Pyoderma gangrenosum is uncommon neutrophilic dermatosis characterized by richness of the mature neutrophilic polynuclear dermal infiltrate. Pyoderma gangrenosum is associated with variable diseases, most commonly inflammatory bowel disease, hematological diseases, malignancies, but it is reported rarely in rheumatoid arthritis. We report a case of pyoderma gangrenosum in rheumoid arthritis patient. A 50-year-old woman admitted to our hospital due to painful pretibial ulcerative skin lesions. She had been treated as rheumatoid arthritis for 8 years. At admission, body temperature was 36.5degrees C and other vital sign was unremarkable. Physical examination revealed right pretibial ulceration, multiple pustules on left pretibial area and both palms. Laboratory studies revealed WBC count 7,600/uL (neutrophils 60.3%, eosinophil 3.2%), hemoglobin 11.4 g/dL, platelet count 319,000/uL, ESR 65 mm/hour. Other lab findings were also unremarkable. Skin biopsy was done, which showed dense dermal infiltrate of neutrophils and wound culture were negative. By 8 weeks after systemic high dose corticosteroid (1 mg/kg/day), cyclosporine A (5 mg/kg/day), sulfasalazine 2 g therapy, symptoms and skin ulceration were being improved. Without skin relapse, she is followed up our hospital with low dose corticosteroid and sulfasalazine.
Arthritis* ; Arthritis, Rheumatoid ; Biopsy ; Body Temperature ; Cyclosporine ; Eosinophils ; Female ; Hematologic Diseases ; Humans ; Inflammatory Bowel Diseases ; Middle Aged ; Neutrophils ; Physical Examination ; Platelet Count ; Pyoderma Gangrenosum* ; Pyoderma* ; Recurrence ; Skin ; Skin Diseases ; Skin Ulcer ; Sulfasalazine ; Ulcer ; Vital Signs ; Wounds and Injuries

Hydroxyurea is an antineoplastic agent with selective cytotoxicity for cells in the DNA synthesizing phase, or S phase, of the cell cycle. It is commonly used in the treatment of myeloproliferative disorders, e.g., chronic myelogenous leukemia, essential thrombocythemia and polycythemia vera. Its major adverse reactions are reversible and dose dependent marrow suppression and gastroenteric disturbances. Cutaneous side effects such as erythema, hyperpigmentation, lichen planus-like dermatitis, nail discoloration and alopecia, atropy of the skin occur, especially with long-term treatment. Painful leg ulcers in association with hydroxyurea have only rarely been reported. The ulcers were usually extremely painful and typically located near the malleoli but were occasionally found over the tibia, on the dorsal aspect of the feet, calves, knees, heels, and hands. Any minor trauma could precipitate skin breakdown and ulceration and these ulcers tended to heal slowly. No consistent correlation between the dose or duration of hydroxyurea therapy and the occurrence of ulcers. Complete wound healing was achieved by simply discontinuing treatment with hydroxyurea. We describe 2 patients who developed spontaneous painful lower leg ulcers during long-term hydroxyurea therapy for a myeloproliferative disorder(chronic myelogenous leukemia and essential thrombo cythemia). All ulcers were painful and typically located both lateral malleoli. These ulcers healed only after hydroxyurea was withdrawn and with conservative therapy including manual debridement and occlusive dressing.
Alopecia ; Bone Marrow ; Cell Cycle ; Debridement ; Dermatitis ; DNA ; Erythema ; Foot ; Hand ; Heel ; Humans ; Hydroxyurea ; Hyperpigmentation ; Knee ; Leg Ulcer* ; Leg* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid ; Lichens ; Myeloproliferative Disorders ; Occlusive Dressings ; Polycythemia Vera ; S Phase ; Skin ; Thrombocythemia, Essential ; Tibia ; Ulcer ; Wound Healing

