BACKGROUND: Generalized sterile pustular eruption with fever which occurs in generalized pustular psoriasis (GPP) and acute generalized exanthematous pustulosis(AGEP) present a diagnostic and therapeutic problems. In Korea, there are a few studies of clinical and histopathologic reviews of these diseases, but long term follow-up and comparative clinicopathologic studies of these two diseases are not available. OBJECTIVE: This study attempts to identify the differences of these two diseases in the aspects of clinical, laboratory, and histopathologic findings. METHODS: We evaluated the clinical features, laboratory and histopathologic findings in 41 patients with generalized pustular eruption who had visited Pusan National University hospital during the past 20 years and reviewed the literature. RESULTS: 1. The ratio of patients with GPP(n=32) to ones with AGEP(n=9) was 3.6:1. 2. The mean age at diagnosis was 32.9(male) and 28.9(female) years in GPP, and 10.3 years(male) and 62.8 years(female) in AGEP. 3. The number of patients of GPP with previous personal history of psoriasis vulgaris were 15/32(46.9%) and the number of ones with previous family history of psoriasis vulgaris were 2/32(6.3%). 4. There was no patient of AGEP with personal or familial history of psoriasis. 5. The number of patients with recent drug intake history were 4/32(12.9%) in GPP and 9/9(100%) in AGEP. And common drugs suspected to cause AGEP were antibiotics(4 cases) and analgesics(3 cases). 6. Associated systemic symptoms were fever(37.5%), arthralgia(18.8%), and itching(62.5%) in GPP, whereas 66.7%, 33.3%, and 55.6%, respectively in AGEP. 7. The mean duration of pustules was 32.9 days in GPP and 7.2 days in AGEP. 8. Reccurences of generalized pustular eruption were 46.9% in GPP and 0% in AGEP. 9. Laboratory findings revealed leukocytosis(34.4%), elevated erythrocyte sedimentation rate(28.1%), hypoalbuminamia(25.0%), and eosinophilia(6.3%) in GPP, whereas 77.8%, 55.6%, 33.3%, and 71.4%, respectively in AGEP. 10. GPP and AGEP are diseases sharing similar clinical features, but these two diseases show distinctive clinical, laboratory, and histologic features. We suggest that it is important to be aware of these distinctions for avoidance of unnecessary aggressive therapy indicated for GPP.
Acute Generalized Exanthematous Pustulosis* ; Blood Sedimentation ; Busan ; Diagnosis ; Fever ; Follow-Up Studies ; Humans ; Korea ; Psoriasis*

Drug-induced pancreatitis accounts for 0.1~2.0% of all pancreatitis cases. Generally, the mechanism of drug-induced pancreatitis is an immune reaction, accumulation of toxic material, and/or ischemia. However, how dapsone causes pancreatitis remains unclear. A 61-year-old man presented with a 2-week history of epigastric discomfort. He had taken dapsone for 2 months to treat psoriasis. Laboratory findings showed high blood glucose levels and metabolic acidosis; however, hemoglobin A1c was low. Serum amylase and lipase levels were elevated to 125/4,479 U/L. Abdominal computed tomography was indicative of pancreatitis. There was no causative history of pancreatitis and no other medication history except dapsone. Thus, we reached a diagnosis of diabetic ketoacidosis (DKA) followed by dapsone-induced pancreatitis. The patient fasted and was treated with insulin administration and fluid hydration in accordance with treatment guidelines. After treatment, amylase and lipase decreased and symptoms subsided, but insulin injection was required to control blood glucose levels. Drug-induced pancreatitis is a very rare adverse effect of dapsone. Only four cases of pancreatitis related to dapsone could be found in a PubMed search. Moreover, diabetes caused by dapsone-induced pancreatitis has not been reported previously. Here, we report a case of DKA caused by dapsoneinduced acute pancreatitis.
Acidosis ; Amylases ; Blood Glucose ; Dapsone ; Diabetic Ketoacidosis* ; Diagnosis ; Humans ; Insulin ; Ischemia ; Lipase ; Middle Aged ; Pancreatitis* ; Psoriasis

Adolescent ; Adult ; CD36 Antigens ; blood ; Diagnosis, Differential ; Female ; Humans ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Platelet Activation ; Psoriasis ; blood ; diagnosis ; Thrombin ; metabolism ; Thrombospondins ; blood

