Publications by authors named "Emilio Berti"

125 Publications

An indolent nasal form of primary cutaneous CD8-positive peripheral T-cell lymphoma not otherwise specified successfully treated with local radiation therapy: A new subtype?

Dermatol Ther 2021 Feb 17:e14887. Epub 2021 Feb 17.

Section of Dermatology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Napoli, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/dth.14887DOI Listing
February 2021

Double expressor and double/triple hit status among primary cutaneous diffuse large B-cell lymphoma: a comparison between leg type and not otherwise specified subtypes.

Hum Pathol 2021 Feb 3;111:1-9. Epub 2021 Feb 3.

Anatomic Pathology, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, 2700, Italy.

Primary cutaneous diffuse large B-cell lymphomas (pcDLBCLs) are rare hematological neoplasms. The pcDLBCL category includes primary cutaneous large B-cell lymphoma leg type (pcDLBCL-LT), characterized by a particularly unfavorable outcome, and primary cutaneous large B-cell lymphoma not otherwise specified (pcDLBCL-NOS), a widely debated subentity with a more indolent course. The negative prognostic impact of double expressor status (DE status, given by coexpression of MYC and BCL2) and double hit/triple hit status (DH/TH status, given by translocations of MYC and BCL2 and/or BCL6) in nodal DLBCL is well known; however, no unanimous conclusions regarding relevance of DE and DH/TH status have been reached in pcDLBCL. Therefore, our purpose has been to investigate the presence and prognostic relevance of DE and DH/TH status among a retrospective multicentric cohort of 16 cases of pcDLBCL-LT and 17 cases of pcDLBCL-NOS. All cases were thoroughly reevaluated, both on a morphological and immunohistochemical level, and tested by means of fluorescence in situ hybridization for MYC, BCL2 and BCL6 rearrangements. DE status was observed in 69% of pcDLBCL-LT cases and in 24% of pcDLBCL-NOS cases; however, it did not impact prognosis in any of the groups examined. Combining molecular results, we highlighted a relevant fraction of DH pcDLBCL cases (three pcDLBCL-LT cases and one pcDLBCL-NOS case) and the very first case of TH pcDLBCL-LT reported to date. All DH cases were characterized by MYC and BCL6 rearrangements. Overall, DH/TH cases represented 15% (5/33) of all pcDLBCLs and were mostly pcDLBCL-LT. DH/TH status and DH status alone were associated with poorer overall survival and disease-specific survival (both p < 0.05) among all pcDLBCLs, without reaching statistical significance in the pcDLBCL-LT and pcDLBCL-NOS groups. In conclusion, MYC, BCL2, and BCL6 cytogenetical testing could be useful in identifying a putative subset of more aggressive pcDLBCLs, although this observation has to be confirmed by further studies.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.humpath.2021.01.006DOI Listing
February 2021

Necrobiotic xanthogranuloma in IgG-κ multiple myeloma.

Br J Haematol 2021 Feb 2. Epub 2021 Feb 2.

UOC Dermatologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/bjh.17300DOI Listing
February 2021

The clinical spectrum of COVID-19-associated cutaneous manifestations: an Italian multicentre study of 200 adult patients.

J Am Acad Dermatol 2021 Jan 18. Epub 2021 Jan 18.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.

Background: COVID-19 is associated with a wide range of skin manifestations.

Objective: To describe the clinical characteristics of COVID-19-associated skin manifestations, and explore the relationships between the six main cutaneous phenotypes and systemic findings.

Methods: Twenty-one Italian Dermatology Units were asked to collect the demographic, clinical and histopathological data of 200 patients with COVID-19-associated skin manifestations. The severity of COVID-19 was classified as asymptomatic, mild, moderate, or severe.

Results: A chilblain-like acral pattern significantly associated with a younger age (p<0.0001) and, after adjusting for age, significantly associated with less severe COVID-19 (p=0.0009). However, the median duration of chilblain-like lesions was significantly longer than that of the other cutaneous manifestations taken together (p <0.0001). Patients with moderate/severe COVID-19 were more represented than those with asymptomatic/mild COVID-19 among the patients with cutaneous manifestations other than chilblain-like lesions, but only the confluent erythematous/maculo-papular/morbilliform phenotype significantly associated with more severe COVID-19 (p=0.015), and this significance disappeared after adjusting for age.

