scholarly journals P046 Pointing the finger: an unusual presentation of dactylitis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Natalia Cernovschi - Feasey ◽  
Julekha Wajed

Abstract Background/Aims  Dactylitis is commonly associated with psoriatic arthritis, and regularly presents at Rheumatology clinics. We discuss a case where progressive systemic symptoms lead to the consideration of alternate diagnoses. Methods  A 46-year-old Nepalese woman presented to the Rheumatology department with a 3 month history of diffuse swelling of the right middle finger proximal interphalangeal joint, with the appearance of dactylitis. There was pain on movement, but no other joint involvement. Simultaneously she noticed blurred and decreased vision, which on review by the ophthalmologists, was diagnosed with bilateral uveitis. There was no history of psoriasis, inflammatory bowel disease, or other past medical history of note. There was no travel history in the past 12 months. A diagnosis of a presumed inflammatory arthritis was made. Results  Blood tests showed elevated c-reactive protein 55 (normal <4 mg/l), erythrocyte sedimentation rate 138 (normal 0-22 mm/hr) and an iron deficiency anaemia. Rheumatoid factor and Anti-CCP antibody were negative. Hand radiographs were reported as normal. MRI of the third digit confirmed an enhancing soft tissue collection at the proximal phalanx of the right middle finger. She was referred for a biopsy of this lesion. Interestingly over the subsequent few months, she developed progressive breathlessness. Chest radiograph showed a left pleural effusion. Further tests showed negative serum ACE, Lyme and Toxoplasma screen. Quantiferon test was negative. Pleural aspirate showed a transudate with negative Acid-fast bacillus (AFB) test and culture. CT chest and abdomen showed a persistent pleural effusion, inflammatory changes in the small bowel and thickening of the peritoneum and omentum. In view of the systemic involvement, a peritoneal tissue biopsy was performed. This confirmed chronic granulomatous inflammation with positive AFB stain for mycobacterium tuberculosis. Our patient was started on quadruple anti- TB antibiotics for 6 months. Her systemic symptoms and dactylitis have improved, although there is on-going treatment for her ocular involvement. Conclusion  Approximately 10% of all cases of extrapulmonary TB have osteoarticular involvement. Dactylitis is a variant of tuberculous osteomyelitis affecting the long bones of the hands and feet. It occurs mainly in young children; however adults may be affected also. The first manifestation is usually painless swelling of the diaphysis of the affected bone followed by trophic changes in the skin. The radiographic changes are known as spina ventosa, because of the ballooned out appearance of the bone, although this was not seen in our case. Fibrous dysplasia, congenital syphilis, sarcoidosis and sickle cell anaemia may induce similar radiographic changes in the metaphysis of long bones of hands and feet, but do not cause soft tissue swelling or periosteal reaction. This case highlights the importance of testing for TB, especially in atypical cases of dactylitis, with other systemic features. Disclosure  N. Cernovschi - Feasey: None. J. Wajed: None.

2014 ◽  
Vol 10 (4) ◽  
pp. 91-94
Author(s):  
A Bhatnagar ◽  
M Deshpande

Servelle Martorelle Syndrome is a congenital vascular malformation associated with soft tissue hypertrophy and bony hypoplasia. This rarely involves whole of an extremity, with involvement of part of limbs reported in literature. We present a case of a twelve year boy who presented to the Department of Plastic Surgery SGPGIMS in April 2011 ,with history of circumferential soft tissue hypertrophy involving whole of left upper limb, scapular region and axilla since birth. The entire left upper limb length was lesser than the right upper limb. Hence this is a very rare case of Servelle Martorelle Syndrome having extensive limb involvement at a very young age. Highlighted is the role of conservative treatment and close follow-up to understand the natural history of the diseases, with prompt treatment of complications. DOI: http://dx.doi.org/10.3126/kumj.v10i4.11011 Kathmandu Univ Med J 2012;10(4):91-94


2020 ◽  
Vol 13 (2) ◽  
pp. 774-782
Author(s):  
Drew A. Fajardo ◽  
Joel France ◽  
Bogna I. Targonska ◽  
H. Bobby Kahlon ◽  
Max J. Coppes

Mantle cell lymphoma (MCL) is a relatively rare B-cell non-Hodgkin lymphoma, typically presenting with extensive lymphadenopathy, bone marrow involvement, and splenomegaly. Extranodal sites can also be involved. We discuss a 73-year-old man whose MCL presented with a 6-month history of a subdermal mass of the right upper thigh and no systemic symptoms.


