Search

Background Cutaneous manifestations in hospitalized children with SARS-CoV-2 have not been studied systematically. Objective To describe the mucocutaneous involvement in pediatric patients with COVID-19 admitted to a pediatric institution in Madrid (Spain), located in a zone reporting among the highest prevalence of COVID-19 in Europe. Methods A descriptive, analytical study was conducted on a series of 50 children hospitalized with COVID-19 between March 1, 2020, and November 30, 2020. Results Twenty-one patients presented with mucocutaneous symptoms: 18 patients with macular and/or papular exanthem, 17 with conjunctival hyperemia, and 9 with red cracked lips or strawberry tongue. Eighteen patients fulfilled criteria for multisystem inflammatory syndrome in children. Patients with mucocutaneous involvement tended to be older and presented to the emergency department with poor general status and extreme tachycardia, higher C-reactive protein and D-dimer levels, and lower lymphocyte counts than patients without skin signs. Mucocutaneous manifestations pose a higher risk of admission to the pediatric intensive care unit (odds ratio, 10.24; 95% confidence interval, 2.23-46.88; P = .003). Conclusions Children hospitalized with COVID-19 frequently had mucocutaneous involvement, with most symptoms fulfilling criteria for multisystem inflammatory syndrome in children. Patients with an exanthem or conjunctival hyperemia at admission have a higher probability of pediatric intensive care admission than patients without mucocutaneous symptoms.

The COVID-19 pandemic has brought innumerable reports of chilblains. The relation between pernio-like acral eruptions and COVID-19 has not been fully elucidated because most reported cases have occurred in patients with negative microbiological tests for SARS-CoV-2.A retrospective study of 49 cases of chilblains seen during the first year of the pandemic in a children's hospital in Madrid, Spain. The incidence of these skin lesions was correlated with the number of COVID-19 admissions and environmental temperatures. Patients were separated into two groups depending on the day of onset (strict lockdown period vs. outside the lockdown period).Most chilblains cases presented during the first and third waves of the pandemic, paralleling the number of COVID-19 admissions. The first wave coincided with a strict lockdown, and the third wave coincided with the lowest ambient seasonal temperatures of the year. Systemic symptoms preceding chilblains were more frequent in the first wave (45.8% vs. 8.0%, p = .002), as was the co-occurrence with erythema multiforme-like lesions (16.7% vs. 0%, p = .033). Laboratory test and skin biopsies were performed more frequently in the first wave (75.0% vs. 12.0%, p .001; and 25.0% vs. 0%, p = .007; respectively). Five patients developed recurrent cutaneous symptoms.An increased incidence of chilblains coincided not only with the two major waves of the pandemic, but also with the strict lockdown period in the first wave and low seasonal temperatures during the third wave. Both increased sedentary behaviors and cold environmental temperatures may have played an additive role in the development of COVID-19-related chilblains.

