Proposal of Slovenian guidelines for the diagnosis of neonatal erythroderma with a case report of Omenn syndrome.

Diagnosing and treating neonatal and infantile erythroderma can be challenging due to the wide variety of potential causes. Neonatal erythroderma is rare and is associated with a high mortality rate due to complications of erythroderma itself and potential life-threatening underlying diseases. Prolonged erythroderma should always be a warning sign and an indication for referral to a hospital where a multidisciplinary team approach is possible. The role of a pediatric dermatologist is to keep in mind the wide spectrum of differential diagnoses that could be causing the condition and the determination of the final diagnosis. To avert a delay in establishing the correct diagnosis, we suggest adhering to specific guidelines. We reviewed available guidelines and adapted a step-by-step approach for use in Slovenia. We also discuss a case of a neonate with erythroderma to illustrate the applicability of the proposed guidelines. Our patient presented with persistent erythroderma, pustules on the trunk and limbs, and intertriginous dermatitis. Despite local corticosteroid treatment, the skin redness persisted. After the exclusion of a systemic infection and additional tests, Omenn syndrome was diagnosed as the underlying cause.

Socioeconomic predictors of cost and length of stay for erythroderma: a cross-sectional analysis of the national inpatient sample.

Erythroderma is an uncommon but serious dermatologic disorder that often requires hospitalization for diagnosis and treatment. However, little is known about predictors influencing cost and patient outcomes. The present study sought to characterize the sociodemographic factors that predict patient outcomes and hospital cost. Data were obtained from the 2016-2018 National Inpatient Sample (NIS) provided by the Healthcare Cost and Utilization Project from the Agency for Healthcare Research and Quality for patients of any age with a primary or secondary diagnosis of exfoliative dermatitis. Regression analyses were performed to find predictors for hospital costs and patient outcomes, represented by the length of stay (LOS). Univariate analysis of LOS revealed urban teaching hospitals were associated with prolonged LOS (p = 0.023). Univariate analysis of hospital cost yielded the following factors associated with increased hospital cost: Black and Asian patients (p = .045), urban teaching hospitals (p = .035), and northeast or south geographic location (p = .004). Multivariable regression analysis revealed prolonged LOS was associated with female sex (p = .043) and large bed capacity (p = .044) while shorter LOS was associated with increased age (p = .025); lower hospital costs were associated with private-owned hospitals -  (p = .025). In patients diagnosed with erythroderma, there appear to be racial, economic, and geographic disparities for patients that lead to greater hospital costs and longer LOS.

The use of Goeckerman therapy in managing erythrodermic psoriasis resistant to multiple medications.

Erythrodermic psoriasis is a relatively rare, more dangerous inflammatory variant of psoriasis associated with high morbidity and mortality. It can be exceptionally challenging to manage, defeating even the most experienced dermatologist's arsenal of treatment strategies. Goeckerman therapy, a regimen of ultraviolet B phototherapy and crude coal tar, has demonstrable efficacy in severe and recalcitrant plaque-type psoriasis. However, its utility in erythrodermic psoriasis has not been explored within the dermatology literature. Herein, we present a patient with a long-standing history of erythrodermic psoriasis refractory to eleven treatment modalities including four biologic agents, who had his erythroderma 'turned around' following Goeckerman therapy. 'Turned around' is used to describe dramatically reducing a patient's cutaneous inflammation so that previously recalcitrant disease can now respond to maintenance therapy. The importance of a one to three week 'cool down' period of topical corticosteroid therapy prior to phototherapy or crude coal tar use is highlighted in this case as well. Although Goeckerman therapy is no longer regularly used, it remains one of the most efficacious treatments available for intractable psoriasis, attracting patients from all over the country desperate for symptom relief. This case suggests it may be useful in 'turning around' extremely difficult-to-treat erythrodermic psoriasis as well.

Erythroderma (exfoliative dermatitis). Part 1: underlying causes, clinical presentation and pathogenesis.

Erythroderma (exfoliative dermatitis), first described by Von Hebra in 1868, manifests as a cutaneous inflammatory state, with associated skin barrier and metabolic dysfunctions. The annual incidence of erythroderma is estimated to be 1-2 per 100 000 population in Europe with a male preponderance. Erythroderma may present at birth, or may develop acutely or insidiously (due to progression of an underlying primary pathology, including malignancy). Although there is a broad range of diseases that associate with erythroderma, the vast majority of cases result from pre-existing and chronic dermatoses. In the first part of this two-part concise review, we explore the underlying causes, clinical presentation, pathogenesis and investigation of erythroderma, and suggest potential treatment targets for erythroderma with unknown causes.

