RESUMEN
Retiform purpura consists of branching purpuric lesions caused by a complete blockage of blood flow in the dermal/subcutaneous vasculature. It is an acute life-threatening disorder characterized by intravascular thrombosis and hemorrhagic infarction of the skin complicated with disseminated intravascular coagulation. It is commonly seen in acute infections following meningococcal and streptococcal infections. Few cases have been described of retiform purpura following rickettsial infections in the literature and rarely with this magnitude. The purpose of this report is to highlight the possibility of this situation in critically ill patients. We report a case of a 62-year-old woman admitted in the intensive care unit with septic shock from Rickettsia conorii. She developed quickly an intense retiform purpura requiring several surgical interventions to control the necrosis. The dermatologic situation was only completely controlled with treatment of underlying situation. This is a severe complication of a critical infectious disease. (AU)
La púrpura retiforme consiste en lesiones purpúricas ramificadas causadas por un bloqueo completo del flujo sanguíneo en la vasculatura dérmica/subcutánea. Es un trastorno agudo potencialmente mortal caracterizado por trombosis intravascular e infarto hemorrágico de la piel complicado con coagulación intravascular diseminada. Se ve comúnmente en infecciones agudas después de infecciones meningocócicas y estreptocócicas. Se han descrito pocos casos de púrpura retiforme tras infecciones por rickettsiosis en la literatura y rara vez de esta magnitud. El propósito de este informe es resaltar la posibilidad de esta situación en pacientes críticamente enfermos. Presentamos el caso de una mujer de 62 años ingresada en la unidad de cuidados intensivos con shock séptico por Rickettsia conorii. Rápidamente desarrolló una intensa púrpura retiforme que requirió varias intervenciones quirúrgicas para controlar la necrosis. La situación dermatológica solo se controló por completo con el tratamiento de la situación subyacente. Esta es una complicación grave de una enfermedad infecciosa crítica. (AU)
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Rickettsia conorii , Púrpura , Enfermedades Transmisibles/complicaciones , NecrosisRESUMEN
BACKGROUND: Antiphospholipid syndrome (APS), defined by thrombotic events or obstetric complications in the presence of persistently high antiphospholipid antibodies, is characterized by a wide variety of clinical presentations and the effects of vascular occlusion can impact almost any organ system or tissue. Since adult-onset APS classification criteria are not well verified in pediatrics (where pregnancy-related problems are rare), estimating childhood prevalence is challenging. Stroke and pulmonary embolism are thromboembolic events occurring in children that can cause considerable long-term morbidity. Children with APS are more prone to recurrent thromboembolism than adults. Cutaneous symptoms are prominent and typically represent the first clue of APS. Although dermatologic findings are exceedingly heterogeneous, it is essential to consider which dermatological symptoms justify the investigation of antiphospholipid syndrome and the required further management. CASE PRESENTATION: We describe a seven-year-old Iranian boy with retiform purpura and acral cutaneous ischemic lesions as the first clinical presentation of antiphospholipid syndrome in the setting of systemic lupus erythematous. CONCLUSION: APS in pediatrics, is associated with a variety of neurologic, dermatologic, and hematologic symptoms. Therefore, it is essential for pediatricians to be aware of the rare appearance of Catastrophic APS as an initial indication of APS.
Asunto(s)
Síndrome Antifosfolípido , Embolia Pulmonar , Accidente Cerebrovascular , Adulto , Masculino , Femenino , Humanos , Niño , Embarazo , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Irán , Accidente Cerebrovascular/etiologíaRESUMEN
Levamisole exposure in cocaine users is a well-recognized cause of retiform purpura, a distinctive net-like maculopapular patch. Prolonged exposure to levamisole can lead to a serious systemic syndrome known as levamisole-induced vasculitis, most commonly involving the kidneys and lungs. More recently, retiform purpura has been observed in patients with the novel coronavirus disease of 2019 (COVID-19). Due to their overlapping dermatologic and systemic manifestations, levamisole-induced and COVID-19-induced retiform purpura may mimic one another in clinical presentation. The possibility that patients may present with one or both syndromes creates a diagnostic challenge. This review of levamisole-induced and COVID-19-induced retiform purpura highlights their corresponding and distinctive features. Additionally, we propose a unique staging system for levamisole-induced retiform purpura that may be valid for future classification of COVID-19-induced retiform purpura.