2011 ◽  
Vol 26 (2) ◽  
pp. 39-41 ◽  
Author(s):  
Mark Angelo C. Ang ◽  
Ariel Vergel De Dios ◽  
Jose M. Carnate

Primary sinonasal ameloblastoma is an extremely rare odontogenic epithelial tumor histomorphologically identical to its gnathic counterparts but with distinct epidemiologic and clinicopathologic characteristics. We present a case of a 46 female with a 1 year history of recurrent epistaxis, nasal obstruction, and frontonasal headache. Clinical examination, CT scan, and subsequent surgical excsion revealed an intranasal mass attached to the lateral nasal cavity with histomorphologic features of ameloblastoma and was signed out as extragnathic soft tissue ameloblastoma of the sinonasal area. Extraosseous extragnathic primary sinonasal ameloblastoma are rare but do occur and should be distinguished from infrasellar craniopharyngiomas.   Keywords: Extraosseous, Extragnathic, Sinonasal, Ameloblastoma                   Ameloblastomas are slow growing locally aggressive odontogenic epithelial tumors of the jaw and are classified into solid/multicystic, unicystic, desmoplastic, and peripheral subtypes.1,2,3 They involve the mandible 80% of the time and are often associated with an unerrupted molar tooth. Extraosseous extragnathic Ameloblastomas are very rare, occurring less than 1.3 to 10% of all ameloblastomas, with all cases reported so far arising from the sinonasal region.1,2,4 We present a case of primary sinonasal ameloblastoma in a Filipino female. Case Report               A 46-year old female consulted at the University of the Philippines - Philippine General Hospital Department of Otorhinolayngology with a one year history of recurrent, spontaneous epistaxis from the right nose, associated with ipsilateral nasal obstruction, thin-brown rhinorrhea, and frontonasal headache relieved by oral paracetamol. Nasal endoscopy revealed a pale pink irregularly shaped polypoid mass attached to the lateral nasal wall, almost completely obstructing the nasal cavity. Plain coronal and sagittal CT images of the nasal cavity and paranasal sinuses showed opacification of the right nasal chamber by soft tissue densities with obstruction of the ipsilateral ostiomeatal unit and sphenoethmoidal recess (Figure 1). The sphenoid, frontal and contralateral paranasal sinuses and nasal vault were uninvolved. Incision biopsy was read as sinonasal exophytic papilloma and the mass was excised via endoscopic sinus surgery under general anesthesia. The submitted specimen consisted of a 2 cm by 0.8 cm cream white solid, soft to rubbery mass. On histologic examination, trabecula and islands of cytologically benign odontogenic epithelium permeate an edematous, myxoid, hypocellular stroma. Columnar cells that display palisading and reverse polarity, line the periphery of the epithelium. At the center of the epithelial islands, loose collections of stellate and spindly cells, similar to the stellate reticulum of the embryonic enamel organ, are found. Acanthomatous changes are present in the superficial layers. There is no atypia and no mitosis (Figures 2 and 3). This case was signed out as extragnathic soft tissue ameloblastoma. Discussion               Most reported cases of ameloblastoma in the sinonasal cavity actually describe tumors that originated from the maxilla and have only secondarily involved the sinonasal area.4 To date, the 26-year review by Schafer et al. of 24 primary sinonasal tract ameloblastomas at the Armed Forces Institute of Pathology remains the single largest series describing this entity.4 Although three additional case reports were recently published, to the best of our knowledge, this is the 1st case of primary sinonasal ameloblastoma in the Philippines.5,6,7 Unlike our patient, primary sinonasal ameloblastomas more commonly affect males with mean age at presentation of 59.7 years.1,4 Patients usually present with an intranasal mass, nasal obstruction, sinusitis and epistaxis of 1 month to several years duration.1,4 Radiologically, sinonasal ameloblastomas are solid masses or opacifications rather than multilocular and radiolucent as those that arise within the jaws.1 The histomorphologic features of primary sinonasal ameloblastomas are identical to their gnathic counterparts and include unencapsulated proliferating nests, islands or sheets of odontogenic epithelium resembling the embryonic enamel organ. The epithelium is composed of a central area of loosely arranged cells similar to the stellate reticulum of the enamel organ and a peripheral layer of palisading columnar or cuboidal cells with hyperchromatic small nuclei oriented away from the basement membrane, the so called reverse polarity.1 Experts believe that primary sinonasal ameloblastomas arise from remnants of odontogenic epithelium, lining of odontogenic cysts, basal layer of the overlying oral mucosa, or heterotopic embryonic organ epithelium.1,4 This is supported by the observation that the ameloblastomatous epithelial proliferations are often seen in continuity with native sinonasal (schneiderian) epithelium.1,4 This entity should be distinguished from an infrasellar craniopharyngioma, which is an important differential diagnosis that is often difficult and often virtually impossible to differentiate from a primary sinonasal ameloblastoma solely on histomorphologic grounds. In most cases, however, clinicopathologic correlation guides the diagnosis8 and special stains are of limited utility.1  Surgical excision is the treatment of choice, the type and extent of which is dictated by the size and localization of the lesion. Recurrence can occur, generally within 2 years, but overall treatment success depends on complete surgical eradication. No deaths, metastases, or malignant transformation have so far been reported1,4 and our patient is free of disease, fifteen months post surgery.


