Inpatient hospitalization of individuals with hidradenitis suppurativa (HS) has increased. Inpatient services may not be familiar enough with this disease to understand how to manage severe HS and/or HS flares. It would be beneficial to the inpatient medical community to establish consensus recommendations on holistic inpatient care of patients with HS. A survey study was developed and distributed by Wake Forest University School of Medicine (Winston-Salem, North Carolina). A total of 26 dermatologists participated in the Delphi process, and the process was conducted in 2 rounds. Participants voted on proposal statements using a 9-point scale (1=very inappropriate; 9=very appropriate). Statements were developed using current published guidelines for management of HS and supportive care guidelines for other severe inpatient dermatologic diseases. A total of 50 statements were reviewed and voted on between the 2 rounds. Consensus was determined using the RAND/UCLA Appropriateness Method. Twenty-six dermatologists completed the first-round survey, and 24 completed the second-round survey. The 40 consensus recommendations generated through these surveys can serve as a resource for providers caring for inpatients with HS.
Abstract Background Acute generalized exanthematous pustulosis (AGEP) is a rare and severe cutaneous drug eruption. Hydroxychloroquine (HCQ) is one of the known culprit drugs with less than 60 cases reported in the literature, and may have a more severe clinical presentation compared to AGEP induced by other drugs. There are no standardized management strategies for HCQ‐induced AGEP outside of drug discontinuation. Objectives Characterize the clinical presentation of HCQ‐induced AGEP and management strategies. Methods Retrospective chart review of nine patients clinically diagnosed with HCQ‐induced AGEP using the EuroSCAR score from 2017 to 2022 at four academic medical centres in the United States. Results All patients in our series were female and the median age was 52. Four patients initially presented with fever, and eight patients had neutrophilia. Median time to rash was 15 days. Biopsies obtained in six patients showed typical characteristics of AGEP. In most patients, rash morphology consisted of generalized pinpoint pustules atop atypical targetoid lesions and annular plaques. Five patients were hospitalized for AGEP. All patients were treated with methylprednisolone and/or prednisone, and the average days treated with systemic corticosteroids was 35. Conclusions Our case series is the largest to date describing HCQ‐induced AGEP. Clinicians should be wary that HCQ‐induced AGEP may present with a longer latency period after drug initiation and with atypical targetoid and annular lesions. Early initiation of systemic corticosteroids should be considered due to the severity of this drug reaction with addition of cyclosporine in refractory cases.
This retrospective cohort study analyzes the presentation, diagnosis, and treatment outcomes of patients who developed drug rash with eosinophilia and systemic symptoms (DRESS) to tuberculosis (TB) therapy in a TB non-endemic region. Anti-TB agents represented 7.5% of all antimicrobial-induced DRESS cases, and rifampin was the most commonly implicated agent among drugs used to treat TB.
Mucocutaneous eruptions are associated with various pathogens, including Mycoplasma pneumoniae, herpes simplex virus (HSV), and influenza, most commonly presenting in younger populations. Here we present a case of an adult with recurrent reactive infectious mucocutaneous eruption (RIME) with extensive ocular involvement, triggered by SARS-CoV-2.
Drug-induced hypersensitivity syndrome (DiHS), also known as drug reaction with eosinophilia and systemic symptoms (DRESS), is a severe cutaneous adverse reaction (SCAR) characterized by an exanthem, fever, and hematologic and visceral organ involvement. The differential diagnosis includes other cutaneous adverse reactions, infections, inflammatory and autoimmune diseases, and neoplastic disorders. Three sets of diagnostic criteria have been proposed; however, consensus is lacking. The cornerstone of management is immediate discontinuation of the suspected drug culprit. Systemic corticosteroids remain first-line therapy, but the literature on steroid-sparing agents is expanding. Longitudinal evaluation for sequelae is recommended. Adjunctive tests for risk stratification and drug culprit identification remain under investigation. Part II of this continuing medical education activity begins by exploring the differential diagnosis and diagnosis of DiHS/DRESS and concludes with an evidence-based overview of evaluation and treatment.
Background: In the 2022 mpox (monkeypox) outbreak, 79,000 global cases have been reported. Yet, limited dermatologic data have been published regarding lesion morphology and progression.Objective: The objective of this study was to characterize skin lesion morphology, symptomatology, and outcomes of mpox infection over time.Methods: The American Academy of Dermatology/International League of Dermatological Societies Dermatology COVID-19, Mpox, and Emerging Infections Registry captured deidentified patient cases of mpox entered by health care professionals.Results: From August 4 to November 13, 2022, 101 cases from 13 countries were entered, primarily by dermatologists (92%). Thirty-nine percent had fewer than 5 lesions. In 54% of cases, skin lesions were the first sign of infection. In the first 1-5 days of infection, papules (36%), vesicles (17%), and pustules (20%) predominated. By days 6-10, pustules (36%) were most common, followed by erosions/ulcers (27%) and crusts/scabs (24%). Crusts/scabs were the predominant morphology after day 11. Ten cases of morbilliform rash were reported. Scarring occurred in 13% of the cases.Limitations: Registry-reported data cannot address incidence. There is a potential reporting bias from the predilection to report cases with greater clinical severity.Discussion: These findings highlight differences in skin findings compared to historical outbreaks, notably the presence of skin lesions prior to systemic symptoms and low overall lesion counts. Scarring emerged as a major possible sequela. ( J Am Acad Dermatol 2023;88:1066-73.)
