Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury without any association with preceding diarrhea. Dysregulation of the complement system is the most common cause of aHUS, and monoclonal humanized anti-C5 antibodies are now recommended as the first-line treatment for aHUS. However, if the complement pathway is not the cause of aHUS, C5 inhibitors are ineffective. In this study, we report the second reported case of aHUS caused by DGKE mutations in Republic of Korea. The patient was an 11-month-old infant who presented with prodromal diarrhea similar to typical HUS, self-remitted with conservative management unlike complement-mediated aHUS but recurred with fever. While infantile aHUS often implies genetic dysregulation of the complement system, other rare genetic causes, such as DGKE mutation, need to be considered before deciding long-term treatment with C5 inhibitors.

Syringoma is the most common type of benign intraepidermal eccrine sweat gland tumor in Korea, and is usually found in women in their forties. It presents mostly as a localized lesion, preferring the lower eyelid, cheek, or forehead, and rarely invades the vulval area, and in the case of children, vulvar invasion is even more rare. Tranilast is an antihistamine used for atopic dermatitis and asthma, and has recently been used for the treatment of keloid. A few previous studies have reported both localized and generalized forms of syringoma being effectively resolved with tranilast. Herein, we report a rare and interesting case of milium-like syringoma, which manifested on the vulval area of 10-year old girl that was successfully treated with tranilast.
Asthma ; Cheek ; Child* ; Dermatitis, Atopic ; Eyelids ; Female ; Forehead ; Humans ; Keloid ; Korea ; Sweat Glands ; Syringoma* ; Vulva

BACKGROUND: Acne has been found to be associated with psychosocial problems, and the influence of acne may vary according to the era. OBJECTIVE: We aimed to compare the present quality of life (QOL) of acne patients with that in the past 10 years. Additionally, financial values that patients attach to the treatment of their acne were evaluated. METHODS: We analyzed the influences of acne on QOL using self-reported validated questionnaires (WHOQOLBREF, Skindex-29, APSEA, CADI) between June 2014 and December 2015. RESULTS: 100 acne patients and 70 controls were enrolled in the study. Compared with that in the past 10 years, the quality of life of acne patients decreased in terms of psychological and social relationships, and the difference between males and females was reduced. Clinical characteristics associated with quality of life were identified as age, duration, education, and self-reported severity score, etc. Patient-based financial valuation of treatment costs was associated with the Skindex-29, WHOQOL-BREF, and CADI scores. CONCLUSION: The impact of acne on QOL, especially psychosocial aspects, is important, and early initiation of the appropriate treatment may help to address the diminished QOL and financial burden of acne patients.
Acne Vulgaris* ; Education ; Female ; Health Care Costs ; Humans ; Male ; Quality of Life*

BACKGROUND: Topical and oral steroids are widely prescribed in dermatology. While interest in steroid use has increased rapidly, people have also acquired misinformation that causes rampant fears. OBJECTIVE: To identify fears and misunderstandings about steroid use by comparing doctors' attitudes and treatment and non-treatment group experiences with steroid use. METHODS: A total of 346 participants including outpatients who visited the Department of Dermatology at Hallym University Sacred Heart Hospital, physicians who work at the hospital, and visitors to its Department of Occupational and Environmental Medicine completed questionnaires. RESULTS: The topical treatment group had a high response rate of 46.7% regarding feeling “somewhat anxious” about using topical steroids, while the oral treatment group had a high response rate of 53.8% regarding feeling “somewhat anxious” about using oral steroids. With respect to information about the adverse effects of steroid use, those in the non-experience group obtained their information mostly from the Internet (67.5%) or the media (47.5%), whereas those in the topical treatment group obtained their information from product guides (48%) and doctors (38.7%). Meanwhile, the oral treatment group acquired information from acquaintances (39.7%) or doctors (32.1%). CONCLUSION: Dermatologists must evaluate the validity of the information that patients have about the adverse effects of topical and oral steroid use as well as the resulting phenomenon of steroid phobia. If the information possessed by patients is inaccurate, clinicians must provide a full understanding and proper education to enhance patient satisfaction and treatment compliance and success rates.
Compliance ; Dermatology ; Education ; Environmental Medicine ; Friends ; Heart ; Humans ; Internet ; Outpatients ; Patient Satisfaction ; Phobic Disorders ; Steroids

A 27-year-old male with underlying history of atopic dermatitis presented with skin lesion on the trunk and upper extremity, occurring a month ago. Scattered erythematous multiple tiny maculopapules and patches were noticed. The lesion was first diagnosed as aggravated atopic dermatitis with folliculitis based on the clinical manifestation, but had failed to improve with antibiotics and medications for atopic dermatitis. KOH examinations were performed on his upper arm's lesion, and with detection of hyphae and isolation of Trichophyton rubrum by fungus culture, he was successfully treated with oral itraconazole 200 mg/day and topical flutrimazole. Tinea incognito refers to dermatophyte infections with atypical clinical presentation caused by previous treatment with steroid. Nowadays, the cases of tinea incognito have been increasing due to the increased usage of steroid and other immunosuppressants. Therefore, it is essential to include fungal infection in the differential diagnosis when cutaneous lesions are noticed in patients taking steroid and other immunosuppressants. Herein, we present a tinea incognito that clinically mimicked folliculitis in atopic dermatitis patient.
Adult ; Anti-Bacterial Agents ; Arthrodermataceae ; Dermatitis, Atopic* ; Diagnosis, Differential ; Folliculitis* ; Fungi ; Humans ; Hyphae ; Immunosuppressive Agents ; Itraconazole ; Male ; Skin ; Tinea* ; Trichophyton ; Upper Extremity

No abstract available.
Mucins* ; Nevus*

BACKGROUND: Basal cell carcinoma is the most common form of skin cancer. Surgical excision is commonly used as the therapeutic modality. However, there is insufficient data on the prognosis of Korean patients who have undergone surgical excision for basal cell carcinoma. OBJECTIVE: We therefore retrospectively analyzed the effectiveness of surgical excision in patients with basal cell carcinoma and compared it with previous studies. METHODS: From 2009 to 2015, we enrolled 138 patients with primary basal cell carcinoma who underwent surgical excision and analyzed the recurrence rate, clinical features, surgical procedures, and histopathological subtypes. RESULTS: The case series comprised 138 patients (M:F=1:1.51) with a mean age of 68.7 years. Recurrence after surgical excision was diagnosed in 0 of the 138 patients with a mean follow-up period of 47.5 months (12~93 months). The mean tumor diameter and surgical margin were 0.9 cm and 3.1 mm, respectively. Most tumors (85.5%) were of the nodular subtype. Primary closure was used most often to repair the surgical defects (44.9%). CONCLUSION: Surgical excision may be a satisfactory method for the treatment of primary basal cell carcinomas, especially in non-high-risk cases.
Carcinoma, Basal Cell* ; Follow-Up Studies ; Humans ; Methods ; Prognosis ; Recurrence* ; Retrospective Studies ; Skin Neoplasms