ABSTRACT
Congenital smooth muscle hamartoma is a benign proliferation of smooth muscles within the dermis. The classic form presents as well-defined, skin-colored, or hyperpigmented plaques associated with hypertrichosis. However, there have been reports of atypical forms, including a follicular spotted appearance, linear atrophic plaques, and morphea-like forms. In such cases, distinguishing congenital smooth muscle hamartomas from other cutaneous diseases can be challenging. Herein, we report on a 16-month-old boy who presented with a hypopigmented patch and hypertrichosis on his back since birth. Histopathological examination revealed mild acanthosis and well-defined smooth muscle bundles haphazardly oriented in the dermis. These bundles stained positively with Masson’s trichrome stain. Based on these findings, a definitive diagnosis of congenital smooth muscle hamartoma was established. In conclusion, an exceptionally rare case of congenital smooth muscle hamartoma with a hypopigmented appearance is reported.
ABSTRACT
Background@#Dupilumab is a human monoclonal antibody against interleukin-4 receptor α. Several clinical trials have demonstrated the rapid and excellent therapeutic effects of dupilumab. Although a growing number of studies have reported data on the real-world efficacy and safety of dupilumab for the treatment of atopic dermatitis, data on real-world experiences in Korea are limited. @*Objective@#To evaluate the real-world efficacy and safety of dupilumab for the treatment of moderate-to-severe atopic dermatitis in Korean patients. @*Methods@#This was a retrospective, single-center study. A total of 179 patients treated with dupilumab for at least 16 weeks were enrolled in this study. Based on electronic medical records, the clinical characteristics, Eczema Area and Severity Index (EASI) score, and adverse events were investigated. @*Results@#The mean baseline EASI score (26.5±7.2) significantly decreased at weeks 16, 40, 52, and 112 (p<0.05). All and 55.2% of patients achieved EASI75 and EASI90 responses at week 52, and all and 75.0% of patients achieved EASI75 and EASI90 responses at week 112, respectively. Common adverse events were facial erythema (31.8%), conjunctivitis (24.0%), and herpes simplex virus infection (11.2%). Three serious adverse events of severe conjunctivitis, mycosis fungoides, and mesenteric venous thrombosis resulted in discontinuation of dupilumab. @*Conclusion@#Dupilumab was effective in real-world clinical practice with a favorable safety profile in Korean patients with moderate-to-severe atopic dermatitis.
ABSTRACT
Dupilumab, a human monoclonal antibody targeting the interleukin-4 receptor α, has exhibited rapid and remarkable therapeutic efficacy in numerous clinical trials focusing on moderate-to-severe atopic dermatitis and prurigo nodularis. Nonetheless, instances of mycosis fungoides have been reported in patients undergoing dupilumab treatment for atopic dermatitis. We present two cases: a 36-year-old woman and a 64-year-old man who presented to our dermatology clinic with erythematous papules and plaques. Following skin biopsy, both patients were diagnosed with chronic eczematous dermatitis with prurigo. Erythematous plaques notably increased after 9 months and 1 month of dupilumab treatment, respectively. Subsequent biopsies confirmed histopathological markers consistent with mycosis fungoides. The first patient underwent chemotherapy for lymph node metastasis, while the second received oral acitretin, narrow-band ultraviolet B, and topical corticosteroids.
ABSTRACT
Psoriasis is a common immune-mediated, inflammatory skin disease. However, unilateral psoriasis is rare and few cases have been reported. A 59-year-old male with a polio-affected right leg presented with exfoliative skin on the left leg for three months. He had been treated under the diagnosis of contact dermatitis with secondary infection. After the exfoliative skin lesions improved, psoriatic papules were noted, which was ultimately diagnosed as psoriasis and successfully treated with topical steroid and vitamin D agents. We propose that the Koebner phenomenon has acted as the triggering and aggravating factor of unilateral psoriasis in this patient.
ABSTRACT
Common atrium is a rare congenital heart disease characterized by complete absence of the interatrial septum, and is commonly accompanied by malformation of the atrioventricular valve. Most patients with common atrium experience symptoms during childhood. Here, we describe a patient with common atrium who experienced his first obvious symptom at 48 years of age.
Subject(s)
Adult , Humans , Heart Atria , Heart Defects, CongenitalABSTRACT
Gemellae is a gram positive cocci that forms part of the oropharyngeal microflora in humans and is anaerobic to aerotolerant. Unlike the other members of the same genus, G. morbillorum rarely causes human infections. Recently, we experienced a case of tubo-ovarian abscess caused by G. morbillorum which was initially suspected to be actinomycosis associated with intrauterine device. This is the first case in the world on tubo-ovarian abscess with G. morbillorum as the culprit.
Subject(s)
Humans , Abscess , Actinomycosis , Gemella , Gram-Positive Cocci , Intrauterine DevicesABSTRACT
Gemellae is a gram positive cocci that forms part of the oropharyngeal microflora in humans and is anaerobic to aerotolerant. Unlike the other members of the same genus, G. morbillorum rarely causes human infections. Recently, we experienced a case of tubo-ovarian abscess caused by G. morbillorum which was initially suspected to be actinomycosis associated with intrauterine device. This is the first case in the world on tubo-ovarian abscess with G. morbillorum as the culprit.