Limitations: Laboratory confirmation of COVID-19 was not possible in all cases.

Conclusions: After adjusting for age, there was no clear-cut spectrum of COVID-19 severity in patients with COVID-19-related skin manifestations although chilblain-like acral lesions were more frequent in younger patients with asymptomatic/paucisymptomatic COVID-19.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2021.01.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7816892PMC
January 2021

Italian expert-based recommendations on the use of photo(chemo)therapy in the management of mycosis fungoides: Results of an e-Delphi consensus.

Photodermatol Photoimmunol Photomed 2021 Jan 18. Epub 2021 Jan 18.

Dip. Scienze della Salute, sezione Dermatologia, Universita' degli Studi di Firenze, Firenze, Italy.

Background: Phototherapy is a mainstay for the treatment of MF. However, there is scarce evidence for its use, mostly due to the lack of a unified schedule.

Aims: The primary aim of this study was to establish the first structured, expert-based consensus regarding the indications and technical schedules of NB-UVB and PUVA for MF. The secondary aim was to determine the consensus level for each specific item.

Materials & Methods: E-delphi study. Item-specific expert consensus was defined as the number of "Totally Agree" results to ≥80% of the panelists. Cronbach alpha index ≥0.7 was used as a measure of homogeneity in the responses among questions related to the same topic.

Results: Overall, there was a high homogeneity among responders (0.78). On specific topics, the highest grade was observed for technical items (0.8) followed by indications for early (0.73) and advanced stages (0.7).

Conclusions: Items related to the most canonical indications of phototherapy and to treatment schedules showed the highest agreements rates. There is consensus about the use of standardized treatment schedules for the induction and consolidation phases for NB-UVB and PUVA in MF.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/phpp.12658DOI Listing
January 2021

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis.

Front Med (Lausanne) 2020 20;7:604945. Epub 2020 Nov 20.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series. To systematically review current literature on treatments and clinical outcomes of PG and to establish recommendations on its therapeutic management. An a priori protocol was designed based on PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched for English-language articles detailing PG treatments and clinical outcomes, published between 1970 and March 2020. In total, 109 articles including 140 PG patients were analyzed. No randomized controlled trials or robust observational studies detailing PG treatment were found. Systemic corticosteroids ± topical corticosteroids and/or antihistamines were the most frequently prescribed treatment modality ( = 74/137; 54%). Complete remission was achieved by 114/136 (83.8%) patients. Sixty-four patients (45.7%) were given more than one treatment modality due to side effects or ineffectiveness. Leaving aside topical corticosteroids as monotherapy ± antihistamines in patients with mild disease, systemic corticosteroids ± topical corticosteroids and/or antihistamines led to complete remission in the highest proportion of patients (83%), while steroid-sparing treatments ± topical corticosteroids and/or antihistamines were associated with the lowest proportion of flares (55.5%). The review has been drafted based on a limited number of single case reports and small case series. Underreporting/underdiagnosis of patients with mild-to-moderate PG, partial/absent follow-up, absence of precise description of neonatal outcomes and lack of validated objective scores for measuring disease severity are other limitations of our study. Our systematic review was affected by publication bias. Systemic corticosteroids are the most frequently used treatment for PG. Whilst most patients achieve complete remission, many of them have refractory/persistent disease requiring multiple lines of therapy. Therefore, we provided an algorithm for PG treatment integrating the results of this systematic review with current knowledge available for bullous pemphigoid. High-quality studies will further help assess the effectiveness of different treatment options for PG.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fmed.2020.604945DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7717454PMC
November 2020

Triage process for the assessment of coronavirus disease 2019-positive patients with cancer: The ONCOVID prospective study.

Cancer 2020 Dec 3. Epub 2020 Dec 3.

Medical Oncology Unit, IRCCS Foundation Ca' Granda Maggiore Hospital Policlinic, Milan, Italy.

