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.

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

BACKGROUND: Topical imiquimod has been shown to be an effective treatment for extramammary Paget disease (EMPD), although available evidence supporting its use is based on case reports and small series of patients. OBJECTIVES: To investigate the therapeutic outcomes and analyse potential clinicopathological factors associated with the imiquimod response in a large cohort of patients with EMPD. METHODS: Retrospective chart review of 125 patients with EMPD 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%) treatments achieving a complete response (CR), 41 (30.6%) a 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 = 0.04) 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 per week; P = 0.112). Among patients who achieved CR, 30 of 69 (43%) treatments resulted in 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 favourable therapeutic response could be expected in larger lesions and those affecting > 1 anatomical site. Based on our results, a three to four times weekly regimen of imiquimod with a treatment duration of at least 6 months could be considered an appropriate therapeutic strategy for patients with EMPD.

Prognostic factors for satellitosis or in-transit metastasis in cutaneous squamous cell carcinoma: A multicentric cohort study.

BACKGROUND: Satellitosis or in-transit metastasis (S-ITM) has clinical outcomes comparable to node-positivity in cutaneous squamous cell carcinoma (cSCC). There is a need to stratify the risk groups. OBJECTIVE: To determine which prognostic factors of S-ITM confer an increased risk of relapse and cSCC-specific-death. METHODS: A retrospective, multicenter cohort study. Patients with cSCC developing S-ITM were included. Multivariate competing risk analysis evaluated which factors were associated with relapse and specific death. RESULTS: Of a total of 111 patients with cSCC and S-ITM, 86 patients were included for analysis. An S-ITM size of ≥20 mm, >5 S-ITM lesions, and a primary tumor deep invasion was associated with an increased cumulative incidence of relapse (subhazard ratio [SHR]: 2.89 [95% CI, 1.44-5.83; P = .003], 2.32 [95% CI, 1.13-4.77; P = .021], and 2.863 [95% CI, 1.25-6.55; P = .013]), respectively. Several >5 S-ITM lesions were also associated with an increased probability of specific death (SHR: 3.48 [95% CI, 1.18-10.2; P = .023]). LIMITATIONS: Retrospective study and heterogeneity of treatments. CONCLUSION: The size and the number of S-ITM lesions confer an increased risk of relapse and the number of S-ITM an increased risk of specific-death in patients with cSCC presenting with S-ITM. These results provide new prognostic information and can be considered in the staging guidelines.

Observational study of chondrodermatitis nodularis helicis treated with methyl aminolevulinate photodynamic therapy.

BACKGROUND: Therapies used to treat chondrodermatitis nodularis helicis (CNH), such as surgical excision, pressure relief, or topical steroids report varying degrees of success. OBJECTIVE: We evaluated the response and safety of methyl aminolevulinate (MAL) photodynamic therapy (PDT) in CNH. METHODS: This retrospective, observational study performed at the University Hospital Ramon y Cajal (Madrid, Spain) and Hospital San Jorge (Huesca, Spain) included all patients diagnosed with CNH and treated with MAL-PDT from 2008 to 2015. Treatment sites were prepared and irradiated as per the conventional MAL-PDT procedure. RESULTS: Patients underwent a mean of 2.3 sessions with between-session intervals ranging from 15 days to 1 month. A complete response to PDT was observed in 33 patients (76.7%), who experienced pain relief and resolution of the inflammatory nodule. Lesion recurrence was recorded in 10 patients (23.3%) during the mean follow-up period of 20 months. Receiving ≥2 PDT sessions was significantly associated with a good response (26/28, 93% success rate, P = .003). LIMITATIONS: Some limitations of the study are the lack of an established between-session interval, the absence of evaluation of curettage effectiveness and the limited sample size. DISCUSSION: The results support the view that PDT is a promising treatment approach for CNH.

Primary cutaneous CD30+ anaplastic large cell lymphoma treated with radiotherapy and methotrexate with development of xanthomas at the sites of prior disease.

Primary cutaneous anaplastic large cell lymphoma is a rare type of cutaneous T-cell lymphoma, and the involvement of the ocular adnexa is extremely rare. Secondary xanthoma-like changes after radiation therapy or chemotherapy have been rarely reported in association with large-cell T-cell anaplastic lymphoma. We report one case of a primary C-anaplastic large cell lymphoma affecting the eyelid with fast progression with multiple nodules in various anatomic sites and development of xanthoma-like lesions after treatment.

Case Report: Extensive Dermatitis Secondary to Severe Malnutrition, Zinc and Vitamin Deficiencies After Malabsorptive Bariatric Surgery.

Bariatric surgery is one of the most effective treatments currently available for obesity and its derived comorbidities. However, complications may occur, especially when malabsorptive surgeries like a biliopancreatic diversion is performed. We present the case of a female patient whose obesity was treated with this technique, and in the 9th year of follow-up developed an extensive dermatitis secondary to zinc deficiency and malnutrition, precipitated by therapeutic non-compliance. A close surveillance of early symptoms and signs of nutritional deficiencies as well as chronic supplementation of vitamins and trace elements is required; this case illustrates the relevance of periodical, lifelong visits to a medical physician with special training and experience in the management of post bariatric surgery patients in order to prevent, diagnosis and early treat related complications.