Author(s):  
Tudor Morar ◽  
Radu Pirlog ◽  
Sonia Vlaicu ◽  
Vasile Bintintan ◽  
Doinita Crisan

Necrotizing myositis represents a rare, aggressive form of bacterial-induced soft tissue necrotizing infection. We present a fulminant case of a 44-year-old patient with a necrotizing soft tissue infection  and a history of rheumatoid arthritis transferred to our service, Cluj-Napoca Emergency County Hospital, from a local hospital where he had been admitted two days before with chills and light-headedness after an accidental minor blunt trauma in the right thigh region. After admission to our hospital and first assessment, broad spectrum antibiotherapy was started with Meropenem, Vancomycin and Metronidazole along with surgical debridement. The evolution was fulminant with rapid development of multiple organ dysfunction syndrome, therefore he was transferred to the intensive care unit, intubated, and started the volemic resuscitation and vasopressor therapy. The blood culture was positive for group A beta-hemolytic streptococcus (GAS) and high dose Penicillin G was added to the therapeutic scheme. Despite all efforts, the patient developed disseminated intravascular coagulation syndrome and died in the next hours. The clinical picture together with the findings from the autopsy were suggestive for a streptococcal toxic shock syndrome developed as a complication of GAS induced necrotizing myositis.


2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Christopher B. Toomey ◽  
Andrew Gross ◽  
Jeffrey Lee ◽  
Doran B. Spencer

Intraocular coccidioidomycosis is a rare condition, with the most commonly reported presentation being an idiopathic iritis in patients who live in or have traveled thorough endemic areas. A paucity of reports exists describing the chorioretinal manifestations of coccidioidomycosis. Here we report a case of unilateral coccidioidal chorioretinitis and meningoencephalitis in an AIDS patient that led to near complete unilateral loss of vision. A 48-year-old Hispanic female with poorly controlled HIV/AIDS in southern California presented with a three-week history of headache, nausea, vomiting, right eye blurry vision, and a one-day history of subjective fever. Examination of the right eye revealed vitritis and several large chorioretinal lesions scattered throughout the periphery and macula with optic disc pallor. Serum coccidioidomycoses complement fixation (CF) was positive (titers of 1 : 256). Neuroimaging revealed a new area of enhancement in the left anterior frontal lobe consistent with meningoencephalitis. The patient was treated with intravenous fluconazole and intravitreal voriconazole with resolution of systemic symptoms and vitritis but persistence of unilateral, severe chorioretinal scarring and vision loss. In conclusion, in spite of the rarity of intraocular coccidioidomycosis, one must carry a degree of suspicion for this vision- and life-threatening condition as a potential etiology of chorioretinitis in individuals with pertinent risk factors.


2019 ◽  
Vol 12 (1) ◽  
pp. e223365
Author(s):  
Kiran Dhaliwal ◽  
Colin Thomas Brewster ◽  
Sivarajasingham Pakeerathan

Acute blue finger syndrome is a rare benign condition that mimics digital ischaemia. We discuss the case of a 32-year-old woman who presented with a 6hour history of blue discolouration of the middle finger of the right hand, associated with pain and swelling. There was no history of trauma and this was the first time that the patient had experienced these symptoms. Examination found blue discolouration of the digit primarily on the volar aspect with associated swelling. All investigations, including blood tests, X-rays and Doppler scanning, were normal. The symptoms resolved spontaneously within 48 hours. There were no recurrent episodes or long-term sequelae. Patients presenting with an acutely blue finger need rapid assessment to exclude digit ischaemia. Knowledge of this rare benign condition may prevent unnecessary distress, invasive investigations and potentially harmful treatment of a healthy patient.


