Collagen vascular disease is a heterogeneous group of autoimmune diseases that affect multiple organ systems. Sjögren syndrome, dermatomyositis, scleroderma, systemic lupus erythematosus, and sarcoidosis are collagen vascular diseases that often present with characteristic cutaneous manifestations. Although less known, various ocular manifestations that affect both external and internal structures of the eye can be seen in these conditions. Multidisciplinary management between dermatologists and ophthalmologists is essential in the early diagnosis and management of collagen vascular diseases affecting both the skin and eye. Part I of our series will discuss the ocular manifestations, their diagnosis, and therapeutic options in Sjögren syndrome and systemic lupus erythematosus.
Background There are few studies focusing on pediatric teledermatology, and the impact of a large-scale pediatric teledermatology program on the accessibility and efficiency of dermatology care remains unclear. This study evaluated the impact of a state-wide implementation of a store-and-forward teledermatology program augmented by the incorporation of dermoscopy in pediatric patients visiting community health centers. Methods It was a descriptive, retrospective cohort study of 876 pediatric dermatology referrals. Results In the traditional referral system, only 60 patients (17.6%) were seen by dermatologists with average waiting times of 75 days due to limited access. In comparison, with an implementation of dermoscopy-aided teledermatology, all 536 teledermatology referrals received dermatological care within 24 h, of which only 64 (12%) patients requires face-to-face (F2F) consultation. Patients referred for F2F consultation via eConsults had a much lower no-show rate as compared to the traditional referral system (39% vs. 71%). Side by side comparison between general population and pediatric population has demonstrated shared features in efficiency and access improvement but revealed specific characteristics of pediatric teledermatology in terms of diagnosis and treatment. Conclusion Coordinated store-and-forward teledermatology platform with incorporation of dermoscopy between large community care network and dermatology provider can greatly improve access to pediatric dermatology care especially in underserved population. The efficiency of teledermatology in access improvement for pediatric population is comparable with adult population in eConsults. There are also unique features and challenges in pediatric teledermatology that require further research.
Collagen vascular disease is a heterogeneous group of autoimmune diseases that affect multiple organ systems. Sjögren syndrome, dermatomyositis, scleroderma, systemic lupus erythematosus, and sarcoidosis are collagen vascular diseases that often present with characteristic cutaneous manifestations. Although less known, various ocular manifestations that affect both external and internal structures of the eye can also be seen in these conditions. Multidisciplinary management between dermatologists and ophthalmologists is essential in the early diagnosis and management of collagen vascular diseases affecting both the skin and eye. In part II of our series, we discuss the ocular manifestations, diagnosis, and therapeutic options of dermatomyositis, scleroderma, and sarcoidosis.
Wells syndrome, also referred to as eosinophilic cellulitis, is a rare and often sporadic inflammatory skin condition whose aetiology remains uncertain. Clinically, this condition presents as a collection of erythematous, oedematous, and tender skin lesions most often affecting the extremities and trunk that can mimic cellulitis. Histologically, Wells syndrome is characterised by inflammatory changes and eosinophilic infiltration of the dermis with the absence of underlying infection, thereby distinguishing it from cellulitis. Due to the rarity of this syndrome and its ambiguous presentation, there remains to be a definitive strategy for treatment. Recent case reports have documented varying success and recurrence with the use of oral and topical corticosteroids, antifungals, antibiotics, immunosuppressants and antihistamines. Here, we report a unique case of progressively worsening neutrophilic-rich Wells syndrome on the vertex of the scalp that was successfully treated with a combination of dupilumab and oral corticosteroids.
Emerging evidence highlights the gut-skin microbiota as a potential therapeutic target for the management of inflammatory-driven cutaneous diseases as well as the interconnection of the gut-skin microbiota in disease pathogenesis and progression. Although not fully understood, recent research has indicated that commensal microbiota and the interaction of the gut-skin axis play an important role in maintaining skin homeostasis. Dysbiosis and disruption of the skin-gut microbiome may lead to impaired skin barrier function, thus triggering downstream inflammatory responses involved in the development of inflammatory skin disorders, especially in atopic dermatitis, psoriasis, and hidradenitis suppurativa. The skin microbiome may also serve as adjunctive therapeutic modalities for treatment. Herein, we review the latest reports on the interrelationship between microbial dysbiosis and inflammatory cutaneous diseases as well as emerging microbiome-directed therapeutics in atopic dermatitis, psoriasis, and hidradenitis suppurativa.
