Non-cirrhotic portal hypertension related to azathioprine therapy in a patient with Crohn’s disease

Azathioprine is widely used for the treatment of Crohn’s disease (CD). Few cases from Western countries have reported idiopathic non-cirrhotic portal hypertension (NCPH) related to thiopurine therapy in patients with inflammatory bowel disease. Idiopathic NCPH is a rare hepatic condition with intrahepatic portal hypertension but no evidence of cirrhosis or chronic liver disease. Patients with idiopathic NCPH present with symptoms of portal hypertension such as thrombocytopenia, splenomegaly and esophageal varices. We report a case of idiopathic NCPH in a 51-year-old male patient with CD who had been taking azathioprine for 5 years. He was admitted due to esophageal variceal bleeding along with splenomegaly and thrombocytopenia. Evaluation of cirrhosis or chronic liver disease showed normal-range results as estimated by FibroScan evaluation, laboratory examination for autoimmune hepatitis or viral hepatitis, and liver biopsy. This case may suggest the need for careful monitoring for manifestations of portal hypertension in Asian patients with inflammatory bowel disease receiving thiopurine treatment.

Non-cirrhotic portal hypertension related to azathioprine therapy in a patient with Crohn’s disease

Azathioprine is widely used for the treatment of Crohn’s disease (CD). Few cases from Western countries have reported idiopathic non-cirrhotic portal hypertension (NCPH) related to thiopurine therapy in patients with inflammatory bowel disease. Idiopathic NCPH is a rare hepatic condition with intrahepatic portal hypertension but no evidence of cirrhosis or chronic liver disease. Patients with idiopathic NCPH present with symptoms of portal hypertension such as thrombocytopenia, splenomegaly and esophageal varices. We report a case of idiopathic NCPH in a 51-year-old male patient with CD who had been taking azathioprine for 5 years. He was admitted due to esophageal variceal bleeding along with splenomegaly and thrombocytopenia. Evaluation of cirrhosis or chronic liver disease showed normal-range results as estimated by FibroScan evaluation, laboratory examination for autoimmune hepatitis or viral hepatitis, and liver biopsy. This case may suggest the need for careful monitoring for manifestations of portal hypertension in Asian patients with inflammatory bowel disease receiving thiopurine treatment.

Feasibility of Non-TBI Conditioning with Busulfan and Fludarabine for Allogeneic Stem Cell Transplantation in Lymphoid Malignancy

BACKGROUND/AIMS: This retrospective study evaluated the transplantation outcomes of patients with adult lymphoid malignancies who received chemotherapy-based conditioning with busulfan and fludarabine (BuFlu) and busulfan and cyclophosphamide (BuCy2). METHODS: Thirty-eight patients (34 with acute lymphoblastic leukemia and 4 with lymphoblastic lymphoma) were included in the current study. The conditioning regimen was BuCy2 for 14 patients and BuFlu for the remaining 24 patients. Eight and 13 patients were high risk disease in the BuCy2 and BuFlu groups, respectively. RESULTS: The cumulative incidence of grade II-IV acute graft-versus-host disease (GVHD) was 56.5% and 55.2% and that of extensive chronic GVHD 17.0% and 55.6% (p = 0.018) for the BuFlu and BuCy2 groups, respectively. The 3-year relapse rate was 27.8% and 31.4% and 3-year overall survival 34.3% and 46.8% for the BuFlu and BuCy2 groups, respectively. Treatment-related mortality (TRM) was significantly lower in the BuFlu group (16.9%) than in the BuCy2 group (57.1%, p = 0.010). In multivariate analyses, the BuFlu regimen was identified as an independent favorable risk factor for TRM (hazard ratio [HR], 0.036; p = 0.017) and extensive chronic GVHD (HR, 0.168; p = 0.034). CONCLUSIONS: Our BuFlu regimen would appear to be an acceptable conditioning option for lymphoid malignancies, including high-risk diseases. It was safely administered with a lower TRM rate than BuCy2 conditioning.

Prospective Randomization Trial of G-CSF-Primed Induction Regimen versus Standard Regimen in Patients with AML / 전남의대학술지

