Tongue Necrosis as a Manifestation of Immune Dysfunction: A Complex Case of Lupus, Histoplasmosis, and Macrophage Activation SyndromeMadalyn Walsh, MD1, Alick Feng, MD2, Petar Lenart, MD PhD2, Bharat Kumar, MD MME21Department of Internal Medicine, University of Iowa Hospitals & Clinics2Division of Immunology, University of Iowa Hospitals & Clinics
INTRODUCTION:Human beings are often infected with species of filariasis which are naturally found in animals. Among these the commonly reported is due to dirofilaria species - D repens and D imitis. It is transmitted by mosquito bite. The review of literature revealed around 800 cases reported worldwide. Most of the pediatric cases reported in India are ocular dirofilariasis. Subcutaneous dirofilariasis cases are mostly reported in adult population. Here we present a series of four cases of subcutaneous dirofilariasis in children presenting as subcutaneous nodules.
Perforated marginal ulcer following Whipple procedure: A case reportAbstractMarginal ulcers are rare complications of pancreatoduodenectomy. Patient can present with varying symptoms such as epigastric discomfort, pain, dysphagia, or can land in emergency with complications like bleeding and perforation.IntroductionWhipple procedures are performed for variety of benign and malignant lesions affecting the pancreatic head, duodenum, and distal bile duct.1 Marginal ulcer, one of the rare long term complications of pancreatoduodenectomy, are ulcerations that occur at or around the gastrointestinal anastomosis.2 Their associated morbidity and mortality have been infrequently described in literature.3 Here we present a case of a gentleman with a 6 year old history of Whipple procedure who presented in emergency department with acute onset abdominal pain and was later diagnosed with perforated marginal ulcer.Case presentationA 64-year-old retired soldier who underwent the Whipple procedure six years ago for carcinoma head of pancreas, adenocarcinoma (well-differentiated adenocarcinoma) presented to emergency department with complaints of severe abdominal pain for a one day on the day of presentation. The pain was acute in onset, continuous, non-radiating, and increasing in severity, which used to be aggravated after ingestion of food and movement. He had three episodes of vomiting since morning on the day of presentation. On his past history, he underwent the Whipple procedure six years ago and has received complete six cycles of chemotherapy after surgery. He was under irregular follow-ups for past two years. The patient had no other comorbid illnesses.On his arrival to the emergency department, his pulse rate was 130beats per minute, regular; oxygen saturation 85 % on room air; blood pressure 110/70 mm Hg, body temperature 38.7 ◦C, and respiratory rate(RR) 22 breaths/min. On his physical examination, his abdomen was distended with diffuse tenderness. There was diffuse guarding and rigidity all over the abdomen. Bowel sounds were absent. Digital rectal examination revealed a normal sphincter tone with a collapsed rectum and absent fecal stain on the gloved finger. He was immediately administered crystalloids and supplemental oxygen at 4 L/min. Nasogastric tube decompression and Foley catheterization were done. His laboratory parameters showed leukocytosis with raised amylase. Liver function test revealed total bilirubin 1.80 mg/dL, conjugated bilirubin 0.8 mg/dL and alkaline phosphatase 712U/L. On radiological examination, supine abdominal X-ray showed prominent dilated small bowel loops and free gas under right hemi diaphragm pointing towards hollow viscous perforation (Fig. 1). Ultrasonography of the abdomen and pelvis was unremarkable with minimal free fluid in the pelvis.After an initial fluid resuscitation, an emergency laparotomy was done. Intraoperatively, The findings were 300 ml of bilious fluid in the peritoneal cavity and dense adhesion between the small bowel loops and previous surgical scar. Adhesions were meticulously released and gastrojejunostomy site perforation was there, which was around 1 cm Fig.1. A thorough peritoneal lavage was done and the gastrojejunostomy site perforation was closed with a well-vascularized omental patch after a biopsy from the ulcer edge. He received Meropenem IV 1 g and Vancomycin IV 500 mg twice daily along with low molecular weight Heparin 60 mg twice daily the following day. His condition gradually improved and was discharged on 10th post operative day.
