Recurrent Oral and Genital Ulcers in an Infant: Neonatal Presentation of Pediatric Behçet Disease.

Behçet disease is a complex, multisystem disease characterized by recurrent oral and genital ulcerations. It rarely occurs in infants or children. Neonatal Behçet disease has been reported in infants whose ulcers resolve at or before 9 weeks of age. Few cases of neonatal Behçet disease persisting into childhood have previously been reported. We report the case of a 1-month-old infant who presented with severe recurrent genital ulcerations and at 6 months developed recurrent oral ulcerations. Her orogenital ulcerations continue to recur. Human leukocyte antigen testing revealed HLA-B51 and B44 positivity. This is a case of pediatric Behçet disease in the neonatal period. Behçet disease should be considered in the differential diagnosis of recurrent genital and oral ulcerations in infants and children.

Perception of xeroderma pigmentosum support group members of xeroderma pigmentosum lifestyle impact.

Xeroderma pigmentosum (XP) is a genetic condition, which can cause an extreme sensitivity to sunlight and an increased risk of skin cancer due to errors in DNA repair. An online survey was administered to a convenience sample of participants who were members of an online support group for XP patients and their families to determine common symptoms and quality of life. The Dermatologic Life Quality Index (DLQI) or the Children's Dermatologic Life Quality Index (CDLQI) was used depending on patient age. A total of four patients and two parents of young patients completed our survey. Quality of life as measured through the DLQI and CDLQI was moderately affected.

Self-reported quality of life after skin cancer in young adults.

INTRODUCTION: Skin cancer incidence is increasing in younger adults. Patient satisfaction and quality of life surveys are limited, and more information is needed on the unique perspectives of these younger patients who may be affected differently. We sought to explore how skin cancer has affected quality of life in younger adults. METHODS: A self-reported questionnaire was administered to patients with skin cancer aged 20-50 years diagnosed from 2007 to 2008. Quality of life was measured using the Skin Cancer Index (SCI) and Skindex-16 with a transformed scale. Descriptive statistics with standard deviations were calculated. RESULTS: One-hundred sixty-one patients were identified; 62 completed surveys (38.5%), with 47 patients eligible for inclusion. Forty participants (85%) reported having made lifestyle changes since being diagnosed with non-melanoma skin cancers, most commonly habitual use of sunscreen (n = 34; 77.3%). Quality of life was most impacted by worries about their skin cancer (Skindex-16 score 24.5 (34.4 standard deviation)), including their future risk of developing further cancers (58.5 (31.8)). LIMITATIONS: Limitations include small sample size and possible response bias. CONCLUSIONS: There is a modest impact on quality of life in young patients with skin cancer based on the Skindex-16 and SCI. Young skin cancer survivors may benefit from patient counseling, which addresses risk assessment and future risk reduction.

Superficial fungal infections in children.

Superficial fungal infections can involve the hair, skin, and nails. Most affected children are healthy, although immunosuppression is a risk factor for more severe presentation. Causative organisms typically are members of the Trichophyton, Microsporum, and Epidermophyton genera (dermatophytes), can be acquired from other infected humans, animals, or soil, and illicit a host inflammatory response. Nondermatophyte infections include pityriasis versicolor. In this article, the most common clinical presentations, diagnostic recommendations, and treatment algorithms for dermatophyte and nondermatophyte mycoses in children and adolescents are described.

MEK2 is sufficient but not necessary for proliferation and anchorage-independent growth of SK-MEL-28 melanoma cells.

Mitogen-activated protein kinase kinases (MKK or MEK) 1 and 2 are usually treated as redundant kinases. However, in assessing their relative contribution towards ERK-mediated biologic response investigators have relied on tests of necessity, not sufficiency. In response we developed a novel experimental model using lethal toxin (LeTx), an anthrax toxin-derived pan-MKK protease, and genetically engineered protease resistant MKK mutants (MKKcr) to test the sufficiency of MEK signaling in melanoma SK-MEL-28 cells. Surprisingly, ERK activity persisted in LeTx-treated cells expressing MEK2cr but not MEK1cr. Microarray analysis revealed non-overlapping downstream transcriptional targets of MEK1 and MEK2, and indicated a substantial rescue effect of MEK2cr on proliferation pathways. Furthermore, LeTx efficiently inhibited the cell proliferation and anchorage-independent growth of SK-MEL-28 cells expressing MKK1cr but not MEK2cr. These results indicate in SK-MEL-28 cells MEK1 and MEK2 signaling pathways are not redundant and interchangeable for cell proliferation. We conclude that in the absence of other MKK, MEK2 is sufficient for SK-MEL-28 cell proliferation. MEK1 conditionally compensates for loss of MEK2 only in the presence of other MKK.