Assessment of the perceived burden associated with Malignant Melanoma with Pictorial Representation of Illness and Self Measure (PRISM) and Melanoma Concerns Questionnaire (MCQ-28).

PURPOSE: The impact of malignant melanoma (MM) on patients' psychophysical well-being has been poorly addressed. We aimed to assess the perceived burden in patients with a diagnosis of MM, using two different tools, one generic and one specific for MM, such as Pictorial Representation of Illness and Self Measure (PRISM) and Melanoma Concerns Questionnaire (MCQ-28), respectively. The correlation between PRISM and MCQ-28 subscales and the relevance of disease and patient-related variables were also investigated. METHODS: This single-centre, cross-sectional study included all adult consecutive MM patients who attended our Dermatology Unit from December 2020 to June 2021. Demographics and disease-related data were recorded. PRISM and MCQ-28 were administered. RESULTS: One hundred and seventy-one patients were included (mean age: 59.5 ±14.9 years.; 48.0% males). Median time from MM diagnosis to inclusion was 36 months. Nearly 80% of the patients had in situ or stage I MM. Overall, 22.2% of the patients reported a PRISM score <100mm and similar percentages provided scores indicating impaired quality of life, as assessed with MCQ-28 subscales. A weak, albeit significant, correlation was found between PRISM scores and ACP, CON and SOC2 subscales. The most relevant association found was that between lower PRISM scores and higher-stage MM. CONCLUSIONS: In the study population, mostly affected with superficial MM, their perception of the burden associated with MM did not appear either particularly dramatic or disabling. PRISM seems a reliable system for capturing and quantifying the domains correlated with the emotive dimension of MM, especially MM-related concerns and willingness to face life.

Multiple perianal ulcers due to suppositories.

We report a case of long-standing inexplicable perianal ulcers. After exclusion of an inflammatory, infectious or neoplastic origin, a thorough personal history revealed that for many years the patient had been using analgesic suppositories containing indomethacin, caffeine, and prochlorperazine dimaleate, four to five times a week, for migraine. On stopping the suppositories, there was complete healing within 12 weeks. We hypothesize that vasoconstriction and vascular damage were the pathogenetic mechanisms behind the perianal ulcers.

Post-traumatic erosive dermatosis of the scalp: A hypergranulated variant.

Erosive pustular dermatosis of the scalp is a slowly progressive chronic inflammatory disease that predominantly affects elderly male patients with marked actinic damage. The clinical evolution consists firstly of keratotic and erosive plaques surmounted by yellow-brown crusts and non-follicular pustules; later, the active crusting lesions regress in number and the scarring process causes diffuse cutaneous thinning and loss of hair follicles. However, manifestations may be atypical, leading to frequent misdiagnosis. We present a case series of post-traumatic erosive pustular dermatosis on the scalp of 4 elderly patients. The characterising feature was the presence of erosion consisting of abundant hypergranulation tissue, with an almost total lack of crusts and pustules. Dermoscopy showed a unique pattern of stretched and dilated linear, telangiectatic and polymorphous on-focus vessels, milky-red areas and white scarring areas. This clinical entity is rarely reported in the literature. The majority of reported cases were located on the legs.

Topical corticosteroids versus "wait and see" in the management of solitary mastocytoma in pediatric patients: a long-term follow-up.

Management of patients affected by mastocytoma (MS) includes avoiding triggering factors of mast cell degranulation, and administration of symptomatic treatment. We evaluated topical steroid treatment efficiency on the clinical course of MS in a group of patients, comparing the results with another untreated group.We retrospectively evaluated clinical data of 176 patients under 15 years of age, affected by MS and referred to our Dermatological Pediatric Service from 1996 to 2010. Ninety-one of 176 children were treated with topical steroids. Follow-up was possible in 130 of 176 patients and lasted for 56.3 months on average. We compared 62 treated and 68 untreated patients. There was not statistic difference between the two groups: (i) in the number of healed or partially improved cases; and (ii) in the time of partial regression, although it is quicker with therapy. The time of healing is 16.4 months (on average) with treatment, and 34.7 months (on average, p = 0.001) without any treatment. The resolution of MS is independent of therapy administration, but the time of healing is statistically faster using the local steroids. An appropriate treatment with them is effective and safe, considering the long time needed for spontaneous resolution.

Psoriasis with Leg Involvement as the New Difficult-To-Treat Area: A Cohort Study of Patients Treated With Risankizumab.

INTRODUCTION: Historically, difficult-to-treat areas in psoriasis included face, scalp, folds, genitalia, nails, and palmoplantar region. Recent studies have found that lower limbs behave like a "new" difficult-to-treat area as they can be the only site of residual disease even in patients undergoing biologic therapies. OBJECTIVES: We aimed to evaluate whether legs had different response rates and response times to treatment with a new biologic drug, risankizumab, compared to other body sites. METHODS: We conducted a real-life, observational, retrospective, multicenter study including patients affected by moderate-to-severe psoriasis with leg involvement and undergoing biological therapy with risankizumab for more than 16 weeks. The Psoriasis Area Severity Index (PASI) and Leg-PASI were collected at T0 and at weeks 16, 28, 40, 52, 64, and 76. Statistical analysis using Student's t test and linear regression analysis were performed. RESULTS: A total of 124 patients were included. The difference between the improvement percentage compared to baseline was statistically significant at weeks 16 and 28, demonstrating that Leg-PASI improved less than PASI. From the linear regression it was deduced that the slope is statistically less steep for Leg-PASI than for overall PASI, confirming that this site responds more slowly to the therapy. CONCLUSIONS: Leg response to risankizumab appears to differ significantly from other body sites in the first weeks of treatment, even if after 28 weeks, statistical significance is lost. Our preliminary finding suggests that risankizumab can be considered an effective treatment for leg psoriasis but with longer response times than other areas, demonstrating the relative nature of resistance to treatment of this district.

Multiple basal cell carcinomas in a 38-year-old woman with Goltz syndrome.

BACKGROUND: Focal dermal hypoplasia (FDH) or Goltz syndrome is a rare genetic multisystem disorder characterized by hypoplasia of ectodermally and mesodermally derived tissues. No cases of development of basal cell carcinomas in patients affected by FDH have previously been reported. METHODS: We followed a 38-year-old woman with FDH who developed, within a period of 3 years, 14 atypical pigmented lesions. All of them were surgically removed and pathologically assessed. In 2007, this patient underwent molecular examination with the multiple amplifiable probe hybridization technique. RESULTS: Histopathological examination showed 6 basal cell carcinomas, 2 basaloid proliferations, 2 tumours of follicular infundibulum and 2 solar lentigines. Molecular examination showed that only 1 copy of the coding exons of PORCN and EBP, respectively, was present, reflecting a microdeletion of one of her X chromosomes, eliminating at least the neighbouring genes PORCN and EBP. CONCLUSIONS: No other cases of association between FDH and multiple cutaneous basal cell carcinomas have previously been reported, so it could be interesting to take into consideration this aspect in the molecular assessment of these patients to improve information on the disease. This is a single case experience, and especially the molecular results need confirmation and validation by other groups involved in the diagnosis.

Use of Apremilast® in the psoriasis treatment: a real-life multicenter Italian experience.

BACKGROUND: Apremilast® (Amgen, Thousand Oaks, CA, USA) is the first small molecule approved for the treatment of moderate-to-severe psoriasis in adult patients; however, real-life data are still limited. We investigated the effectiveness and safety of this drug in a multicenter real-world setting. METHODS: We retrospectively reviewed data from all psoriatic patients who received at least one dose of Apremilast® (Amgen) and collected demographic data and medical history at baseline and periodically for 36 months. RESULTS: A total of 111 patients entered in the study. The mean drug survival duration was 21.8±10.6 months; however, it was significantly shorter when comorbidities were ≥3 and if biologic drugs were previously administered. ΔPASI90 was achieved in 29% of patients and ΔPASI50 in 68% at T4; the rate of ΔPASI improvement increased progressively at T12, T24, T36 in patients who continued to receive Apremilast® (Amgen). At the end of the study 50 patients discontinued the treatment because of adverse events (19.8%), primary failure (19%) or secondary failure (6.3%). CONCLUSIONS: Apremilast® (Amgen) proved to be an effective, safe, and manageable drug, showing effectiveness also in difficult-to-treat patients with psoriasis, with a favorable tolerability profile and a potentially valid weight loss effect. We believe that treating patients with few comorbidities who are naive to biological therapy may result in higher response rates and longer mean drug survival.

Guselkumab for the treatment of psoriasis: a 60-week real-life multicenter retrospective experience.