The sensitization of leukemia cells with hematopoietic growth factors can enhance the cytotoxicity of chemotherapy in acute myeloid leukemia (AML). Therefore, the current trial attempted to evaluate the efficacy of granulocyte colony-stimulating factor (G-CSF) priming in remission induction chemotherapy with an intensified dose of Ara-C for newly diagnosed AML. Patients with newly diagnosed AML were randomly assigned to receive idarubicin (12 mg/m2/24 hr, days 1-3) plus Ara-C (500 mg/m2/12 hr, days 4-8) with G-CSF (250 microg/m2/d, days 3-7) (IAG group) or standard idarubicin (12 mg/m2/24 hr, days 1-3) plus Ara-C (100 mg/m2/12 hr, days 1-7) without G-CSF (IA group). There were no significant differences in sex, age, subtype, or cytogenetic risk between the two groups. Complete remission was achieved in 15 patients (88.2%) from the IAG group and in 14 patients (82.4%) from the IA group (p=0.31). The median time to complete remission was 26 vs. 31 days (p=0.779) for the IA and IAG groups, respectively. The median time to neutrophil recovery (>1x10(9)/L) and platelet recovery (>20x10(9)/L) did not differ significantly between the two groups (26 vs. 26 days, p=0.338; 21 vs. 16 days, p=0.190, respectively). After a median follow-up of 682 days, the 3-year overall survival rate for the IA group was 64.7%, whereas that for the IAG group was 45.6% (p=0.984). No improved clinical outcomes were observed for the AML patients subjected to intensified remission induction with G-CSF priming when compared with standard induction chemotherapy.

Prospective Randomization Trial of G-CSF-Primed Induction Regimen versus Standard Regimen in Patients with AML / 전남의대학술지

The sensitization of leukemia cells with hematopoietic growth factors can enhance the cytotoxicity of chemotherapy in acute myeloid leukemia (AML). Therefore, the current trial attempted to evaluate the efficacy of granulocyte colony-stimulating factor (G-CSF) priming in remission induction chemotherapy with an intensified dose of Ara-C for newly diagnosed AML. Patients with newly diagnosed AML were randomly assigned to receive idarubicin (12 mg/m2/24 hr, days 1-3) plus Ara-C (500 mg/m2/12 hr, days 4-8) with G-CSF (250 microg/m2/d, days 3-7) (IAG group) or standard idarubicin (12 mg/m2/24 hr, days 1-3) plus Ara-C (100 mg/m2/12 hr, days 1-7) without G-CSF (IA group). There were no significant differences in sex, age, subtype, or cytogenetic risk between the two groups. Complete remission was achieved in 15 patients (88.2%) from the IAG group and in 14 patients (82.4%) from the IA group (p=0.31). The median time to complete remission was 26 vs. 31 days (p=0.779) for the IA and IAG groups, respectively. The median time to neutrophil recovery (>1x10(9)/L) and platelet recovery (>20x10(9)/L) did not differ significantly between the two groups (26 vs. 26 days, p=0.338; 21 vs. 16 days, p=0.190, respectively). After a median follow-up of 682 days, the 3-year overall survival rate for the IA group was 64.7%, whereas that for the IAG group was 45.6% (p=0.984). No improved clinical outcomes were observed for the AML patients subjected to intensified remission induction with G-CSF priming when compared with standard induction chemotherapy.

Perianal Granuloma due to Enterobius vermicularis / 대한피부과학회지

Enterobiasis is a common disease among school-aged children, with worldwide distribution and high prevalence in countries with a temperate climate. Enterobius vermicularis usually inhabits the intestinal lumen, and the majority of clinical presentations are perianal pruritus. Extraintestinal infection is uncommon, mostly involving the female genital tract. It has been reported to occur in the peritoneal cavity, ovary, fallopian tube, endometrium, lung, liver, urinary tract and perianal tissue. In Korea, there has been only 1 case of enterobiasis presenting as perianal abscess in the Korean pathologic literature. So we present here an unusual case of perianal granuloma by Enterobius vermicularis in a 62-year-old woman.

Comparative Analysis of Tumescent Liposuction according to the Dermal Curettage Level for the Treatment for Axillary Osmidrosis / 대한피부과학회지

