Treatment of extramammary Paget's disease with imiquimod in a real-life setting: a multicenter retrospective analysis in Spain.

BACKGROUND: Topical imiquimod has shown to be an effective treatment for EMPD, although available evidence supporting its use is based on case reports and small series of patients. OBJECTIVES: To investigate the therapeutic outcomes and analyze potential clinico-pathological factors associated with imiquimod response in a large cohort of EMPD patients. METHODS: Retrospective chart review of 125 EMPD patients treated with imiquimod at 20 Spanish tertiary-care hospitals. RESULTS: During the study period, patients received 134 treatment regimens with imiquimod, with 70 (52.2%) cases achieving complete response (CR), 41 (30.6%) partial response and 23 (17.2%) no response. The cumulative CR rates at 24 and 48 weeks of treatment were 46.3% and 71.8%, respectively, without significant differences between first-time and previously treated EMPD. Larger lesions (≥6 cm; p = 0.038) and EMPD affecting >1 anatomical site (p = 0.002) were significantly associated with a worse treatment response. However, the CR rate did not differ significantly by the number of treatment applications (≤4 vs. > 4 times/week; p = 0.112). Among patients who achieved CR, 30 (42.9%) developed local recurrences during a mean follow-up period of 36 months, with an estimated 3 and 5-year recurrence free-survival of 55.7% and 36.4%, respectively. CONCLUSIONS: Imiquimod appears as an effective therapeutic alternative for both first-line and previously treated EMPD lesions. However, a less favorable therapeutic response could be expected in larger lesions and those affecting >1 anatomical site. Based on our results, a 3-4 times weekly regimen of imiquimod with a treatment duration of at least 6 months could be considered an appropriate therapeutic strategy for EMPD patients.

Therapeutic outcomes and survival analysis of Extramammary Paget's disease: A multicentre retrospective study of 249 patients.

BACKGROUND: Evidence regarding long-term therapeutic outcomes and disease-specific survival (DSS) in Extramammary Paget's disease (EMPD) is limited. OBJECTIVES: To assess the DSS and outcomes of surgical and nonsurgical therapeutic modalities in a large cohort of EMPD patients. METHODS: Retrospective chart review of EMPD patients from 20 Spanish tertiary care hospitals. RESULTS: Data on 249 patients with a median follow-up of 60 months were analyzed. The estimated 5-, 10-, and 15-year DSS was 95.9%, 92.9%, and 88.5%, respectively. A significantly lower DSS was observed in patients showing deep dermal invasion (≥1 mm) or metastatic disease (P < .05). A ≥50% reduction in EMPD lesion size was achieved in 100% and 75.3% of patients treated with surgery and topical therapies, respectively. Tumor-free resection margins were obtained in 42.4% of the patients after wide local excision (WLE). The 5-year recurrence-free survival after Mohs micrographic surgery (MMS), WLE with tumor-free margins, WLE with positive margins, radiotherapy, and topical treatments was 63.0%, 51.4%, 20.4%, 30.1%, and 20.8%, respectively. LIMITATIONS: Retrospective design. CONCLUSIONS: EMPD is usually a chronic condition with favorable prognosis. MMS represents the therapeutic alternative with the greatest efficacy for the disease. Recurrence rates in patients with positive margins after WLE are similar to the ones observed in patients treated with topical agents.

Initial adaptation among university student: The case of the social sciences.

Students' academic and social adaptation is linked to factors such as their initial study motivations, the atmosphere of the academic environment and their perception of academic wellbeing. This paper analyses the initial adaptation of first-degree students in their first semester through a quantitative ex post facto study with a descriptive-exploratory approach, using a questionnaire as the information-gathering instrument. Findings shed light on the concept of initial adaptation itself (through the creation of an index) and validate the study of the construct through three factors: initial motivation, the academic environment and perception of academic wellbeing. Also, the influence on initial adaptation of the academic and social context of the degree course taken is demonstrated:, as the constitutive features of the degree contribute information predicting how students will integrate into the institution. Therefore, it is suggested that institutional means and actions should be designed and put in place in accordance with measurements that indicate how students function best in a specific context (the bachelor's degree), in order to boost motivation and the perception of academic wellbeing.

Cutaneous metastases from internal malignancies: a clinicopathologic and immunohistochemical review.

Skin metastases occur in 0.6%-10.4% of all patients with cancer and represent 2% of all skin tumors. Skin metastases from visceral malignancies are important for dermatologists and dermatopathologists because of their variable clinical appearance and presentation, frequent delay and failure in their diagnosis, relative proportion of different internal malignancies metastasizing to the skin, and impact on morbidity, prognosis, and treatment. Another factor to take into account is that cutaneous metastasis may be the first sign of clinically silent visceral cancer. The relative frequencies of metastatic skin disease tend to correlate with the frequency of the different types of primary cancer in each sex. Thus, women with skin metastases have the following distribution in decreasing order of frequency of primary malignancies: breast, ovary, oral cavity, lung, and large intestine. In men, the distribution is as follows: lung, large intestine, oral cavity, kidney, breast, esophagus, pancreas, stomach, and liver. A wide morphologic spectrum of clinical appearances has been described in cutaneous metastases. This variable clinical morphology included nodules, papules, plaques, tumors, and ulcers. From a histopathologic point of view, there are 4 main morphologic patterns of cutaneous metastases involving the dermis, namely, nodular, infiltrative, diffuse, and intravascular. Generally, cutaneous metastases herald a poor prognosis. The average survival time of patients with skin metastases is a few months. In this article, we review the clinicopathologic and immunohistochemical characteristics of cutaneous metastases from internal malignancies, classify the most common cutaneous metastases, and identify studies that may assist in diagnosing the origin of a cutaneous metastasis.

Superficial thrombophlebitis: A new clinical manifestation of the immune reconstitution inflammatory syndrome in a patient with HIV infection.

Skin manifestations of immune reconstitution inflammatory syndrome in response to highly active antiretroviral therapy may account for up to 50% of the clinical presentations in this syndrome. Viable or dying infective antigens, host antigens, tumoral antigens, and others may target immune reconstitution inflammatory syndrome, resulting in a wide spectrum of clinical manifestations. We describe a 26-year-old HIV-infected man who had started highly active antiretroviral therapy a few months earlier. He developed multiple linear nodules following the superficial veins in both legs. Histopathologic examination demonstrated a mostly septal panniculitis with features of superficial thrombophlebitis. We propose that superficial thrombophlebitis should be added to the list of clinical manifestations of this newly observed immune restoration disease.

Unilateral blaschkolinear psoriasis.

Acquired blaschkolinear psoriasis is a rare entity. It is commonly confused clinically and histopathologically with inflammatory linear verrucous epidermal nevus and other acquired blaschkolinear dermatoses. We describe a case of psoriasis with a striking band-like and linear distribution in a 21 year old woman. The history of asymptomatic acquired erythematous scaly lesions, the peculiar distribution, the histopathology and the favourable response to anti-psoriatic treatment confirmed the diagnosis.

Keratosis lichenoides chronica: response to photodynamic therapy.

Keratosis lichenoides chronica (KLC) is a rare, acquired disorder of keratinization of unknown aetiology. The disease has a chronic and progressive course and is characterized by a poor response to almost all topical treatments and most systemic regimens. We report the first case of KLC in which there was a marked response in localized areas to photodynamic therapy (PDT) with methyl 5-aminolaevulinic acid (ALA).