Publications by authors named "Angelo V Marzano"

99 Publications

Anti-COVID-19 measurements for hidradenitis suppurativa patients.

Exp Dermatol 2021 06;30 Suppl 1:18-22

European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany.

The reported incidence of COVID-19 among cohorts of patients with inflammatory bowel and skin diseases under treatment with biologicals is low. Treatment may further modify disease severity as some biological modifiers, such as anakinra, are also proposed for the management of COVID-19 patients potentially providing HS patients with an advantage. The above preliminary evidence suggests that hidradenitis suppurativa (HS) does probably not provide an increased susceptibility for COVID-19 and that any susceptibility is unlikely to be modified negatively by treatment with biologicals. On the occasion of its 10th International Conference, experts of the European Hidradenitis Suppurativa Foundation e.V. have prepared a consensus statement regarding anti-COVID-19 measurements for HS patients. Based on the available knowledge, patients with HS may be vaccinated against SARS-CoV2 and patients affected by metabolic syndrome constitute a high-risk group for COVID-19 and should be vaccinated at the earliest convenient point in time. HS patients on treatment with adalimumab can be vaccinated with non-living virus anti-SARS-CoV2 vaccines. A possible suboptimal effect of the vaccine may be suspected but might not be expected universally. The management of the biological treatment in HS patients is at the discretion of the dermatologist / responsible physician.
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http://dx.doi.org/10.1111/exd.14339DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8207032PMC
June 2021

Target molecules for future hidradenitis suppurativa treatment.

Exp Dermatol 2021 Jun;30 Suppl 1:8-17

European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany.

The registration of the tumour necrosis factor-α inhibitor adalimumab in 2015 was a major step forward in the treatment of hidradenitis suppurativa/acne inversa (HS). However, it soon became evident that the effectiveness of adalimumab in daily practice was highly variable. A significant unmet medical need of HS patients remained, and the search for novel therapeutic targets was intensified. During the 10th European Hidradenitis Suppurativa Foundation (EHSF) e.V. Conference, reknown international HS investigators virtually presented and discussed the published data on these potential target molecules for future HS treatment. This article addresses the most promising molecules currently under investigation from a pathophysiological and clinical point of view. With phase III trials ongoing, the anti- interleukin (IL)-17 biologics bimekizumab and secukinumab are in the most advanced stage of clinical development showing promising results. In addition, targeting IL-1α with bermekimab has shown encouraging results in two clinical trials. Directing treatment at neutrophil recruitment and activation by targeting IL-36 with spesolimab fits well in the pathogenic concept of HS and clinical phase II trial results are pending. In contrast to in situ evidence, Complement 5a (C5a) and C5a receptor blockade have only shown greater clinical benefit in patients with severe HS. Inhibition of Janus kinase (JAK) 1 signalling in HS showed clinical efficacy only in the highest dosage, highlighting that careful surveillance of the balance between safety and efficacy of JAK inhibition is warranted. Overall, clinical efficacies of all novel treatments reported so far are modest. To guide drug development, more and better-defined translational data on the pathogenesis of this severe and enigmatic inflammatory skin disease are required.
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http://dx.doi.org/10.1111/exd.14338DOI Listing
June 2021

How to Manage COVID-19 Vaccination in Immune-Mediated Inflammatory Diseases: An Expert Opinion by IMIDs Study Group.

Front Immunol 2021;12:656362. Epub 2021 Apr 15.

Gastroenterology Unit, ASST Fatebenefratelli-Sacco, Department of Biomedical and Clinical Sciences (DIBIC) L. Sacco, Università degli Studi di Milano, Milan, Italy.

Since March 2020, the outbreak of Sars-CoV-2 pandemic has changed medical practice and daily routine around the world. Huge efforts from pharmacological industries have led to the development of COVID-19 vaccines. In particular two mRNA vaccines, namely the BNT162b2 (Pfizer-BioNTech) and the mRNA-1273 (Moderna), and a viral-vectored vaccine, i.e. ChAdOx1 nCoV-19 (AstraZeneca), have recently been approved in Europe. Clinical trials on these vaccines have been published on the general population showing a high efficacy with minor adverse events. However, specific data about the efficacy and safety of these vaccines in patients with immune-mediated inflammatory diseases (IMIDs) are still lacking. Moreover, the limited availability of these vaccines requires prioritizing some vulnerable categories of patients compared to others. In this position paper, we propose the point of view about the management of COVID-19 vaccination from Italian experts on IMIDs and the identification of high-risk groups according to the different diseases and their chronic therapy.
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http://dx.doi.org/10.3389/fimmu.2021.656362DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8082137PMC
May 2021

25-Hydroxyvitamin D serum levels inversely correlate to disease severity and serum C-reactive protein levels in patients with hidradenitis suppurativa.