Introduction Dermatomyositis is a rare autoimmune inflammatory condition affecting skin and muscles. The disease can be seen in both adults and children. It can be associated with malignancy. Considering involvement of skin in the disease, many patients consult dermatologists for its treatment. Hence, knowledge about its presentation, complications, prognosis, and treatment is necessary. Objective The objective of this review article is to provide comprehensive information about treatment of dermatomyositis. Methods In this review article, we reviewed the published literature on adult and juvenile dermatomyositis to highlight the treatment. Articles published in peer-reviewed journals including reviews, clinical trials, case series, and case reports published in electronic database (MEDLINE/PubMed) through January 2021, cross references of respective articles and trials from were included for qualitative analysis of the literature. Results Treatment options for dermatomyositis include traditional immunosuppressive agents and immunomodulatory therapy. High-dose corticosteroids represent the first line of treatment while other immunosuppressive agents are also used, either in combination with or as an alternative to corticosteroids, after initial treatment failure. Some biological agents have been used for the treatment of dermatomyositis with variable responses. Conclusion Although several treatment options are available, several questions remain unanswered about the optimal treatment of dermatomyositis.
Background: Dermatomyositis (DM) is a chronic idiopathic inflammatory condition characterized by involvement of the skin and muscles. Amyopathic DM is a subset of DM with classic skin manifestations but no muscle weakness. Many cases are refractory to standard treatment. We present a 66-year-old Hispanic female who presented with a 4-year history of an intensely pruritic rash on the upper back, arms, and scalp with associated alopecia, Gottron's papules, and heliotrope rash. A prior biopsy showed interface dermatitis and lab studies were positive for TIF1-gamma antibodies.
To the Editor: Bullous pemphigoid (BP) is an autoimmune blistering disorder usually seen in elderly patients. Current therapies include corticosteroids, antibiotics, intravenous immunoglobulin, and immunosuppressants; however, some patients have exclusionary comorbidities, recalcitrant BP, or side effects of these medications. 1 Amber K.T. Murrell D.F. Schmidt E. Joly P. Borradori L. Autoimmune subepidermal bullous diseases of the skin and mucosae: clinical features, diagnosis, and management. Clin Rev Allergy Immunol. 2018; 54: 26-51https://doi.org/10.1007/s12016-017-8633-4 Crossref PubMed Scopus (130) Google Scholar Type 2 proinflammatory cytokines may play a role in the pathogenesis of BP and dupilumab, an interleukin 4 receptor-alpha antagonist, has been identified as a potential therapeutic agent. 1 Amber K.T. Murrell D.F. Schmidt E. Joly P. Borradori L. Autoimmune subepidermal bullous diseases of the skin and mucosae: clinical features, diagnosis, and management. Clin Rev Allergy Immunol. 2018; 54: 26-51https://doi.org/10.1007/s12016-017-8633-4 Crossref PubMed Scopus (130) Google Scholar , 2 Abdat R. Waldman R.A. de Bedout V. et al. Dupilumab as a novel therapy for bullous pemphigoid: a multicenter case series. J Am Acad Dermatol. 2020; 83: 46-52https://doi.org/10.1016/j.jaad.2020.01.089 Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar , 3 Muñoz-Bellido F.J. Moreno E. Dávila I. Dupilumab: a Review of present indications and off-label uses. J Investig Allergol Clin Immunol. 2022; 32: 97-115https://doi.org/10.18176/jiaci.0682 Crossref PubMed Scopus (41) Google Scholar Existing literature describes positive patients with BP response to dupilumab, but little information is available regarding long-term outcomes. 2 Abdat R. Waldman R.A. de Bedout V. et al. Dupilumab as a novel therapy for bullous pemphigoid: a multicenter case series. J Am Acad Dermatol. 2020; 83: 46-52https://doi.org/10.1016/j.jaad.2020.01.089 Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar ,4 Zhang Y. Xu Q. Chen L. et al. Efficacy and safety of dupilumab in moderate-to-severe bullous pemphigoid. Front Immunol. 2021; 12738907https://doi.org/10.3389/fimmu.2021.738907 Crossref Scopus (43) Google Scholar We evaluate response maintenance, treatment durability, and adverse events of patients with BP on dupilumab. This study was approved by Tufts Institutional Review Board.