ◽  
Paraneoplastic Pemphigus ◽  
Oral Ulcers ◽  
History Of ◽  
Hands And Feet ◽  
Malignant Neoplasia

Paraneoplastic pemphigus (PNP) is a rare autoimmune disorder associated with underlying benign or malignant neoplasia. Its signs and symptoms may be the first presentation of a concealed malignancy. Due to late diagnosis, prognosis of PNP is not good, so early diagnosis and treatment is of paramount importance. We present a case of 22 years old female who presented to our out patient department (OPD) with history of recurrent, severe, recalcitrant, painful oral ulcers; lichenoid lesions over the hands and feet and widespread blistering and erosions involving the soles. Histopathological examination was consistent with paraneoplastic pemphigus and showed features of lichenoid dermatitis. CT scan revealed retroperitoneal mass suggestive of Castleman disease. The patient was referred to surgical team for further management.

Unilateral facial erythematous patch as the presenting sign of systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1968-1970
Author(s):  
Dae-Lyong Ha ◽  
Gi-Wook Lee ◽  
Kihyuk Shin ◽  
Hoon-Soo Kim ◽  
Hyun-Chang Ko ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Autoimmune Disorder ◽  
Left Cheek ◽  
Systemic Lupus ◽  
Diagnostic Challenge ◽  
Cutaneous Manifestations ◽  
Oral Ulcers ◽  
Rare Manifestation ◽  
History Of

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that can have many cutaneous manifestations including malar rash, discoid rash or oral ulcer. Isolated unilateral involvement of face is uncommon in SLE. It lacks typical clinical features of LE, and may impose a diagnostic challenge for clinicians. Herein we report a case of 62-year-old woman presenting with a 2-year history of erythematous patches on left cheek and eyelid. Initially, she was diagnosed as having recurrent blepharitis or cellulitis that did not respond to conventional treatment with ophthalmic medicaments. As time went by, the patches spread to her left cheek, and she was referred to our dermatologic department. Histopathologic examination was consistent with LE. Further physical and laboratory tests have found that she had oral ulcers, proteinuria, thrombocytopenia and abnormal titer of anti-nuclear antibody satisfying the diagnosis of SLE. From this case, we think unilateral erythematous patches on face could be a rare manifestation of SLE and more intention should be paid to this type of patients, because unilateral facial symptom may mimic other dermatoses.

scholarly journals Invasive Aspergillosis Masquerading as Catastrophic Antiphospholipid Syndrome

2013 ◽  
Vol 22 (5) ◽  
pp. 448-451 ◽  
Author(s):  
Kathryn S. Robinett ◽  
Bethany Weiler ◽  
Avelino C. Verceles
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Invasive Aspergillosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Signs And Symptoms ◽  
Subdural Hemorrhage ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Oral Ulcers ◽  
History Of

A 25-year-old woman with a history of systemic lupus erythematosus who was taking steroids came to the hospital because of vague signs and symptoms of weight loss, constipation, and oral ulcers. Multiorgan dysfunction developed, and catastrophic antiphospholipid syndrome was suspected. She was treated with an intravenous infusion of heparin, but she experienced a subdural hemorrhage and died on day 10 of the hospitalization. An autopsy revealed disseminated invasive aspergillosis. This case illustrates that invasive aspergillosis is a frequently missed diagnosis and should be part of the differential diagnosis for any patient who is immunosuppressed, including patients with autoimmune diseases such as systemic lupus erythematosus.

scholarly journals Eosinophilic Enteritis: A Delayed Diagnosis

2017 ◽  
Vol 5 (4) ◽  
pp. 232470961773424 ◽  
Author(s):  
Ankita Munjal ◽  
Abdulhameed Al-Sabban ◽  
Kathryn Bull-Henry
Keyword(s):  
High Power ◽  
Histopathological Examination ◽  
Delayed Diagnosis ◽  
Signs And Symptoms ◽  
Gi Tract ◽  
High Power Field ◽  
Radiographic Findings ◽  
Wide Range ◽  
History Of ◽  
Allergic Disorders

