Updates to the Diagnosis and Medical Management of Vesiculobullous Disorders

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Dermatology".

Deadline for manuscript submissions: closed (15 July 2021) | Viewed by 87940

Special Issue Editors

Department of Dermatology, Temple University Lewis Katz School of Medicine, Philadelphia, PA 19140, USA
Interests: psoriasis; autoimmune bullous dermatoses; acne vulgaris; skin cancer; eczema
Department of Dermatology, University of Florida College of Medicine, Gainesville, FL 32606, USA
Interests: dermatology; dermatopathology; infectious diseases; squamous cell skin cancer

Special Issue Information

Dear Colleagues,

Multiple inflammatory skin conditions present with vesicles or bullae. Some of these disorders are common and are encountered in everyday practice, whereas others are rarely seen. In the majority of these diseases, clinical diagnosis alone is sufficient. However, given the potential for overlap and the impact on treatment, additional testing is frequently recommended. Adults with Stevens–Johnson syndrome/toxic epidermal necrolysis now benefit from higher quality evidence for treatment, while updates to diagnostic subclassification may enhance the care of affected pediatric patients. Given the sharp contrast in the management and prognosis of erythema multiforme and the fixed drug eruption compared with Stevens–Johnson syndrome/toxic epidermal necrolysis—along with the limited utility of histopathology for differentiation—morphologic clues are crucial to a timely clinical distinction. For bullous pemphigoid, the most common disorder in the pemphigoid group, recent and upcoming therapies target inflammatory mediators to improve the quality of life and reduce pruritus in elderly patients. Clinicians who encounter less common pemphigoid disorders, such as mucous membrane pemphigoid, ocular cicatricial pemphigoid, and epidermolysis bullosa acquisita, may benefit from a review of stepwise therapeutic ladders and a multidisciplinary approach to care. B-cell-directed antibodies and cell engineering will provide novel immunotherapies to patients with pemphigus. While uncommon, clinicians may benefit from a review of tests that distinguish and permit disease monitoring in dermatitis herpetiformis, linear IgA disease, and bullous systemic lupus erythematosus. Clinical diagnosis and selection of empiric therapy in bullous impetigo and staphylococcal scalded skin syndrome are reviewed. Diagnostic distinction and management of porphyria cutanea tarda and pseudoporphyria are highlighted. Lastly, differentiating features and approaches to therapy, pustular psoriasis and acute generalized exanthematous pustulosis are compared and contrasted. In this Special Issue, we present updates to the diagnosis and medical management of vesiculobullous dermatoses.

Prof. Dr. Sylvia Hsu
Prof. Dr. Kiran Motaparthi
Guest Editors

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Keywords

  • Stevens–Johnson syndrome
  • toxic epidermal necrolysis
  • pemphigoid
  • pemphigus
  • dermatitis herpetiformis
  • linear IgA disease
  • erythema multiforme
  • fixed drug eruption
  • bullous impetigo
  • staphylococcal scalded skin syndrome
  • porphyria

Published Papers (11 papers)

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Editorial

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3 pages, 241 KiB  
Editorial
Vesiculobullous Diseases
by Simo Huang, Sylvia Hsu and Kiran Motaparthi
Medicina 2022, 58(2), 186; https://doi.org/10.3390/medicina58020186 - 26 Jan 2022
Viewed by 2198
Abstract
A diverse range of inflammatory dermatoses are characterized by vesicles or bullae [...] Full article

Research

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6 pages, 2376 KiB  
Communication
Diagnosis of Laryngeal Pemphigus Vulgaris Can Be Facilitated Using Advanced Endoscopic Methods
by Lucia Staníková, Martin Formánek, Pavel Hurník, Peter Kántor, Pavel Komínek and Karol Zeleník
Medicina 2021, 57(7), 686; https://doi.org/10.3390/medicina57070686 - 06 Jul 2021
Viewed by 1965
Abstract
Background: Isolated laryngeal pemphigus vulgaris (LPV) is rare; however, early diagnosis is crucial in determining its course and prognosis. This paper aims to describe mucosal vascular changes typical for LPV using advanced endoscopic methods, which include Narrow Band Imaging (NBI), IMAGE1-S video-endoscopy and [...] Read more.
Background: Isolated laryngeal pemphigus vulgaris (LPV) is rare; however, early diagnosis is crucial in determining its course and prognosis. This paper aims to describe mucosal vascular changes typical for LPV using advanced endoscopic methods, which include Narrow Band Imaging (NBI), IMAGE1-S video-endoscopy and enhanced contact endoscopy (ECE). Materials and Methods: Retrospective analysis of all laryngeal mucosal lesion examined using advanced endoscopic methods during 2018–2020 at tertiary hospital was performed. Results: Videolaryngoscopy examination records of 278 patients with laryngeal mucosal lesions were analyzed; three of them were diagnosed with LPV. Epithelial vascularization of LPV included specific pattern. Intraepithelial papillary capillary loops were symmetrically stratified and were organized into “contour-like lines”. This specific vascularization associated with LPV were different from other laryngeal mucosal pathologies. Conclusions: Using advanced endoscopic methods supports early diagnosis of LPV and accelerate the diagnosis and treatment. Full article
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Review

