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Introduction Distinct skin lesions associated with coronavirus disease 2019 (COVID-19) have been described, but data regarding their time of onset during the COVID-19 course are scant. Our objective was to systematically review the studies reporting the time of onset of selected skin lesions with respect to the reported onset of the COVID-19 core symptoms. Methods A comprehensive search of studies published before 21 January 2021 was performed on MEDLINE via PubMed database using a predefined strategy to identify relevant articles. Results Out of 354 references, 87 were selected, reporting a total of 895 patients with skin lesions associated with COVID-19. The most frequent pattern was exanthema (n = 430, 48%), followed by vascular (n = 299, 33%), urticarial (n = 105, 12%) and others (n = 66, 7%). Skin lesions occurred more frequently in the first 4 weeks from the COVID-19 onset (n = 831, 92%), whereas prodromal or late lesions were rarer (n = 69, 8%). The urticarial and exanthema patterns were more frequent in the first 2 weeks. About the vascular pattern some differences were noted among its subtypes. Livedoid lesions occurred mainly in the first 2 weeks, while chilblain-like lesions between weeks 2 and 4. Purpuric/petechial lesions were equally distributed during the first 4 weeks. Several skin manifestations did not fall into the pattern classification, including erythema multiforme, generalized pruritus, Kawasaki disease and others. Conclusion The diversity in the time of onset of skin lesions as well as their polymorphic nature likely reflects the diversity of the pathogenetic underlying mechanisms. PROSPERO database registration number CRD42021236331. Supplementary Information The online version contains supplementary material available at 10.1007/s13555-021-00526-8.

FILS syndrome (facial dysmorphism, immunodeficiency, livedo, and short stature) is a rare autosomal recessive disorder caused by pathogenic alterations in the POLE gene leading to multisystemic manifestations, including poorly characterized skin findings. We report a child with a homozygous variant, c.100C > T (p.Arg34Cys), in POLE and features consistent with poikiloderma, expanding the dermatologic signs associated with this rare disorder. Additionally, we review reported cases of FILS syndrome, discuss possible pathomechanisms for our patient's presentation, and consider implications for management.

Idiopathic hypereosinophilic syndrome (IHES) is a rare disorder defined by (1) eosinophilia (eosinophil count > 1.5 × 109/L) on 2 examinations 1 month apart, (2) organ damage and/or dysfunction attributable to tissue hypereosinophilia, and (3) lack of clear alternate cause.1 Eosinophil-related organ damage consists of eosinophilic infiltrates associated with fibrosis, thrombosis, and cutaneous erythema or ulceration. It occurs predominantly in men ages 20 to 50 years.2 Systemic manifestations include cardiomyopathy, neuropathy, hepatosplenomegaly, and thromboembolism, with skin lesions as the dominant symptom in most patients.2 We report a case of skin-limited IHES presenting as retiform purpura and highlight the importance of early diagnosis and intervention in this heterogeneous condition.

The skin is one of the organs most commonly affected by vasculitis and the only one that is open to direct inspection. Cutaneous vasculitis can be part of a systemic vasculitis, a variant restricted to the skin (e.g. systemic and cutaneous IgA1 vasculitis) or an independent cutaneous form (recurrent macular vasculitis in hypergammaglobulinemia, nodular vasculitis). For the nomenclature and classification of cutaneous vasculitides the scheme of the Chapel Hill Consensus Conference can be used and the vessels mainly affected determine the clinical picture of individual forms of vasculitis. Some cutaneous efflorescences and their distribution are so characteristic for certain forms of vasculitis that they provide diagnostic indications or even diagnostic criteria. A palpable purpura on the legs is typical for involvement of postcapillary venules in the context of immune complex vasculitis. If arterioles or venules in the dermis are additionally affected, the clinical presentation is that of plaques with marginal offshoots and central hemorrhagic blisters or necrosis (retiform purpura). In contrast to the purpura in occluding vasculopathies, which shows no or little surrounding erythema, the purpura in vasculitis is accompanied by an inflammatory erythema. In vasculitides of the arterioles and small-caliber arteries in the subcutaneous tissue, visible or only palpable nodules exist surrounded by an irregular livedo or also retiform purpura. Understanding how vasculitis-induced efflorescence arises and the histological manifestations helps their recognition during physical examinations in daily practice and is an important guide in the diagnosis and classification of vasculitides.

Antiphospholipid antibodies syndrome is a recently described condition. The diagnosis of this condition is based on the presence of thrombotic events without previous predisposition and the positivity of anti-phospholipid antibodies. Its clinical presentation includes a variety of patterns, some of which are not included within the clinical criteria of the diagnosis, but must be known. Dermal involvement as livedo is common. However, dermal necrosis is not usual. Thus, a case of antiphospholipid syndrome with dermal necrosis is presented as a primary manifestation of the disease.

Background Livedo vasculopathy is a cutaneous thrombotic microangiopathy favouring the lower legs characterised by an intense reticulate pigmentation, atrophie blanche and recurrent painful stellate ulcerations. There is a strong association with antiphospholipid syndrome, thrombophilic abnormalities, factor V Leiden mutation and more recently, lipoprotein A. Thrombotic occlusion of the dermal venules is the key pathology in livedo vasculopathyand there is no associated vasculitis. Anticoagulation and other currently recommended conservative therapies do not influence the rate of ulcer healing. Method Concurrent venous hypertension was treated with endovenous ablative methods. In addition, vessels deemed to be involved in the process of vasculopathy, irrespective of their reflux status were identified on duplex ultrasound or by sight and ablated using endovenous laser and/or sclerotherapy. Results Five patients received endovenous intervention while two patients did not undergo any procedures. One patient requested a delay in interventions due to her circumstances and the other patient had multiple contraindications and comorbidities. On long-term follow-up, the intervention achieved complete ulcer healing and resolution of pain without any recurrence in four treated patients. Conclusion We recommend ablative venous interventions in suitable patients with livedo vasculopathy to prevent recurrent ulcerations. Compression should be considered as an adjunct treatment.

Evaluation of skin diseases can be challenging for non-dermatologists. Even obvious well-characterized skin pathologies might be misleading and thus treatment can fail. Particularly the differentiation of surgical treated entities is important, for example the management of a wound healing disturbance profoundly differs from that of a pyoderma gangrenosum. This article outlines several easily mistaken pairs of dermatologic entities on one hand and surgical on the other. For example, a livedo vasculopathy can be confused with a leg ulcer, a nail melanoma with a simple hematoma and finally a hidradenitis suppurativa with an axillary abscess. Typical clinical signs and anamnestic data may often lead to the right diagnosis also assisted by the simple fact to “keep it in mind“.

Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS.

Background Sneddon syndrome (SS) is defined by widespread livedo reticularis (LR) and stroke. There is no single diagnostic test for SS and diagnosis can be solely based on clinical features. This cross-sectional case-control study aimed to determine the diagnostic value of skin biopsies in SS patients. Materials and methods We studied skin biopsies from patients with a clinical diagnosis of SS or isolated LR. We also studied controls with vitiligo or normal skin. Biopsies were considered standardized if 3 biopsies were taken from the white centre of the livedo and reached until the dermis-subcutis border. Biopsies were scored for features of an occlusive microangiopathy without knowledge of the clinical features. Sensitivity and specificity of the biopsy findings were calculated with the clinical criteria as the reference standard. Results We included 34 SS patients, 14 isolated LR patients and 41 control patients. Biopsies of 17 patients with SS (50%), 4 with isolated LR (31%) and 10 control patients (24%) showed at least one artery in the deep dermis with a thickened vessel wall combined with recanalization or neovascularization (sensitivity 50% and specificity 69% with LR as reference). Standardized biopsies increased the sensitivity to 70%. In a post hoc analysis the combination of an occlusive microangiopathy and the presence of a livedo pattern in the superficial dermis increased the specificity to 92%. Conclusions Standardized skin biopsies can support the clinical diagnosis of SS. An occlusive microangiopathy as the only positive criterion for the diagnosis of SS had insufficient specificity for a definite diagnosis.

BACKGROUND: The infection by coronavirus disease 2019 (COVID-19) has been associated with multiple cutaneous manifestations, although characterization of them in Hispanic patients with darker skin phototypes is lacking. The objective of this study is to characterize the clinical dermatological manifestations associated with COVID-19 infection in cases with few or without general symptoms in patients from Latin America. METHODS: Cross-sectional study using a questionnaire that was made for health professionals (physicians with a specialty in dermatology) to investigate dermatological lesions associated with COVID-19 infection in patients from 25 countries of Latin America. The survey was active from June 9 to July 30, 2020. RESULTS: In this study, information was collected from a total of 347 patients. We found a female gender predominance: 179/347 (51.6%). The mean age at presentation was 40.87 years. The most frequent dermatological manifestations were maculopapular rash and urticarial lesions, followed by papulovesicular lesions, vesicular lesions, chilblain-like lesions, papular lesions, ecchymosis, petechial purpura, pityriasis rosea-like lesions, pruritus, palmoplantar dysesthesias, transient livedo, acral necrosis, palpable purpura, livedo racemosa, and retiform purpura. As far as we know, there are no previous reports of pruritus and palmoplantar dysesthesias. CONCLUSIONS: This registry emphasizes skin manifestations as an important criterion for establishing the diagnosis of COVID-19 infection in Latin American countries. This information will be useful for the early identification of suspected cases by health professionals (dermatologists and nondermatologists) and will allow contact tracing to mitigate the impact on health systems at different levels

The coronavirus 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has had enormous health, economic, and social consequences. The clinical spectrum of cutaneous manifestations observed in patients with COVID-19 is both heterogeneous and complex. To date, reports have identified 5 main categories: acral lesions, vesicular rashes, urticarial rashes, maculopapular rashes, and livedoid and necrotic lesions. However, these will probably be modified as new information comes to light. Cutaneous manifestations associated with COVID-19 probably reflect the activation of pathogenic pathways by the virus or a response to inflammatory processes, vascular or systemic complications, or even treatments. Familiarity with the cutaneous manifestations of COVID-19 may enable early diagnosis or help guide prognosis.

Coronavirus disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a beta coronavirus with a characteristic S-glycoprotein spike on the cell surface. Initial reports did not include cutaneous manifestations as a feature of COVID-19; however, there is a growing repertoire of reports demonstrating an array of dermatologic manifestations on the skin in children and adults. Dermatologic afflictions have been summarized into different categories several times, with the most recent analysis identifying six clinical patterns: urticaria, maculopapular-morbilliform eruption, papulovesicular exanthem, chilblain-like acral pattern, livedo reticularis-livedo racemosa pattern, and purpuric vasculitic pattern. In children, the dermatologic features appear to occur before or concomitantly with other COVID-19 manifestations. Dermatologists play a key role in diagnosing patients with COVID-19 who may present for the first time unwittingly exhibiting early signs of COVID-19. We have reviewed the current evidence on the dermatologic impact of COVID-19 in both the adult and pediatric populations.

Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the cause of a current pandemic worldwide. This virus can reach all organs and disturbs the immune system, leading to a cytokine storm in severe forms. We aimed to report cutaneous features among coronavirus disease 2019 (COVID-19) hospitalized patients. Methods We performed a cross-sectional study on 1 given day among all patients hospitalized in acute care for COVID-19 and included all patients with cutaneous features. Follow-up 48 hours later was obtained. Results Among 59 adult patients hospitalized on the day of the study in an infectious diseases ward for SARS-CoV-2 infection who were confirmed by molecular assay and/or radiological findings (computed tomography scan), 40 were included. Several cutaneous manifestations were found: macular exanthema (80%), face edema (32%), livedo (13%), urticarial rash (8%), purpura (5%), oral lichenoid lesions (33%), and conjunctivitis (18%). Cutaneous biopsy was performed in 17 patients. Histological findings showed mast cell hyperplasia (100%), superficial perivascular infiltrate of lymphocytes (94%), and superficial edema (47%) consistent with capillary leak. Conclusions Various dermatological signs can be encountered during COVID-19. A macular rash was the most frequent. All cutaneous features could be related to a vascular leak process., This cross-sectional study showed that cutaneous features are various among hospitalized patients with COVID-19. Macular rash was the most frequent and could be predictive of worsening. They seem related to an immune system disturbance, leading to a vascular leak process.

The disease caused by a new coronavirus, which started in 2019, was named COVID-19 and declared a pandemic on March 11, 2020 by the World Health Organization. Although it is true that the first reports emphasized the respiratory manifestations of this disease as an initial clinical presentation, little by little cases with different initial manifestations began to appear, involving other systems. In cases where central nervous system involvement was identified, the most frequent findings were dizziness, headache, and alteration of alertness. Regarding the cardiovascular system, elevation of cardiac biomarkers and myocarditis are one of the most frequent findings. The main gastrointestinal symptoms described so far are: anorexia, nausea, vomiting, diarrhea, abdominal pain and/or discomfort. Venous thromboembolism is a frequent complication and a public health problem. Skin manifestations remain a field of investigation. Maculopapular rashes, reticular livedo, acral gangrene, among others, have been identified. Health personnel must be updated on new clinical findings and the forms of presentation of this partially known disease, which will make it possible to make more accurate and timely diagnoses, thus impacting the prognosis of these patients.La enfermedad causada por un nuevo coronavirus, que inició en 2019, fue denominada COVID-19 y declarada pandemia el 11 de marzo de 2020 por la Organización Mundial de la Salud. Si bien es cierto que los primeros reportes enfatizaron las manifestaciones respiratorias de esta enfermedad como presentación clínica inicial, poco a poco empezaron a aparecer casos con manifestaciones iniciales distintas, involucrando otros sistemas. En los casos en los que se identificó afectación del sistema nervioso central, los hallazgos más frecuentes fueron mareo, cefalea y alteración del estado de alerta. Respecto al sistema cardiovascular, la elevación de biomarcadores cardiacos y la miocarditis son unos de los hallazgos más frecuentes. Los principales síntomas gastrointestinales descritos hasta el momento son anorexia, náuseas, vómitos, diarrea y dolor o disconfort abdominal. La tromboembolia venosa es una complicación frecuente y un problema de salud pública. Las manifestaciones cutáneas siguen siendo un campo de investigación. Se han identificado exantemas maculopapulares, livedo reticular y gangrena acral, entre otros. El personal sanitario debe estar actualizado sobre los nuevos hallazgos clínicos y las formas de presentación de esta enfermedad solo parcialmente conocida, lo que permitirá hacer diagnósticos más precisos y oportunos, y así impactar en el pronóstico de estos enfermos.

A new type of coronavirus (coronavirus disease 2019; COVID-19), which emerged in the People's Republic of China, spread all over the world over time and became a pandemic. Dermatological symptoms seen during the course of the disease have gained importance over time. Studies have shown that many dermatological findings such as erythematous rash, urticaria, pseudo-chilblain, maculopapular, livedo/necrosis, and vesicular lesions may accompany the disease. In this study, a 24-year-old female patient with maculopapular lesions who had no previous history of allergy or dermatological disease and regressed without any dermatological treatment is presented.

Background COVID-19 cutaneous manifestations have been recently described and classified in five different clinical patterns, including acral erythema-edema (pseudo-chilblain), maculopapular exanthemas, vesicular eruptions, urticarial lesions, and livedo or necrosis. Objectives The objective of this study was to examine the skin of hospitalized patients with a confirmed diagnosis of COVID-19 disease and describe the real prevalence of skin manifestations. Methods A cross-sectional study, which included hospitalized patients in Cruces University Hospital from April 14-30, 2020, with a laboratory-confirmed diagnosis of COVID-19 (with polymerase chain reaction and/or serology tests), was conducted. Entire body surface examination was performed by experienced dermatologists to search for cutaneous manifestations related to COVID-19 disease. Results From a sample of 75 patients, 14 (18.7%) developed cutaneous manifestations possibly related to COVID-19. We found six patients with acral erythema-edema (pseudo-chilblain) (42.8%), four patients with maculopapular exanthemas (28.6%), two patients with urticarial lesions (14.3%), one patient with livedo reticularis-like lesions (7.15%), and one patient with vesicular eruption (7.15%). Conclusions Our study provides a more plausible relationship between the main cutaneous patterns and COVID-19 in hospitalized patients as all of them had a confirmatory laboratory test. Skin manifestations are frequent but mild with spontaneous resolution. These findings are nonspecific and can be similar to other viral infections and adverse drug reactions in hospitalized patients.

Coronavirus 19 (COVID‐19) was declared as a pandemic viral infection by the World Health organization on March 11th 2020. Usual clinical manifestations of COVID‐19 infection include fever, fatigue, myalgia, headache, diarrhea, dry cough, dyspnea that may lead to acute respiratory distress syndrome and death (1). Skin symptoms of COVID‐19 have been poorly described but may include erythematous rash, urticaria and chicken pox like lesions (2‐7). Angiotensin‐converting enzyme 2 (ACE2) is a cellular receptor for COVID‐19.

Since December 2019, coronavirus disease 2019 (COVID‐19) has spread worldwide to become a pandemic. Multiple skin manifestations related to the infection have been described progressively. Recalcati1 asserted that 20.4% of infected patients developed cutaneous manifestations and Galvan‐Casas et al2 have recently proposed 5 clinical patterns (pseudo‐chilblain, vesicular, urticarial, maculopapular and livedo/necrosis). We report a case of COVID‐19 with retiform purpura and its histopathological correlation.

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.

Skin manifestations have been increasingly reported in the setting of COVID‐19. However, their incidence and presentation are debated, and the role, direct or undirect, of SARS‐CoV‐2 in their pathogenesis has yet to be determined. In this work, we aimed to analyze our experience in a French referral center and to perform a systematic review of the literature to evaluate the incidence and prognosis of cutaneous lesions observed in COVID‐19 patients. Cutaneous manifestations were assessed in COVID‐19 patients admitted to Cochin Hospital (Paris, France) between March 16th and April 27th 2020. 759 confirmed moderate‐to‐severe COVID‐19 cases were diagnosed in our institution. Eight patients (1%, 6 males, 2 females, mean age 55.6) presented with skin lesions, mainly disseminated maculo‐papular exanthema, but also digitate papulo‐squamous rash (reported in1), herpes recurrence, papulo‐vesicular rash and Grover’s disease. The mean delay between respiratory/systemic and dermatological signs was 13 days.

Background The infection caused by the recently identified SARS-CoV-2, called coronavirus disease-19 (COVID-19), has rapidly spread throughout the world. With the exponential increase of patients worldwide, the clinical spectrum of COVID-19 is being better defined and new symptoms are emerging. Numerous reports are documenting the occurrence of different cutaneous manifestations in patients with COVID-19. Objectives To provide a brief overview of cutaneous lesions associated with COVID-19. Methods A literature search was performed in the PubMed, Scopus and Web of Science databases up to 30 April 2020. This narrative review summarizes the available data regarding the clinical and histological features of COVID-19-associated skin manifestations. Results The literature reports showed a great heterogeneity in COVID-19-associated cutaneous manifestations, as well as in their latency periods and associated extracutaneous symptoms. Pathogenic mechanisms are unknown, although the roles of a hyperactive immune response, complement activation and microvascular injury have been hypothesized. Based on our experience and the literature data, we subdivided the reported cutaneous lesions into six main clinical patterns: (i) urticarial rash; (ii) confluent erythematous-maculopapular-morbilliform rash; (iii) papulovesicular exanthem; (iv) chilblain-like acral pattern; (v) livedo reticularis-livedo racemosa-like pattern; and (vi) purpuric 'vasculitic' pattern. These six patterns can be merged into two main groups: the first - inflammatory and exanthematous - includes the first three groups listed above, and the second includes the vasculopathic and vasculitic lesions of the last three groups. Conclusions The possible presence of cutaneous findings leading to suspect COVID-19 puts dermatologists in a relevant position. Further studies are needed to delineate the diagnostic and prognostic values of such cutaneous manifestations.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) represents a new public health problem, with a total of 10.577.263 documented COVID-19 cases worldwide and 513.441 deaths up to the present date. Few cases of disease-related cutaneous manifestations have been reported in the literature, and such manifestations are scarce. Integumentary manifestations from COVID-19 include exanthemas and papular dermatoses, urticarial eruptions, atopic dermatitis, vesiculobullous lesions and skin signs of hypercoagulable states, such as acral ischaemia, livedo and retiform purpura. Most common extracutaneous manifestations from the disease include headache, cough, anosmia, ageusia, fever, dyspnoea, nausea, diarrhoea and cardiovascular events. The objectives of this review were to discuss the role of human cell receptors described as interaction targets of SARS-CoV-2, as well to understand the current state of knowledge on skin expression of these receptors, in order to substantiate future research. The authors present a thorough literature review on SARS-CoV-2 and its possible interaction with cell receptors and human tissues including the skin. They discuss a molecular hypothesis to explain the lower prevalence of dermatological manifestations from direct SARS-CoV-2 infection. Distinct human cell receptors binding the virus appear to be less expressed in the skin compared to other organs. Additionally, the presence of resolvins and the disintegrin metalloprotease ADAM17 provide a putative protection to the skin, explaining the majority of COVID-19 manifestations to be extracutaneous. This review represents an excellent opportunity for future studies using skin biopsies from COVID-19 patients to investigate molecular expression in the pathophysiology of cutaneous manifestations of the disease.

La pandemia por SARS-CoV-2 ha causado un gran impacto desde el punto de vista sanitario, económico y social. La semiología dermatológica se ha demostrado heterogénea y compleja. En la actualidad se han definido cinco grupos principales de manifestaciones cutáneas asociadas a la COVID-19: lesiones acrales, exantemas vesiculares, erupciones urticariales, exantemas maculopapulares y lesiones livedoides/necróticas. Sin embargo, es probable que esta clasificación se modifique en el futuro. La clínica cutánea es probablemente el reflejo de distintas vías patogénicas con implicación variable de la infección vírica, del proceso inflamatorio, de las complicaciones vasculares o sistémicas de la enfermedad o incluso de los tratamientos administrados. El conocimiento de las manifestaciones cutáneas puede permitir un diagnóstico precoz o incluso servir como marcador pronóstico.

Cutaneous vasculitis is an inflammatory disease affecting the dermal blood vessel walls. The skin is a privileged organ in the setting of vasculitis since it is easily accessible for physical examination and safe biopsy, allowing an accurate characterization of inflammatory lesions. The skin is often involved. Also, cutaneous vasculitis can reflect a cutaneous component of a systemic vasculitis, a skin-limited or skin-dominant expression or variant of a systemic vasculitis, or be a single-organ vasculitis per se. Vasculitis lesions are multiple and polymorphic. They may induce a wide spectrum of clinical manifestations depending on the location and the size of the vessels involved. The depth of affected vessels is correlated with the type of cutaneous lesions. Involvement of small superficial vessels results mostly in urticarial, but relatively persistent plaques, papules, and palpable purpura. Involvement of vessels in the dermohypodermic junction or hypodermis results in ulcers, nodules, or livedo. The type of inflammatory infiltrate is also a key finding for the diagnosis of cutaneous vasculitis. Leukocytoclastic vasculitis is not a disease per se but the result of a pathophysiological process common to different causes. A better knowledge of the vascular anatomy of the skin, elementary lesions, and histological characteristics of dermatologic manifestations would allow a more relevant and more efficient diagnostic approach. We also propose a list of additional exams to be performed in front of skin lesions suggestive of vasculitis. The aim of our article is to provide an overview of elementary skin lesions and clinicopathologic correlations in cutaneous and systemic vasculitis.

Livedoid vasculopathy is a rare, chronic-recurrent occlusive disorder in the microcirculation of dermal vessels. The clinical appearance is characterized by Livedo racemosa , painful ulceration, located in the distal parts of the lower extremities, followed by healing as porcelain-white, atrophic scars, the so-called Atrophie blanche . Different conditions that can promote a hypercoagulable state, such as inherited and acquired thrombophilias, autoimmune connective-tissue diseases and neoplasms, can be associated with livedoid vasculopathy. Therefore, livedoid vasculopathy is currently considered to be a coagulation disorder, clearly distinguished from inflammatory vasculitis. Although there are hints to hypercoaguability and secondary inflammation, pathophysiology is not completely understood. Diagnosis is made by synopsis of history, clinical and histopathological findings. Early and adequate therapy is essential to maintain life quality and avoid irreversible complications. Better understanding of molecular mechanisms is required to establish appropriate therapy regimens. This article presents the current state of knowledge about livedoid vasculopathy and proposes an algorithmic approach for diagnosis and therapy., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Burg, Mitschang, Goerge and Schneider.)

Sarcoidosis is a multisystem granulomatous disease of unknown etiology. It can affect all age groups, but it is relatively rare in children.1 Clinical manifestations can vary substantially depending on multiple factors, such as the patient's age and the different organs involved. Infants and children younger than 4 years of age usually present with the triad of cutaneous eruption, uveitis, and arthritis, without classic pulmonary disease.1, 2 Cutaneous sarcoidosis is rare and can manifest with different clinical presentations but rarely with livedo reticularis–like eruption.3, 4, 5 However, older children have a classic presentation resembling that of adults, which includes involvement of the lungs and lymph nodes predominantly.6 It can also be accompanied by generalized constitutional symptoms, such as fever, fatigue, malaise, and weight loss. The diagnosis of sarcoidosis is mainly established by confirming a typical noncaseating granuloma on a tissue biopsy specimen and excluding other granulomatous diseases clinically, microbiologically, and histologically. Other causes of granulomatous disease include infections, such as tuberculosis, autoimmune diseases (eg, Sjogren syndrome and Behcet disease), neoplasms (eg, lymphoma), and other diseases.7, 8, 9 There is no significant difference between childhood sarcoidosis and the adult type, although most children have a better prognosis.1 We describe a unique clinical variant of cutaneous sarcoidosis manifesting as livedo reticularis–like eruption in a pediatric patient with a history of intermittent joint pain, cranial nerve involvement, and uveitis for 1 year.

We report a 40-year-old female. The high blood pressure was found in Family history, hospitalized in neurology for recurrent ischemic stroke so the etiological assessment was negative, opinion was sought for lesions of livedo at the level of the members back to 2 years without the concept of miscarriage or Raynaud’s phenomenon, neither photosensitivity nor a dry syndrome. Dermatologic examination showed dusky erythematous to violaceous, irregular, broken circular segments, resulting in a seemingly larger pattern, located on limbs, trunk, and buttocks, exaggerated by the cold and persistent on warming. A report looking for the antiphospholipid antibodies syndrome was positive and the skin biopsy was in favor of a Sneddon syndrome. The patient was treated by anticoagulants and antiplatelet agents with good evolution.

The deficiency of adenosine deaminase 2 (DADA2) has recently been defined as a monogenetic autosomal recessive autoinflammatory disease. DADA2 is mainly characterized by high fever, livedo racemose, early-onset stroke, mild immunodeficiency and clinically polyarteritis nodosa (PAN)-like symptoms. Mutations in CECR1 (cat eye syndrome chromosome region, candidate 1) are responsible for DADA2. Livedoid racemose, lacunar infarct due to involvement in small vessel of the central nervous system, peripheral neuropathy, digital ulcers and loss of fingers are predominantly seen in the disease which could progress to end-stage organ failure and death in some patients. A wide spectrum of severity in phenotype as well as in the age of onset has been reported in the literature. This phenotypic variability is also found in our clinical practice even in patients with the same mutation. Here, we present a family diagnosed with DADA2, with the previously reported p.Gly47Arg mutation in CECR1.

New cases of pneumonia, which spread rapidly and are deadly, have spread throughout the world. This disease is known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) or corona virus disease 2019 (COVID-19) with a viral etiology in the form of novel coronavirus 2019 (2019-nCoV). This disease has extensive clinical manifestations starting from the lungs, hematology system, heart, nerves, reproduction, and the skin. The thing to be aware of is that the skin manifestations in the form of atypical lesions are often found in COVID-19. Common skin manifestations on COVID-19 are erythema-edema with vesicles or pustules (pseudo-chilblain), vesicular eruption, maculopapular rash, urticaria, and livedo lesion. This literature review discusses comprehensively what kind of skin lesions can be suspected of being part of a COVID-19 infection. This literature review is useful for increasing the awareness of doctors about COVID-19 infection and may help in early diagnose of COVID-19 infection and prevent further transmission.

Introduction: Ever since the outbreak of COVID-19, the respiratory system has been the chief focus of researches, however, understanding the impact of this disease on the integumentary system is just as essential. Objectives: We aimed at collecting data on any cutaneous manifestation arising in patients with active and recovering COVID-19 infection, or a direct consequence of the infection's treatment, and correlating these findings with systemic disease severity and duration. Materials and Methods: A prospective observational study was conducted in three tertiary care centers from Rajasthan, India, to acquire data of laboratory-confirmed cases of COVID-19 presenting with any mucocutaneous manifestation. Results: Eight predominant patterns of dermatological involvement were seen, namely, maculopapular (14.59%), urticarial (13.17%), perniotic (12.1%), pityriasis rosea (11.74%), acral erythema/edema (10.3%), petechial (4.63%), vesicular (2.49%), and livedo (1.78%). Rare findings included eruptive pseudoangioma, eruptive hypomelanosis, alopecia parvimaculata, geographic tongue, chikungunya-like hyperpigmentation, and nail changes. On correlating these findings with the gradient of the disease, livedo, vasculitis, exfoliation, and erythroderma were associated with severe disease, whereas perniosis and eruptive pseudoangioma were seen in mild illness. Conclusion: We reported a few previously unpublished skin manifestations of COVID-19, namely, geographic tongue, chikungunya-like pigmentation, eruptive hypomelanosis, and alopecia parvimaculata. This study provides a visual description of the muco-cutaneous manifestations of COVID-19 disease which could aid a dermatologist or physician in early diagnosis of this novel infection, especially in a resource-poor setting.

A 71-year-old man presented with a high fever, polyarthralgia, petechiae and palpable purpura accompanied by livedoid change on his legs and feet. Histopathological findings of the purpura revealed perivascular infiltration of neutrophils, mononuclear cells, and nuclear debris, and extravasation of red cells mainly in the upper dermis: all signs consistent with leukocytoclastic vasculitis. Small vessel thrombi, which are characteristic features of septic vasculopathy, were also observed. Direct immunofluorescence showed negative results. Blood culture revealed the growth of gram-negative bacilli. Subsequently, 16S rRNA sequencing of DNA confirmed the organism as Streptobacillus moniliformis, which is the causative pathogen of rat-bite fever. He had frequently encountered wild rats in his house although there was no evidence of rat bite on his body. Empiric therapy with intravenous administration of ceftriaxone in combination with azithromycin hydrate led to a prompt resolution of the symptoms. Precise history-taking related to contact with rats and detection of skin eruptions suggestive of leukocytoclastic vasculitis on the extremities, especially on the feet, can be clues to Streptobacillus moniliformis infection. Familiarity with its cutaneous features is important for early diagnosis; the evidence herein may also help in understanding its underlying pathogenesis.

Key teaching points •DADA2 is an autosomal recessive syndrome characterized by recurrent fever, early-onset stroke, livedo racemosa, PAN, and hepatosplenomegaly. •Preliminary experience suggests anti–TNF-α therapy may prevent further CVA, improve cutaneous PAN, and improve hepatobiliary function, but further study is needed. •Early identification and confirmation of mutations in the CECR1 gene can potentially decrease morbidity from this disease by guiding selection of appropriate therapy early in the course of disease.

Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62-year-old female was diagnosed with sarcoidosis by chest-abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non-caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis.