widespread livedoid vasculopathy

A 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful ulcerative skin lesions was referred to our department because of a relapse of cutaneous manifestations of the skin lesions involving almost the whole body surface; malar erythema and oedema, non-scarring alopecia and fever were also associated. Routine laboratory data, immunological investigations and coagulation parameters were normal or negative. Histology was consistent with livedoid vasculopathy. A good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline. Livedoid vasculopathy is a rare, distinctive dermatosis that can be associated with systemic autoimmune disorders or present in an ''idiopathic'' form. The latter is at present regarded as a non-inflammatory thrombotic disease that may occur in patients with coagulation abnormalities. It is noteworthy that, in the present case, despite long-standing and dramatic cutaneous features, serious systemic complications have not developed and the patient's seroimmunologic and coagulative profile has remained normal. Key words: autoimmune diseases; coagulation abnormalities; livedoid vasculopathy

Anetodermic lupus panniculitis and antiphospholipid antibodies: Report of three cases

Anetoderma is a rare cutaneous disease characterized by a loss of normal elastic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, most notably lupus erythematosus and antiphospholipid antibodies with or without clinical manifestations of the antiphospholipid syndrome. A retrospective study was conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetodermaassociated lupus erythematosus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 patients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus panniculitis may be regarded as an uncommon variant of cutaneous lupus erythematosus mainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholipid syndrome. Antiphospholipid antibodies could play a role in the elastolytic process, leading to anetoderma. Key words: anetoderma; antiphospholipid antibodies; lupus erythematosus; panniculitis

Extracutaneous involvement of pyoderma gangrenosum

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Biosensing circulating MicroRNAs in autoinflammatory skin diseases: Focus on Hidradenitis suppurativa

MicroRNAs (miRNAs) play a crucial role in the early diagnosis of autoinflammatory diseases, with Hidradenitis Suppurativa (HS) being a notable example. HS, an autoinflammatory skin disease affecting the pilosebaceous unit, profoundly impacts patients’ quality of life. Its hidden nature, with insidious initial symptoms and patient reluctance to seek medical consultation, often leads to a diagnostic delay of up to 7 years. Recognizing the urgency for early diagnostic tools, recent research identified significant differences in circulating miRNA expression, including miR-24-1-5p, miR-146a-5p, miR26a-5p, miR-206, miR338-3p, and miR-338-5p, between HS patients and healthy controls. These miRNAs serve as potential biomarkers for earlier disease detection. Traditional molecular biology techniques, like reverse transcription quantitative-polymerase chain reaction (RT-qPCR), are employed for their detection using specific primers and probes. Alternatively, short peptides offer a versatile and effective means for capturing miRNAs, providing specificity, ease of synthesis, stability, and multiplexing potential. In this context, we present a computational simulation pipeline designed for crafting peptide sequences that can capture circulating miRNAs in the blood of patients with autoinflammatory skin diseases, including HS. This innovative approach aims to expedite early diagnosis and enhance therapeutic follow-up, addressing the critical need for timely intervention in HS and similar conditions

Asthma improvement in patients treated with dupilumab for severe atopic dermatitis

IntroductionAtopic dermatitis (AD) is considered a systemic type 2 immune driven disease, and it is associated to many atopic comorbidities including asthma. The aim of our study was to prospectively evaluate the respiratory outcomes in patients with persistent allergic asthma treated with dupilumab due to severe AD (sAD).MethodsWe enrolled eligible patients with sAD for dupilumab treatment from September 2018 to December 2020. We then selected the subgroup of patients sensitized to perennial allergens. Dupilumab's efficacy and safety on AD and comorbid asthma were assessed at baseline, one month, four months, and then every 4 months up to one year.ResultsA total of 437 patients with sAD were enrolled for dupilumab treatment due to sAD, and 273 reached 48 weeks of therapy. Respiratory outcomes were evaluated in the 85 asthmatic patients with positivity only to perennial allergens. Our patients showed statistically and clinically significant improvement in asthma control (Asthma Control Test and Asthma Control Questionnaire) and airway obstruction parameters (FEV1), in addition to the expected AD-related skin outcomes. Specifically, a significant improvement was achieved at the fourth month of dupilumab therapy, and this trend was maintained up to twelve months, regardless of asthma severity.ConclusionsOur results showed the overall improvement of the clinical picture that dupilumab offers for patients with severe AD and persistent allergic asthma of any severity, highlighting the importance of a global multidisciplinary approach of type 2 driven disease

Mechanisms of Inflammation in Neutrophil-Mediated Skin Diseases

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

A Multidisciplinary Approach to Patients with Psoriasis and a History of Malignancies or On-Treatment for Solid Tumors: A Narrative Literature Review

Psoriasis is a chronic immune-mediated disease that is linked to an increased risk of cancer. Although numerous studies have explored whether neoplasms are concurrent conditions or are induced by psoriasis, a definitive definition remains elusive. In this study, we conducted a comprehensive narrative literature review to offer practical guidance to oncologists and dermatologists regarding the initiation and discontinuation of biologics for psoriasis. The findings indicate that a customized approach is recommended for each patient, and that a history of malignancies does not constitute an absolute contraindication for biologics. Growing evidence supports the treatment of selected patients, emphasizing a nuanced assessment of benefits and risks. There is a lack of data specifying a safe timeframe to initiate biologics following a neoplasm diagnosis due to influences from cancer-related and patient-specific characteristics impacting prognosis. Some patients may continue anti-psoriasis therapy during cancer treatments. Enhanced comprehension of the biological mechanisms in cancer progression and the immune microenvironment of psoriasis holds promise for refining therapeutic strategies. In conclusion, a personalized treatment approach necessitates collaboration between oncologists and dermatologists, considering factors such as cancer prognosis, psoriasis clinical manifestations, patient characteristics, and preferences when making treatment decisions

Post-surgical Thyroid Bed Pyoderma Gangrenosum Mimicking Recurrent Papillary Thyroid Carcinoma

Background: Pyoderma gangrenosum (PG) is a rare inflammatory disease presenting with chronic-recurrent cutaneous ulcers histopathologically hallmarked by neutrophilic infiltrates, which may occur more frequently at sites of surgical traumas. The disease is habitually limited to the skin, but it can virtually involve any organ. Nevertheless, no prior cases of PG involving the thyroid bed have ever been reported.Case Report: A bilateral PG of the breast was diagnosed in a 51-year-old woman and treated with intravenous methylprednisolone pulse-therapy and cyclosporine, with partial improvement. During the hospitalization, cytological examination of two hypoechoic thyroid nodules by fine-needle aspiration (FNA) was consistent with thyroid carcinoma. After total thyroidectomy, histopathology confirmed a papillary thyroid cancer (PTC), and radioactive iodine ablation was performed. At 12-month ultrasonographic follow-up, two hypoechoic avascular areas localized in the empty thyroid bed raised the suspect of PTC recurrence. However, (i) undetectable levels of thyroglobulin without anti-thyroglobulin antibodies, (ii) neutrophilia and increased inflammatory marker levels, and (iii) cytological examination of FNA showing numerous neutrophils induced to suspect thyroid bed PG infiltration. An ex juvantibus approach with high-dose methylprednisolone led to dimensional reduction of the hypoechoic areas on ultrasonography, thus confirming the hypothesis of thyroid bed PG.Conclusion: This case of thyroid bed PG supports the idea that PG reflects a cutaneous phenotype encompassed in the spectrum of systemic neutrophilic diseases. Endocrinologists should be aware that thyroid bed PG involvement is an albeit rare differential diagnosis to consider in patients who had undergone thyroid surgery, especially with a history of PG