Moderate to Severe Psoriasis in Pediatric and Young Patients: The BIOBADADERM Registry Experience. / Psoriasis moderada-grave en pacientes pediátricos y jóvenes: experiencia en el registro BIOBADADERM.

Childhood-onset psoriasis generally follows an indolent course but patients with moderate or severe disease may require systemic treatment. The aim of this study was to determine the relative proportion of children and young people aged up to 21 years with moderate to severe psoriasis in the BIOBADADERM registry and to analyze the characteristics of these patients, treatments used, and adverse events. Of the 3946 patients in the registry, 24 were aged 21 years or younger. They had mean age of 16.1 years on starting treatment. When the registry was started, they had a Psoriasis Area and Severity Index of 9.4 and 67% were being treated with a conventional systemic drug. Treatment was discontinued in 14 patients (58%) due to adverse events or a loss or lack of effectiveness. In conclusion, the BIOBADADERM registry shows that young people account for a small proportion of psoriasis patients receiving systemic treatment, and they are more likely to be treated using conventional systemic drugs.

'Rainbow pattern': a dermoscopic sign of invasive melanoma.

BACKGROUND: The 'rainbow pattern' was initially described as a highly specific dermoscopic feature of Kaposi sarcoma. Since then, it has been reported in many benign and malignant cutaneous tumours, including a few malignant melanomas (MMs). AIM: To determine the frequency and presentation of this dermoscopic pattern in primary cutaneous MMs in comparison to other cutaneous tumours. METHODS: The presence of a rainbow pattern was evaluated in a sample of 1100 dermoscopic images of different melanocytic and nonmelanocytic cutaneous neoplasms. RESULTS: The rainbow pattern was observed in 23 of 245 (9.4%) MM and 44 of 855 (5.1%) non-MM neoplasms. MMs presenting this feature were generally thicker: 82.6% > 1 mm and 43.0% > 2 mm. Compared with non-MMs, rainbow pattern in MMs was more commonly focal (82.7% vs. 36.4% nonfocal, P = 0.001) and associated with > 2 dermoscopic structures associated with MM (100% vs. 9% with fewer, P = 0.001). CONCLUSION: The rainbow pattern is a dermoscopic sign that can occasionally be observed in invasive MMs. In MMs, this feature is usually associated with other dermoscopic criteria of MM and located in a focal and eccentric area, as opposed to a diffuse and isolated presentation in non-MM neoplasms.

Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case. / Dermatofibrosarcoma protuberans pigmentado: Descripción de un caso pediátrico.

INTRODUCTION: Bednar tumor is a rare low-grade sarcoma considered the pigmented variant of dermatofibrosarco ma protuberans (DFSP). OBJECTIVE: To describe the clinical and histopathological characteristics, treatment and evolution of this rare neoplasm. CLINICAL CASE: A 9-year old female presented with a 2-year history of an indurated, asymptomatic papule on the back of her fourth left toe. The incisio nal biopsy was compatible with pigmented DFSP. The immunohistochemical study showed intense positivity for CD34 throughout the lesion, with negative factor XIIIa. We complemented the study with molecular cytogenetics (FISH) for PDGFB gene (22q13.1) which showed an abnormal pattern in tumor cells, but not in the melanocytes or the peritumoral skin. Delayed Mohs surgery and skin substitute dressing were performed without neoplastic recurrence at 5 years of follow up. Conclu sion: Pigmented DFSP is a low-grade sarcoma that is very rare in pediatric patients. The classical and pigmented variants should be suspected in the presence of a single papulonodular lesion of slow and progressive growth, with presence of spindle cells with storiform pattern in the biopsy and positive immunohistochemical study for CD34. It is an entity with good prognosis, with little risk of recurren ce and metastasis, if complete excision is achieved.

Prognostic significance of sentinel node biopsy status in cutaneous melanoma: a 21-years prospective study from a single institution.