J Dermatol 2021 May 24;48(5):715-717. Epub 2021 Feb 24.

Department of Biological and Environmental Sciences, College of Arts and Sciences, University of Qatar, Doha, Qatar.

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http://dx.doi.org/10.1111/1346-8138.15797DOI Listing
May 2021

The efficacy and tolerability of tetracyclines and clindamycin plus rifampicin for the treatment of hidradenitis suppurativa: Results of a prospective European cohort study.

J Am Acad Dermatol 2021 08 20;85(2):369-378. Epub 2021 Jan 20.

Erasmus MC, University Medical Center Rotterdam, Department of Dermatology, Rotterdam, the Netherlands. Electronic address:

Background: Tetracyclines and clindamycin plus rifampicin combination therapy are both considered first-line therapy in current hidradenitis suppurativa guidelines. However, evidence for their efficacy is drawn from small studies, often without validated outcomes.

Objective: To assess the 12-week efficacy of oral tetracyclines and a combination of clindamycin and rifampicin.

Methods: A prospective, international cohort study performed between October 2018 and August 2019.

Results: In total, 63.6% of the included 283 patients received oral tetracyclines, and 36.4% were treated with clindamycin and rifampicin. Both groups showed a significant decrease in International Hidradenitis Suppurativa Severity Score System from baseline (both P < .001). The Hidradenitis Suppurativa Clinical Response (HiSCR) was achieved in 40.1% and 48.2% of patients, respectively (P = .26). Patient characteristics or disease severity were not associated with the attainment of HiSCR or the minimal clinically important differences for the Dermatology Life Quality Index and pain.

Limitations: Cohort study. Respectively, 23.9% and 19.4% of patients had to be excluded from the HiSCR analysis for the tetracycline and combination therapy group because of a low abscess and nodule count at baseline.

Conclusion: This study shows significant efficacy of both tetracycline treatment and clindamycin and rifampicin combination therapy after 12 weeks in patients with hidradenitis suppurativa. No significant differences in efficacy were observed between the 2 treatments, regardless of disease severity.
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http://dx.doi.org/10.1016/j.jaad.2020.12.089DOI Listing
August 2021

Vascularization and fibrosis are important ultrasonographic tools for assessing response to adalimumab in hidradenitis suppurativa: Prospective study of 32 patients.

Dermatol Ther 2021 01 3;34(1):e14706. Epub 2021 Jan 3.

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

Hidradenitis suppurativa (HS) is a debilitating skin disease presenting with nodules, abscesses, and fistulae preferentially in the main folds. Adalimumab is the only licensed biologic for moderate-to-severe HS. Ultrasound demonstrated good sensitivity to provide anatomic and functional information in HS; in particular assessing vascularization, related to inflammation, and fibrosis in HS lesions before and after adalimumab treatment with ultrasound and Color Doppler may integrate clinical evaluation with imaging. Patients with moderate-to-severe HS were enrolled in this observational prospective study. Clinical evaluation (according to Hurley classification and International Hidradenitis Suppurativa Severity Score System score) and ultrasound (according to US HS-PGA)/Color Doppler were performed at baseline and after 12 weeks of adalimumab. Ultrasound was used for assessing fibrosis and Color Doppler for vascularization. For each patient, the three most severe lesions among abscesses and fistulae were chosen for total 96 lesions. Thirty-two patients were included, 18 men (56%) and 14 women (44%) with mean age 41.2. Mean IHS4 was 22.4 at baseline and dropped to 14.7 at week 12. Based on US HS-PGA, 14 out of 32 patients fell down by one or more classes of severity. Interestingly, adalimumab led to overall decrease in vascularization, particularly in lesions with intense vascular flow, which were 78 (81.3%) at baseline and became only 25 (26.04%). Finally, marked increase in fibrosis was seen after adalimumab, notably in lesions without fibrosis, which were 81 (84.4%) at baseline and became 15 (15.6%). This study confirms the efficacy of adalimumab in HS and provides value for vascularization and fibrosis as important ultrasonographic tools integrating clinical scores.
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http://dx.doi.org/10.1111/dth.14706DOI Listing
January 2021

Therapeutic management of chronic spontaneous urticaria in clinical practice: results from a pilot survey.

G Ital Dermatol Venereol 2020 Nov 23. Epub 2020 Nov 23.

