ABSTRACT
Systemic plasmacytosis is an uncommon disorder characterized by widely disseminated macular skin eruptions composed of polyclonal lymphoplasmacytic infiltrates associated with variable extracutaneous involvement. An aggressive clinical course has been observed in a small number of patients, but most cases have followed chronic and benign clinical course without spontaneous remission. Previously reported cases of this entity have been described almost exclusively in Japanese patients. We recently experienced a case of systemic plasmacytosis in a 48-year-old Korean female patient. Initial skin biopsy specimen revealed patchy perivascular and periadnexal infiltrates of mature plasma cells. Serum immunoelectrophoresis revealed polyclonal hypergammaglobulinemia, and polyclonal plasmacytosis was noted on the subsequent biopsy specimens of left supraclavicular and axillary lymph nodes. Multiple tiny pulmonary nodules appeared six years after the initial cutaneous presentation and were found to be of the same histologic appearance. We herein report a rare case of systemic plasmacytosis with a review of the literature.
Subject(s)
Female , Humans , Middle Aged , Asian People , Biopsy , Castleman Disease , Hypergammaglobulinemia , Immunoelectrophoresis , Interleukin-6 , Lymph Nodes , Plasma Cells , Remission, Spontaneous , SkinABSTRACT
Chondroid syringoma is referred as mixed tumor because it has epithelial structure and mesenchymal elements. Mixed tumors could be divided into two distinct types, eccrine and apocrine. Mixed tumor with follicular, sebaceous and apocrine differentiation is considered as an expression of the common embryologic origin of elements of the folliculosebaceous-apocrine unit. A 32-year old manpresented with an asymptomatic firm erythematous nodule on the left cheek. Histologically, it showed typical features of chondroid syringoma with tubules exhibiting branching and cystic dilatation in the myxoid and chondroid stroma. Our case is unique in that the tumor arose from follicular infundibulum with follicular, sebacesous and apocrine differentiation.
Subject(s)
Adult , Humans , Adenoma, Pleomorphic , Cheek , DilatationABSTRACT
BACKGROUND/AIMS: Gabexate, a protease inhibitor, has been known to prevent pancreatic damage following ERCP. We conducted a prospective and randomized study to assess the preventive effect of gabexate. Methods: Of the 96 patients enrolled, 46 were treated with gabexate and 50 with placebo. The groups were similar with regard to sex, age, body-mass index, and the final diagnosis of ERCP. RESULTS: 24 patients (25.0%) had elevated pancreatic-enzyme levels; the frequency was similar in the two groups (P=0.48). Mean serum amylase value at 4 hours after ERCP was similar in patients with elevated basal level (220.5+/-43.2 U/L) and those with normal basal level (170.4+/-31.2 U/L). After the procedures, serum amylase values were lower in the gabexate group (137.1+/-19.8 U/L) than in the placebo group (212.0+/-50.4 U/L). The differences were not significant in the mean levels of amylase between the groups for any of imaging of the pancreatic ducts (pancreatic-duct imaging, 201.5+/-49.4 U/L, bile-duct imaging, 153.7+/-30.0 U/L). But in the patients with pancreatic duct imaging, serum amylase values were significantly higher in the placebo group (295.0+/-97.6 U/L) than in the gabexate group (112.0+/-10.6 U/L)(p<0.05). CONCLUSIONS: Prophylactic treatment with gabexate does not reduce pancreatic damage related to ERCP, but only in the patients with pancreatic duct imaging there were the significant differences between in the gabexate group and in the placebo group.
Subject(s)
Humans , Amylases , Cholangiopancreatography, Endoscopic Retrograde , Diagnosis , Gabexate , Pancreatic Ducts , Pancreatitis , Prospective Studies , Protease InhibitorsABSTRACT
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticulendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytopenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of them showed markedly decreased phytohemagglutinin induced lymphocyte proliferation and reversed CD4/CD8 ratio (0.52) in flowcytometric lymphocyte subset analysis. Aspirate of bone marrow revealed typical features consistent with FHL in two of them. In spite of recent therapeutic approaches, none of them survived.
Subject(s)
Humans , Male , Bone Marrow , Fever , Hypertriglyceridemia , Lymphocyte Subsets , Lymphocytes , Lymphohistiocytosis, Hemophagocytic , Rare Diseases , Serum Albumin , SiblingsABSTRACT
An 84-year-old woman had an ovoid shallow ulcer with an elevated, indurated, pigmented border on the left cheek. Histological examination revealed a moderately differentiated squamous cell carcinoma and a solar keratosis with abundant melanocytes and melanin pigment. Ultrastructurally, the keratinocytes contained numerous melanosomes in their cytoplasms and the melanocytes in the squamous cell carcinoma and the solar keratosis had mature melanosomes.