Takayasu arteritis with pyoderma gangrenosum: case reports and literature review

Abstract Background Takayasu arteritis is a rare, chronic inflammatory arteriopathy affecting mainly the aorta and its branches. Many skin manifestations have been reported in association with this disease. Pyoderma gangrenosum is a skin complication that is a neutrophilic dermatosis characterized by destructive, necrotizing and noninfective skin infiltration. However, there are no related records on these conditions in Chinese patients. Case presentation We reported two Chinese female patients presenting with pyoderma gangrenosum associated with Takayasu arteritis. Pyoderma gangrenosum preceded Takayasu arteritis in both patients. Their skin lesions were diagnosed as pyoderma gangrenosum through skin biopsy and relieved after treating with steroids and immunosuppressants. During the follow-up, both patients developed symptoms caused by vascular stenosis and occlusion, such as dizziness and weakness of upper limb. The results of aortic angiography revealed multiple large arteries narrowed and blocked. According to the criteria of the American College of Rheumatology, the vasculitis in both patients were classified as Takayasu arteritis. Since there was scant evidence of active inflammation and the skin lesions were stable, neither of them was given strong immnosuppressive therapy. The PubMed database was also searched and 16 related well-documented cases of Takayasu with pyoderma gangrenosum were reviewed and summarized. Conclusions Pyoderma gangrenosum could occur at any stage of the Takayasu arteritis disease process. No correlation was found between the location of the skin lesions and the clinical severity and scope of Takayasu arteritis. It is important to remember the rare possibility of Takayasu arteritis in patients with skin lesions indicative of pyoderma gangrenosum of unknown aetiology. Obtaining the relevant history and regular monitoring of the arteries are necessary.

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SWEET SYNDROME AND HASHIMOTO THYROIDITIS: A CASE REPORT AND REVIEW OF THE LITERATURE

AACE Clinical Case Reports ◽

10.4158/accr-2019-0579 ◽

2020 ◽

Vol 6 (4) ◽

pp. e179-e182

Author(s):

Jacob Goodwin ◽

Samuel Ives ◽

Hiba Hashmi

Keyword(s):

Thyroid Dysfunction ◽

Case Reports ◽

Hashimoto Thyroiditis ◽

Skin Lesions ◽

Thyroid Stimulating Hormone ◽

Disease Stage ◽

Thyroid Peroxidase ◽

Thyroid Function Tests ◽

Sweet Syndrome ◽

Pubmed Database

Objective: Sweet syndrome (SS) is characterized by an inflammatory rash that has been associated with a number of drugs and malignant, inflammatory, and infectious conditions. Rare accounts of Hashimoto thyroiditis (HT) presenting with SS exist in the literature. HT is usually identified after the onset of skin lesions and without signs of overt thyroid dysfunction, and the stage of thyroid disease stage at presentation is variable. Methods: A search of the PubMed database was performed using search criteria involving combinations of “Sweet syndrome” and “Hashimoto thyroiditis,” “autoimmune thyroiditis,” or “thyroiditis,” and the search was filtered for clinical case reports. Five case reports were identified to describe the coexistence of Sweet syndrome and Hashimoto thyroiditis, and full-text versions of these reports were obtained and reviewed. Of note, cases involving subacute or other types of thyroiditis were excluded. Results: A 57-year-old man presented with painful eruptions on his hands; he was initially treated with antibiotics for presumed cellulitis without relief. Skin biopsy later confirmed SS and subsequent workup identified underlying HT with an elevated thyroid-stimulating hormone of 19.24 mU/L (normal, 0.30 to 4.30 mU/L) and positive thyroid peroxidase (TPO) antibody at 236.4 IU/mL. Conclusion: Thyroid function tests should be universally evaluated in the workup of SS, and it may be appropriate to test for TPO antibodies even in the absence of objective thyroid dysfunction. Both SS and HT show immune diathesis, so further work should be undertaken to establish whether a common immunologic trigger exists.