Dermatology Private Practice, Bari and Barletta, Italy.

Background: The therapeutic approaches to patients with chronic spontaneous urticaria (CSU) differ among health care professionals and may be influenced by many factors.

Objectives: This cross-sectional survey was aimed at evaluating physicians' attitudes regarding therapeutic management of CSU on clinical practice.

Methods: A study-specific questionnaire was administered to a group of physicians (n=21) with a specialist interest in CSU from different areas of Italy (Group A) and also to other physicians (n=25) who manage CSU only occasionally in their clinical activity (Group B).

Results: In case of ineffectiveness of second-generation antihistamines at standard doses, higher doses of the same drug were always or frequently prescribed by most physicians in both groups, and 64% in group B and one third in group A usually increased the dose up to twice. Old-generation antihistamines were never used in clinical practice by 14% of survey participants in group A and 24% in group B, with the remaining physicians reporting rare or occasional uses. The prescription of systemic corticosteroids appeared to be more common among physicians in group B. The question concerning the use of alternative drugs in refractory CSU produced different answers between the two groups. Costs and access to specialist reference centers were indicated as the most important barriers to the use of medications different from antihistamines.

Conclusions: These preliminary results suggest that therapeutic approaches to CSU seem to be heterogeneous in clinical practice and could be at least in part conditioned by the different medical settings where physicians usually work.
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http://dx.doi.org/10.23736/S0392-0488.20.06761-9DOI Listing
November 2020

Chronic spontaneous urticaria in clinical practice: a pilot survey about attitudes and perceptions on assessment, diagnostic work-up and dietary management.

G Ital Dermatol Venereol 2020 Nov 23. Epub 2020 Nov 23.

Dermatology Private Practice, Bari and Barletta, Italy.

Background: Chronic spontaneous urticaria (CSU) is a heterogeneous condition whose management can be complex and challenging. The 0bjectives is to evaluate physicians' attitudes regarding practical aspects of CSU management, including adherence to international guidelines, criteria and instruments for CSU assessment, prescription of laboratory investigations and role of dietary measures.

Methods: A cross-sectional survey was conducted using a study-specific questionnaire. It was administered to a group of physicians with a specialist interest in CSU from different areas of Italy definable as "CSU experts" (Group A; n=21) and subsequently to other physicians who managed CSU only occasionally in their clinical activity (Group B; n=25).

Results: The EAACI/GA²LEN/EDF/WAO guidelines were considered very or moderately useful by the majority of participants. Significantly more physicians in group A reported that such guidelines were always followed in clinical practice (P=0.0008). Instruments for the assessment of CSU severity/activity and quality of life were used in clinical practice significantly more often by CSU experts as compared to group B. Dietary measures were frequently suggested for CSU patients by nearly three quarters of group B members and by only 5% of CSU experts (P<0.00001). When physicians were asked to indicate the type of laboratory examinations that were commonly performed in patients with longstanding and/or uncontrolled CSU, regardless of history, the investigations most frequently reported were full blood count and thyroid autoantibodies, followed by erythrosedimentation rate and/or C-reactive protein and thyroid function tests.

Conclusions: The results of the present pilot survey seem to suggest the heterogeneity of the approaches used for CSU management in clinical practice.
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http://dx.doi.org/10.23736/S0392-0488.20.06760-7DOI Listing
November 2020

Characterization of Hidradenitis Suppurativa Phenotypes: A Multidimensional Latent Class Analysis of the National Italian Registry IRHIS.

J Invest Dermatol 2021 May 21;141(5):1236-1242.e1. Epub 2020 Oct 21.

Study Center of the Italian Group for Epidemiologic Research in Dermatology (GISED), Bergamo, Italy; Department of Dermatology, San Bortolo Hospital, Vicenza, Italy. Electronic address:

In spite of the large heterogeneity, limited data exist on hidradenitis suppurativa (HS) phenotypes. To identify the HS phenotypes that best explain the disease heterogeneity, a cross-sectional study using latent class (LC) analysis was conducted on a cohort of patients examined at 17 dermatological centers participating in the Italian Registry of Hidradenitis Suppurativa and being enrolled between January 2015 and January 2020. Overall, 965 patients aged 32.0 ± 12.4 years (mean ± SD) were evaluated. A three-class model in LC analysis best fitted the data. Patients in LC1 (20.1%) were females, mostly obese, with a high probability of axillary‒groin (0.85) and mammary (0.59) lesions and the highest HS severity. Patients in LC2 (29.6%) were nonobese males, with moderate disease severity; with a high probability of gluteal (0.50) and genital (0.17) lesions, besides axillary‒groin involvement; and with acne and pilonidal cysts. Patients in LC3 (50%) were nonobese females with a milder disease mostly limited to axillary (0.52) and groin (0.66) areas. The stratification of patients with HS into a severe axillary‒mammary‒groin phenotype with predominantly anterior body involvement in females, an axillary‒gluteal‒groin phenotype of intermediate severity mainly affecting males in the posterior body areas, and an axillary‒groin phenotype with mildest clinical symptoms and limited skin involvement may help in optimizing HS management.
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http://dx.doi.org/10.1016/j.jid.2020.08.032DOI Listing
May 2021

What causes hidradenitis suppurativa ?-15 years after.

Exp Dermatol 2020 12;29(12):1154-1170

European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany.

The 14 authors of the first review article on hidradenitis suppurativa (HS) pathogenesis published 2008 in EXPERIMENTAL DERMATOLOGY cumulating from the 1st International Hidradenitis Suppurativa Research Symposium held March 30-April 2, 2006 in Dessau, Germany with 33 participants were prophetic when they wrote "Hopefully, this heralds a welcome new tradition: to get to the molecular heart of HS pathogenesis, which can only be achieved by a renaissance of solid basic HS research, as the key to developing more effective HS therapy." (Kurzen et al. What causes hidradenitis suppurativa? Exp Dermatol 2008;17:455). Fifteen years later, there is no doubt that the desired renaissance of solid basic HS research is progressing with rapid steps and that HS has developed deep roots among inflammatory diseases in Dermatology and beyond, recognized as "the only inflammatory skin disease than can be healed". This anniversary article of 43 research-performing authors from all around the globe in the official journal of the European Hidradenitis Suppurativa Foundation e.V. (EHSF e.V.) and the Hidradenitis Suppurativa Foundation, Inc (HSF USA) summarizes the evidence of the intense HS clinical and experimental research during the last 15 years in all aspects of the disease and provides information of the developments to come in the near future.
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http://dx.doi.org/10.1111/exd.14214DOI Listing
December 2020

Pyoderma gangrenosum.

Nat Rev Dis Primers 2020 10 8;6(1):81. Epub 2020 Oct 8.

Faculty of Epidemiology and Population Health, London School of Hygiene and Tropical Medicine, London, UK.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that presents with rapidly developing, painful skin ulcers hallmarked by undermined borders and peripheral erythema. Epidemiological studies indicate that the average age of PG onset is in the mid-40s, with an incidence of a few cases per million person-years. PG is often associated with a variety of other immune-mediated diseases, most commonly inflammatory bowel disease and rheumatoid arthritis. The cause of PG is not well understood, but PG is generally considered an autoinflammatory disorder. Studies have focused on the role of T cells, especially at the wound margin; these cells may support the destructive autoinflammatory response by the innate immune system. PG is difficult to diagnose as several differential diagnoses are possible; in addition to clinical examination, laboratory tests of biopsied wound tissue are required for an accurate diagnosis, and new validated diagnostic criteria will facilitate the process. Treatment of PG typically starts with fast-acting immunosuppressive drugs (corticosteroids and/or cyclosporine) to reduce inflammation followed by the addition of more slowly acting immunosuppressive drugs with superior adverse event profiles, including biologics (in particular, anti-tumour necrosis factor (TNF) agents). Appropriate wound care is also essential. Future research should focus on PG-specific outcome measures and PG quality-of-life studies.
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http://dx.doi.org/10.1038/s41572-020-0213-xDOI Listing
October 2020

A Systematic Review of Promising Therapeutic Targets in Hidradenitis Suppurativa: A Critical Evaluation of Mechanistic and Clinical Relevance.

J Invest Dermatol 2021 02 9;141(2):316-324.e2. Epub 2020 Sep 9.

Division of Dermatology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada; Division of Dermatology, Department of Medicine, Women's College Hospital, Toronto, Ontario, Canada.

This systematic review identifies and critically evaluates the mechanistic and clinical evidence of new promising therapeutic targets in hidradenitis suppurativa (HS). Evidence for these targets is largely based on observational data with limited ex vivo and translational data from clinical trials. A number of placebo-controlled studies have been completed or are underway utilizing IL-1, IL-23, IL-17, complement, and Jak inhibition, although there is concern regarding elevated placebo response rates and the questionable validity of clinical scores in some participant subsets. Knowledge gaps are identified suggesting a direction for future mechanistic studies in HS, including more comprehensive inflammatory endotype profiling of disease.
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http://dx.doi.org/10.1016/j.jid.2020.06.019DOI Listing
February 2021

Pyoderma gangrenosum: proposed pathogenesis and current use of biologics with an emphasis on complement C5a inhibitor IFX-1.