Asunto(s)
COVID-19 , Levamisol , Púrpura , Humanos , COVID-19/complicaciones , Levamisol/efectos adversos , Púrpura/inducido químicamente , Púrpura/diagnósticoRESUMEN
BACKGROUND: Clinical data regarding cutaneous manifestations in Chinese patients with livedoid vasculopathy (LV) are limited. OBJECTIVES: To assess clinical features of skin lesions in LV, especially the characteristics of extensive livedo reticularis and pigmented purpuric dermatosis-like lesions in these patients. METHODS: This was a single-center retrospective study of 46 Chinese patients with LV between March 2021 and July 2021. The characteristics of skin lesions in LV were described in detail. RESULTS: A total of 29 females and 17 males were included in this study, with a mean age of 27.7 years (ranging from 13 to 51 years). Twenty (43.5%) patients developed their first skin lesions before age 18. Among 46 patients, 33 presented livedo reticularis with 78.8% (n = 26) of these patients whose livedo reticularis was extensive. Seven patients had lesions simulating pigmented purpuric dermatosis, including four cases of pigmented purpura and three cases of telangiectatic purpura. Numbness was found in 16 patients, mainly in the lower limbs (62.5%), ankles (31.3%), and dorsum of the feet (18.8%). CONCLUSIONS: For patients with symptoms of extensive livedo reticularis, retiform purpura, or numbness, it is necessary to make a differential diagnosis.
Asunto(s)
Livedo Reticularis , Enfermedades de la Piel , Masculino , Femenino , Humanos , Adulto , Adolescente , Livedo Reticularis/diagnóstico , Hipoestesia , Pueblos del Este de Asia , Estudios RetrospectivosRESUMEN
Livedoid vasculopathy (LV) is a thrombo-occlusive vasculopathy that involves the dermal vessels. Clinically, it is characterized by the presence of painful purpuric ulcers on the lower extremities. Histopathologically, it shows intraluminal fibrin deposition and thrombosis, segmental hyalinization, and endothelial proliferation. It is important to notice that the term "atrophie blanche" is descriptive and it includes not only patients with LV but also patients with a combination of vasculitis and vasculopathy, that is, LV and medium-sized vasculitis such as cutaneous polyarteritis nodosa (PANc). Diagnosis is based on a proper clinicopathological correlation, excluding the main differential diagnosis and considering vasculitis as a mimicker or concomitant diagnosis. Coagulation disorders must also be studied although they are not found in all LV. Its frequency is reviewed as well. Treatment of LV is challenging, and different therapies have been attempted. Among them, pain management, wound care, control of cardiovascular risk factors, and both antiplatelets and anticoagulants, mostly rivaroxaban, are the main therapies used. These different therapies as well as their degree of evidence are reviewed.
RESUMEN
A young man was treated in hospital for sepsis, disseminated intravascular coagulation and multi-organ failure. He was a regular intranasal cocaine user up to 1 day prior to symptom onset. Clinical examination revealed extensive retiform purpura affecting both his lower limbs. Skin biopsy revealed widespread thrombosis in the small- and medium-sized vessels of the mid dermis and the subcutaneous fat with surrounding leucocytoclasis. There was also extensive ischaemic necrosis of the upper reticular and papillary dermis and focal ischaemic necrosis of the epidermis. These findings were in keeping with a thrombotic vasculopathy with associated cutaneous ischaemic necrosis, likely associated with levamisole-adulterated cocaine (LAC). An autoimmune screen showed extremely raised levels of anti-B2-glycoprotein IgM, IgG and anti-cardiolipin IgG antibodies, usually seen in antiphospholipid syndrome (APS). The literature describes how APS could be secondary to various underlying conditions, including LAC, and that levamisole toxicity may mimic APS and hence be missed. LEARNING POINTS: Levamisole is a common adulterant found in cocaine; the resultant toxicity can present with cutaneous manifestations, namely retiform purpura and skin necrosis, similar to antiphospholipid syndrome.Patients presenting with such features should be asked about illicit drug use, specifically cocaine, and investigated by screening urine for drugs of abuse and serum antihuman elastase antibody when possible.
RESUMEN
BACKGROUND: The abundance of publications of COVID-19-induced chilblains has resulted in a confusing situation. METHODS: This is a prospective single-institution study from 15 March to 13 May 2020. Thirty-two patients received PCR nasopharyngeal swabs. Of these, 28 patients had a thoracic CT-scan, 31 patients had blood and urine examinations, 24 patients had skin biopsies including immunohistochemical and direct immunofluorescence studies, and four patients had electron microscopy. RESULTS: COVID-19-induced chilblains are clinically and histopathologically identical to chilblains from other causes. Although intravascular thrombi are sometimes observed, no patient had a systemic coagulopathy or severe clinical course. The exhaustive clinical, radiological, and laboratory work-up in this study ruled-out other primary and secondary causes. Electron microscopy revealed rare, probable viral particles whose core and spikes measured from 120 to 133 nm within endothelium and eccrine glands in two cases. CONCLUSION: This study provides further clinicopathologic evidence of COVID-19-related chilblains. Negative PCR and antibody tests do not rule-out infection. Chilblains represent a good prognosis, occurring later in the disease course. No systemic coagulopathy was identified in any patient. Patients presenting with acral lesions should be isolated, and chilblains should be distinguished from thrombotic lesions (livedo racemosa, retiform purpura, or ischemic acral necrosis).
Asunto(s)
COVID-19/complicaciones , COVID-19/diagnóstico , Eritema Pernio/etiología , Eritema Pernio/patología , Dedos del Pie/patología , Adolescente , Adulto , Anciano , Biopsia/métodos , COVID-19/metabolismo , COVID-19/virología , Eritema Pernio/diagnóstico , Eritema Pernio/virología , Niño , Diagnóstico Diferencial , Glándulas Ecrinas/patología , Glándulas Ecrinas/ultraestructura , Glándulas Ecrinas/virología , Endotelio/patología , Endotelio/ultraestructura , Endotelio/virología , Femenino , Humanos , Livedo Reticularis/patología , Masculino , Microscopía Electrónica/métodos , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Púrpura/patología , SARS-CoV-2/genética , Piel/patología , Dedos del Pie/virología , Adulto JovenRESUMEN
Retiform purpura has been described as a relatively frequent cutaneous finding in patients with coronavirus disease 2019 (COVID-19). The etiology is hypothesized to be related to thrombotic vasculopathy based on lesional biopsy specimen findings, but the pathogenesis of the vasculopathy is not completely understood. Here, we present a case of a retiform purpuric patch on the sacrum/buttocks in a hospitalized patient prior to subsequent diagnosis of COVID-19 and an eventual fatal disease course. Two lesional biopsy specimens at different time points in the disease course revealed thrombotic vasculopathy, despite therapeutic anticoagulation. Detailed histopathologic evaluation using immunohistochemical markers suggest the etiology of the vasculopathy involves both persistent complement activation and platelet aggregation, which possibly promote ongoing thrombus formation. This case highlights that sacral/buttock retiform purpuric patches may be a presenting sign of infection with SARS-CoV-2 virus and may represent an ominous sign supporting a future severe disease course. In addition, biopsy specimen findings at separate time points demonstrate that cutaneous vasculopathy may persist despite adequate systemic anticoagulation, possibly due to the combination of persistent complement and platelet activation. Finally, occlusive thrombi in sacral/buttock retiform purpuric patches may contribute to future ulceration and significant cutaneous morbidity in patients who survive COVID-19.
Asunto(s)
Nalgas/patología , COVID-19/complicaciones , COVID-19/patología , Púrpura/diagnóstico , Sacro/patología , Anciano , Anticoagulantes/uso terapéutico , Biopsia/métodos , Nalgas/virología , COVID-19/diagnóstico , COVID-19/inmunología , Calcifilaxia/diagnóstico , Activación de Complemento/inmunología , Diagnóstico Diferencial , Progresión de la Enfermedad , Resultado Fatal , Femenino , Humanos , Pacientes Internos , Agregación Plaquetaria/inmunología , Púrpura/virología , SARS-CoV-2/genética , SARS-CoV-2/aislamiento & purificación , Sacro/virología , Piel/patología , Enfermedades Cutáneas Vasculares/etiología , Enfermedades Cutáneas Vasculares/patologíaRESUMEN
The skin often provides initial clues of hypercoagulability with features such as livedo reticularis, livedo racemosa, retiform purpura, necrosis, and ulcerations. Because these cutaneous manifestations are nonspecific, laboratory testing is often needed to evaluate for underlying causes of hypercoagulability. Importantly, these disorders are reported to be the most common mimicker, resulting in an erroneous diagnosis of pyoderma gangrenosum. Understanding inherent properties of, and indications for, available tests is necessary for appropriate ordering and interpretation of results. Additionally, ordering of these tests in an indiscriminate manner may lead to inaccurate results, complicating the interpretation and approach to management. This second article in this continuing medical education series summarizes information on methodology, test characteristics, and limitations of several in vitro laboratory tests used for the work up of hypercoagulability and vasculopathic disease as it pertains to dermatologic disease.
Asunto(s)
Enfermedades de la Piel/sangre , Enfermedades de la Piel/diagnóstico , Trombofilia/sangre , Trombofilia/diagnóstico , Técnicas de Laboratorio Clínico , Humanos , Enfermedades de la Piel/etiología , Trombofilia/complicacionesRESUMEN
ABSTRACT CONTEXT: Various skin manifestations have been reported in coronavirus disease. It may be difficult to determine the etiology of these lesions in view of the increased frequency of handwashing during the pandemic, along with occurrences of irritant contact dermatitis and allergic contact dermatitis due to disinfectant use; usage of herbal medicine and supplements to strengthen the immune system; and urticarial or maculopapular drug eruptions due to COVID-19 treatment. The variety of associated skin manifestations seen with COVID-19 makes it challenging to identify virus-specific skin manifestations. Petechiae, purpura, acrocyanosis and necrotic and non-necrotic purpura, which can be considered as manifestations of vascular involvement on the skin, have been reported. CASE REPORT: Here, we report a case of eruptive cherry angiomas, which was thought to have developed due to COVID-19, with a papulovesicular rash on distal extremities that progressed over time to reticular purpura. CONCLUSION: The case presented had a papulovesicular rash at the onset, which evolved to retiform purpura, and eruptive cherry angiomas were observed. It should be kept in mind that dermatological signs may vary in patients with COVID-19.
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Púrpura/virología , Piel/virología , Enfermedades Cutáneas Virales/virología , Exantema/virología , COVID-19/complicaciones , COVID-19/virología , Hemangioma/virología , Piel/efectos de los fármacos , Piel/patología , Resultado del Tratamiento , Enfermedades Cutáneas Virales/diagnóstico , Enfermedades Cutáneas Virales/terapia , Prueba de COVID-19 , SARS-CoV-2 , COVID-19/tratamiento farmacológico , COVID-19/terapiaRESUMEN
Las manifestaciones cutáneas en las enfermedades autoinmunes son frecuentes y heterogéneas. En algunas de ellas, como en el caso del lupus eritematoso sistémico, la dermatomiositis, la esclerosis sistémica y el síndrome antifosfolípidico son de tal importancia que se incluyen como criterios clasificatorios de la enfermedad. Los diagnósticos diferenciales varían en gravedad, pudiendo en ocasiones presentar riesgo vital, por lo cual se jerarquizan el diagnóstico y tratamiento oportunos. Se describe el caso de una paciente de 22 años con diagnóstico previo de lupus eritematoso sistémico y síndrome antifosfolípídico, que concurre a la consulta con cuadro agudo caracterizado por lesiones cutáneas dolorosas de aspecto necrótico acompañadas de fiebre y livedo reticularis.
Cutaneous involvement is frequent and heterogeneous in autoimmune diseases. In some of them, such as in systemic lupus erythematosus, dermatomyositis, systemic sclerosis and antiphospholipid syndrome, some manifestations are so relevant that are included in the classification criteria. Differential diagnosis ranges in severity. Since the disease may be life-threatening, a prompt diagnosis and treatment are mandatory. We describe a clinical case of a twenty-two-year-old woman with diagnosis of systemic lupus erythematosus and antiphospholipidic syndrome, presenting with acute, painful cutaneous lesions with necrotic aspect, fever and livedo reticularis.
Asunto(s)
Humanos , Femenino , Púrpura , Terapéutica , Síndrome Antifosfolípido , Lupus Eritematoso SistémicoRESUMEN
BACKGROUND: Coronavirus disease 2019 (COVID-19) has associated cutaneous manifestations. OBJECTIVE: To characterize the diversity of cutaneous manifestations of COVID-19 and facilitate understanding of the underlying pathophysiology. METHODS: Case series from an international registry from the American Academy of Dermatology and International League of Dermatological Societies. RESULTS: The registry collected 716 cases of new-onset dermatologic symptoms in patients with confirmed/suspected COVID-19. Of the 171 patients in the registry with laboratory-confirmed COVID-19, the most common morphologies were morbilliform (22%), pernio-like (18%), urticarial (16%), macular erythema (13%), vesicular (11%), papulosquamous (9.9%), and retiform purpura (6.4%). Pernio-like lesions were common in patients with mild disease, whereas retiform purpura presented exclusively in ill, hospitalized patients. LIMITATIONS: We cannot estimate incidence or prevalence. Confirmation bias is possible. CONCLUSIONS: This study highlights the array of cutaneous manifestations associated with COVID-19. Many morphologies were nonspecific, whereas others may provide insight into potential immune or inflammatory pathways in COVID-19 pathophysiology.
Asunto(s)
Betacoronavirus/inmunología , Infecciones por Coronavirus/complicaciones , Neumonía Viral/complicaciones , Sistema de Registros/estadística & datos numéricos , Enfermedades de la Piel/inmunología , Adolescente , Adulto , Betacoronavirus/aislamiento & purificación , COVID-19 , Infecciones por Coronavirus/diagnóstico , Infecciones por Coronavirus/inmunología , Infecciones por Coronavirus/virología , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Pandemias , Neumonía Viral/diagnóstico , Neumonía Viral/inmunología , Neumonía Viral/virología , SARS-CoV-2 , Índice de Severidad de la Enfermedad , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/epidemiología , Enfermedades de la Piel/virología , Adulto JovenRESUMEN
Retiform purpura is a specific morphology within the spectrum of reticulate eruptions of vascular origin. It develops when blood vessels serving the skin are compromised resulting in downstream cutaneous ischemia, purpura, and necrosis. Identifying retiform purpura is important particularly in the acutely ill patient. It may elucidate the underlying diagnosis, provide prognostic information, and suggest a treatment approach. The differential diagnosis of retiform purpura is vast, reflecting the myriad conditions that can lead to cutaneous vessel wall damage or lumen occlusion. In this article, we give an overview of the differential diagnosis of this cutaneous morphology, provide an approach to workup, and highlight updates in treatment of some of the more common conditions that manifest as retiform purpura.
Asunto(s)
Púrpura/diagnóstico , Enfermedades Cutáneas Vasculares/diagnóstico , Biopsia , Técnicas de Laboratorio Clínico , Diagnóstico Diferencial , Humanos , Anamnesis , Examen Físico , Púrpura/etiología , Púrpura/patología , Púrpura/terapia , Enfermedades Cutáneas Vasculares/etiología , Enfermedades Cutáneas Vasculares/patología , Enfermedades Cutáneas Vasculares/terapiaRESUMEN
In this article we focus on updates in select etiologies of retiform purpura. These causes of retiform purpura, in addition to bacterial or fungal sepsis, disseminated intravascular coagulation, purpura fulminans, and catastrophic antiphospholipid syndrome, are important diagnoses with potential for morbidity and mortality. Important aspects in the pathophysiology, patient demographics and risk factors, updates in the diagnostic workup, histopathology, and treatment of these specific conditions are discussed.