Background and Objectives: In 2021, biologics were approved for treating chronic rhinosinusitis with nasal polyps (CRSwNP) in Korea. However, CRS is a heterogeneous disease, and its characteristics are thought to differ between Western and Korean populations. This study aimed to evaluate the experiences of members of the Korean Rhinologic Society during the first year of biologic usage for the treatment of nasal polyps. Methods: An anonymous survey consisting of 15 items was conducted from November to December 2021. The survey included questions about participant demographics, use of biologics for treating CRSwNP, and expectations regarding the effectiveness of biologics for treating CRSwNP. Results: In total, 44 members participated in the survey. Approximately half of the respondents were in their 40s (50.0%) and had 5–9 years of clinical experience as otorhinolaryngologists (47.7%). The majority of participants held academic positions (95.4%). About half of them worked in Gyeonggi Province. The utilization of biologics did not differ significantly based on clinical experience (p=0.192). When asked about the factors considered for prescribing biologics, the most common reason was recurrence of polyps after surgery (87.2%). The most frequent reason for discontinuing biologics was cost (48.6%). When asked about the extent to which they expected that the availability of biologics for CRSwNP treatment would reduce endoscopic sinus surgery (ESS), 45.5% of members expected a reduction of approximately 10%–29%. In addition, 20.5% expected a reduction of 50% or more. However, 61.4% expected a reduction of less than 10% in primary ESS. In addition, most respondents (93.2%) agreed with the need for Korea-specific guidelines for biologic treatment. Conclusion There are discrepancies between the current guidelines for biologic treatment of CRSwNP and the reality of the situation, highlighting the need for the development of Korea-specific guidelines.

Chronic rhinosinusitis (CRS) is characterized by prolonged inflammation of the nasal and paranasal sinus mucosa lasting over 12 weeks. CRS is divided into two main types based on the presence of nasal polyps: CRS without nasal polyps and CRS with nasal polyps. The condition is further classified into endotypes based on type 1, type 2, and type 3 inflammatory signatures, with differences in terms of disease severity, prognosis, and treatment response. Recent studies have emphasized the importance of the epithelial-mesenchymal transition (EMT) in CRS progression. In CRS, the EMT can be triggered by infections, allergens, hypoxia, and environmental pollutants. Specifically, EMT induction proceeds through the following mechanisms: viral and bacterial infections disrupt the epithelial barrier, house dust mites and other allergens activate the TGF-β and EGFR signaling pathways, hypoxia increases HIF-1α and other mesenchymal markers, and diesel exhaust particles and particulate matter cause oxidative stress. Maintaining the integrity of the epithelial barrier is essential for nasal mucosa homeostasis. In CRS, barrier damage activates repair processes that trigger the EMT, resulting in barrier dysfunction and tissue remodeling. Epithelial barrier dysfunction allows antigens and pathogens to penetrate, perpetuating inflammation and promoting the EMT. This disruption is a hallmark of CRS, emphasizing the importance of barrier integrity in the development of the disease. Key signaling pathways regulating the EMT in CRS include TGF-β, Wnt, HMGB1, AGE/ERK, TNF-α, and various miRNAs. These signaling pathways connect to various downstream pathways, such as the Smad2/3, GSK-3β/β-catenin, RAGE, and NF-κB pathways. This review focuses on the complex mechanisms of the EMT in CRS, emphasizing the role of epithelial barrier dysfunction and subsequent EMT processes in driving the disease’s development and progression. A deeper understanding of these EMT-driven mechanisms will help identify the potential therapeutic targets aimed at restoring epithelial integrity and reversing the EMT.

A patient with idiopathic hypereosinophilic syndrome is reported who developed a drug fever that may be related to the administration of hydroxyurea. Typically, this form of fever develops after a few weeks of exposure to the drug and disappears with withdrawal of the drug and recurs on reexposure to the drug. The mechanism of hydroxyurea-induced fever remains unclear.
Fever* ; Humans ; Hydroxyurea ; Hypereosinophilic Syndrome