Background: Patients with cancer are considered at high risk for the novel respiratory illness coronavirus disease 2019 (COVID-19). General measures to keep COVID-19-free cancer divisions have been adopted worldwide. The objective of this study was to evaluate the efficacy of triage to identify COVID-19 among patients with cancer.

Methods: From March 20 to April 17, 2020, data were collected from patients who were treated or followed at the authors' institution in a prospective clinical trial. The primary endpoint was to estimate the cumulative incidence of COVID-19-positive patients who were identified using a triage process through the aid of medical and patient questionnaires. Based on a diagnostic algorithm, patients with suspect symptoms underwent an infectious disease specialist's evaluation and a COVID-19 swab. Serologic tests were proposed for patients who had symptoms or altered laboratory tests that did not fall into the diagnostic algorithm but were suspicious for COVID-19.

Results: Overall, 562 patients were enrolled. Six patients (1%) were diagnosed with COVID-19, of whom 4 (67%) had the disease detected through telehealth triage, and 2 patients (33%) without suspect symptoms at triage had the disease detected later. Seventy-one patients (13%) had suspect symptoms and/or altered laboratory tests that were not included in the diagnostic algorithm and, of these, 47 patients (73%) underwent testing for severe acute respiratory syndrome coronavirus 2 antibody: 6 (13%) were positive for IgG (n = 5) or for both IgM and IgG (n = 1), and antibody tests were negative in the remaining 41 patients.

Conclusions: The triage process had a positive effect on the detection of COVID-19 in patients with cancer. Telehealth triage was helpful in detecting suspect patients and to keep a COVID-19-free cancer center. The overall incidence of COVID-19 diagnosis (1%) and antibody positivity (13%) in patients with suspect symptoms was similar to that observed in the general population.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/cncr.33366DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7753308PMC
December 2020

Skin Manifestations Associated with COVID-19: Current Knowledge and Future Perspectives.

Dermatology 2021 24;237(1):1-12. Epub 2020 Nov 24.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy,

Background: Coronavirus disease-19 (COVID-19) is an ongoing global pandemic caused by the "severe acute respiratory syndrome coronavirus 2" (SARS-CoV-2), which was isolated for the first time in Wuhan (China) in December 2019. Common symptoms include fever, cough, fatigue, dyspnea and hypogeusia/hyposmia. Among extrapulmonary signs associated with COVID-19, dermatological manifestations have been increasingly reported in the last few months.

Summary: The polymorphic nature of COVID-19-associated cutaneous manifestations led our group to propose a classification, which distinguishes the following six main clinical patterns: (i) urticarial rash, (ii) confluent erythematous/maculopapular/morbilliform rash, (iii) papulovesicular exanthem, (iv) chilblain-like acral pattern, (v) livedo reticularis/racemosa-like pattern, (vi) purpuric "vasculitic" pattern. This review summarizes the current knowledge on COVID-19-associated cutaneous manifestations, focusing on clinical features and therapeutic management of each category and attempting to give an overview of the hypothesized pathophysiological mechanisms of these conditions.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1159/000512932DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7801998PMC
January 2021

MAP2K1-driven mixed Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease and Erdheim-Chester disease, clonally related to acute myeloid leukemia.

J Cutan Pathol 2020 Nov 14. Epub 2020 Nov 14.

Department of Dermatology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Mixed histiocytoses are a rare and recently recognized subset of histiocytic disorders that may involve the skin, characterized by the synchronous or metachronous development of lesions with Langerhans and/or non-Langerhans cell histiocytosis histopathological features. Around 10% of patients diagnosed with histiocytosis may develop a hematological malignancy, often with dramatic prognostic consequences. We hereby describe the exceptional case of a patient developing a MAP2K1-driven mixed histiocytosis with Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease, and Erdheim-Chester disease features and cutaneous involvement, progressing to a fatal and clonally-related acute myeloid leukemia. We reviewed the literature on similar cases and discussed the histopathological difficulties in their diagnosis and their clinical-pathological features.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/cup.13918DOI Listing
November 2020

Italian guidelines for the diagnosis and treatment of classic and iatrogenic Kaposi's sarcoma.

