scholarly journals Case Report: Acute amyopathic dermatomyositis presenting with isolated facial edema

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 60
Author(s):  
Efthymia Pappa ◽  
Marina Gkeka ◽  
Asimina Protogerou ◽  
Leonidas Marinos ◽  
Chariclia Loupa ◽  
...  

A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute amyopathic dermatomyositis. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 60
Author(s):  
Efthymia Pappa ◽  
Marina Gkeka ◽  
Asimina Protogerou ◽  
Leonidas Marinos ◽  
Chariclia Loupa ◽  
...  

A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute, clinically amyopathic dermatomyositis in adults. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).


2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Doo-Ho Lim ◽  
Min Wook So ◽  
Yeon Mee Kim ◽  
Ji Hwa Ryu ◽  
Jae Ha Lee ◽  
...  

Abstract Background In clinically amyopathic dermatomyositis, the hallmark cutaneous manifestations are the key to diagnosis. We report a case of clinically amyopathic dermatomyositis which presented with facial edema as the sole cutaneous manifestation and was later complicated by acute respiratory failure leading to death. Case presentation A 58-year-old woman presented with edema of the face that had developed approximately one year ago. There was no weakness in the extremities, and the serum creatine kinase level was within normal range. On MRI, there was diffuse edematous change in the bilateral masticator and extra-ocular muscles, accompanied by subcutaneous fat infiltration in the face. A shared decision was made to defer muscle biopsy in the facial muscles. The facial swelling almost resolved with medium-dose glucocorticoid therapy but relapsed in days at glucocorticoid doses lower than 15 mg/day. Combination therapy with either azathioprine, mycophenolate, or methotrexate was not successful in maintaining clinical remission, and the swelling became more severe after relapses. A US-guided core-needle biopsy was subsequently performed in the right masseter muscle. On pathologic examination, there was a patchy CD4 + T cell-dominant lymphoplasmacytic infiltration in the stroma, necrosis of the myofibrils and prominent perifascicular atrophy. Based on those findings, a diagnosis of clinically amyopathic dermatomyositis was made. Therapy with gamma-globulin was not effective in maintaining remission. In the sixth week after starting rituximab, she presented to emergency room with altered mental state from acute respiratory failure. Despite treatment with antibiotics, glucocorticoid pulse, cyclosporin, and polymyxin B-immobilized fiber column direct hemoperfusion, she died three weeks later from persistent hypoxemic respiratory failure. Conclusions This case showed the full spectrum and severity of internal organ involvement of dermatomyositis, although the patient presented exclusively with subcutaneous edema limited to the head. The prognosis may be more closely associated with a specific auto-antibody profile than the benign-looking initial clinical manifestation. Close follow-up of lung involvement with prophylactic treatment for Pneumocystis pneumonia and prompt implementation of emerging therapeutic regimens may improve the outcome.


2020 ◽  
Vol 3 (1) ◽  

Rationale and Objectives: Subcutaneous panniculitic T-cell lymphoma (SPTCL) is a rare neoplasm accounting for less than 1% of pediatric Non-Hodgkin Lymphomas. It is most common in young adults with predominance of female cases at 0.5. Symptoms include multiple nodules involving the subcutaneous tissues of the extremities and trunk, neck and face. B symptoms such as fever, chills, night sweats and weight loss, have been reported. Rare extra-cutaneous manifestations include edema, involvement of the bone marrow, lymph nodes, liver, spleen, lungs and viscera. Laboratory abnormalities such as cytopenias and elevated lactate dehydrogenase have been recorded. Hemophagocytic syndrome (HPS) occurs in 33% of cases, which were correlated with fatal outcome. Case Report: We present a rare case of a 17 year-old male diagnosed with subcutaneous pannicultic T-cell lymphoma manifesting with prolonged fever, weight loss, and multiple subcutaneous nodules. He also presented with extra-cutaneous manifestations of facial edema, pleural effusion and ascites, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia. Conclusion and Summary: Subcutaneous Panniculitic T-Cell Lymphoma may present with an unusual finding of facial edema, ascites and pleural effusion, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia alongside skin manifestations of subcutaneous panniculitis. HPS may complicate the course, treatment decisions and outcome of the disease.


2021 ◽  
Vol 15 (12) ◽  
pp. 3476-3477
Author(s):  
Youssef A. Alqahtani ◽  
Ayed A. Shati ◽  
Ahmad A. Alhanshani ◽  
Faisal H. Tobeigei ◽  
Saleh M. Alqahtani