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Pyoderma gangrenosum – an interdisciplinary approach to the disease

HIGHER SCHOOL’S PULSE ◽

10.5604/01.3001.0012.7978 ◽

2018 ◽

Vol 12 (4) ◽

pp. 61-64

Author(s):

Aleksandra Kapuśniak ◽

Aleksandra Czachor ◽

Grażyna Wąsik

Keyword(s):

Pyoderma Gangrenosum ◽

Rapid Development ◽

Calcineurin Inhibitors ◽

Skin Lesions ◽

Neutrophilic Dermatosis ◽

Unknown Etiology ◽

Neutrophilic Infiltration ◽

Inflammatory Infiltration ◽

Laboratory Findings ◽

Immunological Mechanisms

Pyoderma gangrenosum is a rare dermatosis of unknown etiology. It is classified as being a neutrophilic dermatosis, in which there is inflammatory infiltration consisting primarily of mature polynuclear leukocytes. Its pathogenesis is multifactorial and is thought to involve neutrophilic dysfunction, inflammatory mediators in combination with a genetic predisposition for the disease. Neutrophilic infiltration is observed in new lesions, while necrosis associated with fibrosis and granulomas are seen in chronic lesions, however these findings are not pathognomonic. Pyoderma gangrenosum can occur at any age. However, it most commonly develops in young and middle-aged adults predominantly women between the second and fifth decades of life. Grossly, pyoderma gangrenosum is characterized by skin lesions in the form of rapidly spreading ulcers, with cylindrical edges and necrotic bottoms. These ulcers are painful and crusted but have undermined borders. Pyoderma gangrenosum commonly presents with the rapid development of one or more purulent ulcers with undermined borders on sites of normal or traumatized skin. Pyoderma gangrenosum is often associated with other systemic diseases such as ulcerative colitis, Crohn’s disease, monoclonal gammopathies, IgG or IgA myelomas and tumors of internal organs and hematopoietic system diseases, which supports the immunological mechanisms involved in the pathogenesis of the disease. Of note, neutrophilic infiltration associated with other extracutaneous manifestations and different systemic disorders can co-exist with pyoderma gangrenosum. Despite the recent development of immune modulating drugs in the treatment of skin conditions, steroid therapy still plays a pivotal role. For patients with mild pyoderma gangrenosum, the local application of topical corticosteroids or calcineurin inhibitors can be sufficient. Systemic therapy is necessary in patients with more extensive disease. The role of surgery is controversial, as it is associated with the induction of pathergy. The clinical, histopathologic and laboratory findings in pyoderma gangrenosum are non-specific, and a diagnosis can only be made once other diagnoses have been excluded.

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Pyoderma Gangrenosum with Positive Antinuclear Antibody, in the Absence of Systemic Association

Nepal Journal of Dermatology Venereology & Leprology ◽

10.3126/njdvl.v16i1.19418 ◽

2018 ◽

Vol 16 (1) ◽

pp. 66-69

Author(s):

Smriti Shrestha ◽

Alisha Aryal

Keyword(s):

Pyoderma Gangrenosum ◽

Lupus Erythematosus ◽

Antinuclear Antibody ◽

Case Reports ◽

Neutrophilic Dermatosis ◽

Systemic Diseases ◽

Connective Tissue Disorders ◽

Systemic Lupus ◽

Unique Case ◽

Positive Antinuclear Antibody

Pyoderma gangrenosum is an uncommon neutrophilic dermatosis, seen on legs, and infrequently on hands and other anatomical sites. It is associated with systemic diseases in 50-70% of the cases. Antinuclear antibody (ANA) seropositivity has been reported in pyoderma gangrenosum associated with connective tissue disorders. However, there are very few case reports of pyoderma gangrenosum in patients of systemic lupus erythematosus, while we did not find any reports of ANA seropositivity in isolated pyoderma gangrenosum. Hence, we report this unique case of pyoderma gangrenosum with classical clinicohistopathology, positive ANA but no systemic association. As anticipated, our patient responded promptly to steroids.

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Clinical and Histopathological Features and Potential Pathological Mechanisms of Skin Lesions in COVID-19: Review of the Literature

Dermatopathology ◽

10.3390/dermatopathology7010002 ◽

2020 ◽

Vol 7 (1) ◽

pp. 3-16 ◽

Cited By ~ 9

Author(s):

Gürkan Kaya ◽

Aysin Kaya ◽

Jean-Hilaire Saurat

Keyword(s):