Drug-induced hypersensitivity syndrome (DiHS), also known as drug reaction with eosinophilia and systemic symptoms (DRESS), is a severe cutaneous adverse reaction (SCAR) characterized by an exanthem, fever, and hematologic and visceral organ involvement. Anticonvulsants, antibiotics, and allopurinol are the most common triggers. The pathogenesis involves a complex interplay between drugs, viruses, and the immune system primarily mediated by T-cells. DiHS/DRESS typically presents with a morbilliform eruption 2-6 weeks after drug exposure, and is associated with significant morbidity, mortality, and risk of relapse. Long-term sequelae primarily relate to organ dysfunction and autoimmune diseases. Part I of this continuing medical education activity on DiHS/DRESS provides an update on epidemiology, novel insights into pathogenesis, and a description of clinicopathological features and prognosis.
To the Editor: In the United States, cutaneous disorders are estimated to be diagnosed in more than 12% of hospitalized adults and account for more than $5 billion in health expenditures annually.1 However, patients with cutaneous disorders are predominantly admitted to inpatient services attended by non-dermatologists.2 To this end, the dermatology hospitalist model has emerged over the past decade, and the Society for Dermatology Hospitalists (SDH) was established.2,3 Single institution studies have suggested dermatology hospitalist services improve diagnostic accuracy and reduce readmissions.
To the Editor: Since the onset of the COVID-19 pandemic, >612 million cases of SARS-CoV-2 and over 6.5 million deaths have been reported. 12.6 billion vaccine doses have been administered worldwide, but we have not yet reached vaccine equity.1Mathieu E. Ritchie H. Rodés-Guirao L. et al.Coronavirus pandemic (COVID-19).https://ourworldindata.org/coronavirusDate: 2020Date accessed: November 1, 2022Google Scholar The spectrum of cutaneous findings of SARS-CoV-2 are associated with different COVID-19 severities, and in some cases can be the presenting, or only sign of infection. Characterizing cutaneous reactions to COVID-19 vaccination remains important, as booster doses are a key component to limiting COVID-19 related morbidity and mortality. In April 2020, in response to growing reports of SARS-CoV-2 cutaneous findings, the American Academy of Dermatology and the International League of Dermatological Societies COVID-19 Dermatology Registry was generated to rapidly collate SARS-CoV-2 associated dermatologic manifestations. In December 2020, the registry expanded to include COVID-19 vaccine-related reactions. Over 500 cases of SARS-CoV-2 cutaneous findings were submitted within 25 days of launch. As of September 2022, the registry has amassed 1436 reports of COVID-related and 1086 reports of vaccine–related dermatologic manifestations spanning 72 countries (Fig 1). The registry was designed in collaboration with a panel of experts in dermatology and infectious disease and hosted via REDCap's (Vanderbilt University) secure online survey platform. Data entry was restricted to healthcare providers for more accurate clinical and morphological data. Respondents were asked for case follow-up and biopsy results as available. Data requests are available for outside investigations via a data request application and reviewed by an appointed data governance group. The American Academy of Dermatology/International League of Dermatological Societies COVID-19 Dermatology Registry exemplifies collaborative efforts of the dermatologic community during the SARS-CoV-2 outbreak. Case entry led to 24 publications, including 111 authors across 19 different countries (Fig 2), and served as a training platform for 13 trainees. Registry publications led to >190 million media impressions, allowing for rapid dissemination of information to the medical field and the lay public. COVID–related dermatologic registries across the globe amassed over 8000 cases in total and contributed to data sharing across a spectrum of COVID-19 related dermatologic subjects.2Freeman E.E. Chamberlin G.C. McMahon D.E. et al.Dermatology COVID-19 registries: updates and future directions.Dermatol Clin. 2021; 39: 575-585Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar Registry data are useful specifically for hypothesis generation, not as a replacement for epidemiologic data, but as an initial method of rapid collation and harmonization of disparate real-world observations in times of novel disease outbreak when resources are low, but the need for data is high. Efficient data collection and dissemination functions as means of combatting misinformation and facilitating diagnosis in times of novel disease outbreaks. The registry will continue to gather information, particularly as the medical community continues to investigate Long COVID — its origins and impacts on the estimated millions it affects—and as vaccination and booster campaigns remain a key component in infection control.3Statistics NCfHLong COVID household pulse survey.https://www.cdc.gov/nchs/covid19/pulse/long-covid.htmDate: 2022Date accessed: November 3, 2022Google Scholar In response to World Health Organization's declaration of the spread of monkeypox (mpox) as a public health emergency, the registry expanded in August 2022, becoming the American Academy of Dermatology/International League of Dermatological Societies Dermatology COVID-19, Monkeypox (mpox), and Emerging Infections Registry.4Freeman E.E. Galvan Casas C. Prasad S. et al.The American Academy of Dermatology and International League of Dermatological Societies monkeypox registry: expanding the COVID-19 registry to emerging infections.J Am Acad Dermatol. 2022; 87: 1278-1280Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Reports of COVID-19, as well as mpox cases and cutaneous reactions to mpox/smallpox vaccines, are being accepted from healthcare providers worldwide (https://www.aad.org/monkeypoxregistry). Dr Freeman is the Principal Investigator of the AAD/ILDS Dermatology Registry for COVID-19, Monkeypox, and Emerging Infections. Dr Fox is a Board member of the AAD. Dr Lim is a Board member of the ILDS. Dr French is the President of the ILDS. Authors Strahan, Lubov, and Prasad; Dr McMahon; Author Singh; Drs Rosenbach, Desai, Thiers, and Hruza have no conflicts of interest to declare.