AIM: To analyze the accuracy of the sentinel lymphatic node biopsy (SLNB) and to investigate predictive factors for sentinel node (SN) status and prognostic factors for recurrence-free survival (RFS) and disease-specific survival (DSS) in patients with melanoma. MATERIAL AND METHODS: Between June 1997 and June 2017, 440 consecutive patients, who underwent SLNB by a single surgical team, were prospectively included. Descriptive and survival analysis were performed. RESULTS: 119 of 440 patients (26%) had positive SN. SLNB's false-negative rate was 6.3%. Breslow thickness, Clark´s level, ulceration and histological subtype were statistically significant predictive factors of SN metastases. In a multivariate analysis, positive SN (HR = 2.21, p = 0.01), deeper Breslow thickness (HR = 2.05, p = 0.013), male gender (RR = 2.05, p = 0.02), and higher Clark's level (HR = 2.30, p = 0.043) were significantly associated with decreased RFS; and positive SN (HR = 2.58, p < 0.001), deeper Breslow thickness (HR = 2.57, p = 0.006) and male gender (HR = 1.93, p = 0.006) were associated with lower DSS. CONCLUSION: SLNB is a reliable and reproducible procedure with high sensitivity (93.7%). Positive SN metastases, Breslow thickness and male gender were statistically associated with poorer outcomes. Male gender was an independent prognostic factor of tumor thickness or SN status.

Recommendations on Testing Before Outpatient Dermatologic Surgery. / Recomendaciones en la petición de pruebas preoperatorias en cirugía dermatológica ambulatoria.

INTRODUCTION: Short-duration ambulatory surgery for the removal of skin tumors under local anesthesia is increasingly common in dermatology. Preoperative assessment has traditionally targeted the identification of any unknown diseases or other health conditions that might lead to changes in plans for anesthesia or surgery. Hospitals and specialists differ greatly in the tests they order in patients about to undergo outpatient dermatologic surgery given that hardly any finding would be likely to contraindicate or lead to changes in the procedure. This study aimed to provide guidance for those ordering tests before outpatient dermatologic surgery. METHODS: In 2017 our hospital developed a protocol to standardize preoperative testing for outpatient dermatologic surgery. We designed an observational, descriptive, retrospective analysis of tests ordered for patients scheduled for such surgery before and after the protocol was applied. RESULTS: Fewer tests were ordered after the protocol was introduced. We detected no statistically significant differences in relation to type of surgery planned or postoperative complications. CONCLUSIONS: Patients about to undergo outpatient dermatologic surgery under local anesthesia who have no unusual health risks may not require preoperative testing.

Thiopurine methyl-transferase activity and azathioprine metabolite concentrations do not predict clinical outcome in thiopurine-treated inflammatory bowel disease patients.

BACKGROUND: Low thiopurine-methyl-transferase (TPMT) activity and high 6-thioguanine-nucleotide (6TGN) concentrations have been linked to therapeutic success in inflammatory bowel disease patients treated with thiopurines; however, this has not been implemented in clinical practice. AIM: To identify a therapeutic threshold value for TPMT or 6TGN concentrations, and their capability to predict treatment safety and efficacy. METHODS: Prospective multicentre study including steroid-resistant/dependent patients starting thiopurines. The TPMT activity was determined at inclusion (>5 U/mL required). Azathioprine metabolites [6TGN, 6-methyl-mercaptopurine ribonucleotides (6MMP), and 6TGN/6MMP and 6TGN/TPMT ratios] were periodically monitored during steroid tapering and after withdrawal for 6 months or until a new flare occurred. RESULTS: A total of 113 patients were analysed (62% clinical response). Areas under the receiver operating characteristic (ROC) curve (AUC) relating clinical response and metabolite levels at 2, 4 and 6 months after steroid withdrawal were less than 0.7. The AUCs relating final response and initial TPMT activity or metabolite concentrations at 2, 4, 8 and 16 weeks after starting thiopurines were less than 0.7. No cut-off point with worthwhile sensitivity/specificity was found. Eight (7%) patients developed thiopurine-related toxicity that could not be linked to TPMT activity or 6TGN levels. CONCLUSIONS: Our results do not support determination of TPMT activity or 6TGN concentrations to predict treatment outcome, and no useful serum metabolites threshold value to adjust the drug's dose was identified.