Chronic limb-threatening ischemia (CLTI) with severe below-the-ankle (BTA) lesions is often difficult to revascularize with endovascular treatment (EVT) and surgical treatment. We present a case of successful limb salvage using percutaneous deep venous arterialization (pDVA) in a patient with CLTI whose BTA lesion reconstruction failed. A 57-year-old man with diabetes mellitus
Background: The purpose of this article is armed to provide some experience and insight for the clinical treatment of primary mediastinal yolk sac tumor (PMYST) basing on our case report and relevant literature review. Case report and literature review: A 15-year-old boy was diagnosed as PMYST with the volume about 13*12*8cm. The
IntroductionTumoral calcinosis (TC) is a rare clinicopathological entity characterized by the presence of calcified deposits in periarticular soft tissue. The calcified deposits predominantly comprise of calcium hydroxy apatite crystals and amorphous calcium phosphate1. Clinically, they appear as a painless slowly growing mass in the vicinity of large joints such as hips, shoulder, and elbow 2, 3. However, it has also been reported in other areas such as the neck, arms, hands, spine, legs, and feet 3. The progressive growth of the mass may result in pain, nerve compression, and functional impairment4. The overlying skin is usually intact; however, long-standing lesions may lead to skin ulceration and sinus tract formation with chalk white drainage 2.The diagnosis of TC is based on clinical and imaging findings. It has a characteristic radiographic appearance of multilobulated calcification in periarticular soft tissues, commonly on the extensor side of the articulation. Radiolucent lines separate the lobules (fibrous septa)5, 6. Ultrasound can be used to examine lesions, especially with minimal calcification, appearing as heterogeneous multiloculated mass with multiple cavities separated by hyperechoic thin septa. Color doppler may detect blood flow in some of these septa. MRI is superior to CT scan in detecting the extent of the lesions and their relationship with surrounding structures 6. However, histopathological examination is required for definitive diagnosis. Treatment options include both medical and surgical intervention5, 7.We present a case of tumoral calcinosis with peculiar presentation. A case of 4-year-old female with tumoral calcinosis of the extensor indicis proprius tendon on the right wrist.
Benign penile nodules in infants can cause distress to mothers and diagnostic challenges for physicians. Smegma pearls, characterized by desquamated epithelial cells, are a common type of benign nodule that can be managed with reassurance. We present similar case which presented to the rural primary care centre in Nepal.
Introduction:Floppy Eyelid Syndrome (FES) describes the disease process whereby extreme laxity of the eyelid can lead to recurrent irritation of the ocular surface and corresponding palpebral keratoconjunctivitis1,2. Clinical exam characteristically reveals an eyelid that is easy to evert with traction superiorly2. However, FES can masquerade as conditions such as dry eye syndrome, keratitis, conjunctivitis, and dermatochalasis1-3. Incidence in the general population has been estimated to range from 2.3% to 15.8%, though notably it may not always be correctly diagnosed and may be more common1,2.We describe a case of unilateral and chronic keratoconjunctivitis unresponsive to standard medical therapy. Applying upward traction on the upper eyelid confirmed the diagnosis, as it revealed extensive laxity of the tarsal plate with resultant lagophthalmos and exposure keratopathy. Tape splint tarsorrhaphy (TST) has been recently described as a novel and non-invasive treatment modality for persistent corneal epithelial defects4. In this case, the TST was used as a novel and non-invasive diagnostic and temporizing tool to splint the eyelid down to address exposure keratopathy from lagophthalmos. Resolution of signs and symptoms was diagnostic and therapeutic confirmation that the keratoconjunctivitis resulted from the lagophthalmos and FES. This is the first such report to our knowledge discussing the specific use of this technique to more effectively diagnose refractory keratoconjunctivitis due to lagophthalmos and FES.
MULTICENTRIC RETICULOHISTIOCYTOSIS- A RARE AND DISABLING DISEASEOmowunmi Ashaolu1, Selwyn Ng2, Shaun Smale3, Jenny Hughes11Clinical fellow, Department of Dermatology, Princess of Wales Hospital, Bridgend, UK, email@example.comConsultant, Department of Histopathology, Morriston Hospital, Swansea, UK, firstname.lastname@example.orgConsultant, Department of Rheumatology, Princess of Wales Hospital, Bridgend UK, email@example.comConsultant, Department of Dermatology, Princess of Wales Hospital, Bridgend, UK,firstname.lastname@example.org.A 39-year-old man presented with 5-week history of multiple discrete and skin-coloured lesions on his hands, forearms, and face. He also had severely painful, stiff, and swollen fingers. He was otherwise well with no relevant personal or family history. Skin examination showed multiple discrete and coalescing firm, reddish brown, tender papules of varying sizes distributed mainly over the dorsum of the hands and nail folds (Fig 1) with no mucosal involvement. There was bilateral swelling and stiffness of the joints, especially the distal interphalangeal joints.Baseline investigations, autoantibody, viral screen, and staging CT to rule out underlying malignancy