Clinical Symptoms ◽

Case Reports ◽

Clinical Manifestations ◽

Skin Lesions ◽

Vascular Lesions ◽

Cutaneous Lesions ◽

Cutaneous Manifestations ◽

Histopathological Features ◽

Pubmed Database ◽

The Mean

In recent weeks, several reports have emerged of skin lesions with different clinical presentations in COVID-19 cases. All dermatologists should be aware of these cutaneous lesions, which may be early clinical symptoms of infection. We reviewed the literature on cutaneous manifestations in the PubMed database from December 2019 and June 2020. From the cases described as case reports or series in 57 recent articles, it appears that skin lesions (i) are highly varied, (ii) may not be related to the severity of the condition and (iii) resolve spontaneously in a few days. The frequency of these lesions in COVID-19 patients varies between 1.8% and 20.4%. The major clinical forms described were maculopapular eruptions, acral areas of erythema with vesicles or pustules (pseudochilblain), urticarial lesions, other vesicular eruptions and livedo or necrosis. The lesions were mainly localized in the trunk and extremities. The majority of patients were male, aged between 4.5 and 89 years. A minority of the patients were children presenting with acral, chilblain-like lesions, papulo-vesicular eruptions or Kawasaki disease-like pediatric inflammatory multisystem syndrome. The mean duration of the lesions was a few days, but some lasting as little as 20 min and others as long as four weeks have been reported. The mean latency time in the majority of cases was between 1 and 14 days; however, in some patients, lesions appeared 2 to 5 days before the onset of COVID-19 symptoms. The histopathological features of these lesions also vary, corresponding to the diversity of clinical manifestations. These features underline the nature of epidermal and dermal vascular lesions—and in severe cases, microvascular injury and thrombosis—associated with COVID-19, and provide important clues to their pathological mechanisms.

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Recent advances in managing and understanding pyoderma gangrenosum

F1000Research ◽

10.12688/f1000research.19909.1 ◽

2019 ◽

Vol 8 ◽

pp. 2092 ◽

Cited By ~ 7

Author(s):

Josh Fletcher ◽

Raed Alhusayen ◽

Afsaneh Alavi

Keyword(s):

Pyoderma Gangrenosum ◽

Randomized Clinical Trials ◽

Traditional Approach ◽

Wound Care ◽

Case Reports ◽

Case Series ◽

Adaptive Immune System ◽

Neutrophilic Dermatosis ◽

Recent Advances ◽

No Gold Standard

Pyoderma Gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. PG has historically been a challenging disorder to diagnose, leading to the development of new diagnostic criteria rather than the traditional approach of a diagnosis of exclusion. The pathophysiology is thought to involve both innate and adaptive immune system dysregulation, neutrophilic abnormalities, environmental, and genetic factors. As of today, no gold standard therapy exists for the treatment of PG, and the literature is restricted to mainly case reports, case series, and 2 small randomized clinical trials. Topical, systemic, and biologic therapy, as well as adequate analgesia and proper wound care all play a role in the management of PG. Recent studies have identified additional cytokines and signalling cascades thought to be involved in the pathogenesis of PG, ultimately leading to the development of new targeted therapies. This review will focus on recent advances in the pathophysiology, clinical presentation and associated comorbidities, diagnosis, and management of PG.

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Pyoderma Gangrenosum Induced by BNT162b2 COVID-19 Vaccine in a Healthy Adult

Vaccines ◽

10.3390/vaccines10010087 ◽

2022 ◽

Vol 10 (1) ◽

pp. 87

Author(s):

Mazin Barry ◽

Abdulaziz AlRajhi ◽

Khaldoon Aljerian

Keyword(s):

Pyoderma Gangrenosum ◽

Messenger Rna ◽

Skin Lesions ◽

Neutrophilic Dermatosis ◽

Unknown Etiology ◽

Rapid Improvement ◽

Inflammatory Infiltration ◽

Cutaneous Manifestations ◽

Wide Scale ◽

Monitoring And Surveillance

(1) Background: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis of unknown etiology. Coronavirus disease 2019 (COVID-19) vaccines can cause a variety of adverse cutaneous manifestations. PG associated with mRNA vaccines has not previously been described. This case study reports on the first patient to develop PG after receiving BNT162b2. (2) Case Presentation: An otherwise-healthy 27-year-old man developed multiple skin lesions 24 h after receiving the first dose of the messenger RNA-based Pfizer/BioNTech BNT162b2 COVID-19 vaccine. When in hospital, he developed a new painful ulcerative lesion on his right hand. Skin ulcer edge biopsy showed severe epidermal neutrophilic infiltrate with epidermal and dermal edema, underlying superficial dermal necrosis, and characteristic undermining with extensive mixed inflammatory infiltration of the dermis and abscess formation consistent with an ulcer with mixed dermal inflammation compatible with pyoderma gangrenosum. The lesion showed rapid improvement after the initiation of immunosuppressive therapy. (3) Conclusions: PG may be a rare adverse event related to the BNT162b2 vaccine, which could be more frequently encountered with the wide-scale use of mRNA vaccines. The continuous monitoring and surveillance of skin manifestations post-vaccination is essential.