Gianotti-Crosti Syndrome is a rare skin disease affecting children at the first years of age. The condition is clinically featured by blisters on the skin of the legs, buttocks and arms. A history of viral infection preceding the disease was reported for many cases. A 12-month-old boy, free of any medical illnesses, was presented with a history of acute-onset rash for five days prior to presentation that was preceded by a history of fever for two days then subsided. The rashes appeared initially on his buttocks, then spread to his upper and lower extremities and trunk. The child had fever, sore throat and coryza symptoms with lethargy. A polymerase chain reaction (PCR) swab was taken to test for SARS-CoV-2, which was positive two weeks before rash onset. All symptoms resolved in 1 week with supportive therapy and the current rashes appeared two weeks later in the patient. Gianotti–Crosti syndrome may follow the active phase of infection for some cases. It should not be misdiagnosed with dermatological simple eruptive disorders. The clinical features for covid-19 associated infection are typical for the well-known clinical picture of the syndrome. The case was reported to improve clinicians’ awareness and help in better understanding and reporting cutaneous manifestations of COVID-19. Keywords: Gianotti-Crosti Syndrome; Infants; Children; covid-19; Corona


2021 ◽  
Vol 9 ◽  
Author(s):  
Nina Emeršič ◽  
Tanja Tomaževič ◽  
Olga Točkova ◽  
Matjaž Kopač ◽  
Metka Volavšek ◽  
...  

Necrotizing stomatitis is a rare, acute-onset disease that is usually associated with severely malnourished children or diminished systemic resistance. We describe a 1-year-old girl who developed necrotizing stomatitis, vasculitic rash, skin desquamation on the fingers and toes, and persistent hypertension after serologically confirmed SARS-CoV-2 infection. Her laboratory investigations revealed positive IgG anticardiolipin and IgG anti-β2 glycoprotein antibodies, and biopsy of the mucosa of the lower jaw showed necrosis and endothelial damage with mural thrombi. Swollen endothelial cells of small veins in the upper dermis were confirmed also by electron microscopy. As illustrated by our case, necrotizing stomatitis may develop as a rare complication associated with SARS-CoV-2 infection and can be considered as a part of the clinical spectrum of COVID-19 vasculopathy. The pathogenic mechanism could involve a consequence of inflammatory events with vasculopathy, hypercoagulability, and damage of endothelial cells as a response to SARS-CoV-2 infection.


1993 ◽  
Vol 52 (3) ◽  
pp. 401-410
Author(s):  
Tsutomu Yamazaki ◽  
Chihiro Harada ◽  
Munetaka Yamakawa ◽  
Tsutomu Sohma

2021 ◽  
Vol 16 (1) ◽  
pp. 746-751
Author(s):  
Tao Wang ◽  
Qiancheng Xu ◽  
Xiaogan Jiang

Abstract A 29-year-old woman presented to the emergency department with the acute onset of palpitations, shortness of breath, and haemoptysis. She reported having an abortion (56 days of pregnancy) 1 week before admission because of hyperthyroidism diagnosis during pregnancy. The first diagnoses considered were cardiomyopathy associated with hyperthyroidism, acute left ventricular failure, and hyperthyroidism crisis. The young woman’s cardiocirculatory system collapsed within several hours. Hence, venoarterial extracorporeal membrane oxygenation (VA ECMO) was performed for this patient. Over the next 3 days after ECMO was established, repeat transthoracic echocardiography showed gradual improvements in biventricular function, and later the patient recovered almost completely. The patient’s blood pressure increased to 230/130 mm Hg when the ECMO catheter was removed, and then the diagnosis of phaeochromocytoma was suspected. Computed tomography showed a left suprarenal tumour. The tumour size was 5.8 cm × 5.7 cm with central necrosis. The vanillylmandelic acid concentration was 63.15 mg/24 h. Post-operation, pathology confirmed phaeochromocytoma. To our knowledge, this is the first case report of a patient with cardiogenic shock induced by phaeochromocytoma crisis mimicking hyperthyroidism which was successfully resuscitated by VA ECMO.


2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Eyal Meir ◽  
Chovav Handler ◽  
Uri Kaplan ◽  
Doron Kopelman ◽  
Ossama A. Hatoum

Abstract Introduction Primary lymphoma of the colon is exceedingly rare and comprises 0.2–1% of all colon tumors. The most common subtype of lymphoma in the colon is non-Hodgkin lymphoma. Symptoms are often nonspecific, and treatment varies between chemotherapy alone and a combination of surgery and chemotherapy. Case presentation We describe a case of a Ashkenazi Jew patient who presented in the typical way that carcinoma of the colon might present but turned out to have a very rare type of tumor in both its histology and its location. Conclusion There was apparent discordance between the relative bulkiness and gross appearance of the tumor with the unrevealing result of the biopsies, demanding a high level of suspicion as to the actual presence and possible type of such a tumor in the future.


2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Demetrio Larraín ◽  
Andrés Casanova ◽  
Iván Rojas

Ovarian torsion after hysterectomy is a rare event. The diagnosis of ovarian torsion is challenging because symptoms are nonspecific. We present a case of ovarian torsion 2 years after laparoscopic hysterectomy (LH). Furthermore, we performed a literature review about ovarian torsion after hysterectomy. This case shows that, in cases of acute onset pelvic pain in patients with history of hysterectomy, the adnexal torsion must be kept in mind in the differential diagnosis, especially in those women who had undergone LH.


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