Bleomycin-induced flagellate dermatitis: report of four paediatric cases.

The reduced concentration of bleomycin hydrolase enzyme in the skin, pruri- tus and the concomitant radiotherapy could have had a synergistic role in the accumulation of the drug and triggered the flagellate dermatitis

Sézary Syndrome: a clinico-pathological study of 9 cases and review of the literature

BACKGROUND: Sezary Syndrome (SS) is a rare and aggressive variant of cutaneous T-cell lymphoma characterized by erythroderma, generalized lymphadenopathy and atypical lymphocytes in peripheral blood. The aim of the study is to describe our experience with SS patients.METHODS: 9 SS patients were retrospectively identified within 288 patients with cutaneous Tcell lymphomas (CTCLs) followed from 1977 to 2017 in the Unit of Dermatology, IRCCS Policlinico San Matteo Foundation, Pavia.RESULTS: 9 SS patients were described: 5 males and 4 females, mean age at diagnosis 66.1 years (49-87 ys), overall survival (OS) after SS diagnosis was 2.6 years (31.5 ms). All the patients showed erythroderma, pruritus and lymphadenopathy. Palmo-plantar hyperkeratosis, nail lesions, alopecia and ectropion were also present. One patient was excluded for significative differences in management. Three lines treatment -extracorporeal photopheresis plus immunomodulator/s plus photo-photochemotherapy- was the most used first-line option for induction of remission, reached in 4 patients out of 8: 3 with Complete Remission (CR), 1 with Partial Remission (PR). Prognostic variables were investigated by univariate analysis: hypereosinophilia, highly elevated beta2muglobulin >3500 mug/l, male sex and highly elevated LDH >450 U/l resulted with statistical power.CONCLUSIONS: The improved comprehension of SS pathogenesis is progressively increasing the -still poor- survival: 38.5 months (3.2 years) considering only the 6 patients followed in the last five years, versus overall 31.5 months (2.6 years). The correct identification of SS patients remains determinant for the proper overall management. Among unfavorable prognostic markers, levels of beta2muglobulin allow stratification of patients

A Clinico-Pathological Multidisciplinary Team Increases the Efficacy of Skin Biopsy and Reduces Clinical Risk in Dermatology

A clinical risk is an inherent risk in healthcare processes, including skin biopsy procedures, and may lead to misdiagnoses, increased healthcare costs and potential harm to patients. Indeed, clinical and histopathological data must be integrated if we are to reduce clinical risks and improve diagnostic accuracy in the diagnosis of dermatologic diseases. Although dermopathology services used to be part of a dermatologist’s duty, the recent centralization of these laboratories has caused a loss of expertise and increased both complexity and safety issues. Some countries have implemented clinical-pathological correlation programs aimed at facilitating communication between clinicians and dermatopathologists. However, Italy has regulatory and cultural barriers that make the implementation of these programs difficult. Therefore, an internal analysis was carried out to assess the efficacy and impact that skin biopsy procedures for inflammatory and neoplastic conditions have on the quality of care in our dermatology department. As the analysis evidenced a high number of descriptive pathologic reports and discordant diagnoses, a multidisciplinary group of four dermatologists, four general pathologists and one dermatopathologist was set up. Herein, we present the results of this analysis and project and describe the structure of the multidisciplinary group. We also discuss the pros and cons, possibilities and limitations of our project, including the regulatory barriers of the Italian National Health System

Efficacy of propranolol in cutaneous haemangiomas: it is more effective on premature children.

AIM: THE purpose of the present study is to evaluate the efficacy and safety of propranolol for problematic infantile haemangiomas (IH), showing our experience on 24 children, with special focus on prematures. METHODS: A retrospective observational study considered 24 patients who were given oral propranolol for the treatment of "problematic" IH. A multidisciplinary team, composed of a dermatologist, a pediatrician, a pediatric cardiologist, and a neonatologist, took part in the indication for propranolol and follow--up on all the patients. Propranolol was administered orally at the starting dose of 0.5--1 mg/kg/die and was gradually increased to the target dose of 2 mg/kg/die. A clinical gravity score, based on color, major diameter, thickness and texture was calculated for each IH, giving a numeric score before (t 0 ) and after (t f) propranolol therapy. Improvement rate was evaluated in terms of score percentage difference between t 0 and t f . RESULTS: All of the IH except one (96%), showed a variable grade of improvement, with a median score improvement of 69.1%. Median initial score in premature and term infants didn't show any significant difference (p = 0.38). Otherwise the two subgroups showed a significant difference in final scores: medium percentage improvement in premature and term infants, was respectively 80.9% and 49.6% (p < 0,01). No significant side effects were reported during the treatment period. CONCLUSION: As pointed out in our study, IH in premature children showed a significantly better response to propranolol treatment

Propranolol for the treatment of infantile hemangiomas: a nine-year monocentric experience from a tertiary hospital

Background: Propranolol is currently considered the first-line therapy for problematic infantile hemangiomas (IH), the most common benign vascular neoplasm of infancy. Objectives: We present a retrospective observational study aimed at assessing the efficacy of propranolol in 44 IH patients. Materials & methods: A nine-year retrospective review considering clinicodemographical and therapy-related variables was performed on medical records of infants treated for IH with oral propranolol. Each lesion was assessed through a numeric severity score based on size and colour both at baseline and after treatment conclusion (p <0.05 was considered statistically significant). Results: Complete remission was achieved in 90.7% cases of IH with a general mean improvement in severity of 94.94%. No severe adverse effects were reported. Preterm patients showed a superior response compared to term infants, even though the difference was not significant (p=0.185). Conclusion: Propranolol showed high efficacy in terms of safety profile and cosmetic results. Prematurity and precocious therapy could be linked to a superior response