G Ital Dermatol Venereol 2020 Nov 12. Epub 2020 Nov 12.

U.O. Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milano, Italy -

Kaposi's sarcoma (KS) is a lymphangioproliferative disorder associated with human herpesvirus 8 (HHV8) infection. Four clinical subtypes are recognized: classic, endemic, epidemic (HIV-related) and iatrogenic. KS diagnosis is based on clinical features, histopathological assessment, and HHV8 serology. Classic KS is usually skin-limited and has a chronic course, while the iatrogenic variant may show mucosal, nodal or visceral involvement. Clinical staging is fundamental to guide the management. Localized disease may be treated with different local therapies, even if there are no randomized trials comparing these different modalities. Aggressive, disseminated KS and cases with visceral involvement usually require systemic chemotherapy, most commonly vinblastine, bleomycin or paclitaxel. Iatrogenic KS needs immunosuppression tapering/withdrawal and, if possible, switch to m-TOR inhibitors in post-transplant KS. The present work by a panel of Italian experts provides guidelines on KS diagnosis and management based on a critical review of the literature and a long and extensive personal experience.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.23736/S0392-0488.20.06703-6DOI Listing
November 2020

Phenotypical Markers, Molecular Mutations, and Immune Microenvironment as Targets for New Treatments in Patients with Mycosis Fungoides and/or Sézary Syndrome.

J Invest Dermatol 2021 Mar 5;141(3):484-495. Epub 2020 Nov 5.

Dermatologic Clinic, Department of Medical Sciences, University of Turin, Turin, Italy.

Primary cutaneous lymphomas encompass a wide spectrum of rare lymphoproliferative disorders originating in the skin, among which, mycosis fungoides (MF) is the most common subtype. The treatment of this disease is based on skin-directed therapies eventually in association with biologic response modifiers in the early phases, whereas in patients with the advanced stages, several therapeutic strategies can be used including mono and/or polychemotherapy and bone marrow transplantation. In recent years, the identification of specific markers (phenotypical, immunological, and molecular) has led to the development of several studies (including two randomized phase III trials). The results of these studies are modifying our therapeutic strategy toward a personalized treatment approach in which the clinical characteristics of the patients and tumor-node-metastasis-blood stage are considered together with the expression of specific markers (i.e., a CD30-positive expression for the use of brentuximab vedotin). This review will provide a comprehensive scenario of the main phenotypical, molecular, and immunological markers related to MF pathogenesis and disease evolution, which could represent the target for the development of innovative effective treatments in this disease.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jid.2020.07.026DOI Listing
March 2021

Cutaneous-group histiocytoses associated with myeloid malignancies: A systematic review of 102 cases.

Australas J Dermatol 2020 Oct 30. Epub 2020 Oct 30.

Department of Dermatology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Background: Histiocytoses are haematological disorders of bone marrow origin that share many biological and clinical features with haematological neoplasms. The association between histiocytoses of the cutaneous-group and myeloid malignancies is a poorly investigated topic of high biological and clinical impact.

Methods: We performed a systematic review of the scientific literature, compliant with PRISMA guidelines, to unravel the clinical and pathological features of this intriguing association.

Findings: We gathered and analysed 102 patients. Most were children with generalised cutaneous eruptions and displayed risk organ involvement (i.e. bone marrow, spleen, liver). Interestingly, all these features are uncommonly encountered in C-group histiocytosis not associated with haematological neoplasms.

Conclusions: Our review shows that generalised eruptions and risk organ involvement in cutaneous-group histiocytosis should raise a suspicion for a concomitant myeloid neoplasm both in children and in adults and warrant further investigations. A rapid recognition of this association is required to start a prompt and effective therapeutic management given the aggressive behaviour of the associated myeloid neoplasm in most instances.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/ajd.13491DOI Listing
October 2020

Treatment of Autoimmune Bullous Diseases During Pregnancy and Lactation: A Review Focusing on Pemphigus and Pemphigoid Gestationis.