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Manifestações Cutâneas Associadas à COVID-19: Uma Revisão Narrativa

Acta Médica Portuguesa ◽

10.20344/amp.14574 ◽

2020 ◽

Vol 33 (13) ◽

Author(s):

Maria Relvas ◽

Joana Calvão ◽

Raquel Oliveira ◽

José Carlos Cardoso ◽

Margarida Gonçalo

Keyword(s):

Case Reports ◽

Clinical Manifestations ◽

Case Series ◽

Skin Lesions ◽

Cutaneous Manifestations ◽

Pubmed Database ◽

The World ◽

Confirmatory Tests ◽

Substantial Heterogeneity ◽

Pathophysiologic Mechanisms

Introduction: COVID-19 is an infectious disease caused by the new coronavirus, SARS-CoV-2, that has spread rapidly throughout the world. This has resulted in an urgent need to obtain information regarding its pathogenesis, diagnosis and clinical manifestations. More specifically, skin manifestations, seldom reported initially, have been increasingly described.Material and Methods: We performed a literature search in the PubMed database, regarding cutaneous manifestations associated with COVID-19. This article describes the clinical and histological findings of the main skin lesions observed in the context of SARS-CoV-2 infection.Discussion: Cutaneous manifestations associated with COVID-19 have been described in multiple retrospective and prospective studies, case series and case reports. The reported incidence reached 20.4%. Although there was substantial heterogeneity in terms of clinical patterns, the main ones include: erythematous/maculopapular, urticarial, papulovesicular, and purpuric/petechial eruptions, chilblain-like lesions and livedoid/acro-ischemic lesions. In the vast majority, the underlying pathophysiologic mechanisms are not fully understood, although histopathological findings and biomolecular studies can add relevant data.Conclusion: The recognition of cutaneous manifestations associated with COVID-19 is of utmost importance. They may help establishing an early diagnosis, namely in oligosymptomatic patients or when confirmatory tests are impossible to perform. Moreover, chilblain-like lesions and acro-ischemia, also seem to play an important role in terms of prognosis.

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Novel Therapeutic Approaches and Targets for Treatment of Chronic Urticaria: New Insights and Promising Targets for a Challenging Disease

Current Pharmaceutical Biotechnology ◽

10.2174/1389201021666200630140137 ◽

2020 ◽

Vol 22 (1) ◽

pp. 32-45

Author(s):

Emanuela Martina ◽

Federico Diotallevi ◽

Tommaso Bianchelli ◽

Matteo Paolinelli ◽

Annamaria Offidani

Keyword(s):

English Language ◽

Life Experience ◽

Case Reports ◽

Immunosuppressive Agents ◽

Real Life ◽

Chronic Spontaneous Urticaria ◽

New Targets ◽

Current State ◽

Pubmed Database ◽

Novel Drugs

Background: Chronic Spontaneous Urticaria (CSU) is a disease characterized by the onset of wheals and/or angioedema over 6 weeks. The pathophysiology for CSU is very complex, involving mast cells and basophils with a multitude of inflammatory mediators. For many years the treatment of CSU has been based on the use of antihistamines, steroids and immunosuppressive agents with inconstant and frustrating results. The introduction of omalizumab, the only licensed biologic for antihistamine- refractory CSU, has changed the management of the disease. Objective: The aim of this article is to review the current state of the art of CSU, the real-life experience with omalizumab and the promising drugs that are under development. Methods:: An electronic search was performed to identify studies, case reports, guidelines and reviews focused on the new targets for the treatment of chronic spontaneous urticaria, both approved or under investigation. The search was limited to articles published in peer-reviewed journals in the English Language in the PubMed database and trials registered in Clinicaltrials.gov. Results:: Since the advent of omalizumab, the search for new therapies for chronic spontaneous urticaria has had a new impulse. Anti-IgE drugs will probably still be the cornerstone of therapy, but new targets may prove effective in syndromic urticaria or refractory cases. Conclusion:: Although omalizumab has been a breakthrough in the treatment of CSU, many patients do not completely get benefit and even require more effective treatments. Novel drugs are under investigation with promising results.

