Cutaneous vasculitis may be a cutaneous manifestation of systemic vasculitis, a skin-limited variant of systemic vasculitis, or a type of single-organ vasculitis limited to the skin. Careful ...evaluation is necessary to distinguish these possibilities, differentiate skin-limited from systemic disease, and identify important underlying conditions, if present. Appropriate management depends on disease type and severity. In the case of cutaneous small vessel vasculitis (CSVV), treatment is primarily based on case reports and expert opinion, using a range of immunomodulatory or immunosuppressive therapies. This manuscript presents a systematic and stepwise approach to the evaluation of patients presenting with cutaneous vasculitis, as well as the management of those diagnosed with CSVV, using best available evidence. Studies relevant to the classification, evaluation, and management of skin vasculitis are ongoing.
Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features but are unified by the presence of a sterile, predominantly neutrophilic ...infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes. While some neutrophilic dermatoses may resolve spontaneously, most require treatment to achieve remission. Delays in diagnosis and treatment can lead to significant patient morbidity and even mortality. Therapeutic modalities range from systemic corticosteroids to novel biologic agents, and the treatment literature is rapidly expanding. The first article in this continuing medical education series explores the pathogenesis of neutrophilic dermatoses and reviews the epidemiology, clinical and histopathologic features, diagnosis, and management of Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.
Systemic glucocorticoids are an essential therapy for a range of conditions, but their multiple side effects can produce significant morbidity for patients. The objective of this review is to discuss ...these side effects while addressing 3 questions: 1) What dose and duration of glucocorticoid therapy should prompt concern for individual side effects?; 2) How should clinicians counsel patients about these complications?; and 3) How can these problems be prevented or managed? To accomplish these objectives, we have created a series of tables and algorithms based on a review of relevant data to guide counseling, prophylaxis, and management of 11 glucocorticoid side effects. The first article in this 4-part continuing medical education series begins with a review of glucocorticoid pharmacology followed by a discussion of bone health (ie, osteoporosis and osteonecrosis).
Cutaneous vasculitis encompasses a spectrum of disease states, with varied morphology, severity, and potential for systemic involvement. Even vasculitis which is skin-limited can have a significant ...quality-of-life impact, necessitating treatment. This manuscript summarizes the available evidence for management of various types of skin-limited vasculitis and provides a proposed therapeutic ladder based on published studies and expert opinion.
Objective
Cutaneous manifestations of antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and ...eosinophilic granulomatosis with polyangiitis (EGPA), are poorly characterized. This report describes the dermatologic features of AAV and their association with systemic manifestations of vasculitis.
Methods
A cross‐sectional study identifying and comparing the cutaneous manifestations of AAV was performed using data from a large, international, collaborative effort in order to collect comprehensive clinical data on patients with vasculitis.
Results
Data from 1,184 patients with AAV from 130 centers worldwide were available. Cutaneous manifestations were common in all AAV subtypes: GPA (223 of 656, or 34%), MPA (85 of 302, or 28%), and EGPA (106 of 226, or 47%). The most frequent cutaneous manifestation in AAV (all types) was petechiae/purpura, which was observed in 181 patients (15%). Allergic and nonspecific manifestations, such as pruritus, urticaria, and maculopapular rash, were more common in EGPA than in other disease subtypes (all P < 0.01). Skin biopsy, while underutilized (performed in 22–44% of patients), was frequently found to be an effective test suitable for diagnosis of AAV (diagnostic in 68–94% of patients). Compared to patients without cutaneous manifestations, those with skin lesions more frequently had severe systemic manifestations of vasculitis (such as alveolar hemorrhage and glomerulonephritis), specifically patients with GPA or EGPA and cytoplasmic/anti–proteinase 3 (anti‐PR3) ANCA–positive or ANCA‐negative patients (hazard ratio >1.9 for all), but not those with MPA or perinuclear/antimyeloperoxidase ANCAs.
Conclusion
Cutaneous manifestations are common and varied in AAV and are associated with disease severity in patients with GPA, EGPA, cytoplasmic/anti‐PR3 ANCA, or ANCA‐negative disease. These findings underscore the potential diagnostic and prognostic importance of the cutaneous examination in the evaluation and management of AAV.
Part 2 of this 4-part continuing medical education series continues with a discussion of the prevention and management of gastrointestinal side effects associated with corticosteroid use, including ...peptic ulcer disease, gastrointestinal bleeding, and pancreatitis, followed by a review of corticosteroid-related endocrinologic side effects, such as diabetes, adrenal suppression, and Cushing syndrome.
What are the effects of systemic therapies for Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)?
There is limited high-quality evidence to support the use of systemic immunomodulatory ...therapies to decrease mortality rates in SJS/TEN.
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