Clinical Features and Laboratory Findings in Children Diagnosed with Benign Acute Childhood Myositis (BACM) / 대한소아신경학회지

PURPOSE: This study was performed to review the relationship between the clinical features and laboratory findings of patients suspected of benign acute childhood myositis (BACM) in children and adolescents with bilateral calf pain and gait disturbances. METHODS: From January 1, 2010 to December 31, 2016, the clinical and laboratory findings of patients who visited Dongguk University Ilsan Hospital with the sudden onset fever and muscle pain were retrospectively examined. RESULTS: The total number of patients was 29 (21 males and 8 females), and their mean age was 5.5 years. The mean duration of fever was 4.2 days. The mean duration from the onset of fever to show the symptom was 3.2 days. The mean recovery period from myositis was 2.4 days. The mean duration of hospitalization was 2.2 days. Fever and bilateral calf pain were the most common symptoms; however, cough, rhinorrhea, sore throat, headache, and abdominal pain were also observed. The causative agent of BACM was influenza B infection in 23 (79.3%) of the cases. Creatinine phosphokinase (CPK), myoglobin, aspartate aminotransferase (AST), and alanine transaminase (ALT) values were higher in the shorter duration of fever and were statistically significant. The following values were noted: CPK (r=−0.472), myoglobin (r=−0.472), AST (r=−0.443), and ALT (r=−0.459). The longer the pain period, the lower the white blood cell (WBC) count (r=−0.655). CONCLUSION: BACM is mostly associated with the influenza B virus. Time to onset of symptoms after fever and WBC levels are related to muscle enzyme levels and duration of symptoms in BACM.

Differences in Clinical Manifestations and Treatment Responses in Influenza Type A and B in a Single Hospital during 2013 to 2015

PURPOSE: We suspect there is a difference in the clinical manifestations and treatment response to antiviral drugs for influenza A and B. This study was conducted to investigate this difference. METHODS: We collected information on pediatric patients, infected with the influenza virus, admitted to Dongguk University Ilsan Hospital from October 2013 to May 2015. We investigated the clinical manifestations of influenza and differences in treatment response to oseltamivir treatment for the two types of influenza. RESULTS: A total of 138 patients were included. The mean age was 3.5±4.0 years. When comparing the diseases associated with influenza A and B, croup (19.2% vs. 1.7%, P=0.001) was more common with influenza A infection. Myositis (0% vs. 6.7%, P=0.021) and gastroenteritis (29.5% vs. 46.7%, P=0.038) were more common with influenza B infection. When comparing the total fever duration from the start of oseltamivir administration, patients treated with oseltamivir within 2 days of fever had the shortest duration. Among the patients treated with oseltamivir, the duration of fever, after the start of oseltamivir treatment, for was shorter for influenza A infection than for influenza B infection (16.0±19.1 hours vs. 28.9±27.9 hours, P=0.006). CONCLUSIONS: There appear to be differences in the accompanying diseases and antiviral medication responses between the two types of influenza. It is important to administer oseltamivir within 2 days of fever.

Morbidity of Solid Cancer in Behcet's Disease: Analysis of 11 Cases in a Series of 506 Patients

PURPOSE: Behcet's disease (BD) is rarely reported to be associated with malignancies in the literature. However, the frequency of cancer in BD patients remains unknown. This study evaluated cancer morbidity in BD patients compared with that in the general population of Korea. MATERIALS AND METHODS: A retrospective chart review was performed on 506 patients visiting our hospital from 1994 to 2011 for BD. We analyzed the standardized morbidity rate (SMR), which is the ratio of observed to expected malignancies. Furthermore, we reviewed cases of solid cancer in BD patients in the literature. RESULTS: Of the 506 patients with BD, 11 (2.17%) developed cancer. We found a variety of solid cancers without predominance and no hematologic malignancies. The total number of cancers observed was less than expected, which was determined from the statistical data of the National Cancer Information Center of Korea, with an SMR of 0.023 (95% confidence interval, 0.012-0.039). CONCLUSION: BD may be associated with a lower cancer-related morbidity compared with the general population of Korea.

Prevalence, Clinical Characteristics, and Management of Functional Constipation at Pediatric Gastroenterology Clinics

The purpose of this study was to investigate the prevalence, clinical characteristics, and management of functional constipation at pediatric gastroenterology clinics. A prospective survey using the Rome III criteria was distributed to a group of parents of children with a constipation history and its control group in May 2008. The mean prevalence of constipation was 6.4%, which was similar to those in other countries. Statistically significant variables for children without constipation were that more children had a body mass index of below the 10th percentile even though they received more mother's care and ate balanced meals compared to the constipation group. Meanwhile, the constipation group frequently showed a history of constipation in infancy, picky-eating, lack of exercise, and retentive posturing. When analyzed with the Rome III criteria, the children showed greater than 60% rate of hard stools, painful stools, a history of large fecal mass in rectum, and its disappearance of constipation symptoms after passing a large stool. Our study found different approaches amongst pediatric gastroenterologists like rectal examinations, disimpaction, or drug treatment. Several factors addressed in our study can provide better guidelines for clinicians treating constipation and its future research.

Analysis of Colonization and Genotyping of the Exotoxins of Staphylococcus aureus in Patients with Atopic Dermatitis

BACKGROUND: The skin of atopic dermatitis (AD) patients has a high susceptibility to Staphylococcus aureus colonization, and the toxins produced by S. aureus may aggravate AD by acting as superantigens. OBJECTIVE: The purpose of this study was to evaluate the relationship of the skin barrier function, colonization of S. aureus, and the clinical severity of AD. We also examined the predominant toxin genes produced in Korean AD patients. METHODS: Thirty-nine patients with AD were evaluated for clinical severity and skin barrier function by using Severity Scoring of Atopic Dermatitis (SCORAD) index and transepidermal water loss (TEWL). S. aureus was isolated from the forearm, popliteal fossa, and anterior nares of AD patients (n=39) and age-matched controls (n=40); the toxin genes were analyzed by performing multiplex polymerase chain reaction. RESULTS: TEWL showed a statistically significant correlation with clinical severity in patients with AD (p<0.05). TEWL was correlated with the number of S. aureus colonization sites and the presence of nasal colonization, but these results were not statistically significant. S. aureus strains were isolated in 64.1% of the 39 AD patients. The SCORAD index and AD severity were strongly correlated with the number of colonization sites. The predominant toxin gene found in AD patients was staphylococcal enterotoxin a (sea) only, which was produced in 52.6% of patients. The toxin genes sea and toxic shock syndrome toxin-1 (tsst-1) were found together in 42.1%, while tsst-1 only was found in 5.3% of the patients. CONCLUSION: S. aureus strains were isolated in 64.1% of the 39 AD patients. Skin barrier function, as measured by TEWL, revealed a statistically significant correlation with clinical severity in AD patients. The SCORAD index and severity of AD was strongly correlated with the number of colonization. The most common toxin gene was sea in the Korean AD patients and this gene might have an important role in the pathogenesis of AD.

Biological Therapy for Inflammatory Bowel Disease in Children / 대한소아소화기영양학회지

The pathogenesis of inflammatory bowel diseases is not very well understood; it is currently thought to be caused by the interaction between genetic factors, environmental factors, intestinal microbes, and immune factors. Biological agents such as anti-tumor necrosis factor (anti-TNF) are widely being used as therapeutic agents. Infliximab, a chimeric monoclonal IgG1 antibody against tumor necrosis factor, has been demonstrated to have an effect in the induction and maintenance of remission in Crohn's disease in children. The effects of biological agents, typified by anti-TNFs, in inflammatory bowel disease in children; the recent concern on the administration of biological agents in combination with immunomodulators; and 'Top-down' therapy are some of the topics covered in this review.

Cytomegalovirus Infection in Infantile Hepatitis / 대한소아소화기영양학회지

PURPOSE: The aims of this study was to compare and evaluate the clinical characteristics, laboratory data, and prognosis for infants under age 1 year with CMV hepatitis and those with viral hepatitis of unknown etiology. METHODS: A retrospective study was conducted of infants under age 1 year who were admitted with acute hepatitis. The exclusion criteria consisted of: autoimmune, genetic, metabolic, toxic, HAV, HBV, HCV, toxoplasma, rubella, herpes simplex, and Epstein-Barr virus. The 30 patients included were divided into two groups based on markers for CMV (IgM anti-CMV, CMV PCR in urine, CMV culture in urine). RESULTS: The median age of patients (n=15) was 2.8 months. No other organ involvement was detected in any patient. Peak serum total bilirubin levels (n=4) ranged from 2.6 to 6.7 mg/dL. Peak serum ALT levels ranged from 51 to 1,581 IU/L. The duration of ALT elevation ranged from 1.5 weeks to 26 weeks (median 9 weeks). All had recovered in full without ganciclovir; there were no cases of hearing loss. The median age of controls (n=15) was 2.5 months. Peak serum total bilirubin levels (n=4) ranged from 1.6 to 9.1 mg/dL. Peak serum ALT levels ranged from 26 to 1,794 IU/L. No significant differences were observed between both groups regarding the peak serum ALT levels, peak serum total bilirubin levels, duration of hyperbilirubinemia and ALT elevation. CONCLUSION: Although it was not possible to differentiate congenital infection with perinatal infection in this study, the prognosis of patients with CMV hepatitis without other organ involvement was good without ganciclovir treatment.

Endoscopic Hemostasis for Bleeding Gastric Ulcer Caused by Ibuprofen in a 16-month-old Infant / 대한소아소화기영양학회지

Gastric ulcers are rare in children and are typically seen in cases of Helicobacter pylori (H. pylori) infection, non-steroidal anti-inflammatory drugs (NSAIDs) use, and critical illnesses such as sepsis. The risk of a bleeding ulcer due to use of NSAIDs is dependent on the dose, duration, and the individual NSAIDs, but the bleeding may occur soon after the initiation of NSAID therapy. An experience is described of a 16-month-old infant with a bleeding gastric ulcer after taking the usual dosage of ibuprofen for 3 days. The infant was also successfully treated with endoscopic hemostasis. Even a small amount of ibuprofen may be associated with bleeding gastric ulcers in infant.

Eccrine Angiomatous Hamartoma Mimicking a Traumatic Hemorrhage

Eccrine angiomatous hamartoma (EAH) is a rare benign disease that is characterized by an abnormal proliferation of eccrine glands and vascular elements. It is generally congenital, but it can appear before puberty. It usually presents as a single plaque or nodule, but multiple patch-like lesions are also possible. EAH is mostly asymptomatic, but it is sometimes associated with pain or hyperhidrosis. It generally does not require aggressive treatment, but the lesion can be excised due to pain, enlargement and cosmetic reasons. A 3-week-old Korean female presented with a hemorrhagic skin lesion on the right foot since birth. There was no specific birth history. The lesion first appeared on the third toe of the right foot and quickly spread to almost half of the right foot. Histopathology examination revealed acanthosis in the epidermis and a proliferation of eccrine ducts, glands and capillaries. The eccrine glands were immunohistochemically-positive for carcinoembryonic antigen.

Sweet Syndrome as an Initial Presentation in a Patient with Systemic Lupus Erythematosus / 대한피부과학회지

Sweet syndrome is also called acute febrile neutrophilic dermatosis and this malady is characterized by an abrupt onset of fever, leukocytosis and tender erythematous plaques that are infiltrated by neutrophils. It most commonly occurs in women who are between 30 to 50 years of age. About 50% of the cases are associated with autoimmune disease, sarcoidosis, Behcet's disease and pregnancy. We present here a 48-year-old woman who had tender erythematous tender plaque and nodules with vesicles and pustules over both extremities. She complained of general weakness, chills and arthralgia. We biopsied the nodule and performed laboratory tests. The results of the biopsy were compatible with Sweet syndrome and the laboratory test showed systemic lupus erythematosus. We report here on a case of Sweet syndrome as an initial presentation of systemic lupus erythematosus.

A Malignant Melanoma Associated with a Blue Nevus of the Lip

Blue nevi are characterized by a collection of pigment-producing melanocytes in the dermis. These lesions clinically present as well demarcated cerulean-blue or bluish black colored papules or plaques that usually measure less than 1 cm in diameter. They are typically found on the dorsal surface of the hands and feet or in the head and neck region; however, they are rarely found in the oral cavity. These lesions are usually benign and stable over time. However, malignant melanomas developing in or associated with a blue nevus (which is also called malignant blue nevus) have been only rarely reported. A malignant blue nevus might develop in a common blue or cellular blue nevus, a giant congenital nevus or in a nevus of Ota, or it may be malignant from the start. Malignant blue nevi most commonly are found on the scalp. A malignant blue nevus of the lip has not been previously reported in the medical literature. We report here on a patient with a malignant melanoma associated with a blue nevus of the lip. The malignant melanoma was presumed to have developed from a blue nevus that was present on the upper lip of a 50-year-old male.

Chromoblastomycosis Caused by Phialophora richardsiae

Chromoblastomycosis is a chronic fungal disease of the skin and subcutaneous tissues caused by a group of dematiaceous (black) fungi. The most common etiologic agents are Fonsecaea pedrosoi and Cladophialophora carrionii, both of which can be isolated from plant debris. The infection usually follows traumatic inoculation by a penetrating thorn or splinter wound. Several months after the injury, painless papules or nodules appear on the affected area; these papules then progress to scaly and verrucose plaques. We report a case of chromoblastomycosis caused by Phialophora richardsiae, which has been rarely associated with chromoblastomycosis. The case involved a 43-year-old male, who for the past 2 months had noted an erythematous, pustulous plaque that was somewhat dark brown in color on his right shin; the plaque also had intermittent purulent discharge and crust formation. On histopathological examination, chronic granulomatous inflammation and sclerotic cells were seen. The tissue fungus culture grew out the typical black fungi of P. richardsiae, which was confirmed by polymerase chain reaction. The patient has been treated with a combination of terbinafine and itraconazole for 3 months with a good clinical response.

The Clinical Presentation and Etiologic Factors of Demodicosis / 대한피부과학회지

BACKGROUND: Demodicosis is a common cutaneous disease that is caused by demodex mites, which reside in the pilosebaceous follicles. However, any data of the clinical features and the factors that induce or aggravate demodicosis is lacking in the Korea literature. OBJECTIVE: We aimed to demonstrate the clinical feature and determine the medical or personal history that influences demodicosis. METHODS: We performed a retrospective study to review clinical features, the histopathologic findings and the past medical and personal history of Korean patients who suffer with demodicosis. RESULTS: Thirty-four patients were diagnosed with demodicosis based on the clinical features, the potassium hydroxide examination and/or the skin biopsy specimen. There were 11 males and 23 female patients (mean age: 44.4 years). The mean duration of disease was 15.5 months. The clinical features were classified to acne rosacea-like (n=26), perioral dermatitis-like (n=3), granulomatous rosacea-like (n=2), pityriasis folliculorum (n=2) and demodex abscess (n=1). Skin biopsy was performed on 10 patients and the histopathologic findings showed demodex mites in the follicular infundibulum or sebaceous ducts with a perifollicular and perivascular inflammatory infiltrate in all case. According to the medical history, 17 patients had history of chronic use of topical steroid and calcineurin inhibitor and 5 patients didn't use the soap during washing their face. CONCLUSION: Our findings indicate that demodicosis demonstrates various clinical presentations. If patients with facial eruption chronically use topical steroid or calcineurin inhibitor or they do not use soap, demodicosis should be considered in the differential diagnosis and a potassium hydroxide examination or skin biopsy may be helpful to diagnose and treat the facial eruption.

A Case of Elephantiasis Nostras Verrucosa Caused by Recurrent Infection after Skin Grafting / 대한피부과학회지

Elephantiasis nostras verrucosa is an uncommon disorder that is characterized by dermal fibrosis, hyperkeratotic verrucous and papillomatous lesions and this is all caused by chronic non-filarial lymphedema secondary to infections, surgeries, tumor obstructions, radiations, congestive heart failure and obesity. We report here on a case of elephantiasis nostras verrucosa that occurred on the left lower leg of a 54-year-old woman who had a past history of extensive skin grafts and recurrent infection.

Ecthyma Gangrenosum: A Rare Cutaneous Manifestation Caused by Stenotrophomonas maltophilia in a Leukemic Patient

Ecthyma gangrenosum (EG) is a well-recognized cutaneous infection that most commonly affects immunocompromised patients. It typically occurs on the extremities, or in gluteal and perineal regions. Although Pseudomonas aeruginosa is the most well-known pathogen causing EG, other organisms have been reported to cause EG. Herein we report a rare case of ecthyma gangrenosum presenting as aggressive necrotic skin lesions in perioral and infraorbital areas in a 47-year-old patient with acute myelocytic leukemia after allogeneic bone marrow transplantation. It was caused by Stenotrophomonas maltophilia, which is an aerobic, gram-negative pathogen that has been associated only rarely with cutaneous disease. Blood culture and tissue culture were positive for S. maltophilia. Histological examination revealed numerous tiny bacilli in the dermis and perivascular area. Early recognition of skin lesions caused by S. maltophilia is important to decrease associated mortality in immunosuppressed patients.

Three Cases of Pilomatricoma That Clinically Presented as Hemangioma on the Eyelid / 대한피부과학회지

Pilomatricoma is a common benign neoplasm that originates from the matrix of the hair root. The tumor usually presents as a deep-seated, solitary, firm nodule with overlying epidermis. It occurs more frequently in the head and neck region of children and adolescents, and it often involves the eyelid or eyebrow. Pilomatricoma is often misdiagnosed clinically and the correct diagnosis can be established only after excision and histological examination. The pathologic diagnosis of pilomatricoma is based on finding large masses of shadow cells, combined with basophilic cells, inflammation, foreign body giant cells, calcification and ossification. We report here on 3 cases of pilomatricoma that clinically presented as hemangioma on the eyelid. Punch and excisional biopsy were done after ultrasonography, and we diagnosed the lesions as eyelid pilomatricoma.

A Case of Lichen Nitidus That Was Improved with Topical Tacrolimus / 대한피부과학회지

Lichen nitidus is a rare chronic inflammatory disease that was first described by Pinkus in 1907. It is characterized by asymptomatic multiple minute, flesh-colored, shiny dome shaped papules that occur on the abdomen, chest, extremities and genitalia of children or young adults. This disorder is most often localized and only a few cases of generalized lichen nitidus have been reported. Because lichen nitidus tends to be asymptomatic and it spontaneously resolves within several years, it usually does not require treatment except in the symptomatic, persistent and generalized cases. We report here on a case of a 27-year-old woman who was diagnosed with lichen nitidus, and her condition was refractory to general treatment. She was successfully treated with the application of 0.03% tacrolimus ointment.

A Case of Varicella Combined with Hand-foot-mouth Disease in a Healthy Child

We report here on an 11-year-old child with a pustulovesicular eruption on her whole body, including her palms, soles and oral mucosa, and this was accompanied with fever. A serologic test was positive for IgM varicella zoster antibody and coxsackievirus A16 antibody. The histopathologic examination from the palm revealed intraepidermal pustules that showed neither inclusion bodies nor multinucleated giant cells. We made the final diagnosis, according to the serologic tests and histopathologic findings, as varicella combined with hand-foot-mouth disease caused by coxsackievirus A16 in a previously healthy child

Metastatic Malignant Fibrous Histiocytoma Mimicking Keratoacanthoma / 대한피부과학회지

Malignant fibrous histiocytoma (MFH) has been considered to be the most common malignant soft tissue tumor of middle and late adulthood. This tumor is usually a skin-colored subcutaneous nodule. It metastasizes to the lung, lymph node, bone and liver. Metastasis to the skin is very rare. We present a case of a 69-year-old man who presented with an asymptomatic dome-shaped large nodule with central umbilical ulceration on his scalp that had grown abruptly over 3 months. The lesion clinically mimicked keratoacanthoma. Biopsy of the nodule was done to differentiate keratoacanthoma from MFH confirmed the latter. The result of biopsy was MFH. Diagnosis was MFH of unknown primary tumor origin. We report a case of metastatic MFH mimicking keratoacanthoma.

A Case of Cellulitis Associated with Coral Injury

Corals are the marine organism that belongs to the phylum Cnidaria, and are one of the common causes of superficial injury in the marine environment. In addition to acute reactions such as burning or stinging pain and erythema, coral injuries may present with complications such as foreign body reactions, bacterial infections, and/or localized eczematous reactions. A 23-year-old male presented with an erythematous edematous tender patch with centrally grouped vesicles on the left ankle; the injury had occurred during skin-scuba diving 2 days before. A biopsy of the lesion treated with hematoxylin-eosin stain showed epidermal necrosis with subepidermal blisters and neutrophilic panniculitis. Herein we report a case of cellulitis caused by the nematocyst stings of corals.