2021 ◽  
pp. 35-36
Author(s):  
Tannistha Chakraborty ◽  
Vamsi Krishna ◽  
T. Raghupathy

Lipoma, a benign neoplasm of adipocytes, is the most common mesenchymal soft tissue tumor of adulthood, but rarely present with huge sizes in their cutaneous localization. Liposarcomas are the second most common soft tissue sarcoma following malignant brous histiocytoma. They comprise approximately 16-18% of all malignant soft tissue tumours. It is most common in the trunk followed by lower extremity, upper extremity and head and neck.. While lipomas can be clinically diagnosed , transformation of lipomas into liposarcoma is rare. Here we report the case of a 55 gentleman, who came with a history of swelling over the right shoulder with a probable malignant transformation due to history of long duration of presence of swelling. Management is usually by wide excision. Local radiation and chemotherapy may be necessary for high grade lesions.


2020 ◽  
Vol 13 (3) ◽  
pp. 1082-1090
Author(s):  
Rosy Setiawati ◽  
Vivid Umi Varidha ◽  
Giuseppe Guglielmi ◽  
Filippo Del Grande

Traumatic lesions of the axillary artery itself are limited to 2.9–9% of major arterial injuries. Pseudoaneurysms represent a pulsating encapsulated hematoma in communication with the lumen of a ruptured vessel. Traumatic pseudoaneurysm of the axillary artery is a rare sequela of injury to the shoulder region. We describe a case of posttraumatic pseudoaneurysm involving the axillary artery, which was initially misdiagnosed as an aggressive soft tissue tumor. The man presented 10 years after an injury from a fall from a tree with a slowly growing mass in the right upper limb region and reduced range of movement. This is a neglected case with a history of traditional massage. The patient presented a pathologic fracture of the right proximal humerus and dislocation of the glenohumeral joint. At the beginning, it was suggested to be a primary soft tissue tumor, but after several examinations, including comparable X-ray, ultrasound, and histopathology, the results did not support a soft tissue tumor. Magnetic resonance imaging and computed tomography angiography (CTA) finally confirmed the finding of a pseudoaneurysm of the right axillary artery associated with a huge hematoma with different age of the bleeding product and granulation tissue. This case demonstrates the necessity of early diagnosis of axillary artery pseudoaneurysm to prevent complications after a history of trauma. CTA is a useful modality to evaluate vascular injury and provides valuable information.


2009 ◽  
Vol 69 (3) ◽  
pp. 542-545 ◽  
Author(s):  
Abdurrahman Tufan ◽  
Ingrid Meulenbelt ◽  
Jessica Bijsterbosch ◽  
Herman M Kroon ◽  
Sita M A Bierma-Zeinstra ◽  
...  

BackgroundTibiofemoral alignment has a role in knee osteoarthritis (OA), but which factors contribute to alignment is unknown.ObjectiveTo investigate familial aggregation of tibiofemoral alignment in participants of the GARP (Genetics ARthrosis and Progression) study.MethodsThe tibiofemoral anatomical angle on semiflexed knee radiographs was measured in sibling pairs (mean age 60 years, 81% women) with primary OA with multiple joint involvement. Radiographic OA was assessed according to the Kellgren–Lawrence (KL) method. Heritability estimates of the tibiofemoral angle were calculated by comparing twice the between-sibling variance with the total variance; adjustments were made for age, gender, body mass index, history of meniscectomy, lower limb fracture and in analyses including all knees, for KL score.Results360 subjects representing 180 families were studied. The mean (SD) tibiofemoral angle of right and left knees in the probands was 182.7 (2.9)° and 182.8 (2.6)°, respectively; similar angles were measured in the siblings. Radiographic knee OA (KL score ≥2) was present in 27% of the knees. Stratified analyses in sib pairs with non-osteoarthritic right or left knees showed adjusted heritability estimates of the tibiofemoral angle of the right and left knees of 0.42 (95% CI 0.02 to 0.82) and 0.56 (95% CI 0.19 to 0.93). In addition, adjusted heritability estimates of the tibiofemoral angle in all right and left knees were calculated, being 0.48 (95% CI 0.18 to 0.78) and 0.50 (95% CI 0.21 to 0.79), respectively.ConclusionThe alignment of the tibiofemoral joint is influenced by familial factors, implying that tibiofemoral malalignment may add to the genetic predisposition for knee OA development. These results need to be confirmed in other study populations.


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