To the Editor: In July 2022, the World Health Organization declared "mpox" a public health emergency. Since outbreak onset, >91,000 cases have been reported. 1 CDC. 2022 Monkeypox Outbreak Global Map. 2022. https://www.cdc.gov/poxvirus/monkeypox/response/2022/world-map.htmlDate accessed: November 6, 2022 Google Scholar During the 2022 mpox outbreak, most disease courses were mild. 2 Yon H. Shin H. Shin J.I. et al. Clinical manifestations of human Mpox infection: a systematic review and meta-analysis. Rev Med Virol. 2023; 33e2446https://doi.org/10.1002/rmv.2446 Crossref Scopus (12) Google Scholar In some cases, however, severe outcomes were reported and included hospitalization and death. People living with HIV/AIDS (PLWH) have been disproportionally affected, and all immunocompromised individuals are at a higher risk of severe outcomes. 3 Mitjà O. Alemany A. Marks M. et al. Mpox in people with advanced HIV infection: a global case series. Lancet. 2023; 401: 939-949https://doi.org/10.1016/s0140-6736(23)00273-85 Crossref PubMed Scopus (0) Google Scholar ,4 Benites-Zapata V.A. Ulloque-Badaracco J.R. Alarcon-Braga E.A. et al. Clinical features, hospitalisation and deaths associated with monkeypox: a systematic review and meta-analysis. Ann Clin Microbiol Antimicrob. 2022; 21: 36https://doi.org/10.1186/s12941-022-00527-1 Crossref PubMed Scopus (86) Google Scholar Hospitalization in those diagnosed with mpox ranges from 14% to 59%. 4 Benites-Zapata V.A. Ulloque-Badaracco J.R. Alarcon-Braga E.A. et al. Clinical features, hospitalisation and deaths associated with monkeypox: a systematic review and meta-analysis. Ann Clin Microbiol Antimicrob. 2022; 21: 36https://doi.org/10.1186/s12941-022-00527-1 Crossref PubMed Scopus (86) Google Scholar In cases requiring hospitalization, death was reported in 4% of the cases, but it is as high as 25% in PLWH. 3 Mitjà O. Alemany A. Marks M. et al. Mpox in people with advanced HIV infection: a global case series. Lancet. 2023; 401: 939-949https://doi.org/10.1016/s0140-6736(23)00273-85 Crossref PubMed Scopus (0) Google Scholar ,4 Benites-Zapata V.A. Ulloque-Badaracco J.R. Alarcon-Braga E.A. et al. Clinical features, hospitalisation and deaths associated with monkeypox: a systematic review and meta-analysis. Ann Clin Microbiol Antimicrob. 2022; 21: 36https://doi.org/10.1186/s12941-022-00527-1 Crossref PubMed Scopus (86) Google Scholar Reports of dermatologic findings and associated clinical outcomes in PLWH are limited.
IntroductionCalciphylaxis is a vasculopathy characterized by vascular calcification, ischemia, and subsequent skin necrosis. While uremic calciphylaxis presents in the setting of end-stage renal disease, nonuremic calciphylaxis (NUC) is associated with a variety of factors, including liver disease, warfarin or systemic corticosteroid use, hypercoagulability, and autoimmune diseases. Diffuse dermal angiomatosis (DDA) is a variant of reactive angioendotheliomatosis that is triggered by local ischemia or vascular inflammation, causing the upregulation of vascular endothelial growth factor. Prior reports have demonstrated a rare association of uremic calciphylaxis with DDA.1Ayoubi N. Francois R.A. Braswell D.S. Ramos-Caro F.A. Motaparthi K. Diffuse dermal angiomatosis with clinical features simulating calciphylaxis in the setting of end-stage renal disease.JAAD Case Rep. 2020; 6: 826-828https://doi.org/10.1016/j.jdcr.2020.06.041Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar, 2Steele K.T. Sullivan B.J. Wanat K.A. Rosenbach M. Elenitsas R. Diffuse dermal angiomatosis associated with calciphylaxis in a patient with end-stage renal disease.J Cutan Pathol. 2013; 40: 829-832https://doi.org/10.1111/cup.12183Crossref PubMed Scopus (21) Google Scholar, 3Vavricka B.M.P. Barry C. Victor T. Guitart J. Diffuse dermal angiomatosis associated with calciphylaxis.Am J Dermatopathol. 2009; 31: 653-657https://doi.org/10.1097/DAD.0b013e3181a59ba9Crossref PubMed Scopus (30) Google Scholar Both DDA and calciphylaxis present with painful, purpuric plaques/nodules with necrotic eschars. These similarities can pose a diagnostic and treatment conundrum. We present a case of NUC with histopathologic features of DDA that was responsive to treatment with sodium thiosulfate (STS).Report of a CaseA 41-year-old woman with alcoholic hepatitis and acute kidney injury presented with a 3-month history of painful, indurated, subcutaneous nodules, without overlying cutaneous changes and plaques, with overlying retiform purpura and central black eschars, on the abdomen and both thighs (Fig 1). The patient was a smoker without a history of cardiovascular disease or warfarin use.The initial workup was notable for positive antinuclear antibodies (1:80, speckled), low protein C, and borderline-low antithrombin III. Subsequent testing, including for neutrophil cytoplasmic antibodies, cryoglobulins, serum protein electrophoresis, urine protein electrophoresis, immunofixation electrophoresis, and rheumatoid factor levels, was unremarkable. Given concern for vasculopathy secondary to hypercoagulability, the patient was started on 2.5 mg rivaroxaban daily, then increased to 5 mg daily. This was subsequently dose reduced and ultimately discontinued due to acute anemia from hemorrhoids. As the patient had no history of deep vein thromboses despite numerous thrombotic challenges (4 pregnancies and oral contraceptive use), the low protein C was deemed to have been acquired from liver dysfunction.The initial skin biopsy demonstrated the proliferation of thin-walled vessels in a mixed lobular and diffuse pattern throughout the dermis and subcutis (Fig 2). Von Kossa staining was negative for calcium. After the patient was admitted, a repeat biopsy demonstrated similar findings. An additional, deeper biopsy with sampling of the subcutis also showed zones of diffuse, small vessels surrounded by pericytes in the dermis. Von Kossa staining in the last 2 biopsies showed focal calcium deposition on elastin fibers (Fig 3). Magnetic resonance imaging and plain films demonstrated only soft tissue