A Case of Incontinentia Pigmenti in a Boy with Klinefelter Syndrome / 대한피부과학회지

Incontinentia pigmenti (IP) is an uncommon X-linked, dominantly inherited disorder due to a mutation in the NEMO (NF-kappaB essential modulator) gene on the X chromosome. IP mostly occurs in female infants, it is usually embryonic lethal in males. The mechanisms for survival of affected males are explained by the presence of an extra X chromosome (Klinefelter's syndrome), hypomorphic mutations, and somatic mosaicism. We report here a rare case of incontinentia pigmenti in a 13-year-old boy with Klinefelter's syndrome, NEMO gene mutation, and whorled, hyperkeratotic, hyperpigmented, linear lesions along the lines of Blaschko on the trunk and leg.

A Case of Posttraumatic Ectopic Nail / 대한피부과학회지

Ectopic nail is characterized by growth of nail-like tissue in a location other than the nail bed. It is an extremely rare disorder that can be either congenital or acquired from incidents such as trauma. An 8-year old female presented with a complaint of a nail-like lesion on the dorsal surface around the center of the right fifth toe. It had been present for 3 years, while the patient continuously clipped it upon growth. The fifth toe was initially injured by a chair that had fallen on that toe 4 years prior to her visit. There was no associated bony deformity or other physical changes in the nail. A clinical diagnosis of ectopic nail was made and it was surgically excised under local anesthesia. The histopathological examination revealed a normal nail unit and there was no recurrence during the 6 month follow-up period.

Disseminated Superficial Porokeratosisin a Patient with Gastric Cancer

Disseminated superficial porokeratosis (DSP) is a rare variant of porokeratosis, which is characterized histologically by cornoid lamella and clinically by central atrophy with elevated borders. DSP is usually associated with immunosuppressive states and hematopoietic malignancies, but rarely with malignancies of visceral organs. A 65-year-old male presented with numerous brownish macules with elevated borders on the trunk and limbs that had been present for 1 year. Before the visit to our clinic, gastric cancer was diagnosed at about the same time the skin lesions suddenly increased in size and number. Clinical and histopathological examination revealed that the lesions were consistent with DSP. We herein report a rare case of disseminated superficial porokeratosis that occurred in association with gastric cancer.

A Case of Onycholysis with Subungual Suppuration Secondary to Paclitaxel / 대한피부과학회지

Paclitaxel is one of the taxoid-antineoplastic agents. These agents are used in the treatment of breast, lung and ovary cancer. Nail changes due to treatment with the taxanes (mainly docetaxel) are reported in 30~40 percent of patients. Paclitaxel is not commonly associated with dermatological reactions, although localized skin reactions and tissue necrosis following extravasation have been reported. Reports of the incidence of nail changes associated with paclitaxel is noted as the pigmentation or discoloration of the nail bed, vary from 2~20%. We report a patient with onycholysis and subungual suppuration during treatment with paclitaxel.

Treatment-Seeking Behaviors and Related Epidemiological Features in Korean Acne Patients

Little is known about the treatment-seeking behaviors of acne patients, especially Asian acne patients. This study was performed to obtain detailed information about the treatment-seeking behaviors in Korean acne patients. Patients who visited the dermatology departments at 17 university hospitals completed a self-administered questionnaire. Most patients obtained information about acne from doctors or the Internet. The most important criteria for selecting a treatment method or choosing a particular clinic were effectiveness and accessibility. Patients used traditional medicine, visited beauty clinics, drank more water, and used over-the-counter topical agents more frequently than they sought doctors during the worsening period. The degree of satisfaction in treatment was found to depend on the total cost of treatment, number of places visited, site affected by acne, and emotional stress. Those who had experienced a side effect tended to have been treated for longer, to have paid more for treatment, and to have an associated skin disease. Treatments prescribed by dermatology clinics had the lowest aggravating rate, although improvement rates for family medicine clinics were also fairly high. This is the first study to investigate in detail the demographic features and characteristics of the treatmentseeking behaviors of acne patients in Asia.