Subject(s)
Humans , Abscess , Actinomycosis , Gemella , Gram-Positive Cocci , Intrauterine DevicesABSTRACT
Retinal pigment epithelium (RPE) constituting the outer blood-retina barrier plays an important role in ocular defense mechanism. Many studies reported that RPE participates in ongoing immune responses in the retina. However, the exact mechanism is still uncertain. Toll-like receptors (TLRs) participate in the recognition of pathogen-associated molecular patterns (PAMP), such as LPS, zymosan, lipoprotein, and dsRNA. The expression and function of TLRs in human RPE have not been established. In this study, we investigated TLRs expression in human fetal RPE and their recognition of PAMP to determine how human RPE participates in ocular defense mechanism against microbial component. RT-PCR and real time PCR revealed that TLR1 through 5 were constitutively expressed in human fetal RPE, and their expressions were slightly increased by LPS. We determined the TNF-alpha, IL-6, and IL-8 expression in human fetal RPE after treatment with LPS, zymosan, petidoglycan, or poly I:C. RT-PCR demonstrated that LPS and poly I:C treatment increased the production of TNF-alpha, IL-6, and IL-8 in human fetal RPE. LPS showed more potent effects on TNF-alpha and IL-8 production. Peptidoglycan and zymosan did not induce the production of TNF-alpha. CD14, the co-receptor of LPS was weakly expressed and functioned in recognizing LPS in human fetal RPE. These results suggest that human RPE may participate in ocular defense mechanism against microbial component through toll-like receptors.
Subject(s)
Humans , Epithelial Cells , Interleukin-6 , Interleukin-8 , Lipoproteins , Peptidoglycan , Real-Time Polymerase Chain Reaction , Retina , Retinal Pigment Epithelium , Retinaldehyde , Toll-Like Receptors , Tumor Necrosis Factor-alpha , ZymosanABSTRACT
In this study, we examined the expression of Toll-like receptor3 (TLR3) by human retinal pigment epithelial cells (RPE) and determined whether exposure to the TLR3 agonist polyinosinic-polycytidylic acid (poly I:C) would induced the expression of cytokines in these cells. RT-PCR revealed that TLR3 was constitutively expressed in human RPE, and its expression was increased by treatment with poly I:C. After treatment with poly I:C, we determined the expression levels of pro-inflammatory cytokines in human RPE using RT-PCR and ELISA. We demonstrated that poly I:C treatment increased the production of TNF-alpha, IL-6, and IL-8 in human RPE. Upon exposure to poly I:C, human RPE initiated antiviral response resulting in the induction of IFN-beta mRNA expression and 2',5'-oligoadenylate synthetase mRNA expression. These results suggest that human RPE may participate in ocular defense mechanism against viral infection through TLR3.
Subject(s)
Humans , 2',5'-Oligoadenylate Synthetase , Cytokines , Enzyme-Linked Immunosorbent Assay , Epithelial Cells , Interferons , Interleukin-6 , Interleukin-8 , Poly I-C , Retinal Pigment Epithelium , Retinaldehyde , RNA, Messenger , Tumor Necrosis Factor-alphaABSTRACT
PURPOSE: We evaluated the safety and efficacy of radial optic neurotomy (RON) in eyes with central retinal vein occlusion (CRVO). METHODS: We performed standard tri-port pars plana vitrectomy and RON in 7 eyes (7 patients) which were diagnosed with CRVO at Konyang University Hospital from December 2001 to November 2002. The eyes had best corrected visual acuities of less than 20/200 and revealed macular edema and hemorrhage on optical coherence tomography and fluorescein angiography. Visual acuity, fundus examination, fundus photography, optical coherence tomography, and fluorescein angiography were performed both preoperatively and postoperatively in all cases. Visual fields were performed preoperatively and postoperatively in 2 eyes. RESULTS: No intraoperative complications occurred in any case except bleeding from incision sites, but we noted defects corresponding to the incision sites of RON on visual field tests in 2 eyes. In 6 of the 7 eyes, the findings improved on fundus photography, optical coherence tomography, and fluorescein angiography. In 2 of the 6 eyes, the visual acuities improved to better than 20/30, but the other 4 eyes showed no improvement. CONCLUSIONS: The surgical decompression of CRVO via RON was a relatively safe procedure, and demonstrated the benefit of rapid reperfusion of retina. However, further studies are needed to establish the safety and long term efficacy of the surgery.
Subject(s)
Decompression, Surgical , Fluorescein Angiography , Follow-Up Studies , Hemorrhage , Intraoperative Complications , Macular Edema , Photography , Reperfusion , Retina , Retinal Vein , Tomography, Optical Coherence , Visual Acuity , Visual Field Tests , Visual Fields , VitrectomyABSTRACT
PURPOSE: To report the arteriovenous (AV) adventitial sheathotomy for the treatment of macular edema associated with branch retinal vein occlusion (BRVO). METHODS: Seven eyes of seven patients with best corrected visual acuity of less than 20/200 secondary to BRVO received pars plana vitrectomy and AV adventitial sheathotomy, and were followed postoperatively for 1, 2, and 4 months. RESULTS: Intraoperative decompression of the AV crossing was achieved in all 7 patients. All patients showed clinical improvement as determined by fundus examination, optical coherent tomography, and fluorescein angiography. Postoperative visual acuity was better in 6 of the 7 patients (86%) with an average of two lines of vision being gained. CONCLUSIONS: AV adventitial sheathotomy may be beneficial for selected patients with poor vision secondary to BRVO.