RESUMO
Oral-facial-digital syndrome type 1 (OFD1), first described by Papillon-Léage in 1954, is transmitted as an X-linked dominant condition and is characterized by a combination of malformations in the face, oral cavity, and digits. Malformations of the brain and polycystic kidney disease are also commonly associated with OFD1. An 11-month-old female presented with multiple tiny whitish papules on her face that had been present since birth. The histopathologic examination was consistent with milium. She also had congenital anomalies, including incomplete cleft palate, bifid tongue, short frenulum, anomalous deformities of both toes, and clino-brachy-syndactyly. Based on the characteristic dysmorphic features of her face, mouth, and hands, a clinical diagnosis of OFD1 was made. Herein, we report a rare case of OFD1 featuring congenital milia, which has not been previously reported in the Korean literature.
RESUMO
Congenital melanocytic nevus is a hamartoma derived from the neural crest that is present at birth. Regression following surgical excision with an apparent halo phenomenon through suture lines has never been reported. A nine-year-old boy presented with a solitary symmetric, oval-shaped, blackish pigmented patch on his right forearm.He reported increasing size of the lesion with no other subjective symptoms. Histological examination of the first excisional biopsy revealed congenital melanocytic nevus, and serial excisions were planned. Interestingly, at the second visit at 18 months after the first biopsy, the size of the congenital melanocytic nevus was reduced with a peripheral whitish halo. Linear regression through suture lines and a peripheral halo was observed after the second and third serial excisions. The mechanism of the halo phenomenon remains elusive but is suggested theorized to be caused by destruction of melanocytes by immune responses of autoantibodies or cytotoxic T cells.
RESUMO
Congenital melanocytic nevus is a hamartoma derived from the neural crest that is present at birth. Regression following surgical excision with an apparent halo phenomenon through suture lines has never been reported. A nine-year-old boy presented with a solitary symmetric, oval-shaped, blackish pigmented patch on his right forearm.He reported increasing size of the lesion with no other subjective symptoms. Histological examination of the first excisional biopsy revealed congenital melanocytic nevus, and serial excisions were planned. Interestingly, at the second visit at 18 months after the first biopsy, the size of the congenital melanocytic nevus was reduced with a peripheral whitish halo. Linear regression through suture lines and a peripheral halo was observed after the second and third serial excisions. The mechanism of the halo phenomenon remains elusive but is suggested theorized to be caused by destruction of melanocytes by immune responses of autoantibodies or cytotoxic T cells.
RESUMO
BACKGROUND@#Cyclosporine (CS) is widely used to treat various skin diseases. Gastrointestinal (GI) discomfort is the most common adverse effect of orally administered CS for dermatologic indications. However, few studies on CS-associated adverse GI events have been conducted.@*OBJECTIVE@#In this study, we aimed to describe the major features of adverse GI events associated with CS therapy using a validated symptom questionnaire, and to investigate the factors contributing to their development. We also evaluated the effectiveness of three empirical GI medications in relieving adverse GI events.@*METHODS@#This study consisted of 2 phases. Phase I was a prospective observational cohort study to investigate the characteristics of CS-associated adverse GI events in 942 consecutive patients treated with CS. Phase II was a randomized controlled trial to evaluate the efficacy of three different classes of GI medications.@*RESULTS@#CS-associated adverse GI events occurred in 119 patients (12.6%). GI complications were more common in female patients (p=0.04), patients with a history of GI disorders (p=0.02), and patients whose initial CS doses were greater than 3 mg/kg/day (p=0.05). In patients treated with any one of the three GI medications, the mean Gastrointestinal Symptom Rating Scale scores significantly decreased (p<0.001).@*CONCLUSION@#This study demonstrated that adverse GI events are common during early CS treatment, especially in women, patients receiving high doses of CS, and those with a history of GI disorders. Our results suggest that new-onset CS-associated GI side effects can be effectively managed with the addition of GI medications.
RESUMO
BACKGROUND: Cordyceps militarys water extract (CME) has been reported to exert antitumor and immunomodulatory activities in vivo and in vitro. However, the therapeutic mechanism has not yet been elucidated. In this study, we examined the effects of CME on the antigen presenting function of antigen presenting cells (APCs). METHODS: Dendritic cells (DCs) were cultured in the presence of CME, and then allowed to phagocytose microspheres containing ovalbumin (OVA). After washing and fixing the efficacy of OVA, peptide presentation by DCs were evaluated using CD8 and CD4 T cells. Also, we confirmed the protein levels of proinflammatory cytokines through western blot analysis. RESULTS: CME enhanced both MHC class I and class II-restricted presentation of OVA in DCs. In addition, the expression of both MHC class I and II molecules was enhanced, but there was no changes in the phagocytic activity of exogenous OVA. Furthermore, CME induced the protein levels of iNOS, COX-2, proinflammatory cytokines, and nuclear p65 in a concentration-dependent manner, as determined by western blot. CONCLUSION: These results provide an understanding of the mechanism of the immuno-enhancing activity of CME on the induction of MHC-restricted antigen presentation in relation to their actions on APCs.