edema and focal skin thickening, without vessel calcification.Fig 2Diffuse dermal angiomatosis manifesting with many thin, compressed vessels between dermal collagen bundles. (Hematoxylin-eosin stain; original magnification: ×200.)View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 3Sparse calcification of elastin fibers present in the deep dermis and subcutis. (Von Kossa stain; original magnification: ×200.)View Large Image Figure ViewerDownload Hi-res image Download (PPT)Given the suspicion for NUC due to the recent alcoholic hepatitis and acute renal injury, the patient was started on STS infusions (25 g, thrice weekly). Upon treatment initiation, the patient reported a significant reduction in pain. Within 1 week, there was softening of the induration surrounding her lesions and improved healing at the existing ulcers.DiscussionNUC classically affects individuals who are women, White, overweight, or taking warfarin.4Bajaj R. Courbebaisse M. Kroshinsky D. Thadhani R.I. Nigwekar S.U. Calciphylaxis in patients with normal renal function: a case series and systematic review.Mayo Clin Proc. 2018; 93: 1202-1212https://doi.org/10.1016/j.mayocp.2018.06.001Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar Our patient met 3 of these criteria and had alcoholic liver disease, which has also been associated with NUC. Vitamin K antagonism is one of the most common concomitant conditions reported with NUC4Bajaj R. Courbebaisse M. Kroshinsky D. Thadhani R.I. Nigwekar S.U. Calciphylaxis in patients with normal renal function: a case series and systematic review.Mayo Clin Proc. 2018; 93: 1202-1212https://doi.org/10.1016/j.mayocp.2018.06.001Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar; notably, patients with liver disease are at risk of vitamin K deficiency from malabsorption and malnutrition. It is posited that aberrant matrix Gla protein activity, a key inhibitor of calcification activated by vitamin K, may be a driving force in NUC pathogenesis.5Nigwekar S.U. Wolf M. Sterns R.H. Hix J.K. Calciphylaxis from nonuremic causes: a systematic review.Clin J Am Soc Nephrol. 2008; 3: 1139-1143https://doi.org/10.2215/CJN.00530108Crossref PubMed Scopus (294) Google Scholar Liver disease has also been shown to impact the expression of nuclear factor–κB, receptor activator of NF-κB ligand, and osteoprotegrin, which are involved in extraskeletal mineralization.5Nigwekar S.U. Wolf M. Sterns R.H. Hix J.K. Calciphylaxis from nonuremic causes: a systematic review.Clin J Am Soc Nephrol. 2008; 3: 1139-1143https://doi.org/10.2215/CJN.00530108Crossref PubMed Scopus (294) Google Scholar Additionally, decreased protein C levels, as seen in our patient, and decreased protein S levels can occur in the setting of liver disease and may promote local hypercoagulability, leading to NUC.5Nigwekar S.U. Wolf M. Sterns R.H. Hix J.K. Calciphylaxis from nonuremic causes: a systematic review.Clin J Am Soc Nephrol. 2008; 3: 1139-1143https://doi.org/10.2215/CJN.00530108Crossref PubMed Scopus (294) Google ScholarThe incidence of NUC is rising, but its diagnosis remains challenging. DDA may be a feature of calciphylaxis, with studies demonstrating histopathologic features of DDA in biopsies from patients with calciphylaxis.3Vavricka B.M.P. Barry C. Victor T. Guitart J. Diffuse dermal angiomatosis associated with calciphylaxis.Am J Dermatopathol. 2009; 31: 653-657https://doi.org/10.1097/DAD.0b013e3181a59ba9Crossref PubMed Scopus (30) Google Scholar,6McMullen E.R. Harms P.W. Lowe L. Fullen D.R. Chan M.P. Clinicopathologic features and calcium deposition patterns in calciphylaxis: comparison with gangrene, peripheral artery disease, chronic stasis, and thrombotic vasculopathy.Am J Surg Pathol. 2019; 43: 1273-1281https://doi.org/10.1097/pas.0000000000001302Crossref PubMed Google Scholar A recent report highlighted a case with clinical features of uremic calciphylaxis with histopathology suggestive of DDA.1Ayoubi N. Francois R.A. Braswell D.S. Ramos-Caro F.A. Motaparthi K. Diffuse dermal angiomatosis with clinical features simulating calciphylaxis in the setting of end-stage renal disease.JAAD Case Rep. 2020; 6: 826-828https://doi.org/10.1016/j.jdcr.2020.06.041Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar Our case uniquely demonstrates that NUC may present with histopathologic features of DDA and without diagnostic features of calciphylaxis yet still respond to STS treatment.Ischemia appears to be critical in both calciphylaxis and DDA development. While calciphylaxis and DDA are associated, the sequence of events in which they cooccur is unclear. Two possibilities have been posited. First, the conditions that induce initially low-grade local ischemia promote vascular endothelial growth factor production, resulting in DDA, serving as a harbinger of the frank ischemia of calciphylaxis.3Vavricka B.M.P. Barry C. Victor T. Guitart J. Diffuse dermal angiomatosis associated with calciphylaxis.Am J Dermatopathol. 2009; 31: 653-657https://doi.org/10.1097/DAD.0b013e3181a59ba9Crossref PubMed Scopus (30) Google Scholar Second, the vascular occlusion of larger dermal vessels in calciphylaxis causes a compensatory increase in vascular endothelial growth factor that promotes the development of DDA.2Steele K.T. Sullivan B.J. Wanat K.A. Rosenbach M. Elenitsas R. Diffuse dermal angiomatosis associated with calciphylaxis in a patient with end-stage renal disease.J Cutan Pathol. 2013; 40: 829-832https://doi.org/10.1111/cup.12183Crossref PubMed Scopus (21) Google ScholarThere are limited reliable tools to aid in the diagnosis of NUC. We recommend maintaining a high degree of clinical suspicion for NUC in patients with appropriate clinical features. Biopsy sensitivities range from 20% to 86%, influenced by factors such as biopsy location and technique.7Dobry A.S. Nguyen E.D. Shah R. Mihm M.C. Kroshinsky D. The role of skin biopsy in diagnosis and management of calciphylaxis: a retrospective analysis.J Am Acad Dermatol. 