Eosinophilic gastrointestinal disorders are a rare and complex group of disorders that are characterized by eosinophilic infiltration of the gastrointestinal tract. Patients often present with a wide range of signs and symptoms as any length or layer of the GI tract can be involved such as mucosal, muscular, or serosal. As a part of the workup, patients frequently undergo computed tomography scans and multiple endoscopies before the diagnosis is finally made as was true in our case of a 59-year-old male patient presenting with 2 months of nausea, abdominal pain, and weight loss. He underwent esophagogastroduodenoscopies, colonoscopies, video capsule study, and balloon enteroscopy before the diagnosis was confirmed histologically. Endoscopic and radiographic findings can be variable and are usually unpredictable. The diagnosis is confirmed on histopathological examination of biopsies that must show >15-50 eosinophils/high-power field based on the location in the GI tract. In our patient, erythema, scalloping, whitish exudate, and patches of villous blunting were noted in the duodenum to proximal ileum endoscopically with >50 eosinophils/high-power field confirming the diagnosis of eosinophilic enteritis. This class of diseases is often found in patients with a history of allergic disorders suggestive of hypersensitivity in the etiology of the disease although our patient had no such known history. Elimination diets and steroids are the mainstay of therapy and often lead to complete resolution of symptoms as well as endoscopic and radiographic findings in up to 90% of patients as was seen in our patient, although some patients have a chronic remitting course.

scholarly journals Multiloculated peritoneal inclusion cyst: a case report

2020 ◽  
Vol 8 (1) ◽  
pp. 388
Author(s):  
Sunita Prakash Jain ◽  
Arun C. M. ◽  
Doreswamy K. ◽  
Amasa Vishwanath Reddy
Keyword(s):  
Histopathological Examination ◽  
Previous History ◽  
Pelvic Mass ◽  
Signs And Symptoms ◽  
Emergency Laparotomy ◽  
Inclusion Cyst ◽  
Peritoneal Inclusion Cyst ◽  
Healthy Female ◽  
Perimenopausal Women ◽  
History Of

Multiloculated peritoneal inclusion cysts (MPIC) are uncommon abdominopelvic cysts seen in perimenopausal women. We describe a perimenopausal woman presenting with pelvic mass. This patient presented to us with all signs and symptoms involving abdomen and the pelvis. On emergency laparotomy, it was seen that multioculated cyst occupied the whole of abdomen arising from the peritoneum. The condition was successfully treated with surgery. Histopathological examination (HPE) report proved it to be multi loculated peritoneal inclusion cyst. This case is presented for its rarity in itself, presenting as intestinal obstruction, in a healthy female, that too without previous history of any surgery.

scholarly journals Castleman disease with rapidly progressive pemphigus: a case report

2020 ◽  
Vol 2 (4) ◽  
pp. 223-225
Author(s):  
Ruiying Fu ◽  
◽  
Yingzhi He ◽  
Liang Wang ◽  
Keyword(s):  
Autoimmune Disorder ◽  
Pemphigus Vulgaris ◽  
Hematologic Malignancies ◽  
Skin Lesions ◽  
Castleman Disease ◽  
Pathological Examination ◽  
Malignant Neoplasms ◽  
Retroperitoneal Mass ◽  
Paraneoplastic Pemphigus ◽  
Pet Ct

Paraneoplastic pemphigus (PNP) is a rare autoimmune disorder of the skin, which is associated with various underlying benign or malignant neoplasms. We report a case of a 34-year-old man with rapidly progressed and extensive skin lesions, and significant weight loss. He was diagnosed as pemphigus Vulgaris with a skin biopsy firstly, then the PET/CT scan showed a retroperitoneal mass. Pathological examination of the retroperitoneal mass confirmed the diagnosis of Castleman disease with paraneoplastic pemphigus. By reporting this case, we aimed to improve the clinical alertness and awareness of Castleman disease in patients characterized by paraneoplastic pemphigus. Keywords: Paraneoplastic pemphigus, Castleman disease, paraneoplastic syndrome, hematologic malignancies

scholarly journals Clinical characteristics and prognosis of castleman disease patients in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor

2021 ◽  
Author(s):  
Yibo Hua ◽  
Chao Liang ◽  
Jie Yang ◽  
Luyang Wang ◽  
Lei Xi ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Prognostic Factors ◽  
Cox Regression ◽  
Univariate Analysis ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Castleman Disease ◽  
Paraneoplastic Pemphigus ◽  
Clinical Complications