Expert Opin Investig Drugs 2020 Nov 29;29(11):1179-1185. Epub 2020 Sep 29.

Division of Dermatology, Department of Medicine, University of Toronto , Toronto, ON, Canada.

Introduction: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis with no FDA-approved treatment. The complement pathway has received renewed attention because it is elevated in inflammatory cutaneous conditions such as hidradenitis suppurativa (HS) and psoriasis. IFX-1 is a complement C5a inhibitor which inhibits neutrophil activation, chemotaxis, and reduces inflammatory signaling and complement driven tissue damage in various diseases.

Areas Covered: The article discusses a proposed pathogenesis of PG, early clinical investigations of IFX-1 for the treatment of HS and PG, its potential as a treatment for PG, and those other biologics currently under investigation.

Expert Opinion: Further studies should explore how patients with PG and other neutrophilic conditions may respond to complement inhibitors such as IFX-1. C5a blockade led to a reduction in inflammatory tunnels in HS, and alteration in neutrophil migration and activation supports the role of this pathway in the development of PG. The main challenges to the approval of IFX-1 are the identification of the optimal dose, duration, and stage-dependent factors in cutaneous inflammatory disorders. Further studies are required; however, complement inhibitors such as IFX-1 could find a place in clinical practice in years to come for severe, resistant PG that does not respond to conventional therapies.
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http://dx.doi.org/10.1080/13543784.2020.1819981DOI Listing
November 2020

PAPA spectrum disorders.

G Ital Dermatol Venereol 2020 Oct 2;155(5):542-550. Epub 2020 Jul 2.

Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy -

Pyogenic arthritis, pyoderma gangrenosum (PG) and acne (PAPA) syndrome is an autosomal dominant autoinflammatory syndrome due to mutations in proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) gene and presenting with cutaneous and articular manifestations. Other autoinflammatory syndromes caused by mutations in PSTPIP1 gene or characterized by clinical findings overlapping with those found in PAPA syndrome have been recently included in the group of PAPA spectrum disorders. These disorders are PASH (PG, acne and hidradenitis suppurativa [HS]), PAPASH (PASH associated with pyogenic sterile arthritis), PsAPASH (PASH combined with psoriatic arthritis [PsA], PASS (PG, acne, ankylosing spondylitis, with or without HS), PAC (PG, acne and ulcerative colitis [UC]) and PAMI syndrome (PSTPIP1-associated myeloid-related-proteinemia inflammatory syndrome). Except for PAPA and PAMI, no specific pathogenetic mutations have been identified in these syndromes. Dermatologists should be aware that PG, acne and HS may represent cutaneous signs hiding the presence of these rare entities. Systemic corticosteroids, a number of immunosuppressants and biologics, such as interleukin (IL)-1 antagonists and tumour necrosis factor (TNF) α inhibitors, are nowadays therapy for these diseases. A pathogenesis-driven treatment is the near future in the management of these conditions.
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http://dx.doi.org/10.23736/S0392-0488.20.06629-8DOI Listing
October 2020

Lymph node involvement in hidradenitis suppurativa: Ultrasound and color Doppler study of 85 patients.

Skin Res Technol 2020 Nov 25;26(6):960-962. Epub 2020 Jun 25.

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

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http://dx.doi.org/10.1111/srt.12896DOI Listing
November 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.

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

Varicella-like exanthem associated with COVID-19 in an 8-year-old girl: A diagnostic clue?

Pediatr Dermatol 2020 May 19;37(3):435-436. Epub 2020 May 19.

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

It has been reported that the novel coronavirus disease (COVID-19) may be associated with a papulovesicular skin eruption predominantly involving the trunk. We hereby present a case of COVID-19-associated varicella-like exanthem in an 8-year-old girl with mild systemic symptoms.
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http://dx.doi.org/10.1111/pde.14201DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7264614PMC
May 2020

Autoantibody Profile of a Cohort of 54 Italian Patients with Linear IgA Bullous Dermatosis: LAD-1 Denoted as a Major Auto-antigen of the Lamina Lucida Subtype.

Acta Derm Venereol 2020 02 29;100(4):adv00070. Epub 2020 Feb 29.

Section of Dermatology, Department of Health Sciences (Di.S.Sal.), University of Genoa, 16132 Genoa, Italy.

Linear IgA bullous dermatosis (LABD) is characterized by presence of multiple IgA autoantibodies, and a comparatively lesser number of IgG antibodies, directed against different hemidesmosomal antigens. The main autoantigens are LAD-1, LABD-97, BP180 and BP230, type VII collagen and laminin 332. We retrospectively studied the serology of 54 Italian patients with LABD using enzyme-linked immunosorbent assay (ELISA), immunoblotting assay, and indirect immunofluorescence on monkey oesophagus and salt-split skin. Among these, indirect immunofluorescence of salt-split skin elicits the greatest sensitivity. Sixty-three percent of the sera were observed to be positive, with a lamina lucida pattern observed in 48%, a sub-lamina densa pattern in 2% and a mixed pattern in 13% of the cases. IgA reactivity to LAD-1 on immunoblotting was found in 52% of sera, to BP180-NC16A by ELISA in 32% and to BP230 in 26%. Only 17% of patients possessed circulating IgG autoantibodies. LAD-1 was determined to be a major autoantigen of the lamina lucida subtype. Combined serological assays demonstrated a high sensitivity (82%), suggesting that this approach could support diagnosis when a biopsy is not feasible or direct immunofluorescence results are negative.
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http://dx.doi.org/10.2340/00015555-3415DOI Listing
February 2020

Ultrasonographic assessment of fibrosis in hidradenitis suppurativa fistulae helps in addressing treatment.

Skin Res Technol 2020 05 24;26(3):445-446. Epub 2019 Nov 24.

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

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http://dx.doi.org/10.1111/srt.12805DOI Listing
May 2020

Editorial: Neutrophil-Mediated Skin Diseases: Immunology and Genetics.

Front Immunol 2019 9;10:2377. Epub 2019 Oct 9.

Department of Physiopathology and Transplantation, Università Degli Studi di Milano, Milan, Italy.

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http://dx.doi.org/10.3389/fimmu.2019.02377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6794441PMC
October 2020

Coexistence of steatocystoma multiplex and hidradenitis suppurativa: Assessment of this unique association by means of ultrasonography and Color Doppler.

Skin Res Technol 2019 Nov 28;25(6):877-880. Epub 2019 Jul 28.

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

Background: Steatocystoma multiplex (SM) is an uncommon skin disease manifesting as multiple sebum-containing cysts arising in pilosebaceous unit-rich body areas. Hidradenitis suppurativa (HS) is a debilitating chronic inflammatory disease affecting the apocrine gland-bearing skin and presenting with both pseudocystic and inflammatory nodules, abscesses and fistulas. Considering that genetics has been reported to play a role in both entities, the albeit rare association between them suggests a shared genetic background. Although histology remains the gold standard for the diagnosis of SM, ultrasonography can be an useful diagnostic tool. This method is largely used in combination with Color Doppler for assessing disease severity in HS.

Materials And Methods: We report three cases of coexisting SM and HS and describe the ultrasonography and Color Doppler features of the two entities.

Results: SM lesions appeared on ultrasonography as hypoechoic nodules with well-defined hyperechoic borders and posterior acoustic enhancement, in the absence of Color Doppler signal. HS lesions had the ultrasonographic features of the fistulas, abscesses and pseudocystic nodules, some of which including hair fragments, with an intense Color Doppler signal within or around inflamed lesions.

Conclusion: The combination of ultrasonography and Color Doppler proved to be a reliable instrument for differentiating between SM and HS lesions, particularly distinguishing HS pseudocystic nodules from true cysts of SM.
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http://dx.doi.org/10.1111/srt.12751DOI Listing
November 2019

Infrared thermography and color Doppler: Two combined tools for assessing inflammation in hidradenitis suppurativa.

Skin Res Technol 2020 01 24;26(1):140-141. Epub 2019 Jul 24.

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

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http://dx.doi.org/10.1111/srt.12750DOI Listing
January 2020

Baseline D-dimer plasma levels correlate with disease activity but not with the response to omalizumab in chronic spontaneous urticaria.

Allergy 2019 12 26;74(12):2538. Epub 2019 Jun 26.

Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.

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http://dx.doi.org/10.1111/all.13936DOI Listing
December 2019

Mechanisms of Inflammation in Neutrophil-Mediated Skin Diseases.

Front Immunol 2019 8;10:1059. Epub 2019 May 8.

Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.

Neutrophil-mediated skin diseases, originally named neutrophilic dermatoses (NDs), are a group of conditions due to an altered neutrophil recruitment and activation, characterized by polymorphic cutaneous manifestations with possible internal organ involvement. Although a number of diseases are included in this setting, the two prototypic forms are pyoderma gangrenosum (PG) and Sweet's syndrome (SS) which usually present with skin ulcers and plaque-type lesions, respectively. They have central features significantly overlapping with autoinflammatory conditions which manifest as repeated episodes of tissue inflammation. However, in contrast to appropriate inflammatory responses to insults or to autoimmune disease, there is an absence of identifiable pathogens, autoantibodies, or autoreactive lymphocytes. The recognition of monogenic autoinflammatory diseases which can present with NDs has led to study several genes involved in autoinflammation in NDs. Based on discovering of a number of mutations involving different autoinflammatory genes, neutrophil-mediated skin diseases are nowadays regarded as a spectrum of polygenic autoinflammatory conditions. Although disease mechanisms have not yet been completely elucidated, NDs are recognized as diseases involving dysfunctional cellular signaling mediated by pathways mainly related to inflammasome and IL-1 with the contributory role of IL-17 and other effector molecules. The precise elucidation of the above-mentioned pathologic mechanisms may pave the way to tailored treatments for patients with different neutrophil-mediated skin diseases.
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http://dx.doi.org/10.3389/fimmu.2019.01059DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6519315PMC
June 2020

An Integrated Approach to Unravel Hidradenitis Suppurativa Etiopathogenesis.

Front Immunol 2019 25;10:892. Epub 2019 Apr 25.

Department of Advanced Diagnostics, Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy.

Hidradenitis suppurativa/acne inversa (HS) is a chronic inflammatory disease involving hair follicles that presents with painful nodules, abscesses, fistulae, and hypertrophic scars, typically occurring in apocrine gland bearing skin. Establishing a diagnosis of HS may take up to 7 years after disease onset. HS severely impairs the quality of life of patients and its high frequency causes significant costs for health care system. HS patients have an increased risk of developing associated diseases, such as inflammatory bowel diseases and spondyloarthropathies, thereby suggesting a common pathophysiological mechanism. Familial cases, which are around 35% of HS patients, have allowed the identification of susceptibility genes. HS is perceived as a complex disease where environmental factors trigger chronic inflammation in the skin of genetically predisposed individuals. Despite the efforts made to understand HS etiopathogenesis, the exact mechanisms at the basis of the disease need to be still unraveled. In this review, we considered all OMICs studies performed on HS and observed that OMICs contribution in the context of HS appeared as not clear enough and/or rich of useful clinical information. Indeed, most studies focused only on one aspect-genome, transcriptome, or proteome-of the disease, enrolling small numbers of patients. This is quite limiting for the genetic studies, from different geographical areas and looking at a few aspects of HS pathogenesis without any integration of the findings obtained or a comparison among different studies. A strong need for an integrated approach using OMICs tools is required to discover novel actors involved in HS etiopathogenesis. Moreover, we suggest the constitution of consortia to enroll a higher number of patients to be analyzed following common and consensus OMICs strategies. Comparison and integration with the findings present in the OMICs repositories are mandatory. In a theoretic pipeline, the Skin-OMICs profile obtained from each HS patient should be compared and integrated with repositories and literature data by using appropriate InterOMICs approach. The final goal is not only to improve the knowledge of HS etiopathogenesis but also to provide novel tools to the clinicians with the eventual aim of offering a tailored treatment for HS patients.
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http://dx.doi.org/10.3389/fimmu.2019.00892DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6494959PMC
July 2020

Photobiomodulation as potential novel third line tool for non-invasive treatment of hidradenitis suppurativa.

G Ital Dermatol Venereol 2020 Feb 23;155(1):88-98. Epub 2019 Apr 23.

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

Hidradenitis Suppurativa (HS) is a severe inflammatory pathology of the skin characterized by chronic recurrent inflamed lesions, nodules, sinus tracts and abscesses usually manifests after puberty, which involves scalp, neck, axillae, perineum and infra-mammary areas. Nowadays treatment options range from short or long courses of antibiotics, anti-inflammatory and biologic drugs, to surgery. Other suggested treatments consider the employment of laser devices, mainly microsurgical lasers (such as CO2 and intense pulsed lasers) and photodynamic therapy. This review explores the potential use of photobiomodulation (PBM), already used for the treatment of other skin conditions, such as acne, hypertrophic scars, wrinkles, and burns, as potential novel therapy for HS. PBM has been reported to have beneficial effects on promoting wound healing, angiogenesis, vasodilation, and relieving from pain and inflammation, as recently demonstrated in an in-vitro model mimicking HS disease. In addition, PBM, specifically set at the blue wavelength, has been recently reported as exerting an anti-bacterial activity. Therefore, considering all these PBM features especially its ability to decrease pain and inflammation and to lead to faster wound healing, thus improving patients' quality of life, we hypothesize its employment as adjuvant third line treatment for the management of HS both in young and adult patients.
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http://dx.doi.org/10.23736/S0392-0488.19.06247-3DOI Listing
February 2020

Extracutaneous involvement of pyoderma gangrenosum.

Arch Dermatol Res 2019 Aug 29;311(6):425-434. Epub 2019 Mar 29.

Department of Dermatology and OHSU Wound and Hyperbaric Medicine, Oregon Health and Science University, 3303 SW Bond Ave Center for Health and Healing Building 1, Suite 16, Portland, OR, 97239, USA.

Pyoderma Gangrenosum (PG) is an inflammatory neutrophilic dermatosis (ND) associated with underlying chronic inflammation and/or malignancy. Diagnosis remains to be challenging as a gold standard diagnostic test is lacking. Initial manifestations may include papules, vesicles, or pustules that subsequently develop into ulceration with features of undermining and violaceous borders. Timely recognition of pyoderma gangrenosum is impeded by clinical findings shared with other etiologies, such as granulomatosis with polyangiitis, polyarteritis nodosa, and antiphospholipid syndrome. As with any other ND, extracutaneous involvement may also occur preceding, during, or following the appearance of skin lesions. Sterile neutrophilic infiltrates have been found to affect internal organs supporting the concept of PG being a systemic disease, with lung being the most common extracutaneous manifestation followed by ocular and visceral compromise. Therefore, in this review, we describe the current knowledge of extracutaneous involvement of PG and its respective clinical manifestations to aid dermatologists in diagnosis, management, and determining prognosis.
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http://dx.doi.org/10.1007/s00403-019-01912-1DOI Listing
August 2019

Total IgE and atopic status in patients with severe chronic spontaneous urticaria unresponsive to omalizumab treatment.

Allergy 2019 08 11;74(8):1561-1563. Epub 2019 Mar 11.

Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.

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http://dx.doi.org/10.1111/all.13754DOI Listing
August 2019

Hidradenitis suppurativa: the "bright side" of autoinflammation and hidden diseases.

G Ital Dermatol Venereol 2018 Jun;153(3 Suppl 2):1-2

Department of Dermatology, ASST Spedali Civili di Brescia, University of Brescia, Brescia, Italy.

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http://dx.doi.org/10.23736/S0392-0488.18.06031-5DOI Listing
June 2018

Autoimmune bullous diseases during pregnancy: insight into pathogenetic mechanisms and clinical features.

G Ital Dermatol Venereol 2019 Jun 29;154(3):256-262. Epub 2018 Oct 29.

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

Pemphigoid gestationis (PG), also known as herpes gestationis, is the prototypic pregnancy-associated autoimmune bullous disease (AIBD), but also the other AIBDs, notably pemphigus vulgaris, may begin or exacerbate during pregnancy. Although the increase in concentration of T and B regulatory cells makes pregnancy a state of increased immunologic tolerance toward the semiallogeneic fetal antigens, a prevalent T helper (Th) 2 profile, that is reported to be associated with pregnancy, may cause exacerbation of pemphigus and AIBDs in general during this period. Active disease may lead to stillbirth, spontaneous abortion, preterm pregnancy, low birthweight, and neonatal pemphigus. PG is a rare AIBD usually starting during the third trimester of pregnancy and healing in the postpartum. It is due to the formation of autoantibodies directed against different epitopes of bullous pemphigoid (BP) 180 as a consequence of the aberrant expression of BP180 in the placental tissue of genetically predisposed women. PG is characterized by vesicles with herpetiform distribution, blisters and urticarial elements typically involving the periumbilical area and the distal portion of the upper limbs. Diagnosis is based on: 1) physical examination; 2) histopathological pattern consisting of a dermal inflammatory infiltrate rich in eosinophils; 3) direct immunofluorescence test demonstrating linear deposits of complement fraction 3 and immunoglobulin G along the basement membrane zone; 4) detection of circulating autoantibodies by means of indirect immunofluorescence or enzyme linked immunosorbent assay. Here, we provide an updated overview on the pathophysiologic mechanisms of pregnancy-associated or pregnancy-exacerbated AIBDs, focusing also on peculiar clinical features of these disorders.
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http://dx.doi.org/10.23736/S0392-0488.18.06153-9DOI Listing
June 2019
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