2021; 85: 765-767https://doi.org/10.1016/j.jaad.2020.05.101Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar,8Cassius C. Moguelet P. Monfort J.B. et al.Calciphylaxis in haemodialysed patients: diagnostic value of calcifications in cutaneous biopsy.Brit J Dermatol. 2018; 178: 292-293https://doi.org/10.1111/bjd.15655Crossref PubMed Scopus (14) Google Scholar Often, biopsies are nondiagnostic due to inadequate sampling or nonspecific histologic features.9Alniemi D.T. Kanner C. Stowman A.M. et al.Diagnosing calciphylaxis: a series of cases with both imaging and tissue biopsy.J Am Acad Dermatol. 2020; 8 (Published online June 8, 2020. https://doi.org/10.1016/j.jaad.2020.05.111)Google Scholar Although incisional biopsies are recommended, these may not be appropriate when considering patient comfort, infection risk, and wound healing. Plain films, computed tomography, and ultrasound can be useful diagnostic modalities, along with contrast-based vascular imaging to detect calcification.9Alniemi D.T. Kanner C. Stowman A.M. et al.Diagnosing calciphylaxis: a series of cases with both imaging and tissue biopsy.J Am Acad Dermatol. 2020; 8 (Published online June 8, 2020. https://doi.org/10.1016/j.jaad.2020.05.111)Google Scholar, 10Bonchak J.G. Park K.K. Vethanayagamony T. Sheikh M.M. Winterfield L.S. Calciphylaxis: a case series and the role of radiology in diagnosis.Int J Dermatol. 2016; 55: e275-e279https://doi.org/10.1111/ijd.13043Crossref PubMed Scopus (21) Google Scholar, 11Yang H. Ahmed I. Mathew V. Schroeter A.L. Diffuse dermal angiomatosis of the breast.Arch Dermatol. 2006; 142: 343-347https://doi.org/10.1001/archderm.142.3.343Crossref PubMed Scopus (38) Google Scholar Given our patient's acute renal injury, contrast-based imaging was not pursued.This case suggests that empiric calciphylaxis treatment can be initiated in the correct clinical context. Treatments for calciphylaxis are limited. The efficacy of STS has been demonstrated widely, although no randomized controlled trials exist.12Ning M.S. Dahir K.M. Castellanos E.H. McGirt L.Y. Sodium thiosulfate in the treatment of non-uremic calciphylaxis.J Dermatol. 2013; 40: 649-652https://doi.org/10.1111/1346-8138.12139Crossref PubMed Scopus (25) Google Scholar,13Auriemma M. Carbone A. Liberato L.D. et al.Treatment of cutaneous calciphylaxis with sodium thiosulfate: two case reports and a review of the literature.Am J Clin Dermatol. 2011; 12: 339-346https://doi.org/10.2165/11587060-000000000-00000Crossref PubMed Scopus (33) Google Scholar The use of STS in NUC has also been demonstrated and is associated with a survival benefit.12Ning M.S. Dahir K.M. Castellanos E.H. McGirt L.Y. Sodium thiosulfate in the treatment of non-uremic calciphylaxis.J Dermatol. 2013; 40: 649-652https://doi.org/10.1111/1346-8138.12139Crossref PubMed Scopus (25) Google Scholar,14Altman K. Shinohara M. Demographics, comorbid conditions, and outcomes of patients with nonuremic calciphylaxis.JAMA Dermatol. 2019; 155: 251-252https://doi.org/10.1001/jamadermatol.2018.4937Crossref PubMed Scopus (4) Google Scholar Our patient's improvement on STS builds on this prior evidence. STS is thought to act as a chelating agent, dissolving calcium deposits in the blood vessels.12Ning M.S. Dahir K.M. Castellanos E.H. McGirt L.Y. Sodium thiosulfate in the treatment of non-uremic calciphylaxis.J Dermatol. 2013; 40: 649-652https://doi.org/10.1111/1346-8138.12139Crossref PubMed Scopus (25) Google Scholar It is also proposed to have antioxidant properties, ultimately restoring nitric oxide production in endothelial cells to promote vasodilation. Another proposed mechanism includes hydrogen sulfide, an STS metabolite, exerting vasodilatory, antiinflammatory, and analgesic effects.13Auriemma M. Carbone A. Liberato L.D. et al.Treatment of cutaneous calciphylaxis with sodium thiosulfate: two case reports and a review of the literature.Am J Clin Dermatol. 2011; 12: 339-346https://doi.org/10.2165/11587060-000000000-00000Crossref PubMed Scopus (33) Google Scholar Gastrointestinal side effects can preclude patients from receiving optimal doses, and supportive therapies, such as antiemetics, are often required.NUC carries high morbidity and mortality risks yet remains difficult to diagnose. This case asserts that DDA may be considered a diagnostic clue in presentations suspicious for NUC and prompt the timely initiation of therapies. IntroductionCalciphylaxis is a vasculopathy characterized by vascular calcification, ischemia, and subsequent skin necrosis. While uremic calciphylaxis presents in the setting of end-stage renal disease, nonuremic calciphylaxis (NUC) is associated with a variety of factors, including liver disease, warfarin or systemic corticosteroid use, hypercoagulability, and autoimmune diseases. Diffuse dermal angiomatosis (DDA) is a variant of reactive angioendotheliomatosis that is triggered by local ischemia or vascular inflammation, causing the upregulation of vascular endothelial growth factor. Prior reports have demonstrated a rare association of uremic calciphylaxis with DDA.1Ayoubi N. Francois R.A. Braswell D.S. Ramos-Caro F.A. Motaparthi K. Diffuse dermal angiomatosis with clinical features simulating calciphylaxis in the setting of end-stage renal disease.JAAD Case Rep. 2020; 6: 826-828https://doi.org/10.1016/j.jdcr.2020.06.041Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar, 2Steele K.T. Sullivan B.J. Wanat K.A. Rosenbach M. Elenitsas R. Diffuse dermal angiomatosis associated with calciphylaxis in a patient with end-stage renal disease.J Cutan Pathol. 2013; 40: 829-832https://doi.org/10.1111/cup.12183Crossref PubMed Scopus (21) Google Scholar, 3Vavricka B.M.P. Barry C. Victor T. Guitart J. Diffuse dermal angiomatosis associated with calciphylaxis.Am J Dermatopathol. 2009; 31: 653-657https://doi.org/10.1097/DAD.0b013e3181a59ba9Crossref PubMed Scopus (30) Google Scholar Both DDA and calciphylaxis present with painful, purpuric plaques/nodules with necrotic eschars. These similarities can pose a diagnostic and treatment conundrum. We present a case of NUC with histopathologic features of DDA that was responsive to treatment with sodium thiosulfate (STS).