BACKGROUND: Real-world data for guselkumab, the first interleukin-23 inhibitor approved to treat moderate-to-severe psoriasis, are scarce. This study represents the first 60-week, real-life, multicenter, retrospective experience to investigate the effectiveness, safety, tolerability, and drug retention of guselkumab in psoriatic patients. RESEARCH DESIGN AND METHODS: Clinical information was collected at baseline and at weeks 12, 24, 36, 48, and 60. RESULTS: The mean baseline Psoriasis Activity Severity Index (PASI) reduced from 14.2 to 3.1 at week 12 and decreased to around 0 at weeks 36, 48, and 60. PASI 75, PASI 90, and PASI 100 were 100%, 96.8%, and 83.9% at week 60, respectively. Multiple logistic regression analysis showed that neither body mass index >30, smoking, ≥3 comorbidities, difficult-to-treat areas, nor a failure to ≥2 prior biologic treatments significantly influenced PASI reduction (p > 0.05). CONCLUSIONS: Our findings confirm guselkumab as an appropriate therapeutic option in routine clinical practice, especially when dealing with complex patients with comorbidities or previous failure to biologic treatments.

An angiomatoid fibrous histiocytoma over the left pre auricular region in a 13-year-old boy.

A 13-year-old boy presented to us for the evaluation of a slowly growing mass over the left preauricular region. Physical examination showed a nodular swelling, firm on palpation. The patient had no lymphadenopathy. The findings of magnetic resonance imaging (MRI) and vascular MRI led us to suspect a vascular growth. An incisional biopsy was performed and revealed an angiomatoid fibrous histiocytoma (AFH). Angiomatoid fibrous histiocytoma (AFH) is a rare tumor of the soft tissue with fibrohistiocytic and vascular differentiation that shows an intermediate malignancy grade, mainly occurs in patients younger than 20 years of age, and is usually localized on the extremities. Angiomatoid fibrous histiocytoma is considered to be a tumor of intermediate malignancy because of its less aggressive course in contrast to conventional malignant fibrous histiocytoma (MFH). Our case of AFH is peculiar because of its localization in the pre auricular region and because it appeared in a young patient. Surgical excision with maxillo-facial, chest-abdomen, and neck CT and prolonged follow-up is recommended because rare cases of metastasizing AFH have been reported.

Dispelling the myth of the "benign hair sign" for melanoma.

The vast majority of melanocytic lesions with hair, such as congenital melanocytic nevi, are benign. However, there is a notion that the presence of one or more hairs in a melanocytic lesion is confirmatory for the benign nature of the lesion. To dispel this notion, we present 3 examples of melanocytic lesions that showed terminal hairs on clinical and dermoscopic evaluation, but in which the final diagnosis was invasive melanoma. Thus, integrating all clinical and dermoscopic findings, rather than relying on a single criterion for the lesion at hand should guide clinicians to the correct diagnosis.

Treatment of refractory pemphigus vulgaris with anti-CD20 monoclonal antibody (rituximab): five cases.

BACKGROUND: Pemphigus vulgaris is an autoimmune disease characterized by blisters and widespread erosions, involving skin and mucous membranes, caused by autoantibodies to desmoglein 1 and 3. This pathology is associated with increased morbidity and mortality if untreated. The treatment of pemphigus vulgaris requires multiple immunosuppressive agents, but often it is particularly resistant. OBJECTIVE: To evaluate the efficacy and safety of rituximab therapy in refractory pemphigus vulgaris. METHODS: Five patients diagnosed as having pemphigus vulgaris were treated with anti-CD20 monoclonal antibody (rituximab). Each patient was treated with rituximab intravenously at a dosage of 375 mg per square metre of body surface area once weekly for 4 weeks. RESULTS: All the patients presented clinical resolution. No adverse effects were observed. It is important to observe the clinical evolution in the future, but our experience is still limited to a short lifetime and follow-up. CONCLUSION: In our experience rituximab has been an effective and safe treatment for refractory pemphigus vulgaris.

Cutaneous granulomatous reaction to injectable hyaluronic acid gel: another case.

We report a case of a granulomatous reaction in the melolabial folds, occurring 10 days after treatment with Restylane. The patient, who had previously been treated with the same product in the last 2 years without any adverse effect, developed an unusual early fibrotic reaction that we hypothesized related to hypersensitivity after repeated use. The lesions slowly disappeared with topical steroid therapy. An improved knowledge of the modality of these uncommon adverse effects is necessary to assess the long-term safety and efficacy of this product.