BACKGROUND: Psoriasis is an immune-mediated, chronic inflammatory disease affecting multiple aspects of patients' lives. Its epidemiology varies regionally; however, nationwide epidemiologic data on psoriasis depicting profile of Korean patients has not been available to date. OBJECTIVE: To understand nationwide epidemiologic characteristics and clinical features of adult patients with psoriasis visited university hospitals in Korea. METHODS: This multicenter, non-interventional, cross-sectional study recruited 1,278 adult patients with psoriasis across 25 centers in Korea in 2013. Various clinical data including PASI, BSA, DLQI, SF-36 and PASE were collected. RESULTS: A total of 1,260 patients completed the study (male:female=1.47:1). The mean age was 47.0 years with a distribution mostly in the 50s (24.9%). Early onset (<40 years) of psoriasis accounted for 53.9% of patients. The mean disease duration was 109.2 months; mean body mass index was 23.9 kg/m²; and 12.7% of patients had a family history of psoriasis. Plaque and guttate types of psoriasis accounted for 85.8% and 8.4%, respectively. Patients with PASI ≥10 accounted for 24.9%; patients with body surface area ≥10 were 45.9%. Patients with DLQI ≥6 accounted for 78.8%. Between PASI <10 and PASI ≥10 groups, significant difference was noted in age at diagnosis, disease duration, blood pressure, waist circumference of female, and treatment experiences with phototherapy, systemic agents, and biologics. CONCLUSION: This was the first nationwide epidemiologic study of patients with psoriasis in Korea and provides an overview of the epidemiologic characteristics and clinical profiles of this patient population.
Adult ; Biological Products ; Blood Pressure ; Body Mass Index ; Body Surface Area ; Cross-Sectional Studies* ; Diagnosis ; Epidemiologic Studies ; Epidemiology ; Female ; Hospitals, University ; Humans ; Korea* ; Phototherapy ; Psoriasis* ; Waist Circumference

OBJECTIVETo explore the correlation between the pathogenesis of psoriasis patients of blood heat syndrome (BHS) and blood stasis syndrome (BSS) and peripheral blood Th1/Th2 cells axis drift, and to observe different expressions of peripheral blood Th1/Th2 cells between healthy subjects and psoriasis patients of BHS and BSS.

METHODSThere were 15 patients in the BHS group and 15 in the BSS group. There were 16 patients in the healthy control group. The expressions of CD4+ gamma-interferon (IFN-gamma) and interleukin-4 (IL-4) in the peripheral blood mononuclear cells (PBMCs) were measured by flow cytometry (FACS). The Th1 main cytokines such as IFN-gamma and Th2 cytokines such as IL-4 in the serum of psoriasis patients of different syndromes were detected by enzyme-linked immunosorbent assay (ELISA). The Psoriasis Area and Severity Index score (PASI) were conducted.

RESULTSFACS results showed that the expression level of CD4+ IFN-gamma+ in the PBMCs was significantly higher in the BHS group than in the BSS group and the healthy control group (P < 0.05). Besides, it was positively correlated with the PASI (P < 0.05). ELISA results showed that the peripheral serum level of IFN-gamma was significantly higher in the BHS group than in the BSS group and the healthy control group (P < 0.05). The plasma level of IFN-gamma was positively correlated with the PASI score in the BHS group (P < 0.05). The plasma level of IFN-gamma was negatively correlated with the PASI score in the BSS group (P < 0.05). The peripheral serum level of IL-4 was significantly lower in the BHS group than in the BSS group and the healthy control group (P < 0.05).

CONCLUSIONSPeripheral Th1 cells had dominant state in psoriasis patients of BHS. When psoriasis patients of BHS were transformed to BSS or to the normal level, the expression of peripheral blood Th1 cells decreased.

Adult ; Aged ; Case-Control Studies ; Female ; Humans ; Interferon-gamma ; blood ; Interleukin-4 ; blood ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Psoriasis ; blood ; diagnosis ; Th1 Cells ; metabolism ; Th1-Th2 Balance ; Th2 Cells ; metabolism ; Young Adult

Ceramides are the main lipid component maintaining the lamellae structure of stratum corneum, as well as lipid second messengers for the regulation of cellular proliferation and/or apoptosis. In our previous study, psoriatic skin lesions showed marked decreased levels of ceramides and signaling molecules, specially protein kinase C-alpha (PKC-alpha) and c-jun N-terminal kinase (JNK) in proportion to the psoriasis area and severity index (PASI) scores, which suggested that the depletion of ceramide is responsible for epidermal hyperproliferation of psoriasis via downregulation of proapoptotic signal cascade such as PKC-alpha and JNK. In this study, we investigated the protein expression of serine palmitoyltransferase (SPT) and ceramidase, two major ceramide metabolizing enzymes, in both psoriatic epidermis and non-lesional epidermis. The expression of SPT, the ceramide generating enzyme in the de novo synthesis in psoriatic epidermis, was significantly less than that of the non-lesional epidermis, which was inversely correlated with PASI score. However, the expression of ceramidase, the degradative enzyme of ceramides, showed no significant difference between the lesional epidermis and the non-lesional epidermis of psoriatic patients. This might suggest that decreased expression of SPT protein is one of the important causative factors for decreased ceramide levels in psoriasis.
Adolescent ; Adult ; Amidohydrolases/*biosynthesis/metabolism ; Apoptosis ; Cell Proliferation ; Ceramidases ; Ceramides/chemistry ; Child ; Epidermis/metabolism ; Female ; Humans ; JNK Mitogen-Activated Protein Kinases/metabolism ; Male ; Models, Biological ; Protein Kinase C-alpha/metabolism ; Psoriasis/*blood/diagnosis ; Serine C-Palmitoyltransferase/*biosynthesis