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Neurofibromatosis type 2 is a rare autosomal dominant disease. Vestibular schwannoma, which causes hearing loss, tinnitus, and imbalance, is the hallmark of the disease and is often the first symptom of neurofibromatosis type 2. Interestingly, children with neurofibromatosis type 2 can present with skin and central nervous system tumors other than vestibular schwannoma. A 10-year-old boy visited our clinic with multiple skin tumors that had been present since he was 1-year-old. Initially, his features did not fulfill the diagnostic criteria of neurofibromatosis type 2, but brain tumors subsequently developed within 2 years. Here, we report a case of neurofibromatosis type 2, that initially presented with multiple cutaneous plexiform schwannomas.
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Background@#Tinea capitis, a superficial dermatomycosis, is caused by a dermatophyte infection on the scalp and hair. Recently, the number of adult patients with tinea capitis have been increasing; therefore, the clinical and mycological features of tinea capitis may be different from those of previous reports. However, only a few studies on adult tinea capitis have been conducted in Korea. Objective: This study aimed to analyze the clinical and mycological features of Korean adult patients with tinea capitis. @*Methods@#A retrospective review of 61 adult patients with tinea capitis was conducted. Clinical features including age, sex, clinical type, and treatment outcome were obtained from medical records and images. The mycological features, including the result of KOH smear and fungal culture, were also analyzed. @*Results@#The mean onset age was 61.3 years, and the male-to-female ratio was 1:4.1. Two patients (3.3%) had a family history of developing the same symptoms, and 17 (27.9%) had a history of close contact with animals. The most predilection site was the vertex, followed by the frontal and parietal areas. Microsporum canis was cultured in 66.7% patients, and Trichophyton rubrum was cultured in 28.6% patients. The number of adult patients with tinea capitis has been increasing constantly. The proportion of patients infected by T. rubrum has been increasing since 2000. Of the 51 patients who received treatments, 42 showed improvements. No significant difference was observed between patients treated with terbinafine and itraconazole. @*Conclusion@#This is the large-scale study that analyzed the clinical and mycological features of Korean adult patients with tinea capitis.
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Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu syndrome, is a rare autosomal dominant genetic disorder that results in vascular malformation in the skin, mucous membrane, and other internal organs, such as the gastrointestinal tract, lung, liver, and brain. A 49-year-old man presented with purpuric punctate, spider-like telangiectasia on the face, hands, and fingers. He had episodes of recurrent nasal bleeding. The family members of the patient had similar events of recurrent nasal bleeding and cutaneous telangiectasia. Abdominal computed tomography revealed multiple arteriovenous malformations and cysts in the liver. Gastroendoscopy and colonoscopy showed angiodysplasia of the fundus of the stomach and mucous membrane of the colon. Histopathologic examination revealed dilated vessels lined with flat endothelial cells in the dermis. We report herein the case of HTT because it is rare and significant for differential diagnosis.
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Transient acantholytic dermatosis, or Grover's disease, usually appears as pruritic erythematous papules and vesicles on the trunk in middle-aged men. The pathogenesis remains unclear, though sun exposure, heat, and sweating may be aggravating factors. A 58-year-old male visited our clinic for evaluation of an asymptomatic erythematous patch on the left temple that developed 40 years ago. Here, we report the rare case of Grover's disease with atypical features presenting as one large patch on the face.
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Humanos , Masculino , Pessoa de Meia-Idade , Acantólise , Temperatura Alta , Dermatopatias , Sistema Solar , Suor , SudoreseRESUMO
BACKGROUND: Henna is a natural dye derived from the leaves of the tree Lawsonia inermis known for its very low allergic properties. Recently, however, cases of pigmented contact dermatitis after henna dyeing have been increasing. OBJECTIVE: To analyze the clinical and histologic features of pigmented contact dermatitis caused by henna dyeing. METHODS: We retrospectively reviewed the clinical and histopathologic features of patients diagnosed with pigmented contact dermatitis after henna dyeing from June 2014 to August 2017. RESULTS: A total of 22 patients with a history of henna dyeing were included. All patients were female, and mean age was 58.9±8.2 years. They presented with rapidly spreading dark brownish patches located mostly adjacent to the hairline, such as at the forehead, temple, and lateral cheek. The mean duration between symptom onset and henna dyeing was 4.52 months (0~15). Standard patch test and as is test using henna showed no significant results. There were 18 patients with pruritus, which was only present at the early stage. Histopathologic examinations showed lichenoid inflammation with dermal melanin incontinence, vacuolar alteration, epidermal apoptotic cells, and perivascular inflammation. CONCLUSION: These results suggest that pigmented contact dermatitis observed after henna dyeing was caused by the toxicity of henna itself, not by other additives. The active ingredient of henna is lawsone (2-hydroxy-1,4-naphthoquinone), which may have induced pigmented contact dermatitis by its own cellular toxicity. Dermatologists should consider henna dyeing as a potential cause of pigmentation of the face and neck.