Background: Cutaneous reactions after COV1D-19 vaccination have been commonly reported; however, histopathologic features and clinical correlations have not been well characterized. Methods: We evaluated for a history of skin biopsy all reports of reactions associated with COVID-19 vaccination identified in an international registry. When histopathology reports were available, we categorized them by reaction patterns. Results: Of 803 vaccine reactions reported, 58 (7%) cases had biopsy reports available for review. The most common histopathologic reaction pattern was spongiotic dermatitis, which clinically ranged from robust papules with overlying crust, to pityriasis rosea-like eruptions, to pink papules with fine scale. We propose the acronym "V-REPP" (vaccine-related eruption of papules and plaques) for this spectrum. Other clinical patterns included bullous pemphigoid-like (n = 12), dermal hypersensitivity (n = 4), herpes zoster (n = 4), lichen planus-like (n = 4), pernio (n = 3), urticarial (n = 2), neutrophilic dermatosis (n = 2), leukocytoclastic vasculitis (n = 2), morbilliform (n = 2), delayed large local reactions (n = 2), erythromelalgia (n = 1), and other (n = 5). Limitations: Cases in which histopathology was available represented a minority of registry entries. Analysis of registry data cannot measure incidence. Conclusion: Clinical and histopathologic correlation allowed for categorization of cutaneous reactions to the COVID-19 vaccine. We propose defining a subset of vaccine-related eruption of papules and plaques, as well as 12 other patterns, following COVID-19 vaccination.
This cross-sectional study examines the rates of group A Streptococcus skin and soft tissue infections among persons experiencing homelessness.
To the Editor: We thank the authors for their reply to our article and would like to clarify the concerns described.1McMahon D.E. Amerson E. Rosenbach M. et al.Cutaneous reactions reported after Moderna and Pfizer COVID-19 vaccination: a registry-based study of 414 cases.J Am Acad Dermatol. 2021; 85: 46-55Abstract Full Text Full Text PDF PubMed Scopus (603) Google Scholar The letter noted that the distribution of COVID-19 vaccine skin reactions was unclear and suggested we delineate each reaction pattern as local, distant, or generalized.1McMahon D.E. Amerson E. Rosenbach M. et al.Cutaneous reactions reported after Moderna and Pfizer COVID-19 vaccination: a registry-based study of 414 cases.J Am Acad Dermatol. 2021; 85: 46-55Abstract Full Text Full Text PDF PubMed Scopus (603) Google Scholar While we did not specify the body part affected by each reaction, we would also like to highlight the challenges with adopting the proposed local/distant/generalized approach. Patients often experienced multiple reactions simultaneously, including reactions both local and distant to the injection site. For instance, patients can exhibit concurrent, delayed large local reactions at the injection site and papulovesicles of the elbow/hand.2Blumenthal K.G. Freeman E.E. Saff R.R. et al.Delayed large rocal Reactions to mRNA-1273 vaccine against SARS-CoV-2.N Engl J Med. 2021; 384: 1273-1277Crossref PubMed Scopus (208) Google Scholar Others had local reactions combined with generalized morbilliform eruptions.1McMahon D.E. Amerson E. Rosenbach M. et al.Cutaneous reactions reported after Moderna and Pfizer COVID-19 vaccination: a registry-based study of 414 cases.J Am Acad Dermatol. 2021; 85: 46-55Abstract Full Text Full Text PDF PubMed Scopus (603) Google Scholar We do agree that at the level of the reaction pattern, certain eruptions were typically more generalized (such as morbilliform, urticaria, or vaccine-related eruption of papules and plaques3McMahon D.E. Kovarik C.L. Damsky W. et al.Clinical and pathologic correlation of cutaneous COVID-19 vaccine reactions including V-REPP: a registry-based study.J Am Acad Dermatol. September 10, 2021; https://doi.org/10.1016/j.jaad.2021.09.002Abstract Full Text Full Text PDF Scopus (102) Google Scholar), while others usually occurred at/near the injection site (local injection site reactions and delayed large local reactions). Others are harder to classify with regard to location: erythema multiforme and vasculitis, for example, could be either distal, in one location (for example, hands/feet only) or generalized. Our study also captured an extensive vaccine reactogenicity profile. Reactogenicity is defined as a subset of reactions occurring shortly after vaccination, including local signs and symptoms, such as pain, redness, swelling and induration, and systemic manifestations, such as fever, myalgia, headache, and rash.4Hervé C. Laupèze B. Del Giudice G. Didierlaurent A.M. Tavares Da Silva F. The how's and what's of vaccine reactogenicity.NPJ Vaccines. 2019; 4: 1-11PubMed Google Scholar We described reactogenicity for each dose of the Moderna/Pfizer vaccines, including fatigue, myalgia, headache, fever, arthralgia, nausea, chills, lymphadenopathy, diarrhea, vomiting, nasal congestion, and others.1McMahon D.E. Amerson E. Rosenbach M. et al.Cutaneous reactions reported after Moderna and Pfizer COVID-19 vaccination: a registry-based study of 414 cases.J Am Acad Dermatol. 