were normal. Radiographs of both hands showed soft tissue swelling and subtle perierosions in the small joints of the hands. Histopathology of the skin biopsy showed infiltration of the dermis and subcutaneous fat by mononuclear and multinucleated histiocytes with granular eosinophilic cytoplasm that showed positivity for CD45, CD68 and factor 13a and negativity for S100- and CD1a (Fig 2). Based on these findings a diagnosis of multicentric reticulohistiocytosis was made.He was initially treated with non-steroidal anti-inflammatory, Morphine, Alendronate and Oral corticosteroids. With worsening disease activity, Denosumab, oral methotrexate, hydroxychloroquine, infliximab, tocilizumab and leflunomide were added sequentially, however there was no symptom improvement. He was then considered for Tofacitinib 5mg twice daily with improvement after 4 weeks of initiating this therapy evidenced by improved sleep, skin symptoms and joint pains.Multicentric reticulohistiocytosis is a rare, multisystem inflammatory disease with unknown aetiology. It was first reported by Weber and Freudenthal in 1937, however Goltz and Laymon coined the term multicentric reticulohistiocytosis in 1954. Approximately 300 cases have been reported in the literature with most from Europe and United States. It is slightly predominant in women1 and symptoms manifest from the fourth decade of life2. Associated involvement of the joints impacts significantly on patients’ quality of life due to limitations on activities of daily living which inadvertently increases the risk of increased anxiety and depression.The cause of MRH is not known but activated macrophages and increased levels of TNF-alpha, IL-12, IL-1, and IL-63 have been documented.Cutaneous manifestation of MRH includes firm, discrete clustered reddish brown to flesh-coloured papules or nodules that gradually enlarge in size. It typically occurs over the joints of fingers and wrists giving a characteristic coral bead appearance1 as seen in our patient. Other commonly affected include the face giving leonine facies. However any part of the body can be affected including nail4 , mucosal surfaces1, lungs and heart. Fever, weight loss and malaise have also been reported. In addition, symmetrical polyarthritis mainly affecting the hands can potentially progress to arthritis mutilans. Differential diagnosis includes rheumatoid arthritis and psoriatic arthritis.Although MRH is not a paraneoplastic disease, solid organ especially breast and stomach carcinomas and haematological malignancies have been reported in 31% of cases5 therefore underlying malignancy should always be ruled out in a patient with MRH.There is no laboratory test specific for MRH. Histological studies show multinucleated giant cells with pale, fine, granular eosinophilic cytoplasm in the dermis. Stains are negative for S100, CD34, factor XIIIa and alpha-1-antitrypsin but positive for PAS and histiocytes (vimentin, CD68, CD45 and MAC387)2. Langerhans granules are absent on electron microscopy.There are no established treatment guidelines owing to the rarity and lack of controlled studies of the disease. The disease can be self-limiting, however this may have caused severe joint destruction. Prednisolone, immunomodulatory and disease modifying antirheumatic drugs, TNF inhibitors either as monotherapies or combination therapies have shown varying response. Tofacitinib, a selective JAK inhibitor, licenced for the treatment of rheumatoid arthritis has also been reported to be of benefit in the treatment of MRH6.MRH is a rare disease that cause disfiguring skin lesions and disabling arthritis therefore early recognition, and treatment is important to prevent long term deformities. In conclusion, we present a patient with a very rare non-Langerhans cell histiocytosis with typical clinical, histopathological and immunohistochemical findings.aFig 1: Showing coral bead appearance of multicentric reticulohistiocytosis
1. INTRODUCTIONOsteochondromas (OC) are benign tumors that account for 20-50% of benign and 10% of all bone tumors. The incidence rate of OC in males is twice that of females and its peak is usually in the second decade of life (1). These lesions are inherited in an autosomal dominant manner and cause the formation of isolated lesions or multiple exostoses during the development of bones in the process of enchondral ossification in the long bones (2). Also, OC is associated with the mutation of some tumor suppressor genes, including EXT1 or EXT2 genes. OC have a periosteal origin and are formed in the active parts of bones, including the metaphysis of long bones and the cartilage at their ends. Studies show that OC can be caused after surgery/radiation-induced injury and hematopoietic stem cell transplantation (3).Osteocartilaginous exostosis is mainly detected in childhood and in the form of palpable masses, with chronic pain and sometimes with edema (4). The presence of misplaced bone masses in the joint capsule and sometimes with cartilaginous coating in radiographic images is one of its common manifestations. The most common joints involved in OC are the hip and knee joints. Patellar OC is rare and mostly affects the patellar bursa. Moraes et al. (2014) reported a patellar OC measuring 8 × 6 × 3 cm anterior to the patella in a 60-year-old man who was painless and without limitation of flexion-extension in the knee joint (5). In the present case, rare retro-patellar OC was observed in the area of the patellar ligament.