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Feminino , Humanos , Bochecha , Dermatite de Contato , Testa , Inflamação , Lawsonia (Planta) , Melaninas , Pescoço , Testes do Emplastro , Pigmentação , Prurido , Estudos Retrospectivos , ÁrvoresRESUMO
BACKGROUND: Acute generalized exanthematous pustulosis (AGEP) is typically a medication-induced acute febrile eruption. Few large-scale studies have reported clinical data regarding AGEP in Korea. OBJECTIVE: This study analyzed the clinical and histopathological features of AGEP in Koreans to identify recent trends in this context. METHODS: This study retrospectively reviewed 31 patients with AGEP. Age, sex, clinical features, etiologies, laboratory findings, histopathological features, and treatment outcomes were obtained from patients' medical records and photographs. RESULTS: The mean age of onset was 43.6 years, and the male:female ratio was 2.1:1. All patients showed non-follicular tiny pustules and erythema. Fever was reported in 13 patients and neutrophilia in 17 patients. Medications were considered the most common etiological contributors. Twenty patients showed drug-induced AGEP, and 11 of 20 patients showed antibiotic-induced AGEP. Serum C-reactive protein and lactate dehydrogenase levels increased in 93.8% and 77.8% of the patients, respectively. Histopathologically, all patients showed subcorneal or intraepidermal pustules, followed by exocytosis and neutrophilic dermal infiltrate. Twenty-five patients improved within a mean period of 7.5 days after the onset of the skin rash. The percentage of women in the drug-induced AGEP group was significantly higher than that in the non-drug-induced AGEP group. CONCLUSION: This report describes a large-scale study that analyzed the clinical and histopathological features of AGEP in Koreans and seems to accurately reflect the recent trends in this context. Clinically, it is important to note that the percentage of women in the drug-induced AGEP group was higher than that in groups showing other etiologies of AGEP.
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Feminino , Humanos , Pustulose Exantematosa Aguda Generalizada , Idade de Início , Proteína C-Reativa , Eritema , Exantema , Exocitose , Febre , Coreia (Geográfico) , L-Lactato Desidrogenase , Prontuários Médicos , Neutrófilos , Estudos RetrospectivosRESUMO
BACKGROUND: Acral melanomas are known to have a low frequency of BRAF mutation, in contrary to higher KIT mutation. Recently, VE1 immunostaining was reported to have a good correlation with BRAF mutation status. OBJECTIVE: We aimed to evaluate the clinicopathological features of BRAF-mutated acral melanomas and validate the correlation of the VE1 immunohistochemical stains in those cases. METHODS: The clinical features (age, sex, anatomical site), and histopathological characteristics of 41 patients with acral melanoma were evaluated. We performed a next-generation sequencing to detect BRAF mutation status. We also determined the correlation of VE1 immunohistochemical staining with BRAF mutation status. RESULTS: Among 19 acral melanomas with BRAF mutation, common histopathological subtype was acral lentiginous melanoma (8/19, 42%) and nodular melanoma (8/19, 42%) and superficial spreading melanoma (3/19, 16%) followed. VE1 immunostaining results were positive in all 15 cases with BRAF V600E mutation (sensitivity 100%), and negative in 4 cases of BRAF non-V600E mutation. However, VE1 immunostaining was negative in all 22 patients with BRAF wild-type. CONCLUSION: VE1 immunostaining had a good correlation with BRAF V600E mutation status.