T-cell prolymphocytic leukemia (T-PLL) is a T-cell lymphoproliferative disorder that frequently involves the skin. The objective was to describe two cases of T-PLL with cutaneous involvement and to present a review of the literature concerning the clinical characteristics, differential diagnosis and treatment of these patients.1) 79 year-old man, with a previous diagnosis of T-PLL based on a laboratory incidental finding. He had been treated with alemtuzumab, but it had to be interrupted due to recurrent infections. After interrupting the treatment, the patient developed a symmetrical rash on his extremities. The skin biopsy demonstrated TPLL infiltration. 2) 28 year-old man that presented with asthenia and lymphocytosis. He also showed a purpuric rash on his trunk and facial erythema. Histopathology of the skin and bone marrow confirmed the diagnosis of T-PLL with cutaneous involvement.T-cell prolymphocytic leukemia accounts for 2% of mature leukemias in adults. Skin involvement is reported in 20-50% of the patients. The characteristic features are facial involvement, purpuric lesions and symmetry of the rash, although there are atypical manifestations as well. Differential diagnosis includes other T-cell lymphoproliferative disorders with hematologic and skin involvement, such as Sézary syndrome. Patients with T-PLL may show cutaneous infiltration at the moment of debut or relapse of the disease. The skin is an accessible organ for taking samples to study and diagnose these patients.La leucemia prolinfocítica T (LPL-T) es una neoplasia hematológica del grupo de síndromes linfoproliferativos T que con frecuencia produce infiltración cutánea. Se presentan dos casos de LPL-T con afectación cutánea y se revisa la literatura en cuanto a características clínicas, diagnóstico diferencial y tratamiento de estos pacientes.1) Varón de 79 años diagnosticado de LPL-T tras un hallazgo analítico incidental. Tras suspender el tratamiento con alemtuzumab por infecciones recurrentes, comenzó con lesiones cutáneas maculopapulosas eritematopurpúricas que afectaban la raíz de las extremidades. La biopsia cutánea confirmó la infiltración por su enfermedad de base. 2) Varón de 28 años que debutó con astenia y hallazgos analíticos de leucocitosis. Había comenzado además con lesiones purpúricas en el tronco y eritema malar bilateral. El estudio de médula ósea y la biopsia cutánea confirmaron el diagnóstico de LPL-T con infiltración cutánea.La LPL-T corresponde al 2% de las leucemias linfocíticas maduras en los adultos. Entre el 20% y el 50% de los pacientes presentan afectación cutánea, con predominio en la región facial, y son característicos el eritema, la púrpura y la simetría, aunque existen manifestaciones atípicas. El diagnóstico diferencial incluye otros síndromes linfoproliferativos T con afectación cutánea y en sangre periférica, entre los que destaca el síndrome de Sézary. Los pacientes con LPL-T pueden presentar afectación cutánea en el debut o en una recidiva de la enfermedad. La piel representa un órgano accesible para la toma de muestras y para el diagnóstico y el estudio de estos pacientes.

Resumen Introduccion La esclerodermia engloba un conjunto de enfermedades que provocan fibrosis de la piel y en el tejido subcutaneo. Se distinguen dos subtipos: esclerodermia sistemica (ES), con afectacion visceral asociada, y esclerodermia localizada o morfea, limitada a la piel y a los tejidos subyacentes. Existen pocos estudios que evaluen las caracteristicas de estos pacientes. El objetivo de nuestro estudio fue analizar las caracteristicas clinicas y epidemiologicas de los pacientes con esclerodermia. Metodologia Se analizaron los datos de los pacientes con diagnostico de esclerodermia en seguimiento en la consulta monografica de colagenosis de un hospital de tercer nivel. Resultados Se analizaron un total de 100 pacientes con diagnostico de esclerodermia (80 con morfea y 20 con ES). La mediana de edad al diagnostico fue de 45 anos, con predominio femenino. Los pacientes fueron clasificados segun el subtipo de esclerodermia. Se analizo ademas la asociacion a otras enfermedades autoinmunes (presente en el 28%) y a liquen escleroatrofico (5%). Propusimos otra clasificacion para los pacientes con morfea segun la distribucion de las lesiones: morfea isomorfica (lesiones en zonas de roce) y no isomorfica (en otras localizaciones). Los pacientes con morfea isomorfica presentaron mayor tasa de sobrepeso, obesidad y sindrome metabolico. Los pacientes con morfea no isomorfica requirieron con mayor frecuencia tratamiento sistemico. Conclusiones Recomendamos subclasificar a los pacientes segun la distribucion de las lesiones, ya que los pacientes con morfea isomorfica y no isomorfica parecen presentar diferentes caracteristicas.