Abstract Background: Castleman disease (CD) is a rare lymphoproliferative disorder that has had limited clinical research. This study aims to detect the clinical manifestations, pathological features, and prognostic factors of this disease.Methods: This study retrospectively analyzed the information of 54 patients with CD hospitalized in a single centre. A Cox regression model was employed to perform univariate analysis and multivariate analysis in order to identify independent prognostic factors for survival.Results: Based on clinical classification, 30 patients (55.6%) had unicentric CD (UCD) and 24 patients (44.4%) had multicentric CD (MCD). Moreover, pathological classification identified 32 cases (59.3%) with hyaline vascular variant (HV), 3 (5.6%) with mixed cellular variant (Mix), and 19 (35.2%) with plasmacytic variant (PC). The MCD patients commonly exhibited clinical signs and symptoms, including fever, splenomegaly, and pleural effusion and/or ascites. Several clinical complications, such as liver injury, anemia, and polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes syndrome (POEMS) were more common in MCD patients. Univariate analysis showed that presence of paraneoplastic pemphigus (PNP) and elevated C-reactive protein (CRP) were unfavorable factors relating to CD patient survival. Multivariate analysis identified the presence of PNP as an independent prognostic factor in patients with CD.Conclusions: This study provided a panoramic elaboration of CD cases and showed the presence of PNP was an independent unfavorable factor.

scholarly journals An elderly-onset rheumatic syndrome: is it RS3PE?

2017 ◽  
Vol 11 (3) ◽  
pp. 335
Author(s):  
Marta Oliveira ◽  
Filipa Ferreira ◽  
Erica Ferreira ◽  
Rosa Jorge
Keyword(s):  
White Blood Cells ◽  
Signs And Symptoms ◽  
Good Prognosis ◽  
Normocytic Anemia ◽  
Reactive Protein ◽  
Underlying Malignancy ◽  
History Of ◽  
Elderly Onset ◽  
Hands And Feet ◽  
Pitting Edema

It is presented a case of a 75-year-old Caucasian male patient who was admitted to hospital with a history of painful swollen hands and feet, associated with worsening mobility. Examination revealed bilateral pitting edema of dorsum of hands and feet. Initial blood test showed 10.7 g/L hemoglobin with normochromic and normocytic anemia, raised inflammatory markers (erythrocyte sedimentation rate 60 mm, C-reactive protein 16.73 mg/dL) and normal white blood cells count. Autoantibody screen and rheumatoid factor were negative, with positive HLA-B27. Radiological findings of hands and feet did not show any erosions. The patient was also screened and investigated for associated malignancies; the tumor markers and the computed tomography thorax/abdomen were reported as normal. The patient responded extremely well to low dose prednisolone at 20 mg daily dosage. Further follow up 5 weeks later on tapering dose of prednisolone showed complete resolution of signs and symptoms without any further flare-ups. Remitting seronegative symmetrical synovitis with pitting edema is a definite syndrome, a rare subset of polyarthritis with favorable outcome and has a good prognosis in older patients. This case illustrates the interesting diagnosis exercise that is needed, once the multiple conditions that may be related with the disease support that this is a syndrome with potentially heterogeneous etiologies. Looking for underlying malignancy is recommended.

Rare Case of Castleman Disease Involving the Pancreas

2007 ◽  
Vol 73 (12) ◽  
pp. 1284-1287 ◽  
Author(s):  
Vonny W. Tunru-Dinh ◽  
Abdul Ghani ◽  
Yuel D. Tom
Keyword(s):  
Lymph Node ◽  
Pancreatic Neoplasm ◽  
Signs And Symptoms ◽  
Castleman Disease ◽  
Pancreatic Mass ◽  
Angiofollicular Lymph Node Hyperplasia ◽  
Vascular Type ◽  
Common Location ◽  
Hyaline Vascular Type ◽  
History Of