Front Pharmacol 2020 2;11:583354. Epub 2020 Oct 2.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Pregnancy may induce the onset or exacerbation of autoimmune bullous diseases such as pemphigus or pemphigoid gestationis. A shift toward T helper (Th) 2 immune response and the influence of hormonal changes have been evoked as possible triggering factors. Therapeutic management of this setting of patients may represent a challenge, mainly due to safety concerns of some immunosuppressive drugs during pregnancy and lactation. In this narrative review, we provided a comprehensive overview of the therapeutic management of autoimmune bullous diseases in pregnant and breastfeeding women, focusing on pemphigus and pemphigoid gestationis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fphar.2020.583354DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7566587PMC
October 2020

Cutaneous tricholemmal carcinoma: a 15-years single centre experience.

G Ital Dermatol Venereol 2020 Oct 16. Epub 2020 Oct 16.

Unità Operativa di Anatomia Patologica, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy -

Background: Clear cell morphology has been described in several cutaneous neoplasms either as a specific feature of some entities either as a morphological variant in the spectrum, and these two entities are frequently considered together in the differential diagnosis.

Methods: We reviewed our series of cases occurred in our laboratory in order to further quantify the number of cases showing morphological features of tricholemmal differentiation and to investigate other clinical or histological difference. We retrieved 91 cases and, for each of them, all the clinical data regarding age, sex, clinical features, and clinical suspicious were collected, when available.

Results: The revision of the specimens concluded with a final diagnosis of tricholemmal carcinoma in 15 cases (17%), all the other cases were thus considered as squamous cell carcinoma with clear cell features. No statistically significant correlations were observed with the demografic or clinicopatholagical parameters such as age, sex or dimensions, but morphological revision highlighted a potentially greater "vertical" growth frequently not matched by a concomitant radial one in tricholemmal carcinoma than in squamous tumors.

Conclusions: The debate upon the diagnostic distinction of these tumours is still ongoing with authors proposing the tricholemmal carcinoma as a variant of a squamous cell carcinoma rather than a distinct entity. Further studies are needed to confirm our data and to evaluate the reproducibility of this feature.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.23736/S0392-0488.20.06672-9DOI Listing
October 2020

Transarterial embolization and surgical excision as treatment for angiolymphoid hyperplasia with eosinophilia.

Dermatol Ther 2020 11 5;33(6):e14138. Epub 2020 Sep 5.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/dth.14138DOI Listing
November 2020

Transitory Dissemination of Molluscum Contagiosum in a Patient Treated with Dupilumab for Atopic Dermatitis.

Acta Derm Venereol 2020 Jul 2;100(14):adv00207. Epub 2020 Jul 2.

Department of Dermatology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, IT-20100 Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2340/00015555-3565DOI Listing
July 2020

What is the role of a dermatologist in the battle against COVID-19? The experience from a hospital on the frontline in Milan.

Int J Dermatol 2020 Jul 7;59(7):e238-e239. Epub 2020 May 7.

UOC Dermatologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/ijd.14926DOI Listing
July 2020

Erdheim-Chester disease: A challenging diagnosis for an effective therapy.

Clin Neurol Neurosurg 2020 07 15;194:105841. Epub 2020 Apr 15.

Division of Human Anatomy and I Division of Neurology and Neurophysiopathology, University of Campania "Luigi Vanvitelli", Naples, Italy. Electronic address:

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clineuro.2020.105841DOI Listing
July 2020

Clinical Response and Quality of Life in Patients with Severe Atopic Dermatitis Treated with Dupilumab: A Single-Center Real-Life Experience.

J Clin Med 2020 Mar 13;9(3). Epub 2020 Mar 13.