Resumen Introduccion La pustulosis palmoplantar (PPP) se caracteriza por la presencia de pustulas esteriles recurrentes en las palmas y plantas. Las caracteristicas epidemiologicas y su asociacion con otras enfermedades difieren entre los estudios publicados. Las mutaciones en el gen IL36RN se han identificado recientemente en pacientes con formas pustulosas, sobre todo generalizadas, pero tambien en algunos pacientes con PPP. El objetivo del presente estudio es describir las caracteristicas clinicas, epidemiologicas y la comorbilidad de enfermedades inflamatorias en PPP, asi como estudiar la frecuencia de las mutaciones descritas en el gen IL36RN. Material y metodo Presentamos un estudio observacional, retrospectivo y longitudinal en el que se reviso a todos los pacientes con diagnostico de PPP con control fotografico, atendidos en nuestro centro entre los anos 2009 y 2017. Resultados Se incluyeron 41 pacientes (90% mujeres) con una edad media de 55,8 anos. El 94% eran fumadores o exfumadores, el 24% presentaba antecedentes de psoriasis en placas, el 39% de psoriasis ungueal y el 27% antecedentes familiares. En cuanto a la asociacion con otras IMID (immune mediated inflammatory disorders), un 7% presentaba una enfermedad inflamatoria intestinal, un 10% artritis reumatoide y un 22% otros tipos de artritis. En ninguno de los 11 pacientes en los que se hizo estudio genetico se encontraron mutaciones descritas en el gen de IL36RN. Conclusiones Los pacientes con PPP son mayoritariamente mujeres fumadoras en la quinta decada de la vida y con una incidencia de IMID superior a lo esperado, con una alta prevalencia de antecedentes personales y familiares de psoriasis. Introduction Palmoplantar pustulosis (PPP) is characterised by the presence of sterile association with other diseases differ among published studies. IL36RN gene mutation has recently been identified in patients with mainly generalised pustular forms, but also in some patients with PPP. The objective of this study is to describe clinical and epidemiological characteristics, the incidence of inflammatory diseases in PPP, and to study the frequency of IL36RN gene mutations. Methods An observational, retrospective, longitudinal study is presented, which includes all patients attended in our centre between 2009 and 2017 with a PPP diagnosis if only they were controlled by photography. Results 41 patients were included (90% women; average age of 55, 8). 94% were smokers or ex-smokers, 24% had a history of plaque psoriasis, 39% ungueal psoriasis and 27% a positive familiar background. On analysing IMIDs (immune mediated inflammatory disorders) incidence in PPP patients, 7% had inflammatory bowel disease, 10% psoriatic arthritis, and 22% other types of arthritis. No IL36RN gene mutations were found in any of the 11 patients in whom a genetic study was performed. Conclusions Patients with PPP are mostly women who smoke, in the fifth decade of life, with an incidence of IMIDs higher than expected, as well as with a high prevalence of personal and family history of psoriasis.

Anorexia nervosa is an eating disorder frequently associated with cutaneous manifestations. A rare type of purpura, known as diffuse reticulate purpura, has been described in patients with anorexia nervosa and severe malnutrition. Typical characteristics of this condition include a purpuric reticulate rash predominantly affecting the trunk that rapidly resolves with adequate feeding. We report two male adolescent patients with anorexia nervosa and severe malnutrition who developed diffuse reticulate purpura.

BACKGROUND T-cell prolymphocytic leukemia (T-PLL) is a T-cell lymphoproliferative disorder that frequently involves the skin. The objective was to describe two cases of T-PLL with cutaneous involvement and to present a review of the literature concerning the clinical characteristics, differential diagnosis and treatment of these patients. CASE REPORTS 1) 79 year-old man, with a previous diagnosis of T-PLL based on a laboratory incidental finding. He had been treated with alemtuzumab, but it had to be interrupted due to recurrent infections. After interrupting the treatment, the patient developed a symmetrical rash on his extremities. The skin biopsy demonstrated TPLL infiltration. 2) 28 year-old man that presented with asthenia and lymphocytosis. He also showed a purpuric rash on his trunk and facial erythema. Histopathology of the skin and bone marrow confirmed the diagnosis of T-PLL with cutaneous involvement. CONCLUSIONS T-cell prolymphocytic leukemia accounts for 2% of mature leukemias in adults. Skin involvement is reported in 20-50% of the patients. The characteristic features are facial involvement, purpuric lesions and symmetry of the rash, although there are atypical manifestations as well. Differential diagnosis includes other T-cell lymphoproliferative disorders with hematologic and skin involvement, such as Sezary syndrome. Patients with T-PLL may show cutaneous infiltration at the moment of debut or relapse of the disease. The skin is an accessible organ for taking samples to study and diagnose these patients.