A Case of Acneiform Eruption and Paronychia Occurring after Use of Erlotinib (Tarceva(R)) / 대한피부과학회지

Erlotinib (Tarceva(R)) is a new anti-cancer agent which acts by inhibiting epidermal growth factor receptor (EGFR) signal transduction. It is currently used in the treatment of advanced stage non-small cell lung cancer and pancreatic cancer. We report a case of acneiform eruption and paronychia induced by erlotinib in a 69-year-old man. The patient visited our clinic with multiple erythematous papules and pustules on the face, periungual erythema and pus discharge, xerosis, fissures on the sole. He had taken erlotinib for the treatment of recurred lung cancer for 4 weeks. The skin lesions were partially improved with oral pyridoxine, corticosteroid and topical antibiotics.

Clinical Analysis of Ramsay Hunt Syndrome / 대한피부과학회지

BACKGROUND: Ramsay Hunt syndrome (herpes zoster oticus) is characterized by vesicles on the pinna, otalgia, facial nerve palsy and sensorineural hearing loss. It is not such a common dermatological disease like herpes zoster. However, reactivation of the latent varicella zoster virus causes facial palsy and therapeutic onset is one of the prognostic factors in Ramsay Hunt syndrome. OBJECTIVE: The aim of this study was to elucidate the epidemiology, the clinical characteristics of Ramsay Hunt syndrome and prognosis according to therapeutic ways. METHODS: The 84 cases diagnosed as Ramsay Hunt syndrome from January 2000 to July 2007 were assessed in regard to age, sex, clinical characteristics, electromyography (EMG) results, onset of neurological recovery according to medication and onset of rehabilitation therapy by review of medical records. RESULTS: Ramsay Hunt syndrome consisted 4.7% (84 cases of 1787) of total herpes zoster patients. The ratio of male to female patients was 37:47. Frequency was slightly higher in female patients. The mean age was 62+/-14.7 and the age distribution was in the range of 26~87 years. The most common clinical appearance was vesicular eruptions and facical nerve palsy including change in nasolabial fold and location of the lip (73.8%), followed by vesicular eruptions and vestibulocochlear symptoms like tinnitus and vertigo (16.7%), facial nerve palsy and vestibulocochlear symptoms appearing together (9.5%). EMG showed abnormal results in 67.9%. More than one kind of abnormality was commonly observed. The most common abnormal EMG result was fibrillation potential (42.2%), followed by positive sharp wave (31.3%), polyphasia (10.8%), high frequency discharge (8.4%), increased insertional activity (4.8%) and fasciculation (2.5%). There was no significant difference in onset of neurological recovery between acyclovir injections and oral famciclovir or valaciclovir treatment. The patients who had received physical therapy a week after the symptoms had developed, had a faster onset of recovery (2.8 week Vs 4.1 week, p<0.05). CONCLUSION: This study suggests that antiviral agents of a different kind may not affect recovery onset and earlier rehabilitation therapy seems to be effective in neurological recovery in Ramsay Hunt syndrome.

A Case of Infantile Acne / 대한피부과학회지

Infantile acne is a much less common disease than adolescent and postadolescent acne. It occurs in children approximately at 3 to 6 months of age. An 8 month-old male infant, accompanied by his mother, visited our clinic with a history of erythematous skin lesions on both cheeks, which started 5 months ago. Physical examination revealed closed comedones, several relatively well demarcated, erythematous papules, nodules and pustules. Inflammatory lesions left scars scattered over the face. For further evaluation of endocrinologic abnormalities or other systemic diseases, an additional abdominal ultrasound was done after a visit to the pediatrician. A skin biopsy revealed findings compatible with acne. Skin lesions were improved by treatment with oral and topical antibiotics, adapalene and isotretinoin. We report herein a case of infantile acne which has not been reported in Korean dermatological literature.