2021; 85: 46-55Abstract Full Text Full Text PDF PubMed Scopus (603) Google Scholar We would also like to highlight new data on skin reactions to other COVID-19 vaccines, including AZD1222, Johnson & Johnson's Ad26.COV2.S, Sputnik V, and Sinovac-Coronavac (Table I). As of August 16, 2021, the American Academy of Dermatology/International League of Dermatological Societies registry included 2063 cutaneous vaccine reactions from 870 patients. We identified 24 reactions after AZD1222 vaccination, most commonly local erythema and pain. Johnson & Johnson's Ad26.COV2.S, another adenovirus vector vaccine, generated 15 reactions, including pityriasis rosea and alopecia. One individual developed urticaria and pruritus to the Sputnik V vaccine. Our data on skin reactions to other vaccine classes, such as the inactivated virus-based vaccine, Sinovac's CoronaVac, are limited. Four cases of reactions have been reported to the registry, including pityriasis rosea and zoster. Clinical trials frequently lump skin reactions into a nonspecific "rash" category, but real-world data better characterizing these cutaneous manifestations can provide mechanistic clues.Table ICharacteristics of dermatologic vaccine reactions reported after COVID-19 vaccination to the AAD/ILDS COVID-19 Dermatology Registry∗December 24, 2020—August 16, 2021.Moderna dose 1Moderna dose 2Pfizer dose 1Pfizer dose 2Astrazeneca dose 1Astrazeneca dose 2Johnson & JohnsonSputnik V dose 1Sputnik V dose 2Sinovac-Coronavac dose 1Sinovac-Coronavac dose 2Unknown dose 1Unknown dose 2TotalNumber of individuals427214114140921101311921870Patient age (Median, IQR)49 (37-66)47 (37-61)46 (35-56)51 (37-64)49 (44.5-55.5)39.5 (37-42)51 (28-60)-3842 (19-85)4261 (45-75)60 (49-75)-Patient sex (F)366 (85.7%)181 (84.5%)77 (67.5)103 (73.6%)5 (55.5%)1 (50%)10 (90.9%)-0 (0)3 (100%)1 (100%)16 (84.2%)13 (61.9%)776 (89.2%)Number of vaccine reactions99852620822822215023127312063Local reactions Local swelling151971214201000022281 Local erythema1691011315301000032307 Local pain116811414300000012231 Delayed local hypersensitivity reaction230501217200000012314Distal and/or generalized reactions Pruritus138642325202010053263 Urticaria28222421102010002101 Morbilliform2617161510100003281 Zoster195111301100104459 Vesicular148111400000000047 Pityriasis rosea1063520100112031 Pernio/chilblains735700000004026 Erythema multiforme1331410000001124 Bullous disease715900100000023 Erythromelalgia772400000000020 Filler reaction771200000000017 Angioedema653020000000016 Contact dermatitis530500000000114 Vasculitis315100000000111 Alopecia12110020000029 Petechiae13310000000008 Reaction in breast-fed infant01210000000004 Livedo reticularis00300000000014 New dermatologic condition7†Moderna first dose: lichen planus (4); psoriasis (1); possible leukocytoclastic vasculitis (1); Acne vulgaris (1).3‡Moderna second dose: granuloma annulare (1); lichen planus (1); psoriasis (1).1§Pfizer first dose: herpes zoster (1).8‖Pfizer second dose: lichen planus (2), granuloma annulare (1), morphea (1), Raynaud (1), pityriasis lichenoides (1); "lichen striatus versus inflammatory linear verrucous epidermal nevus versus Wolf isotopic response" (1); unspecified toe rash (1).000000002¶Unknown second dose: granuloma annulare (1); sarcoidosis (1).21 Flare of existing dermatologic condition91315910000000249 Other24232023213001012100F, Female; IQR, interquartile range.∗ December 24, 2020—August 16, 2021.† Moderna first dose: lichen planus (4); psoriasis (1); possible leukocytoclastic vasculitis (1); Acne vulgaris (1).‡ Moderna second dose: granuloma annulare (1); lichen planus (1); psoriasis (1).§ Pfizer first dose: herpes zoster (1).‖ Pfizer second dose: lichen planus (2), granuloma annulare (1), morphea (1), Raynaud (1), pityriasis lichenoides (1); "lichen striatus versus inflammatory linear verrucous epidermal nevus versus Wolf isotopic response" (1); unspecified toe rash (1).¶ Unknown second dose: granuloma annulare (1); sarcoidosis (1). Open table in a new tab F, Female; IQR, interquartile range. Poulas and Farsalinos3McMahon D.E. Kovarik C.L. Damsky W. et al.Clinical and pathologic correlation of cutaneous COVID-19 vaccine reactions including V-REPP: a registry-based study.J Am Acad Dermatol. September 10, 2021; https://doi.org/10.1016/j.jaad.2021.09.002Abstract Full Text Full Text PDF Scopus (102) Google Scholar hypothesized the spike glycoprotein from vaccination drives these skin phenomena, but the underlying mechanism is likely multifaceted and may vary by vaccine reaction. For instance, delayed large local reactions suggest a delayed hypersensitivity response to vaccination or a T-cell-mediated response resulting from molecular mimicry to viral epitopes. Other manifestations, such as viral reactivation, bullous pemphigoid, and leukocytoclastic vasculitis, may be explained by off-target immune activation postvaccination.3McMahon D.E. Kovarik C.L. Damsky W. et al.Clinical and pathologic correlation of cutaneous COVID-19 vaccine reactions including V-REPP: a registry-based study.J Am Acad Dermatol. September 10, 2021; https://doi.org/10.1016/j.jaad.2021.09.002Abstract Full Text Full Text PDF Scopus (102) Google Scholar RNA-mediated activation of innate immunity via Toll- and RIG-like receptors could also result in type I interferon release. Analysis of the underlying mechanisms for each pattern of skin reaction and systematic characterization of these cutaneous manifestations are paramount to understanding how these side effects influence vaccine adoption, particularly as additional doses, boosters, and vaccine mixing become increasingly common. Drs Freeman, Hruza, and Fox are part of the American Academy of Dermatology (AAD) COVID-19 Ad Hoc Task Force. Dr French is the President of the ILDS. Dr Freeman is an author of COVID-19 dermatology for UpToDate. Drs Sun, McMahon, and Blumenthal have no conflicts of interest to declare. Authors Singh, Fathy, and Tyagi have no conflicts of interest to declare. Response to McMahon et al's "Cutaneous reactions reported after Moderna and Pfizer COVID-19 vaccination: A registry-based study of four hundred fourteen cases"Journal of the American Academy of DermatologyVol. 