Vismodegib for Basal Cell Carcinoma and Beyond: What Dermatologists Need to Know.

Basal cell carcinoma (BCC) is the most common cutaneous malignancy. Although indolent, BCCs can be locally aggressive if untreated. Dysregulated hedgehog (Hh) signaling leads to uncontrolled proliferation in BCC. Vismodegib is a small-molecule antagonist of the Hh pathway that binds to smoothened (SMO), a transmembrane protein, and causes inhibition of an aberrant activation of the Hh pathway. Vismodegib is the first drug approved by the US Food and Drug Administration (FDA) for the management of locally advanced or metastatic BCC.

Pemphigus Vulgaris-Associated Anterior Scleritis: A Case Study.

Ocular involvement in pemphigus vulgaris (PV) is relatively rare. The conjunctiva and eyelids are considered the most common affected sites in ocular pemphigus. Scleritis is rarely reported as a manifestation of PV. We present a case report of anterior scleritis as a manifestation of PV and its response to rituximab therapy.

Confirming the recessive inheritance of PERP-related erythrokeratoderma.

Erythrokeratoderma (EK) is heterogeneous clinical entity characterized by excessive scaling with resulting erythrokeratotic plaques. Several genes have been linked to EK and they encode a number of proteins that are important for the integrity of the keratinocyte layer of the epidermis. PERP is a transcription factor that is activated by both p53 and p63. However, its deficiency in a mouse model appears to only recapitulate p63-mediated role in skin development and organization. We report an extended multiplex consanguineous family in which an EK phenotype with a striking similarity to that observed in Perp-/- mice, is mapped to an autozygous region on chromosome 6 that spans PERP. Whole-exome sequencing revealed a novel variant in PERP that fully segregated with the phenotype. Functional analysis of patient- and control-derived keratinocytes revealed a deleterious effect of the identified variant on the intracellular localization of PERP. A previous report showed that PERP mutation causes a dominant form of keratoderma but a single patient in that report with a homozygous variant in PERP suggests that recessive inheritance is also possible. Our results, therefore, support the establishment of an autosomal recessive PERP-related EK phenotype in humans.

Twenty-five year-old female with sudden onset itchy skin eruption over her upper back and chest three weeks after starting a ketogenic diet.

Prurigo pigmentosa is a rare pruritic inflammatory dermatosis with a unique staged clinicopathological presentation. It was first reported by Nagashima in 1971, and recently, more cases have been reported We introduce a case of a young Saudi female who developed biopsy proved prurigo pigmentosa after she followed strict ketogenic diet. Her condition resolved after she resumed a regular diet.

Unusual presentation of dermatofibroma on the face: Case report.

Dermatofibroma (DF; Benign Fibrous Histiocytoma) seldom presents on the face. We present an unusual case presentation of translucent nodule on the upper right cheek of the face which was diagnosed clinically as basal cell carcinoma. Physician should include DF in the differential of such lesions.

Ipilimumab in anti-PD1 refractory metastatic melanoma: a report of eight cases.

Targeted therapy and immunotherapy in metastatic melanoma have led to a marked improvement in patients' survival and their quality of life. Although there are data on anti-programmed-death-receptor-1 (anti-PD1) after ipilimumab, only few data are available on ipilimumab following anti-PD1 as the first-line treatment. The aim of our study was to evaluate tolerance and survival of patients treated with ipilimumab as the second-line immunotherapy among metastatic melanoma patients following anti-PD1 treatment. Retrospective and descriptive epidemiological studies were carried out at the Dermatology Department of the University Hospital of Lille. We describe a case series of patients treated with ipilimumab after anti-PD1 failure for metastatic melanomas. For each patient, we assessed disease extension since ipilimumab introduction using RECIST 1.1. The time between ipilimumab introduction and other systemic treatment and overall survival (between ipilimumab introduction and last patient visit) was assessed. The effect of ipilimumab after anti-PD1 treatment was evaluated in eight patients. Four patients responded to ipilimumab: three showed a complete response and one showed a partial response. For these patients, the time period between the first ipilimumab injection and another systemic treatment ranged from 209 to 391 days and the overall survival ranged from 314 to 581 days. One patient showed grade 3 chorioretinitis, an unusual toxicity with ipilimumab or anti-PD1 to our knowledge. We have described the efficacy of ipilimumab following anti-PD1 in metastatic melanoma in eight patients. Several comparative studies are still in progress, and their results will be important to develop an optimal therapeutic strategy for our patients.

Perceptions, attitudes, and satisfaction concerning resident participation in health care among dermatology outpatients.

BACKGROUND: A limited number of published studies have discussed patient attitudes toward resident physicians' participation in dermatology clinics. A literature search failed to identify any such study in the Middle East. OBJECTIVE: The aim of this study was to explore patient perceptions and attitudes toward resident participation in dermatology outpatient clinics. METHODS: A self-administered questionnaire focused on patient attitudes toward dermatology resident participation was distributed randomly to all adult outpatients attending dermatology clinics at a university hospital in Saudi Arabia between July and September 2010. RESULTS: The questionnaire was returned by 742 of 900 patients, for an 82% response rate. The mean patient age was 30.58 ± 11.67 years. Forty-two percent (311 of 742) of the respondents were male. The major reason for visiting the hospital was a medical dermatology consultation (80.4%). Only 35% of the patients self-reported an accurate understanding of the "resident" designation. In total, 86.4% of patients were satisfied with the residents' behavior. Furthermore, 98.4% of the patients were satisfied with the medical care provided by the residents. The patients agreed with resident participation in their health care. The majority of the patients expressed their willingness to provide a medical history and receive counseling from residents (87.6% and 86.3%, respectively). There was no gender-associated effect on the understanding of the resident position or the decision to receive a physical examination by a resident. CONCLUSION: Dermatology outpatients are satisfied and have positive perceptions and attitudes toward resident participation in the dermatology clinic.