BACKGROUND: The efficacy of tumescent liposuction in the treatment of axillary osmidrosis has been widely established. But there have been few studies that have conducted a comparative analysis according to the level of dermal curettage with respect to the efficacy and safety. OBJECTIVE: The aim of this study was to evaluate the clinical efficacy and complications of tumescent liposuction according to the degree of dermal curettage. METHODS: From September 2004 through August 2009, a total of 33 patients with axillary osmidrosis (66 axillae) were included in this study. Twelve patients (24 axillae) were treated by tumescent liposuction with dermal curettage at the level of the mid-dermis (the classical technique group), and the other 21 patients (42 axillae) were treated by tumescent liposuction with dermal curettage at the level of the dermo-subcutis junction (the modified technique group). The preoperative and postoperative disease severity was measured as 0, 1, 2 and 3. The improvement of symptoms was graded by the patient as excellent, good, fair and poor. We also evaluated the postoperative complications, including skin necrosis, scar, hematoma, sensory disturbance and infection. Biopsies of the axillary skin were performed in 7 patients to confirm the level of dermal curettage and the reduction of apocrine glands after treatment. RESULTS: The follow-up ranged from 6 to 60 months. Six months after treatment, 95.8% (23/24) of the axillae in the classical technique group and 95.2% (40/42) of the axillae in the modified technique group had reduced disease severity below 1. Among the 12 patients treated with the classical technique, 11 (91.7%) had excellent to good results, while 95.2% (20/21) of the patients treated with the modified technique had excellent to good results by the patients' own grading. The complications included skin necrosis, scar and hematoma. In the classical technique group and the modified technique group, the incidence of skin necrosis was 12.5% (3/24) and 2.4% (1/42), respectively, the incidence of scar formation was 4.2% (1/24) and 0%, respectively, and the incidence of hematoma was 8.3% (2/24) and 0%, respectively. The postoperative histologic examinations confirmed that the dermal curettage level was at the mid-dermis in the classical technique and at the dermo-subcutis junction in the modified technique. Interestingly, the histologic examination from the classical technique group revealed vacuolar alteration of keratinocytes and partial separation of the dermo-epidermal junction. CONCLUSION: The efficacy of the modified technique is compatible with that of the classical technique, but the modified technique offers fewer complications. So, the modified technique may improve the cosmetic outcome without reducing the treatment efficacy for the patient with axillary osmidrosis.

A Case of p-ANCA-Positive Propylthiouracil-Induced Pyoderma Gangrenosum

Propylthiouracil is a common medication used in patients with hyperthyroidism; it can cause perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) in some patients with Graves' disease. This antibody has been associated with various forms of vasculitis and neutrophilic dermatosis. Herein, we report a patient who presented with cutaneous manifestations of pyoderma gangrenosum with simultaneous development of p-ANCAs during PTU therapy for Graves' disease.

A Case of Lichen Spinulosus Arising at the Site of Antecedent Seborrheic Dermatitis / 대한피부과학회지

Lichen spinulosus (LS), a disorder of keratinization, is characterized by 1 to 3 mm sized follicular keratotic papules with a central horny spine that are grouped into large patches. Involved lesions were symmetrically distributed over extensor surfaces of the arms and legs, back, chest, face and neck. Although the exact etiology is unknown, several factors such as atopic dermatitis, infections and genetic components have been proposed as playing a role in LS. Additional associations reported in the literature include Crohn's disease, Hodgkin's disease, HIV infection, administration of omeprazole, and seborrheic dermatitis. Herein, we report a case of wide spread lichen spinulosus occurring on a preexisting seborrheic dermatitis lesion in a 50-year-old woman.

Surgical Excision and Postoperative IPL Epilation for Preventing Recurrence of Pilonidal Sinus / 대한피부과학회지

Pilonidal sinus (PS) is one of follicular occlusion tetrad, and this is a chronic inflammatory disease that's characterized by a sinus or cyst with entrapped hair in the midline of the sacral region. The exact pathogenesis of PS remains unknown, but hair insertion may be the essential cause of the disease. PS is usually treated by surgical excision, marsupialization or incision and curettage. However, these surgical options often exhibit high recurrence rate, and especially for hirsute patients. Laser epilation has recently been recommended as an effective postoperative adjunctive therapy for PS and this treatment may reduce recurrence of PS. We report here on a 37-year-old male patient with PS, and the PS was treated by surgical excision and postoperative intense pulsed light (IPL) epilation.

A Case of Cutaneous Endometriosis Combined with Epidermal Cyst in the Cesarean Section Scar / 대한피부과학회지

Endometriosis is the abnormal growth of endometrial glands and stroma outside the uterine cavity. Cutaneous endometriosis is a very rare form of extrapelvic endometriosis, and this most commonly occurs at the site of a previous abdominal or pelvic procedure such as hysterectomy, laparoscopy, episiotomy and cesarean section. The classic symptom is a painful nodule that becomes bigger and tenderer during menstruation. Because cutaneous endometriosis is unfamiliar to dermatologists, it can be easily misdiagnosed as other skin tumors. We present here a case of cutaneous endometriosis combined with epidermal cyst, and this developed in the cesarean section scar of a 28-year-old woman.

A Case of Melanotrichoblastoma / 대한피부과학회지

Melanotrichoblastoma is a variant of pigmented trichoblastoma that shows highly pigmented epithelial lobules and differentiation toward the hair bulb. Histologically, the tumor presents features of trichoblastoma, and the histological findings are remarkable by virtue of the heavy melanin deposits found within and around the tumor cell nests. For the immunohistochemistry, abundant dendritic melanocytes (with the expressions of S100 protein, tyrosinase and most importantly, gp100/HMB45) are found within the tumor masses. We report here on a rare case of melanotrichoblastoma in a 57 year-old female patient.