Leiner's disease (erythroderma desquamativum): A review and approach to therapy.

Leiner's disease (LD) is a rare and serious syndrome of infantile erythroderma of severe and progressive generalized seborrheic-like dermatitis, recalcitrant diarrhea, malabsorption and wasting, and recurrent local and systemic infections. The purpose of this study is to provide an updated review on management with a summarized review of available peer-reviewed articles on LD. The mechanisms underlying this disease process remain unclear. The diagnosis includes demonstration of deficient opsonic activity along with the clinical tetrad of erythroderma, persistent gastrointestinal disturbance, superimposed bacterial or candidal infection, and marked wasting. An important correlation between LD and defective yeast and Staphylococcus aureus opsonization has been established. For the familial form of LD, an association of either complement three deficiency or complement five dysfunction has been made. LD should be distinguished from other types of infantile erythroderma, including Omenn syndrome. Treatment includes fluid and nutrition replacement, antibiotics to control infection, and fresh-frozen plasma therapy. The prognosis is unclear; it depends on treatment. LD is a life-threatening condition that requires prompt identification and hospitalization. Affected infants who receive vigorous treatment not only have the prospect of surviving, but also generally lead a normal life after infancy.

Therapeutic options and prognosis of chronic idiopathic erythroderma in older adults.

The present authors have previously reported that chronic idiopathic erythroderma (CIE) that lasts for more than 3 months without specific causes occurs almost exclusively in older men. No standardized therapeutic treatments for the condition have been established to date. To assess the efficacies and safeties of current treatments for CIE, the present authors reviewed cases of CIE. Twenty cases were identified, of which CIE was treated with oral corticosteroids, oral cyclosporine A (CsA), ultraviolet therapy, or a combination of these therapies. CIE was cured in 8/20 (40%) of these cases, and the average time to cure was 19.3 months. Oral CsA was the most effective treatment at relatively low doses (50-100 mg/day), which resulted in cures in four cases. Ultraviolet therapy also effectively cured two subjects, and oral corticosteroids cured one patient. In patients that were treated with oral CsA or corticosteroids, the terminations of the treatments within 2 to 3 months resulted in relapses in multiple patients. The maintaining of the treatments for a certain period may be necessary in cases that involve treatments with these orally administered drugs. Although CIE is a chronic and potentially refractory disease, it can evidently be cured or well controlled via appropriate long-term therapies.

Special Considerations in the Treatment of Mycosis Fungoides.

Mycosis fungoides is the most common form of cutaneous T cell lymphoma. Although normally presenting to physicians at an early stage and with an indolent course, mycosis fungoides can have a varied presentation. The National Comprehensive Cancer Network (NCCN) has created guidelines for the treatment and staging of mycosis fungoides. Although comprehensive, in practice these guidelines do not provide specific treatment regimens for lesions located in difficult locations and those recalcitrant to the recommended therapy. Because of this, suggestions based on the practices and decisions made at the multidisciplinary cutaneous lymphoma clinic at the Sidney Kimmel Cancer Center at Thomas Jefferson University, Philadelphia, PA, USA, are presented here. Lesions located in areas such as the face and intertriginous zones are often challenging to treat because first-line therapies are often inappropriate, with the locations increasing the possibility of side effects. Additionally, lesions located in the bathing suit distribution are often nonresponsive to first-line therapies for reasons still undetermined. Finally, although well-described, erythroderma secondary to mycosis fungoides is challenging to treat, with controversy surrounding various methods of control. This article both highlights difficult clinical scenarios and reviews the recommended treatment as provided by the NCCN guidelines and provides alternative therapy for lesions that are either difficult to treat because of the location or are recalcitrant to the recommended therapy. With suggestions for the apparent gaps in guidelines, providers can better treat patients who present with more complicated conditions.

