Upper and Lower Respiratory Mucous Membrane Plasmacytosis with a Cobblestone Appearance: a Case Report.

A 53-year-old woman with a recurrent sore throat, xerostomia, nasal obstruction, and nasal discharge came to our hospital. At the first visit, she did not have a fever, but a blood test showed an intense inflammatory response. She had oral mucosal erosion. She developed dyspnea 2 months later. Nasendoscopy, laryngoscopy, and bronchoscopy showed upper and lower respiratory mucosa cobblestone appearance. Microscopy of the nasal and pharyngeal mucosa biopsy found regular mononuclear inflammatory cell dense submucosal infiltrate, mainly plasma cells. A mucous membrane plasmacytosis diagnosis was made. Prednisolone 1 mg/kg/day rapidly improved bronchial symptoms and cobblestone appearance.

Ichthyosis Prematurity Syndrome: A Rare Form but Easily Recognizable Ichthyosis.

Ichthyosis prematurity syndrome is a rare autosomal recessive genodermatosis that is associated with mutations in the SLC27A4 gene. Its onset occurs in early childhood and presents with the clinical triad of premature birth, thick caseous desquamating epidermis, and neonatal asphyxia. Here, we describe a prematurely born baby patient (33 weeks of gestation) with a homozygous variant at the initiation codon site (c.1 A> G, p.Met1Val) in the SLC27A4 gene to raise awareness of this rare syndrome despite its distinctive features as we believe it is still underdiagnosed.

Chronic active EBV infection in refractory enteritis with longitudinal ulcers with a cobblestone appearance: an autopsied case report.

BACKGROUND: Chronic active Epstein-Barr virus infection (CAEBV) is defined as Epstein-Barr virus (EBV)-positive T/NK cell-related neoplasia, and its major clinical symptom is systemic inflammation presenting as infectious mononucleocytosis, whereas enteritis and diarrhea are minor clinical symptoms. The complex mixture of tumorigenic processes of EBV-positive cells and physical symptoms of systemic inflammatory disease constitutes the varied phenotypes of CAEBV. Herein, we describe a case of CAEBV that was initially diagnosed as Crohn's disease (CD) based on ileal ulcers and clinical symptoms of enteritis. CASE PRESENTATION: A 19-year-old woman complained of abdominal pain and fever. Blood examination showed normal blood cell counts without atypical lymphocyte but detected modest inflammation, hypoalbuminemia, slight liver dysfunction, and evidence of past EBV infection. The esophagogastroduodenoscopic findings were normal. However, colonoscopy revealed a few small ulcers in the terminal ileum. The jejunum and ileum also exhibited various forms of ulcers, exhibiting a cobblestone appearance, on capsule endoscopy. Based on these clinical findings, she was strongly suspected with CD. In the course of treatment by steroid and biologics for refractory enteritis, skin ulcers appeared about 50 months after her initial hospital visit. Immunohistology of her skin biopsy revealed proliferation of EBV-encoded small RNA (EBER)-positive atypical lymphocytes. We retrospectively assessed her previous ileal ulcer biopsy before treatment and found many EBER-positive lymphocytes. Blood EBV DNA was also positive. Therefore, she was diagnosed with extranodal NK/T-cell lymphoma with CAEBV-related enteritis rather than CD. She was treated with cyclosporine and prednisolone combination therapy for CAEBV-related systemic inflammation and chemotherapy for malignant lymphoma. Unfortunately, her disease continued to progress, leading to multiple organ failure and death at the age of 23 years. CONCLUSION: Clinicians need to remember the possibility of CAEBV as a differential diagnosis of refractory enteritis. Enteritis with intestinal ulcer is a rare symptom of CAEBV, and it is impossible to acquire a definitive diagnosis by ulcer morphology only. In cases where the possibility of CAEBV remains, tissue EBVR expression should be checked by in situ hybridization and blood EBV DNA.

Cytomegalovirus Colitis with a Cobblestone Appearance.

Cytomegalovirus (CMV) colitis with a cobblestone appearance is rare, and its endoscopic and pathological features remain poorly described. A 78-year-old woman was admitted to our hospital for disturbance of consciousness, high-grade fever, and diarrhea occurring up to 7 times a day. Sigmoidoscopy revealed a circumferential ulcer with mucosal defect, an ulcer with a cobblestone appearance extending from the upper rectum to the sigmoid colon, and an irregular ulcer on the lower rectum. She was histopathologically diagnosed with CMV colitis and intravenously treated with ganciclovir (5 mg/kg) for 7 weeks. Colonoscopy after treatment revealed an improvement of the ulcers extending from the rectum to the sigmoid colon, but no significant improvement of her general condition. She died from sepsis 4 months after hospitalization. To our knowledge, this report represents a valuable addition to the CMV literature describing a rare case of CMV colitis with a cobblestone appearance.

Assessment of the skin tissue structure using an ultrasonic diagnostic imaging device in patients undergoing open surgery.

BACKGROUND: Perioperative skin injury is a major issue; therefore, several preventative measures have been developed. However, no previous studies have visualized the effects of stromal edema caused by surgical invasion of skin tissue, and therefore, the details remain unknown. We used an ultrasonic diagnostic imaging device to clarify changes in the skin tissue structure of patients after open surgery. MATERIALS AND METHODS: Twenty subjects who underwent open hepatectomy were enrolled. We selected the lateral abdomen, upper arms, and lower legs as ultrasonic imaging measurement sites. We performed measurements on the day before surgery and on postoperative days 1, 3, and 5. We calculated the epidermal/dermal tissue thickness, subcutaneous tissue thickness, and skin tissue thickness. We performed a one-way analysis of variance with repeated measurements for each of the postoperatively measured values on the basis of the preoperative values. Significantly different variables were subjected to the Bonferroni method. We evaluated ultrasonic imaging findings and skin injury. RESULTS: Epidermal/dermal tissue thickness at all measurement sites exhibited sustained thickening on postoperative day 5 compared to that preoperatively. The lateral abdomen exhibited thickening of the subcutaneous tissue and skin tissue on postoperative day 1. In addition, increased echogenicity, increased opacity of the layer structure, and a cobblestone appearance occurred during the postoperative course. Postoperatively, 80% of subjects exhibited skin injury. CONCLUSION: We evaluated the effects of surgical invasion on skin tissue over time. Continual observation and protective skincare are necessary near the surgical wound, where significant invasiveness occurs. Prevention of skin injury due to skin tissue thickening requires further study.