An increasing number of publications have brought attention to COVID-19-associated cutaneous lesions. Histopathological descriptions and clinical correlation of the histopathological findings of COVID-19 skin lesions are lacking. In this manuscript, we reviewed and described the histopathological characteristics of COVID-19 infection cutaneous patterns reported in the literature.

Sorafenib is a multikinase inhibitor increasingly used for the treatment of several solid tumors. Different types of keratotic lesions, such as squamous cell carcinoma, actinic keratosis, or infundibular cyst, have been reported in association with this therapy. We present a 15-year-old male diagnosed with desmoid fibromatosis who developed multiple penile and scrotal infundibular cysts while receiving treatment with sorafenib.

Pigmented purpuric dermatoses (PPDs) are a group of idiopathic, chronic, and self-resolving conditions easily recognizable in adults but extremely uncommon in infants. We present the youngest patient with PPD reported to date.

Dermoscopic studies about cutaneous metastases of malignant melanoma (CMMM) are few. Our objective was to analyze the dermoscopic features of CMMM and propose a new dermoscopic pattern classification based on color pigmentation and some specific dermoscopic features. A retrospective evaluation of 150 dermoscopic images of CMMM taken from 40 patients was performed. One hundred CMMM images were individually evaluated by six dermatologists in order to classify them according to four dermoscopic patterns: (i) blue pattern; (ii) pink pattern; (iii) brown pattern; and (iv) mixed pattern. One hundred and fifty dermoscopic images including 50 CMMM and 100 benign lesions were evaluated by five dermatologists to calculate the accuracy of these patterns in the recognition of CMMM. An intra- and interobserver reproducibility agreement study between all different dermoscopic pattern classifications was performed. Seventy-five percent of our cases of CMMM showed a monochromatic pattern. Light brown pigmented halo, peripheral gray spots and polymorphic atypical vessels were the most significant focal dermoscopic structures. The reproducibility of the color-based dermoscopic pattern classification was superior to previous dermoscopic pattern classification. In summary, a dermoscopic pattern classification based on color pigmentation and some specific dermoscopic features may be useful in recognizing early cutaneous melanoma metastasis. Multicentric studies are recommended in order to lower the impact of interobserver variability.

The aim of this study was to identify clinical factors associated with dose reduction and dose escalation in the treatment with ustekinumab in patients with moderate-to-severe plaque psoriasis.An observational, longitudinal and retrospective study was conducted using patients with moderate-to-severe plaque psoriasis. We reviewed clinical histories and variables were recorded on a database (patients' characteristics, pharmacotherapeutics, effectiveness and safety). We evaluated correlation between dose reduction, dose escalation and used dose with other variables.Of the study's 62 patients, Ustekinumab dose was adjusted in 45.2% (22.6% with reduced doses and 22.6% with increased doses). We found a statistically significant correlation between extending the dosing interval and the absence of psoriatic arthritis, no concomitant systemic therapies, treatment time with ustekinumab, lower PASI at week 28 and achieving PASI75 at week 28. There was also a statistically significant correlation between dose escalation and diabetes mellitus, psoriatic arthritis, prior biological treatments, concomitant systemic therapies, concomitant phototherapy and not achieving PASI75 at week 28.Dose-reduction strategies would increase ustekinumab efficiency in patients that achieve PASI 75 without psoriatic arthritis, diabetes mellitus, previous BT and concomitant treatment with conventional systemic drugs.