The differentiation between Malignancy-Related Ascites(MRA) and Non-Malignant Ascites (NMA) is important for further diagnostic and therapeutic procedures. Althought many parameters were investigated, none has provided a complete distinction between MRA and NMA. We investigated several ascitic fluid parameters to determine the differential power, and to ifferentiate malignant-related from nonmalignant-related ascites with a sequence of sensitive parameters followed by specific parameters. For the present sturdy, 80 patients with ascites were divided into two groups: MRA and NMA. The MRA group was consisted of 27 patients with proven malignancy by image study, biopsy, and follow up; 21 of these patients had peritoneal carcinomatosis, but the remaining 6 showed no evidence of peritoneal carcinomatosis. The NMA group was consisted of 53 patients with no evidence of malignancy; among these patients, one had SLE, and others had liver cirrhosis. The samples of blood and ascites were obtained simultaneously, and then the levels of ascites cholesterol, CEA, protein, LDH, cytology, albumin gradient, ascites/serum concentration ratios of LDH(LDH A/S), and ascites/serum concentration ratios of protein(protein A/S) were measured. Applying cut-off limits for determined parameters, we estimated the diagnostic efficacy of each parameter. Among the eight parameters investigated, ascites fluid cholesterol yielded the best sensitive value of 93%(cut-off value 30mg/dl), and cytologic examination and the protein A/S(cut-off value 0.5) showed the most specific value of 100% and 96%, respectively. Based on the above result, the diagnostic sequence with cholesterol as a sensitive parameter, followed by the combination of cytologic examination and protein A/S as specific parameters, was tested in 80 patients. This diagnostic sequence identified 81.5% of patients with malignancy, and all patients with peritoneal carcinomatosis were classified as malignancy-related ascites. In spite of many limitations, this proposed diagnostic sequence may permit a cost-effective and simple differentiation of malignacy-related ascites from nonmalignant ascites
Ascites* ; Ascitic Fluid* ; Biopsy ; Carcinoma ; Cholesterol ; Follow-Up Studies ; Humans ; Liver Cirrhosis

Well-characterized in chronic rhinosinusitis, type 2 inflammation is frequently associated with nasal polyps, comorbid asthma, and nonsteroidal anti-inflammatory drug hypersensitivity. Despite medical and surgical treatment, it recurs in a significant proportion of patients. Thus, severe uncontrolled type 2 chronic rhinosinusitis with nasal polyps is the most difficult-to-treat phenotype of chronic rhinosinusitis. Recently, dupilumab, a monoclonal antibody against IL-4 receptor α, and omalizumab, a monoclonal antibody against immunoglobulin E, were approved for patients with chronic rhinosinusitis with nasal polyps in the United States, Europe, and Korea. Therefore, rhinologists should understand novel biologics and their use. Here, we provide a literature review of several biologics with their indications, effectiveness, and safety.

BACKGROUND: Colony stimulating factors have been shown to accelerate recovery from severe neutropenia after intensive chemotherapy. To prove its clinical effectiveness, we conducted this study of administration of G- CSF in acute myelogenous leukemia after remission induction chemotherapy. METHODS: Thirty six patients with acute myelogenous leukemia were assigned to one of two groups (21 to G-CSF treated group, 15 to control group) after remission induction che motherapy administration. Treatment with G-CSF (lenograstim, 200ng/m2/d) was started 5 days after the end of chemotherapy and continued until the neutrophil count rose above 1,500/mm3. RESULTS: Treatment with G-CSF shortened neutropenic period after chemotherapy. The median time to recovery to neutrophil counts more than 500/mm3 from the end of chemotherapy was 19 days in G-CSF treated group and 25 days in control group. The incidence of infection was 19 cases in G-CSF treated group and 13 cases in control group and febrile periods were 12 days in G-CSF treated group and 15 days in control group, but there were no statistically significant differences. The duration of antibiotics treatment in G-CSF treated group was shorter than that of control group. There was no evidence that G-CSF could increase remission duration and overall survival. CONCLUSION: Recombinant G-CSF is safe and useful in patients after intensive chemotherapy, accelerating neutrophil recovery and thereby reducing the duration of antibiotics administration.
Anti-Bacterial Agents ; Colony-Stimulating Factors ; Drug Therapy* ; Granulocyte Colony-Stimulating Factor ; Humans ; Incidence ; Leukemia, Myeloid, Acute* ; Neutropenia ; Neutrophils ; Remission Induction*

The postpartum thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a rare complication of normal pregnancy and delivery that is described as a constellation of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. We report a case in patient with postpartum thrombotic thrombocytopenic purpura-hemolytic uremic syndrome who was successfully treated with plasama exchange and prednisolone. Relevant literature was reviewed.
Acute Kidney Injury ; Anemia, Hemolytic ; Hemolytic-Uremic Syndrome ; Humans ; Postpartum Period* ; Prednisolone ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia