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Biomedical subjects

Albert C Yan

Publications and source records attributed to Albert C Yan.

At least 19 recordsLinked to original sources
PubMed2006

Current concepts in acne management.

Acne vulgaris is a nearly universal phenomenon among adolescents in the western world and continues to remain problematic for a significant proportion of adults. During adolescence, emotional and physical changes must be successfully integrated into the emerging sense of self, and skin disorders such as acne, which alter that self-image, may engender distressing feelings of embarrassment, shame, and disgust. While most patients eventually achieve spontaneous remission, approximately one quarter of teenagers will show evidence of permanent acne scarring by 18 years of age. This article reviews current information regarding the pathophysiology, clinical manifestations, differential diagnosis, and therapy of the adolescent patient who has acne, and emphasizes recent advances in acne management.

Acne Vulgaris↗
PubMed2006

PTU-associated vasculitis in a girl with Turner Syndrome and Graves' disease.

Palpable purpura is a concerning clinical finding in pediatric patients and can have many causes, including infectious and autoimmune processes. A rare cause, drug-induced vasculitis, may result from the production of antineutrophil cytoplasmic antibodies (ANCAs) in response to a medication. We report a girl with Turner syndrome and Graves' disease who presented with palpable purpuric lesions. The diagnosis of propylthiouracil (PTU)-associated vasculitis was made by observation of consistent clinical features, the detection of elevated ANA and ANCA in the blood, and the observed clinical resolution of symptoms following withdrawal of PTU. Subsequent treatment of persistent hyperthyroidism with radioablation did not result in an exacerbation of the vasculitis, a complication described in prior case reports.

Antithyroid Agents↗
PubMed2006

Treatment of pediatric localized scleroderma with methotrexate.

OBJECTIVE: To analyze the effectiveness of methotrexate (MTX) for the therapy of pediatric localized scleroderma (LS). METHODS: A retrospective chart review was performed for 17 pediatric patients with LS who failed topical therapy and were subsequently treated with MTX (12.5-25 mg weekly) with or without oral corticosteroids. A structured followup telephone call to the families was used to assess patient satisfaction. RESULTS: Skin findings improved in 16 of 17 patients with a median time to improvement of 2.25 and 2.0 months for MTX alone or in combination with corticosteroids. Only one patient had active lesions at the most recent followup visit. Fifteen of 17 families reported improvement in their child's lesions after beginning MTX. Twelve of 17 patients were treated with MTX and oral corticosteroids. There were no major adverse events. CONCLUSION: MTX appears to be a safe and effective therapy for pediatric LS.

Adolescent↗
PubMed2006

Methicillin-resistant Staphylococcus aureus skin abscesses in a pediatric patient with atopic dermatitis: a case report.

Recent trends indicate an increasing incidence of community-acquired methicillin-resistant Staphylococcus aureus (CAMRSA) in the general population, which is especially worrisome for clinicians caring for patients with atopic dermatitis (AD). Patients with AD are heavily colonized with S aureus and have impaired skin integrity and abnormal immune responses, which predisposes them to more invasive cutaneous infections (eg, cellulitis, furuncles, abscesses). In this report, we describe a child with severe AD who presented with CAMRSA skin abscesses. The presence of an atypical skin infection in patients with AD, particularly those unresponsive to conventional penicillinase-resistant penicillins and cephalosporins, should alert the clinician to the possibility of MRSA as the underlying etiology, and intervention should be directed accordingly.

Child↗
PubMed2005

Two novel TP63 mutations associated with the ankyloblepharon, ectodermal defects, and cleft lip and palate syndrome: a skin fragility phenotype.

BACKGROUND: Ankyloblepharon, ectodermal defects, and cleft lip and palate (AEC) syndrome is a rare autosomal dominant disorder caused by mutations in the sterile alpha motif region of TP63, a homologue of the tumor suppressor TP53. Recent structure-function studies have identified complexities in the genotype-phenotype correlation of the p63 syndromes. OBSERVATIONS: We report 2 sporadic cases of AEC syndrome in infants. Both patients demonstrated skin erosions with prominent scalp involvement. Histologic studies demonstrated mild basal layer vacuolization and rare dyskeratotic keratinocytes, with evidence of both acantholysis and cytolysis at the blister edge. Immunohistochemistry using anti-p63 monoclonal antibody demonstrated basal epidermal nuclear staining in both healthy control and patient tissue samples. Ultrastructural studies showed focal disruption of anchoring fibrils near the blister edge of one patient and normal desmosomes, hemidesmosomes, and basement membrane zone in the nonblistered skin of the other patient. The DNA analysis of each patient revealed 2 novel missense mutations in the TP63 gene that resulted in L514S and R555P amino acid substitutions within the sterile alpha motif region of the p63 protein. CONCLUSIONS: We report 2 novel TP63 mutations resulting in AEC syndrome. The R555P mutation is the most carboxy-terminal of all the reported AEC missense mutations of p63. The presence of skin fragility, manifested as erosive skin lesions in body areas in addition to the scalp, is postulated to be an important diagnostic feature of AEC syndrome.

Abnormalities, Multiple↗
PubMed2005

Aquagenic wrinkling of the palms in patients with cystic fibrosis homozygous for the delta F508 CFTR mutation.

BACKGROUND: Aquagenic wrinkling of the palms (AWP) is a rare condition characterized by the rapid and transient formation of edematous whitish plaques on the palms on exposure to water (the so-called hand-in-the-bucket sign). The changes may be asymptomatic or accompanied by pruritic or burning sensations. First described in 1974 in patients with cystic fibrosis--and still primarily reported in these patients--this condition has been previously described only in females. Specific mutations in CFTR, the gene responsible for cystic fibrosis, have not been reported previously in patients with AWP. OBSERVATIONS: We describe 2 patients with AWP, both of whom are homozygous for the delta F508 mutation in CFTR: a 17-year-old boy--the first male reported to have this condition--who has had AWP for 3 years and a 13-year-old girl who has had AWP for 6 months. CONCLUSIONS: Cystic fibrosis should be considered in patients with AWP, and patients with cystic fibrosis should be asked about symptoms of this condition. Although the etiology of AWP is unknown, the association with cystic fibrosis, and with marasmus and cyclooxygenase-2 inhibitors, suggests that exposure of the skin to abnormally high concentrations of salt may play a role in its pathogenesis.

Adolescent↗
PubMed2005

Rice nightmare: Kwashiorkor in 2 Philadelphia-area infants fed Rice Dream beverage.

A 14-month-old girl and a 7-month-old boy each presented with a diffuse dermatitis, whole body edema, and hypoalbuminemia. The diets of both infants consisted almost entirely of Rice Dream, a rice-based, protein-poor beverage. Both infants were diagnosed with kwashiorkor, which resolved with protein supplementation. Clues from the physical examination, a diet history, appropriate laboratory examinations, and an index of suspicion are crucial in promptly diagnosing and treating infants with kwashiorkor. Manufacturers of rice beverages should appropriately warn parents about the dangers of using their products as infant nourishment.

Dermatitis↗
PubMed2005

Neonatal cutaneous fungal infections.

PURPOSE OF REVIEW: Cutaneous fungal infections are not uncommon in newborns and are seen in premature or otherwise immunocompromised neonates as well as in healthy full-term neonates. Healthy newborns can develop clinical manifestations as a result of infection with Candida species or as a result of skin colonization with Malassezia species; cutaneous infection with other fungal pathogens is rare. Immunocompromised and premature neonates, however, are susceptible to infection with opportunistic pathogens and are also at higher risk for invasive infection with common pathogens such as Candida. This review discusses the fungal species associated with cutaneous fungal infection in neonates, emphasizes the relevant clinical features, and also reviews the use of newer antifungal agents, including lipid-associated amphotericin B, voriconazole, and caspofungin. RECENT FINDINGS: Neonatal cutaneous infections with opportunistic fungal pathogens, including Aspergillus and the Zygomycetes, have been reported with increasing frequency as advances in neonatal care have improved the survival rate in very low birthweight neonates. Although these infections are frequently fatal, survival in some neonates has been reported with the use of aggressive surgical debridement and systemic antifungal therapy. Newer antifungal agents, including voriconazole and caspofungin, show promise in the treatment of potentially fatal fungal infections in neonates. SUMMARY: Cutaneous fungal infections in neonates range from generally benign conditions such as congenital candidiasis and neonatal cephalic pustulosis to potentially fatal infections with opportunistic pathogens in very low birthweight or immunocompromised neonates. The prompt recognition and appropriate treatment of cutaneous fungal disease in neonates is critical to the prevention of adverse outcomes.

Amphotericin B↗
PubMed2005

How and when to treat molluscum contagiosum and warts in children.

Warts and molluscum contagiosum are common skin diseases in children and are usually self-limiting. The decision of whether to treat children with molluscum or warts should be individualized to the patient and his or her family. Considerations include how symptomatic the lesions are, the extent and duration of disease, the ability of the child and the parents to tolerate and comply with treatment recommendations, and any underlying medical conditions (Table, see page 219). Recurrences of molluscum contagiosum and especially warts are common, and realistic expectations regarding the potential for treatment failure and recurrence should be discussed with the child and his or her family prior to initiating any therapy. As pediatric practitioners, we all remain acutely aware of our patients' physical and psychological development and the potential for any intervention to influence this development. Although various treatment modalities now exist for the treatment of these viral diseases, any intervention should be balanced against these considerations.

Adhesives↗
PubMed2005

Addisonian pigmentation of the oral mucosa.

Cutaneous pigmentation is a hallmark of Addison disease. When present, the hyperpigmentation generally localizes to sun-exposed surfaces. This case highlights a less well-recognized cutaneous feature that is pathognomonic for the disease: oral mucous membrane hyperpigmentation. We describe this unique type of discoloration in detail and contrast it with other forms of oral pigmentation.

Addison Disease↗
PubMed2004

Necrolytic acral erythema: response to combination therapy with interferon and ribavirin.

Necrolytic acral erythema is a papulosquamous and sometimes vesiculobullous eruption bearing clinical and histologic similarity to other necrolytic erythemas such as necrolytic migratory erythema, pseudoglucagonoma, and nutritional deficiency syndromes. Necrolytic acral erythema is distinguished by its association with hepatitis C infection and its predominantly acral distribution. We describe a pediatric patient with necrolytic acral erythema whose eruption resolved with hyperalimentation and combination interferon and ribavirin therapy, despite the persistence of detectable viral load and continued hepatic and renal insufficiency.

Acrodermatitis↗
PubMed2004

Nonpseudomonal ecthyma gangrenosum.

Ecthyma gangrenosum is a cutaneous infection associated most commonly with pseudomonal sepsis in the patient who is immunocompromised. We describe an 8-month-old girl with acute myelocytic leukemia who developed perineal ecthyma gangrenosum caused by Citrobacter freundii, a gram-negative pathogen that has been rarely associated with cutaneous disease. We also review the literature to categorize the range of pseudomonal and nonpseudomonal pathogens associated with ecthyma gangrenosum.

Biopsy↗