Palmoplantar pustulosis and acrodermatitis continua of Hallopeau: demographic and clinical comparative study in a large multicentre cohort.

BACKGROUND: Acral pustular disease within the pustular psoriasis/psoriasis-like spectrum mainly includes palmoplantar pustulosis (PPP) and acrodermatitis continua of Hallopeau (ACH). Scarce data argue for a distinction between these two entities, but no study has compared the clinical and epidemiologic characteristics of ACH and PPP. OBJECTIVES: We aimed to perform a comparative description of the epidemiological and clinical characteristics of PPP and ACH in a multicentre retrospective cohort. METHODS: In this multicentre national retrospective cohort study, we compared the epidemiological characteristics, comorbidities and psoriasis characteristics of patients with PPP and ACH. RESULTS: A total of 234 patients were included: 203 (87%) with PPP, 18 (8%) with ACH and 13 (6%) with both, according to 2017 ERASPEN criteria. As compared with ACH, PPP was associated with female sex, smoking activity and higher median BMI (P = 0.01, P = 0.02 and P = 0.05 respectively). A family background of psoriasis was more frequent in PPP than ACH. Age of onset of palmoplantar disease was similar between PPP and ACH patients, median age 44 and 48 years respectively. Peripheral joint inflammatory involvement was the only rheumatic disease associated with ACH. The association with another psoriasis type was similar in PPP and ACH (57.6% and 61.1% respectively). CONCLUSION: Our study confirms in a large PPP cohort the predominance of females and a high prevalence of smoking and elevated body mass index but also shows an association of these features in PPP as compared with ACH. In addition, it highlights peripheral arthritis as the only arthritis endotype associated with ACH. Increased knowledge of the immunogenetic backgrounds underlying these two entities is warranted to better stratify pustular psoriasis or psoriasis-like entities for precision medicine.

Acrodermatitis enteropática en lactante femenina de 6 meses de edad / Acrodermatitis enteropathica in 6- months-old female infant

Introducción: La acrodermatitis enteropática es una dermatosis nutricional heredada o adquirida por deficiencia de zinc que clínicamente se caracteriza por eritema, escamocostras y erosiones, especialmente en la región perioral, en zonas acras y en la región anogenital. Además de una ingesta oral inadecuada, hay causas secundarias de esta deficiencia nutricional. Objetivo: Referir una dermatosis nutricional secundaria infrecuente en lactante femenina. Presentación de caso: Pequeñita de 6 meses de edad, fototipo III, alimentada con lactancia materna exclusiva, que acude a consulta especializada de dermatología en el hospital William Soler por cuadro clínico de 3 meses de evolución caracterizado por lesiones eritematocostrosas en regiónes acrales y periorificiales. Los padres refirieron lactancia materna exclusiva. Se realizaron complementarios Conclusiones: Las deficiencias de vitaminas y oligoelementos pueden ocasionar un amplio rango de hallazgos mucocutaneos. En el caso presentado diagnosticado como acrodermatitis enteropática adquirida, hay un aporte inadecuado de zinc en la leche materna(AU)

Introduction: Acrodermatitis enteropathica is an inherited or acquired nutritional dermatosis by zinc deficiency that is clinically characterized by erythema, squamous crusts and erosions, especially in the perioral region, in acral areas and in the anogenital region. In addition to inadequate oral intake, there are secondary causes of this nutritional deficiency. Objective: To refer a nutritional dermatosis uncommon in female infants. Case presentation: 6-months-old baby girl, skin phototype III, fed with exclusive breastfeeding whom was attended in the specialized consultation in Dermatology of William Soler Pediatric Hospital due to clinical framework of 3 months of evolution characterized by eritemato crusty lesions in acral and periorifices´ regions. Parents commented on exclusive breastfeeding. There were made complementary blood tests. Conclusions: The deficiencies of vitamins and trace elements may cause a wide range of mucocutaneous findings. In the presented case diagnosed as adquired acrodermatitis enteropathica, there is an inadequate intake of zinc in breast milk(AU)

Pathogenetic implications of the age at time of diagnosis and skin location for acrodermatitis chronica atrophicans.

BACKGROUND: The pathogenesis of acrodermatitis chronica atrophicans (ACA) is not well understood. OBJECTIVE: The purpose of this study was to gain a better understanding of ACA by utilizing a large data set of adult Slovenian patients with Lyme borreliosis. METHODS: The age of 590 ACA patients was compared with that of patients with other manifestations of Lyme borreliosis. The location of the ACA lesion on the body was compared with that of erythema migrans (EM). RESULTS: Patients diagnosed with ACA were on average 14.3 years older than patients with EM (p<0.001). ACA patients were also significantly older than patients with Lyme neuroborreliosis or Lyme arthritis (p<0.001). The average delay in diagnosis of ACA was 1.6 years (range 0.1-20 years). For 572 (96.9%) of the ACA patients, the site of the skin lesion(s) was confined to an extremity vs. 79.6% for patients with EM, p<0.001. For the 20 ACA patients who reported a preceding untreated EM lesion at the same body site, the mean time between the development of the EM and the onset of ACA was 3.0±4.4 (median 1.3, range 0.1-15.0) years. CONCLUSIONS: ACA is more likely to be diagnosed in older individuals than any other manifestation of Lyme borreliosis. ACA is more likely than EM to be localized anatomically to the extremities. Available data favor the hypothesis that ACA occurs most often on the extremities of older individuals because of predisposing age-related anatomic or physiologic changes, but more data are needed to define the latency period and other aspects of the pathogenesis of this skin condition.