86Issue 4PreviewTo the Editor: McMahon et al1 reported cutaneous reactions that occurred after the administration of messenger RNA (mRNA) COVID-19 vaccines. The authors recorded 414 unique patients and observed a broad spectrum of reactions after vaccination, from local injection site reactions and delayed large local reactions to urticaria and morbilliform eruptions. Several unusual reactions (erythromelalgia, pernio/chilblains, filler reactions, and pityriasis rosea-like eruptions) were also observed. All these lesions were characterized as local (near the injection site), according to the authors. Full-Text PDF
The World Health Organization declared the global monkeypox outbreak a public health emergency of international concern in July 2022. In response, the American Academy of Dermatology and International League of Dermatological Societies expanded the existing COVID-19 Dermatology Registry to become the “AAD/ILDS Dermatology COVID-19, Monkeypox, and Emerging Infections Registry.” The goal of the registry is to rapidly collate cases of monkeypox and other emerging infections and enable prompt dissemination of findings to front-line healthcare workers and other members of the medical community. The registry is now accepting reports of monkeypox cases and cutaneous reactions to monkeypox/smallpox vaccines. The success of this collaborative effort will depend on active case entry by the global dermatology community.
To the Editor: Booster doses for COVID-19 messenger RNA (mRNA) vaccines ≥6 months after the completion of a patient’s primary vaccine series are now in use across the globe.1,2 Although cutaneous reactions have been widely reported in response to the original series of mRNA vaccines,3-5 little is known about adverse cutaneous reactions to the administration of booster doses. We sought to evaluate cutaneous side effects after mRNA COVID-19 booster to (1) describe reaction sequences after first, second and booster doses, and (2) characterize cutaneous reaction morphology following vaccine booster administration.
To the Editor: Successful transition from the inpatient environment to the outpatient setting is important for patient care,1 with various medical disciplines improving patient outcomes by facilitating postdischarge follow-up.2,3 Despite many hospitalized dermatology patients requiring close outpatient follow-up, the transition from hospital to clinic has not been studied among dermatology patients.
Background:In summer 2021, several countries including the U.S. authorized COVID-19 mRNA vaccine booster doses ≥6 months after completion of a patient's primary vaccine series. The aim of this study was to characterize vaccine cutaneous reactions following a booster dose of mRNA vaccine reported to the American Academy of Dermatology (AAD) & International League of Dermatologic Societies (ILDS) COVID-19 Dermatology registry. Methods:In December 2020, the AAD/ILDS registry was adapted to include COVID-19 vaccine skin reactions. In September 2021 the registry also solicited COVID-19 vaccine booster reactions either as new cases or updates to existing entries. Results:From Dec 2020-Jan 2022, 994 cases of vaccine skin reactions were entered in the registry, of which 44 records indicated the presence or absence of cutaneous reactions following a booster dose. Of 44 records, 31(71%) developed a cutaneous reaction to the booster dose and 29% developed a reaction to the 1st and/or 2nd dose but not the booster. Of the 31 patients who developed a reaction to the booster dose, 22 reacted to the booster alone, 1 reacted to the 1st & booster, 3 reacted to the 2nd & booster, and 5 reacted to all three doses. The most common morphologies among all booster reactions were local injection site reactions (n=31), delayed large local reaction (n=7), erythromelalgia (n=3), and vesicular reactions (n=3). Conclusion:Booster reactions represent a small portion of COVID vaccine reactions in the registry. Infrequent reporting could be due slow booster uptake, reporter fatigue, and/or booster reactions may truly be less frequent than reactions to the initial series. Dermatologists should be aware that cutaneous reactions to boosters are possible, even when reactions to dose 1 & 2 did not occur;none of the reactions were life-threatening.
Background: Pediculus humanus humanus (body lice) survive on blood meals taken from human hosts. Case reports have described profound iron deficiency anemia associated with extensive body lice infestation, but larger epidemiologic studies have not been performed.