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15 pages, 33897 KiB  
Review
An Updated Review of Pemphigus Diseases
by Ali M. Malik, Sarah Tupchong, Simo Huang, Abhirup Are, Sylvia Hsu and Kiran Motaparthi
Medicina 2021, 57(10), 1080; https://doi.org/10.3390/medicina57101080 - 09 Oct 2021
Cited by 23 | Viewed by 8840
Abstract
Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is [...] Read more.
Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Considering the risks associated with a delayed diagnosis or misdiagnosis and the potential for overlap in clinical features and treatment, evaluation for suspected pemphigus disease often requires thorough clinical assessment and laboratory testing. Diagnosis is focused on individual biopsies for histopathology and direct immunofluorescence. Additional laboratory methods used for diagnosis include indirect immunofluorescence and enzyme-linked immunosorbent assay. Recent advancements, including anti-CD20 therapy, have improved the efficacy and reduced the morbidity of pemphigus treatment. This contribution presents updates on the pathophysiology, clinical features, diagnostic work-up, and medical management of pemphigus. Improved strategies for diagnosis and clinical assessment are reviewed, and newer treatment options are discussed. Full article
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14 pages, 2697 KiB  
Review
Bullous Pemphigoid and Other Pemphigoid Dermatoses
by Valeryia Pratasava, Vikram N. Sahni, Aishwarya Suresh, Simo Huang, Abhirup Are, Sylvia Hsu and Kiran Motaparthi
Medicina 2021, 57(10), 1061; https://doi.org/10.3390/medicina57101061 - 04 Oct 2021
Cited by 12 | Viewed by 6372
Abstract
The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous pemphigoid. Its variable clinical presentation, with or without frank bullae, is linked by significant pruritus afflicting the elderly. Mucous membrane [...] Read more.
The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous pemphigoid. Its variable clinical presentation, with or without frank bullae, is linked by significant pruritus afflicting the elderly. Mucous membrane pemphigoid is an umbrella term for a group of subepidermal blistering dermatoses that favor the mucosal membranes and can scar. Epidermolysis bullosa acquisita is a chronic blistering disorder characterized by skin fragility, sensitivity to trauma, and its treatment-refractory nature. Clinicians that encounter these pemphigoid disorders may benefit from an overview of their clinical presentation, diagnostic work-up, and therapeutic management, with an emphasis on the most frequently encountered pemphigoid disease, bullous pemphigoid. Full article
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12 pages, 2557 KiB  
Review
Pustular Psoriasis and Acute Generalized Exanthematous Pustulosis
by Morgan Sussman, Anthony Napodano, Simo Huang, Abhirup Are, Sylvia Hsu and Kiran Motaparthi
Medicina 2021, 57(10), 1004; https://doi.org/10.3390/medicina57101004 - 23 Sep 2021
Cited by 15 | Viewed by 6143
Abstract
The similarity between pustular psoriasis (PP) and acute generalized exanthematous pustulosis (AGEP) poses problems in the diagnosis and treatment of these two conditions. Significant clinical and histopathologic overlap exists between PP and AGEP. PP is an inflammatory disorder that has numerous clinical subtypes, [...] Read more.
The similarity between pustular psoriasis (PP) and acute generalized exanthematous pustulosis (AGEP) poses problems in the diagnosis and treatment of these two conditions. Significant clinical and histopathologic overlap exists between PP and AGEP. PP is an inflammatory disorder that has numerous clinical subtypes, but all with sterile pustules composed of neutrophils. AGEP is a severe cutaneous adverse reaction that is also characterized by non-follicular sterile pustules. Clinical features that suggest a diagnosis of PP over AGEP include a history of psoriasis and the presence of scaling plaques. Histologically, eosinophilic spongiosis, vacuolar interface dermatitis, and dermal eosinophilia favor a diagnosis of AGEP over PP. Importantly, PP and AGEP vary in clinical course and treatment. PP treatment involves topical steroids, oral retinoids, and systemic immunosuppressants. Newer therapies targeting IL-36, IL-23, IL-1, and PDE-4 have been investigated. The removal of the offending agent is a crucial part of the treatment of AGEP. Full article
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11 pages, 3453 KiB  
Review
A Review of Fixed Drug Eruption with a Special Focus on Generalized Bullous Fixed Drug Eruption
by Hannah J. Anderson and Jason B. Lee
Medicina 2021, 57(9), 925; https://doi.org/10.3390/medicina57090925 - 01 Sep 2021
Cited by 28 | Viewed by 11117
Abstract
Fixed drug eruption (FDE) is a cutaneous adverse drug reaction characterized by the onset of rash at a fixed location on the body each time a specific medication is ingested. With each recurrence, the eruption can involve additional sites. Lesions can have overlying [...] Read more.
Fixed drug eruption (FDE) is a cutaneous adverse drug reaction characterized by the onset of rash at a fixed location on the body each time a specific medication is ingested. With each recurrence, the eruption can involve additional sites. Lesions can have overlying vesicles and/or bullae, and when they cover a significant percentage of body surface area, the eruption is referred to as generalized bullous fixed drug eruption (GBFDE). Due to the widespread skin denudation that can be seen in this condition, GBFDE may be confused clinically with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). While treatments described for GBFDE include supportive care, topical and/or systemic steroids, and, recently, cyclosporine, the mainstay of management involves identifying and discontinuing the causative drug. This review article will provide an overview of FDE with an emphasis on its generalized bullous variant. Full article
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8 pages, 552 KiB  
Review
Recent Updates in the Treatment of Erythema Multiforme
by Alexa Soares and Olayemi Sokumbi
Medicina 2021, 57(9), 921; https://doi.org/10.3390/medicina57090921 - 01 Sep 2021
Cited by 16 | Viewed by 11040
Abstract
Erythema multiforme (EM) is an immune-mediated condition that classically presents with discrete targetoid lesions and can involve both mucosal and cutaneous sites. While EM is typically preceded by viral infections, most notably herpes simplex virus (HSV), and certain medications, a large portion of [...] Read more.
Erythema multiforme (EM) is an immune-mediated condition that classically presents with discrete targetoid lesions and can involve both mucosal and cutaneous sites. While EM is typically preceded by viral infections, most notably herpes simplex virus (HSV), and certain medications, a large portion of cases are due to an unidentifiable cause. EM can be confused with other more serious conditions like Stevens–Johnson syndrome (SJS); however, clinical research has provided significant evidence to classify EM and SJS as separate disorders. Treatment of EM is highly variable, depending on the etiology, the involvement of mucosal sites, and the chronicity (acute vs. recurring) of the disease. If the etiology or causal medication/infection is identified, then the medication is stopped and/or the infection is treated prior to initiating symptomatic treatment. Treatment for acute EM is focused on relieving symptoms with topical steroids or antihistamines. Treatment for recurrent EM is most successful when tailored to individual patients. First line treatment for recurrent EM includes both systemic and topical therapies. Systemic therapies include corticosteroid therapy and antiviral prophylaxis. Topical therapies include high-potency corticosteroids, and antiseptic or anesthetic solutions for mucosal involvement. Second-line therapies for patients who do not respond to antiviral medications include immunosuppressive agents, antibiotics, anthelmintics, and antimalarials Full article
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15 pages, 1566 KiB  
Review
Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management
by Robert Frantz, Simo Huang, Abhirup Are and Kiran Motaparthi
Medicina 2021, 57(9), 895; https://doi.org/10.3390/medicina57090895 - 28 Aug 2021
Cited by 62 | Viewed by 21276
Abstract
Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. [...] Read more.
Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. In this scoping review, Excerpta Medica dataBASE and PubMed were searched for publications that addressed recent advances in the diagnosis and management of the disease. Multiple proteins (galectin 7 and RIP3) were identified that are promising potential biomarkers for SJS/TEN, although both are still in early phases of research. Regarding treatment, cyclosporine is the most effective therapy for the treatment of SJS, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN. Due to the rare nature of the disease, there is a lack of prospective, randomized controlled trials and conducting these in the future would provide valuable insights into the management of this disease. Full article
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15 pages, 1611 KiB  
Review
Dermatitis Herpetiformis: An Update on Diagnosis, Disease Monitoring, and Management
by Christopher N. Nguyen and Soo-Jung Kim
Medicina 2021, 57(8), 843; https://doi.org/10.3390/medicina57080843 - 20 Aug 2021
Cited by 14 | Viewed by 7376
Abstract
Dermatitis herpetiformis (DH), Duhring disease, is caused by gluten sensitivity and affects 11.2 to 75.3 per 100,000 people in the United States and Europe with an incidence of 0.4 to 3.5 per 100,000 people per year. DH is characterized by a symmetrical blistering [...] Read more.