Castleman disease, also known as angiofollicular lymph node hyperplasia, is an uncommon tumor involving lymph node tissues. The most common location is in the thorax presenting as a mediastinal mass. In the abdomen, most of the lesions are located in the pelvis, mesentery, and retroperitoneum. Only nine other cases of Castleman disease involving the pancreas have been described. We report a case of Castleman disease (hyaline vascular type) presenting as a pancreatic mass and an enlarged spleen. A 23-year-old woman presented with a 2-month history of abdominal pain localized to the left upper quadrant. Ultrasound and CT demonstrated a large retrogastric pancreatic mass and an enlarged spleen. Distal pancreatectomy and splenectomy were performed. Histopathologic examination revealed a markedly enlarged lymph node showing germinal centers with hyalinization and prominent follicular dendritic cells meshwork surrounded by concentric layers of small lymphocytes. Within the interfollicular area were regions of increased vascularity. BCL2, CD20, and CD79a show expanded mantle zones with a concentric targetoid arrangement of small lymphocytes. This is a case of unicentric Castleman disease of the hyaline-vascular type, which presented with signs and symptoms of a primary pancreatic neoplasm.

Popliteal artery thrombosis secondary to a tibial osteochondroma

VASA ◽  
2011 ◽  
Vol 40 (3) ◽  
pp. 251-255 ◽  
Author(s):  
Gruber-Szydlo ◽  
Poreba ◽  
Belowska-Bien ◽  
Derkacz ◽  
Badowski ◽  
...  
Keyword(s):  
Case Report ◽  
Magnetic Resonance Angiography ◽  
Popliteal Artery ◽  
Doppler Ultrasonography ◽  
Histopathological Examination ◽  
Previous History ◽  
Plain Radiography ◽  
Artery Thrombosis ◽  
History Of ◽  
The Right

Popliteal artery thrombosis may present as a complication of an osteochondroma located in the vicinity of the knee joint. This is a case report of a 26-year-old man with symptoms of the right lower extremity ischaemia without a previous history of vascular disease or trauma. Plain radiography, magnetic resonance angiography and Doppler ultrasonography documented the presence of an osteochondrous structure of the proximal tibial metaphysis, which displaced and compressed the popliteal artery, causing its occlusion due to intraluminal thrombosis..The patient was operated and histopathological examination confirmed the diagnosis of osteochondroma.

A Rare Case of Spinal Leprosy Mimicking Spinal Tuberculosis

2019 ◽  
pp. 33
Author(s):  
K Thuraikumar ◽  
V Naveen ◽  
Mustaqim A ◽  
Arieff AA ◽  
K Shri ◽  
...  
Keyword(s):  
Back Pain ◽  
Soft Tissue ◽  
Histopathological Examination ◽  
Posterior Instrumentation ◽  
Spinal Tuberculosis ◽  
Previous History ◽  
Skin Lesions ◽  
Titanium Implants ◽  
Paravertebral Abscess ◽  
History Of

Introduction: Spinal tuberculosis is the most common manifestation of extrapulmonar y tuberculosis. A combination of leprosy and tuberculosis is a rare entity.Case report: A 44-year-old male patient working as a laborer presented to our hospital with complaints of severe back pain and swelling over the back, difficulty in walking, associated with constitutional symptoms. On admission, he was febrile and had leukocytosis. Initial spine X-ray showed end plate destruction and increase in soft tissue shadow at the level of T8-T9. CT spine revealed thoracic paravertebral collection extending from T7 to T9 levels, suggest ive of tuberculous spondylitis with cold abscess. Patient refused a transpedicular biopsy and was started on anti-tubercular therapy. Two weeks after commencement of treatment, he developed worsening back pain and weakness of the lower extremities. MRI spine showed a paravertebral abscess and posterior soft tissue edema involving level of T7 to T11. Patient underwent a posterior decompression, debridement and posterior instrumentation. He was discharged well, there was improvement of his lower limb power. Upon clinic review, he complained of multiple hyperpigmented, painless, nonpruritic skin lesions over the trunk and back. No previous history of eczema, psoriasis and Tinea corporis. Given the history of allergy, initial impression was hypersensitivity reaction towards the titanium implants, and he was started on anti-histamines. However, there was no improvements seen. Histopathological examination of skin lesions revealed presence of granuloma within the dermis layer, composed of epitheloid, histiocytes, lymphocytes and plasma cells. Wade-Fite stain for Mycobacterium leprae is positive. Slit skin smear shows multibacillary leprosy. Patient was started on multidrug therapy (rifampicin, clofazimine and dapsone) for 1 year. He has recovered well.International Journal of Human and Health Sciences Supplementary Issue: 2019 Page: 33

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