A case-control study on the association of pulse oral poliomyelitis vaccination and Gianotti-Crosti syndrome.

BACKGROUND: Since 1995, the Indian government has been launching two National Immunization Days (NIDs) annually to administer oral polio vaccines (OPVs) to children under the age of 5. Our aim was to investigate the association between OPVs and Gianotti-Crosti syndrome (GCS). METHODS: A board-certified dermatologist in solo practice conducted the examinations. The patients consulted without the need of a referral. We retrieved files of all children under the age of 5 who were diagnosed with GCS in 18 months. There were three NIDs during these months. We charted the number of children 1 month before, 1 week before, 1 week after, and 1 month after the three NIDs. RESULTS: A total of 116 children (49 boys and 67 girls) under the age of 5 with GCS were found (average age: 2.9 years) within these 18 months of three NIDs. Eleven (9.5%) and 105 (90.5%) children developed GCS 1 month before and 1 month following OPV administration, respectively (RR: 1.81; 95% CI: 1.40-2.35; P < 0.0001). Three (2.6%) and 58 (50.0%) children developed GCS 1 week before and 1 week after OPV administration, respectively (RR: 1.90; 95% CI: 1.12-3.22; P < 0.0001). CONCLUSIONS: The administration of OPV is significantly associated with the occurrence of GCS in the part of the world that we investigated. As we demonstrated a temporal relationship, this association is likely to be causal.

Is Gianotti-Crosti Syndrome Associated with Atopy? A Case-Control Study and a Postulation on the Intrinsic Host Factors in Gianotti-Crosti Syndrome.

OBJECTIVES: To investigate whether Gianotti-Crosti syndrome (GCS) in children is associated with atopy. METHODS: The setting was two outpatient clinic. Diagnoses of asthma and atopic dermatitis (AD) were made according to internationally accepted diagnostic criteria. Allergic rhinitis, atopic urticaria, and allergic conjunctivitis were diagnosed clinically. Participants were children with GCS diagnosed over the previous 5 years. For any child with GCS, we extracted the record of the subsequent age and sex pair-matched child seen for problems unrelated to the skin as controls. RESULTS: We retrieved the records of 37 pairs of study and control subjects; 28 (76%) children with GCS and 9 (24%) controls had AD (risk ratio [RR] = 3.11[95% confidence interval {CI} 1.73, 5.73]), 31 (84%) children with GCS and 19 (51%) controls had at least one atopic condition (RR = 1.63 [95% CI 1.13, 2.18]) and 11 (30%) children with GCS and 2 (5%) controls had at least three atopic conditions (RR = 5.50 [95% CI 1.29, 35.35]). CONCLUSION: GCS is significantly associated with AD and the presence of atopic conditions.

Cutaneous infectious diseases: Kids are not just little people.

The changes in immune response that occur with age play a significant role in disease presentation and patient management. Evolution of the innate and adaptive immune systems throughout life, influenced partly by hormonal changes associated with puberty, plays a role in the differences between pediatric and adult response to disease. We review a series of manifestations of dermatologic infectious diseases spanning bacterial, viral, and fungal origins that can be seen in both pediatric and adult age groups and highlight similarities and differences in presentation and disease course. Therapeutic options are also discussed for these infectious diseases, with particular attention to variations in management between these population subgroups, given differences in pharmacokinetics and side effect profiles.

Acrodermatitis enteropathica and other nutritional diseases of the folds (intertriginous areas).

The appropriate intake and metabolism of vitamins and minerals are critical to maintaining homeostasis. Imbalance in essential nutrients, either through dietary excess or deficiency or disorders in metabolism, can result in a spectrum of dermatologic and systemic manifestations. Certain nutrient deficiencies produce a characteristic pattern of cutaneous eruption. Recognition of these patterns is important, as they can alert the physician to an underlying nutritional disease. We review nutritional diseases involving zinc, biotin, essential fatty acids, vitamin B6 (pyridoxine), and riboflavin that present specifically with intertriginous eruptions.

Epstein-Barr virus and skin manifestations in childhood.