UOC Dermatologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Dupilumab is an anti-interleukin-4 receptor monoclonal antibody that was recently approved for the treatment of atopic dermatitis (AD). In this single-center retrospective study, clinical baseline data of 117 severe AD patients treated with dupilumab were collected. At baseline and at weeks 4 and 16, disease severity was assessed through the Eczema Area and Severity Index (EASI) and quality of life through the Dermatology Life Quality Index (DLQI) questionnaire, Patient-Oriented Eczema Measure (POEM), Hospital Anxiety and Depression Scale (HADS), Peak Pruritus Numerical Rating Scale (NRS-itch), and VAS-sleep. Response to dupilumab was defined as an improvement of ≥75% in EASI from baseline (EASI75). At multivariate analysis, AD onset before 18 years [OR, 2.9; 95% CI, 1.2-7.2; = 0.0207] and absence of hypereosinophilia [OR, 2.24; 95% CI, 1.03-4.86; = 0.0412] were identified as significant predictive parameters for response to dupilumab in terms of EASI75 at week 4 but not at week 16. Significant reductions in EASI, DLQI, POEM, HADS, NRS-itch, and VAS-sleep were found between week 4 versus baseline ( < 0.0001 for all) and week 16 versus baseline ( < 0.0001 for all). Early AD onset and absence of hypereosinophilia may be suggested as predictive markers of early response to dupilumab. We confirmed the efficacy and safety of this agent along with the improvement of life quality in severe AD patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/jcm9030791DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141229PMC
March 2020

Hematoma-like primary cutaneous peripheral T-cell lymphoma: a rare clinical presentation.

G Ital Dermatol Venereol 2020 Feb 13. Epub 2020 Feb 13.

Department of Pathology, IRCCS San Raffaele Scientific Institute, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.23736/S0392-0488.20.06468-8DOI Listing
February 2020

Radiotherapy of primary cutaneous anaplastic large cell lymphoma: our experience in 30 cases.

Int J Dermatol 2020 Apr 9;59(4):469-473. Epub 2020 Jan 9.

UO Dermatologia, Università degli Studi di Milano, Milano, Italy.

Background: Since the observation of new cases of primary cutaneous anaplastic large cell lymphoma (PCALCL) with a seemingly aggressive clinical presentation and a favorable response to radiation therapy (RT), a review of our series has been performed to evaluate the results of RT.

Materials And Methods: The study is a retrospective analysis of 30 patients with PCALCL treated with conventional energy RT.

Results: About 55 fields of irradiation were performed. Complete clinical response (CCR) was obtained in 29 cases and a partial clinical response (PCR) in one. Two lesions had a marginal relapse at 7 and 37 months, respectively. In eight cases, new lesions appeared in the same body district and in six cases, in body sites at distance from the field of treatment. Total radiation dose ranged from 15 to 35 Gy (median 25 Gy). Follow-up range was 2-218 months (median 38.5 months) with a local control rate of 85% and a relapse-free rate of 49% at 5 years from RT. Three cases developed a nodal involvement.

Conclusions: PCALCL belongs to the group of primary cutaneous CD30 lymphoproliferative disorders, accounting for 25-30% of cutaneous T-cell lymphomas. RT is considered of choice as alternative to surgical excision of solitary lesions. Recent studies have focused on ideal dose to obtain local control indicating 30 Gy as adequate, but others have hypothesized that lower doses may suffice. Our study confirms the excellent role of RT in the local control of the disease with total doses ≤25 Gy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/ijd.14754DOI Listing
April 2020

Whole-genome analysis uncovers recurrent IKZF1 inactivation and aberrant cell adhesion in blastic plasmacytoid dendritic cell neoplasm.

Genes Chromosomes Cancer 2020 05 31;59(5):295-308. Epub 2019 Dec 31.

Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy with a poorly understood pathobiology and no effective therapeutic options. Despite a few recurrent genetic defects (eg, single nucleotide changes, indels, large chromosomal aberrations) have been identified in BPDCN, none are disease-specific, and more importantly, none explain its genesis or clinical behavior. In this study, we performed the first high resolution whole-genome analysis of BPDCN with a special focus on structural genomic alterations by using whole-genome sequencing and RNA sequencing. Our study, the first to characterize the landscape of genomic rearrangements and copy number alterations of BPDCN at nucleotide-level resolution, revealed that IKZF1, a gene encoding a transcription factor required for the differentiation of plasmacytoid dendritic cell precursors, is focally inactivated through recurrent structural alterations in this neoplasm. In concordance with the genomic data, transcriptome analysis revealed that conserved IKZF1 target genes display a loss-of-IKZF1 expression pattern. Furthermore, up-regulation of cellular processes responsible for cell-cell and cell-ECM interactions, which is a hallmark of IKZF1 deficiency, was prominent in BPDCN. Our findings suggest that IKZF1 inactivation plays a central role in the pathobiology of the disease, and consequently, therapeutic approaches directed at reestablishing the function of this gene might be beneficial for patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/gcc.22831DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7079160PMC
May 2020

Lichen planopilaris and dermatitis herpetiformis: a fortuitous association?