Dermatitis herpetiformis (DH), Duhring disease, is caused by gluten sensitivity and affects 11.2 to 75.3 per 100,000 people in the United States and Europe with an incidence of 0.4 to 3.5 per 100,000 people per year. DH is characterized by a symmetrical blistering rash on the extensor surfaces with severe pruritus. The diagnosis continues to be made primarily by pathognomonic findings on histopathology, especially direct immunofluorescence (DIF). Recently, anti-epidermal transglutaminase (TG3) antibodies have shown to be a primary diagnostic serology, while anti-tissue transglutaminase (TG2) and other autoantibodies may be used to support the diagnosis and for disease monitoring. Newly diagnosed patients with DH should be screened and assessed for associated diseases and complications. A gluten-free diet (GFD) and dapsone are still mainstays of treatment, but other medications may be necessary for recalcitrant cases. Well-controlled DH patients, managed by a dermatologist, a gastroenterologist, and a dietician, have an excellent prognosis. Our review comprehensively details the current diagnostic methods, as well as methods used to monitor its disease course. We also describe both the traditional and novel management options reported in the literature. Full article
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10 pages, 612 KiB  
Review
Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review
by Leah Shin, Jeffrey T. Gardner II and Harry Dao, Jr.
Medicina 2021, 57(8), 818; https://doi.org/10.3390/medicina57080818 - 12 Aug 2021
Cited by 9 | Viewed by 3510
Abstract
Background and Objectives: Linear IgA disease (LAD) is a rare autoimmune blistering disease with linear IgA deposits along the basement membrane zone. Direct immunofluorescence remains the gold standard for diagnosis, but other diagnostic measures reported in recent literature have proven useful in the [...] Read more.
Background and Objectives: Linear IgA disease (LAD) is a rare autoimmune blistering disease with linear IgA deposits along the basement membrane zone. Direct immunofluorescence remains the gold standard for diagnosis, but other diagnostic measures reported in recent literature have proven useful in the setting of inconclusive preliminary results. Dapsone is a commonly used treatment, but many therapeutic agents have emerged in recent years. The objective of this study is to provide a comprehensive overview of updates on the diagnosis and management of LAD. Materials and Methods: A literature search was conducted from May to June of 2021 for articles published in the last 5 years that were related to the diagnosis and management of LAD. Results: False-negative results in cases of drug-induced LAD and the presence of IgG and IgM antibodies on immunofluorescence studies were reported. Serration pattern analysis has been reported to be useful in distinguishing LAD from sublamina densa-type LAD. Rituximab, omalizumab, etanercept, IVIg, sulfonamides, topical corticosteroids, and others have been used successfully in adult and pediatric patients with varying disease severity. Topical corticosteroids were preferred for pediatric patients while rituximab and IVIg were used in adults with recalcitrant LAD. Sulfonamides were utilized in places without access to dapsone. Conclusion: In cases where preliminary biopsy results are negative and clinical suspicion is high, repeat biopsy and additional diagnostic studies should be used. Patient factors such as age, medical comorbidities, and disease severity play a role in therapeutic selection. Full article
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11 pages, 3079 KiB  
Review
Dyshidrosiform Bullous Pemphigoid
by Philip R. Cohen
Medicina 2021, 57(4), 398; https://doi.org/10.3390/medicina57040398 - 20 Apr 2021
Cited by 7 | Viewed by 6370
Abstract
Dyshidrosiform bullous pemphigoid is a variant of bullous pemphigoid. At least 84 patients with dyshidrosiform bullous pemphigoid have been described. Dyshidrosiform bullous pemphigoid usually presents with pruritic blisters in elderly individuals; the hemorrhagic or purpuric lesions on the palms and soles can be [...] Read more.
Dyshidrosiform bullous pemphigoid is a variant of bullous pemphigoid. At least 84 patients with dyshidrosiform bullous pemphigoid have been described. Dyshidrosiform bullous pemphigoid usually presents with pruritic blisters in elderly individuals; the hemorrhagic or purpuric lesions on the palms and soles can be the only manifestation of the disease. However, bullae may concurrently or subsequently appear on other areas of the patient’s body. Patients typically improve after the diagnosis is established and treatment is initiated. The mainstay of therapy is systemic corticosteroids, with or without topical corticosteroids, and systemic dapsone or immunosuppressants. Drug-related or nickel-induced dyshidrosiform bullous pemphigoid improves after stopping the associated agent; however, systemic therapy has also been required to achieve resolution of the blisters. Similar to classic bullous pemphigoid, neurologic conditions and psychiatric disorders have been observed in dyshidrosiform bullous pemphigoid patients. The new onset of recurrent or persistent blisters on the palms, soles, or both of an elderly individual should prompt the clinician to consider the diagnosis of dyshidrosiform bullous pemphigoid. Full article
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