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Humanos , Corantes , MelanomaRESUMO
BACKGROUND: Ashy dermatosis is a disorder of pigmentation, characterized by asymptomatic symmetric blue-gray-colored macules of unknown pathophysiology. OBJECTIVE: Our aim was to evaluate ashy dermatosis in Koreans and to describe its clinical and histopathological features. METHODS: Medical records, photographs, and biopsy specimens obtained from 74 patients were assessed. RESULTS: Of the 74 patients, 38 were men and 36 were women. Clinically, most patients showed lesions on the trunk (83.8%), followed by the extremities (44.6%). At the time of the mean age was 29.9 years, and disease duration was 18.4 months before the institution. We tentatively classified the lesions into the following 3 groups: generalized confluent, generalized macular, and localized type. Clinically, the most common lesion was the generalized macular type (67.6%). Peripheral erythematous borders or concomitant erythematous lesions were observed in 18 patients (24.3%). Histopathologically, the lesions showed perivascular inflammation, dermal melanophages, and pigmentary incontinence. CONCLUSION: Our study classified ashy dermatosis into 3 groups, and this categorization may contribute to a better understanding of ashy dermatosis.
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Feminino , Humanos , Masculino , Biópsia , Extremidades , Inflamação , Prontuários Médicos , Pigmentação , DermatopatiasRESUMO
BACKGROUND@#Malassezia, a lipophilic yeast, is a causative agent for dandruff and seborrheic dermatitis. Many biological agents have been studied for anti-Malassezia effect but further studies are needed for their clinical application.@*OBJECTIVE@#The study was conducted to evaluate the inhibitory effect of different natural essential oils and a fruit extract on Malassezia species in an in vitro study and a clinical trial.@*METHODS@#The antifungal effects of natural essential oils and a fruit extract on Malassezia species (M. furfur and M. sympodialis) were evaluated by measuring the minimum inhibitory concentration (MIC) and minimum fungicidal concentration (MFC) and using the disc diffusion method. Natural essential oils of citron seed, lavender, and rosemary and citrus junos fruit extract were used for the in vitro study. The clinical trial was conducted with a shampoo containing four ingredients. A total of 22 subjects used the shampoo every day for 4 weeks and were evaluated using clinical photography, trichoscopy, and sebumeter at baseline, 2 weeks, and 4 weeks after treatment.@*RESULTS@#Antifungal activity of agents was relatively lower in lavender and rosemary essential oils at MIC and MFC. Disc diffusion method revealed same results. In the clinical trial, the amount of sebum decreased statistically significantly and erythema, dandruff, and lesion extent also improved.@*CONCLUSION@#The natural essential oils and fruit extract are effective for suppressing Malassezia activity, therefore these might be used as an alternative for treatment of dandruff and seborrheic dermatitis.
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Nevus sebaceus is a congenital hamartoma with epidermal, follicular, and apocrine elements. Several skin appendage tumors can evolve secondarily from nevus sebaceus. Two different tumors often develop simultaneously on a nevus sebaceus lesion, but the growth of more than two tumors is rare. Herein, we report a case of four appendage tumors including trichoblastoma, tumor of the follicular infundibulum, sebaceoma, and sebaceous hyperplasia within a single nevus sebaceus lesion on the face of a 76-year-old female.
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Idoso , Feminino , Humanos , Hamartoma , Hiperplasia , Nevo , Hipófise , PeleRESUMO
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.
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Adulto , Feminino , Humanos , Produtos Biológicos , Pressão Sanguínea , Índice de Massa Corporal , Superfície Corporal , Estudos Transversais , Diagnóstico , Estudos Epidemiológicos , Epidemiologia , Hospitais Universitários , Coreia (Geográfico) , Fototerapia , Psoríase , Circunferência da CinturaRESUMO
Autoimmune progesterone dermatitis is a rare cyclic premenstrual reaction to progesterone produced during the luteal phase of the menstrual cycle. The clinical symptoms of autoimmune progesterone dermatitis overlap with other forms of dermatosis such as erythema multiforme, eczema, fixed drug eruption, urticaria, and angioedema. We experienced 3 cases of autoimmune progesterone dermatitis. All patients had a recurrent history of monthly skin eruptions. Skin lesions normally began a few days before menstruation and resolved a few days later. Patients were confirmed to have autoimmune progesterone dermatitis by the results of the progesterone intradermal test. All three patients had different clinical findings such as erythema annulare centrifugum, urticaria, contact dermatitis, and rosacea. Because patients presented with variable clinical manifestations, they could have been easily misdiagnosed. The patients were treated with oral contraceptive, antihistamine and steroids for symptom control. We propose that dermatologists should consider autoimmune progesterone dermatitis in cases of recurrent cyclic skin eruptions in female patients. Further, if this condition is suspected, thorough history taking including that on menstrual cycle and intradermal progesterone test should be performed.