Epstein-Barr virus (EBV) is a human B-lymphotropic herpes virus and one of the most common viruses in humans. Specific skin signs related to EBV infection are the exanthem of mononucleosis, which is observed more frequently after ingestion of amoxicillin, and oral hairy leukoplakia, a disease occurring mostly in immunocompromised subjects with HIV infection. Other more uncommon cutaneous disorders that have been associated with EBV infection include virus-related exanthems or diseases such as Gianotti-Crosti syndrome, erythema multiforme, and acute genital ulcers. Other skin manifestations, not correlated to virus infection, such as hydroa vacciniforme and drug-induced hypersensitivity syndrome have also been linked to EBV. The putative involvement of EBV in skin diseases is growing similarly to other areas of medicine, where the role of EBV infection is being investigated in potentially debilitating inflammatory diseases. The prognosis of EBV infection in healthy, immunocompetent individuals is excellent. However, lifelong infection, which is kept in check by the host immune system, determines an unpredictable risk of pathologic unpredictable scenarios. In this review, we describe the spectrum of non-tumoral dermatological manifestations that can follow EBV primary infection or reactivation of EBV in childhood.

Does tobacco smoking influence the occurrence of hand eczema?

BACKGROUND: Tobacco smoking is known to influence various inflammatory skin diseases and an association between tobacco smoking and hand eczema has been proposed in some studies. OBJECTIVES: To examine a possible association between reported current tobacco smoking and the occurrence of hand eczema. SUBJECTS AND METHODS: Previously collected questionnaire data on the occurrence of hand eczema in three occupational cohorts and corresponding controls from the general population were studied. The questionnaires used included questions on 1-year prevalence of hand eczema and questions on smoking habits. For one occupational group, hairdressers and their controls, information on amount of smoking was obtained. Information on age, sex and history of atopy was also available. RESULTS: In total, answers regarding smoking and hand eczema were obtained from 13,452 individuals. Out of 3493 smokers, 437 (12.5%) reported hand eczema compared with 1294 out of 9959 nonsmokers (13.0%) (P = 0.51). With regard to the number of cigarettes smoked, 22.6% of the hairdressers smoking more than 10 cigarettes per day reported hand eczema compared with 17.4% of those smoking 0-10 cigarettes per day (P = 0.01). Corresponding figures for the controls were 14.5% and 11.7%, respectively (P = 0.06). CONCLUSIONS: No clear association was found between 1-year prevalence of hand eczema and smoking. Heavy smoking, more than 10 cigarettes per day, may give a slightly increased risk of hand eczema. Further studies with information on the amount of tobacco consumption and on possible confounders are needed to evaluate smoking as a risk factor for hand eczema.

Acrodermatitis enteropathica and an overview of zinc metabolism.

Acrodermatitis enteropathica is a rare autosomal recessive disorder of zinc deficiency. The genetic defect has been mapped to 8q24 and the defective gene identified as SLC39A4, which encodes the zinc transporter Zip4. The diagnosis is made by way of clinical presentation together with histopathology and laboratory tests. Here we provide an overview of zinc metabolism and a description of inherited and acquired zinc deficiency.

[Skin manifestations of Lyme borreliosis]. / Skórne postacie boreliozy z Lyme.

Lyme borreliosis is the most common tick-borne disease which is caused by Borrelia burgdorferi and transmitted in the Poland, as well as Europe, primarily by Ixodes ticks. After inoculation spirochetes spreads in the skin, activate locally immune response mechanisms and cause characteristic skin lesions like erythema migrans, lymphadenosis benigna cutis, erythema migrans multiplex and acrodermatitis chronica atrophicans. The correct clinical diagnosis of skin lesions and antibiotic treatment is most important for regression of symptoms and prevention of late manifestations of Lyme disease.

Supresión del tratamiento con zinc en un caso de Acrodermatitis Enteropática / The elimination of the zinc treatment in an Acrodermatitis Enteropathica case

La Acrodermatitis Enteropática es una enfermedad de baja incidencia, se trata de una genodermatosis autonómica recesiva. Sus manifestaciones clínicas son variadas considerándose penta sintomática con lesiones cutáneas, síntomas digestivos, alopecia, distrofia de uñas así como alteraciones psíquicas, fotofobia y depresión mental. En este caso se trata de una paciente, de 29 años de edad, que ha sido tratada en el Servicio de Dermatología Provincial de Matanzas, desde los 6 meses de edad, por presentar lesiones cutáneas alrededor de la boca, vulva y ano, así como en dorso de ambos pies y codos. Esta paciente mediante el cuadro clínico, la biopsia de piel y la dosificación de Zn en sangre y orina se diagnóstico como portadora de Acrodermatitis enteropática congénita, por lo que ha recibido tratamiento con zinc desde esa edad, con recaídas en varias oportunidades, lo que motivó varios ingresos en etapa infantil y además en etapa adulta. En esta oportunidad no les fue posible la adquisición del medicamento, por lo que desde hacía dos meses no realizaba tratamiento, apareciendo lesiones cutáneas como en años anteriores, lo cual motivó el ingreso en nuestro servicio. Se indicó tratamiento con: Sulfato de Zinc remitiendo el cuadro dermatológico una vez iniciado el tratamiento, destacándose que los medicamentos tópicos indicados fueron de escasa efectividad en nuestro caso, por lo que consideramos de gran importancia la presentación de este caso para la práctica médica, dada las variadas manifestaciones clínicas que puede presentar y que sólo pueden remitir con el tratamiento descrito...(AU)