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Risk Factors, and Clinical and Etiological Characteristics of Ischemic Strokes in COVID-19-Infected Patients: A Systematic Review of Literature

Cerebrovascular Diseases ◽

10.1159/000514267 ◽

2021 ◽

pp. 1-4

Author(s):

Simone Vidale

Keyword(s):

Risk Factors ◽

Systematic Review ◽

Clinical Features ◽

Case Reports ◽

Clinical Severity ◽

Large Artery ◽

Causal Association ◽

Review Of Literature ◽

Increased Risk ◽

Long Time

Background and Purpose: Coronavirus disease 2019 (COVID-19) infection is an ongoing pandemic and worldwide health emergency that has caused important changes in healthcare systems. Previous studies reported an increased risk of thromboembolic events, including stroke. This systematic review aims to describe the clinical features and etiological characteristics of ischemic stroke patients with COVID-19 infection. Method: A literature search was performed in principal databases for studies and case reports containing data concerning risk factors, clinical features, and etiological characteristics of patients infected with COVID-19 and suffering from stroke. Descriptive and analytical statistics were applied. Results: Overall, 14 articles were included for a total of 93 patients. Median age was 65 (IQR: 55–75) years with prevalence in males. Stroke occurred after a median of 6 days from COVID-19 infection diagnosis. Median National of Institute of Health Stroke Scale (NIHSS) score was 19. Cryptogenic (Cry) strokes were more frequent (51.8%), followed by cardioembolic etiology, and they occurred a long time after COVID-19 diagnosis compared with large-artery atherosclerosis strokes (ptrend: 0.03). The clinical severity of stroke was significantly associated with the severity grade of COVID-19 infection (ptrend: 0.03). Conclusions: Ischemic strokes in COVID-19-infected patients were clinically severe, affecting younger patients mainly with Cry and cardioembolic etiologies. Further multicenter prospective registries are needed to better describe the causal association and the effect of COVID-19 infection on stroke.

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Villoglandular adenocarcinoma of the uterine cervix: a systematic review and meta-analysis

Archives of Gynecology and Obstetrics ◽

10.1007/s00404-021-06077-9 ◽

2021 ◽

Author(s):

Anna K. Dietl ◽

Matthias W. Beckmann ◽

Konrad Aumann

Keyword(s):

Systematic Review ◽

Uterine Cervix ◽

Case Reports ◽

Case Series ◽

Disease Free Survival ◽

Conservative Group ◽

Pubmed Database ◽

Significant Difference ◽

Stage Ia ◽

Invasive Management

Abstract Purpose Villoglandular adenocarcinoma (VGA) of the uterine cervix has been classified as a rare subtype of cervical adenocarcinoma with good prognosis. A conservative surgical approach is considered feasible. The main risk factor is the presence of other histologic types of cancer. In this largest systematic review to date, we assess oncological outcomes associated with conservative therapy compared to those associated with invasive management in the treatment of stage Ia and Ib1 VGA. Methods Case series and case reports identified by searching the PubMed database were eligible for inclusion in this review (stage Ia–Ib1). Results A total of 271 patients were included in our literature review. 54 (20%) patients were treated by “conservative management” (conization, simple hysterectomy, and trachelectomy) and 217 (80%) by “invasive management” (radical hysterectomy ± radiation, hysterectomy, and radiation). Recurrences of disease (RODs) were found in the conservative group in two (4%) cases and in the invasive group in nine (4%) cases. There was no significant difference in disease-free survival (DFS) according to conservative or invasive treatment (p = 0.75). The histology of VGA may be complex with underlying usual adenocarcinoma (UAC) combined with VGA. Conclusion The excellent prognosis of pure VGA and the young age of the patients may justify the management of this tumor using a less radical procedure. The histological diagnosis of VGA is a challenge, and pretreatment should not be based solely on a simple punch biopsy but rather a conization with wide tumor-free margins.

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