A Case of Drug Eruption with Periorbital Edema and Exfoliative Dermatitis Induced by Imatinib Mesylate (STI571, Gleevec(TM)) / 대한피부과학회지

STI571 (imatinib mesylate, Gleevec(TM)), a selective inhibitor of the bcr-abl, c-kit, platelet-derived growth factor receptor tyrosine kinases, is a new anticancer drug used for chronic myelogenous leukemia and gastrointestinal stromal tumors. Cutaneous adverse reactions of periorbital edema and exfoliative dermatitis related to STI571 are rare and there have been no previous reports in the Korean literature. We herein report a case of periorbital edema and exfoliative dermatitis due to STI571 and discuss the possible mechanism of periorbital edema related to STI571.

A Case of Subcutaneous Fat Necrosis of Newborn (SCFNN) Associated with Meconium Aspiration Syndrome / 대한피부과학회지

Subcutaneous fat necrosis of newborn (SCFNN) is a rare disease. Characteristically, full-term infants with associated perinatal problems such as hypoxia, hypothermia, and obstetric trauma are affected in the first week of life. We report a case of SCFNN, which presented as an erythematous subcutaneous mass on the back of a male neonate who suffered from meconium aspiration pneumonia with neonatal sepsis.

Pulsed Intravenous Immunoglobulin Therapy in Livedo Vasculitis: Report of Two Cases / 대한피부과학회지

Livedo vasculitis is a chronic dermatosis characterized by recurrent painful ulceration of the lower limbs, which heals to leave atrophie blanche. The precise pathophysiology is not known. Antiplatelet, anticoagulant, fibrinolytic therapies and anabolic steroids have been reported to be helpful in this syndrome. However, no consistent benefit has been demonstrated with any treatment modality. Recently, pulsed intravenous immunoglobulin therapy has been reported to be effective in some refractory cases. We herein report two cases of recalcitrant livedo vasculitis which were effectively treated with pulsed intravenous immunoglobulin therapy. These were the first trials carried out in Korea.

Treatment of Extramammary Paget's Disease with Topical Imiquimod / 대한피부과학회지

BACKGROUND: Extramammary Paget's disease (EMPD) is an intraepidermal adenocarcinoma which usually affects elderly individuals. Due to its multifocal nature and indistinct demarcation from normal skin, recurrences are common, even after invasive surgical treatment. Topical application of imiquimod, an immune response modifier, has been reported to be effective in the treatment of not only anogenital warts, but also cutaneous malignancies such as actinic keratoses and basal cell carcinoma. There are only a few case reports on the treatment of EMPD with topical imiquimod in the literature. OBJECTIVE: The purpose of this study was to evaluate the effectiveness of topical imiquimod for the treatment of EMPD. METHODS: Seven elderly patients (6 male and 1 female), whose ages ranged from 60 to 81 years (average, 68.4 years), were diagnosed with anogenital EMPD, which were further confirmed by skin biopsy. None of the patients were found to have associated internal neoplasms. Considering the patients' ages and morbidity associated with invasive treatment options, they were instructed to topically apply 5% imiquimod cream on their skin lesions once to three times a week. Imiquimod was applied for an average of 29.5 weeks. The patients were followed up on a monthly or bimonthly basis for any adverse affects or changes to the lesions. Residual lesions were surgically excised in 2 patients after 5 and 8 months of imiquimod application. RESULTS: With long-term imiquimod application, all 7 subjects experienced transient irritation, erythema, focal erosion, or hypopigmentation of the application site. One patient experienced flu-like symptoms. Clinical cure was observed in all 7 patients during treatment, and/or early observation period. However, a pathologic cure was observed in 2 patients only. The lesions recurred in 3 patients during the follow-up period. CONCLUSION: Since imiquimod has strong advantages in the preservation of function and cosmetics, it could be an effective primary treatment option for primary limited cutaneous EMPD, especially in elderly patients.