Eur J Dermatol 2019 Aug;29(4):426-428

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico,, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1684/ejd.2019.3570DOI Listing
August 2019

New Insights Into the Pathogenesis of Bullous Pemphigoid: 2019 Update.

Front Immunol 2019 2;10:1506. Epub 2019 Jul 2.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

There are several lines of evidence indicating that the physiopathological bases of bullous pemphigoid (BP), the most common subepidermal autoimmune bullous disease, are hallmarked by the production of autoantibodies directed against the hemidesmosomal anchoring proteins BP180 and BP230. In contrast to the robustness of the latter assumption, the multifaceted complexity of upstream and downstream mechanisms implied in the pathogenesis of BP remains an area of intense speculation. So far, an imbalance between T regulatory cells and autoreactive T helper (Th) cells has been regarded as the main pathogenic factor triggering the autoimmune response in BP patients. However, the contributory role of signaling pathways fostering the B cell stimulation, such as Toll-like receptor activation, as well as that of ancillary inflammatory mechanisms responsible for blister formation, such as Th17 axis stimulation and the activation of the coagulation cascade, are still a matter of debate. In the same way, the pathomechanisms implied in the loss of dermal-epidermal adhesion secondary to autoantibodies binding are not fully understood. Herein, we review in detail the current concepts and controversies on the complex pathogenesis of BP, shedding light on the most recent theories emerging from the literature.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fimmu.2019.01506DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6614376PMC
July 2020

Adhesive Tape as the Key to Solve a Case: A Quiz.

Acta Derm Venereol 2019 Oct;99(11):1063-1064

Department of Pathophysiology and Transplantation, Università degli Studi di Milano - Foundation IRCSS Cà Granda Ospedale Maggiore Policlinico, Via Pace 9, IT-20121 Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2340/00015555-3259DOI Listing
October 2019

Linear IgA bullous dermatosis in adults and children: a clinical and immunopathological study of 38 patients.

Orphanet J Rare Dis 2019 05 24;14(1):115. Epub 2019 May 24.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Pace, 9, 20122, Milan, Italy.

Background: Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepithelial vesiculobullous disease due to IgA autoantibodies directed against different antigens of the basement membrane zone (BMZ) of the skin and/or mucosae. It affects mainly preschool-aged children and adults, with only few studies on large series. The aim of this study was to assess possible differences between adults and children regarding clinical presentation, immunopathologic features, management and course of the disease.

Methods: A retrospective review of 38 LABD patients, followed-up from November 2006 to September 2018, was performed.

Results: Of 38 patients, 27 were adults and 11 children. Mean age at diagnosis was 5.4 years and 60.6 years in the pediatric and adult group, respectively. Considering both groups, limbs were the most commonly involved site (73.7%), followed by trunk (55.3%), head (36.8%) and buttocks (13.2%). Interestingly, head (p = 0.008), particularly perioral (p = 0.001), involvement, as well as "string of pearls" arrangement (p = 0.03), were more prevalent in children. Mucosal involvement was seen in 9 (23.7%) patients and was more frequent in children than adults (45.5% vs 14.8%, respectively, p = 0.09). Linear IgA deposits along the BMZ were observed in 30 patients (78.9%), while linear/granular IgA deposits in 8 patients (21.1%). Dapsone was the most commonly used drug (78.9%) and complete remission was achieved in most cases (81.6%).

Conclusions: Our epidemiological and clinicopathological findings relative to a large cohort of LABD patients are mostly consistent with the literature data. Interestingly, head, notably perioral, involvement and "string of pearls" arrangement occurred more frequently in the paediatric than adult group. The above clinical parameters may be regarded as diagnostic tools for LABD in children.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13023-019-1089-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6534856PMC
May 2019

Nonalcoholic fatty liver disease prevalence in an Italian cohort of patients with hidradenitis suppurativa: A multi-center retrospective analysis.

World J Hepatol 2019 Apr;11(4):391-401

Department of Women, Child and General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples 80138, Italy.

Background: Nonalcoholic fatty liver disease (NAFLD) includes two distinct conditions, with different histologic features and prognosis: non-alcoholic fatty liver (NAFL) and non-alcoholic steatohepatitis (NASH). Furthermore, NASH is the more aggressive necro-inflammatory form, which may accumulate fibrosis and result in End stage liver disease (ESLD). NAFLD is also linked to systemic inflammatory conditions such as psoriasis. NAFLD is currently the most common cause of ESLD in Western countries, becoming a serious public health concern. Hidradenitis suppurativa (HS) is a systemic inflammatory/autoinflammatory disease of the terminal follicular epithelium of the apocrine gland with a prevalence of 0.05% to 4.10%. Due to its systemic inflammatory behavior several comorbidities were recently associated, however liver ones were scarcely assessed.

Aim: To evaluate the prevalence and characteristics of NASH/NAFL in HS patients.

Methods: This retrospective study is a sub-analysis of a larger study carried out in 4 Italian dermatological centers. In this cohort, there were 83 patients: 51 patients with HS only, 20 patients with HS/NAFL and 12 with HS/NASH.

Results: Inflammatory comorbidities were present in 3.9% of HS only patients, 25% of HS/NAFL patients and 58.3% of HS/NASH patients ( 0.001). Similarly, mean Autoinflammatory Disease Damage Index (ADDI) was significantly higher among patients with HS/NASH (5.3 ± 2.2, 0.001) compared to patients with HS/NAFL or HS only (2.8 ± 1.6 and 2.6 ± 1.4 respectively). Furthermore, ADDI correlates with IHS4 in HS, HS/NAFL and HS/NASH. Diabetic patients have higher Hurley score than not diabetic ones. Ultrasound examination was significantly different in the three groups.

Conclusion: HS patients displayed a high prevalence of NASH/NAFLD and ultrasound examination should be particularly addressed to patients that display high ADDI scores.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4254/wjh.v11.i4.391DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6504856PMC
April 2019

Paradoxical Skin Reactions to Biologics in Patients With Rheumatologic Disorders.

Front Pharmacol 2019 26;10:282. Epub 2019 Mar 26.

UOC Dermatologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Targeted immune-modulating treatment with biological agents has revolutionized the management of immune-mediated inflammatory diseases, including rheumatologic conditions. The efficacy and tolerability of biological agents, from the initial tumour necrosis factor (TNF)-α inhibitors to the new anti-cytokine monoclonal antibodies, have dramatically changed the natural history of debilitating conditions such as rheumatoid arthritis and seronegative spondyloarthropathies. The widening use of biologics across several rheumatologic diseases has been associated with a new class of adverse events, the so-called paradoxical reactions. These events are inflammatory immune-mediated tissue reactions, developing paradoxically during treatment of rheumatologic conditions with targeted biologics that are commonly used for treating the idiopathic counterparts of these drug-induced reactions. The skin is frequently involved, and, even if considered rare to uncommon, these cutaneous manifestations are an important cause of biologic agent discontinuation. TNF-α antagonist-induced psoriasis, which can manifest or as exacerbation of a pre-existing form, is the prototypic and most frequent paradoxical skin reaction to biologics while other reactions, such as eczematous and lichenoid eruptions, hidradenitis suppurativa, pyoderma gangrenosum, Sweet's syndrome and granulomatous skin diseases, occur much more rarely. Management of these reactions consists of topical or systemic skin-directed therapies, depending on the severity and extension of the cutaneous picture, and it is generally associated with switching over to other disease-modifying regimens for treating the underlying rheumatologic condition. Here, we review in detail the current concepts and controversies on classification, pathogenesis and clinical management of this new class of cutaneous adverse events induced by biologics in rheumatologic patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fphar.2019.00282DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443901PMC
March 2019