Eritrodermia en el adulto: un enfoque práctico para el diagnóstico y tratamiento / A Practical Approach to the Diagnosis and Treatment of Adult Erythroderma

La eritrodermia es un síndrome inflamatorio de la piel caracterizado por descamación y eritema en más del 90% de la superficie corporal. Representa la etapa final de muchas enfermedades dermatológicas en el adulto. La causa más frecuente es la psoriasis, le siguen las enfermedades eccematosas, las reacciones medicamentosas, la pitiriasis rubra pilaris y los linfomas cutáneos de células T. El abordaje diagnóstico debe incluir una historia y examen físicos exhaustivos. Si se desconoce la etiología de la eritrodermia es posible que múltiples biopsias a lo largo del curso de la enfermedad aumenten las posibilidades de un diagnóstico correcto. El abordaje inicial de la eritrodermia debe incluir la evaluación de un experto en nutrición, la valoración del balance hidroelectrolítico, medidas para mantener la función de barrera de la piel, antihistamínicos con efecto sedante y la exclusión de infecciones bacterianas secundarias. Presentamos una revisión práctica de la etiología, diagnóstico y tratamiento de esta entidad

Erythroderma is an inflammatory skin syndrome that involves desquamation and erythema of more than 90% of the body surface area. It represents a final clinical endpoint for many adult dermatological conditions. The most frequent cause of erythroderma is psoriasis followed by eczematous conditions, drug-induced reactions, pityriasis rubra pilaris and cutaneous T-cell lymphomas. Diagnostic approach must include a thorough history and clinical examination. If the etiology of erythroderma is uncertain multiple skin biopsies may enhance diagnostic accuracy. The initial management of erythroderma must include a nutrition expert evaluation, fluid imbalance assessment, maintaining skin barrier function, sedative antihistamines and exclusion of secondary bacterial infection. We present a practical review of the etiology, diagnosis, and treatment of this entity

Profile of erythroderma in children in hospital dermatology departments in Lomé, Togo : study of 28 cases.

The aim of this study was to describe the epidemiological, clinical, and therapeutic profile as well as the outcomes of children with erythroderma (also known as exfoliative dermatitis) in hospital dermatology departments in Lomé, Togo. This retrospective descriptive study examined the records of patients aged 0 to 15 years who were treated for erythroderma in dermatology departments in Lomé from January 1997 to December 2016. The study included 28 patients, with a mean age of 6.3 ± 5 years. The mean duration of progression of erythroderma before the consultation was 39.2 days. Pruritus was present in 60 % of the children, fever in 13.3 %, and impairment of general status in 3.3 %. Among the children aged 3 months and older, 84.2 % had dry squamous erythrodermic dermatosis, and among those younger than 3 months, 15.8 %. Among those with erythrodermic dermatosis, atopic dermatitis (70.6 %) was the most frequent dermatosis. No cause was found in 32.1 % cases. Course and outcome were favorable in 53.3 % of cases; one child died, and the rate of loss to follow-up was 43.3 %. Erythroderma is a rare disease in children in hospital dermatology departments in Lomé. Erythrodermic dermatosis, particularly atopic eczema, remains the most frequent cause.

A Practical Approach to the Diagnosis and Treatment of Adult Erythroderma. / Eritrodermia en el adulto: un enfoque práctico para el diagnóstico y tratamiento.

Erythroderma is an inflammatory skin syndrome that involves desquamation and erythema of more than 90% of the body surface area. It represents a final clinical endpoint for many adult dermatological conditions. The most frequent cause of erythroderma is psoriasis followed by eczematous conditions, drug-induced reactions, pityriasis rubra pilaris and cutaneous T-cell lymphomas. Diagnostic approach must include a thorough history and clinical examination. If the etiology of erythroderma is uncertain multiple skin biopsies may enhance diagnostic accuracy. The initial management of erythroderma must include a nutrition expert evaluation, fluid imbalance assessment, maintaining skin barrier function, sedative antihistamines and exclusion of secondary bacterial infection. We present a practical review of the etiology, diagnosis, and treatment of this entity.

A case series of dermatological emergencies - Erythroderma.

Erythroderma can be life-threatening, primarily because of its metabolic burden and complications. It is mandatory to establish its etiopathology in order to facilitate precise and definitive management. This disorder may be the morphologic presentation of a variety of cutaneous and systemic diseases. Detailed history and thorough work-up is therefore essential. Management of erythroderma involves multi-disciplines with progress monitoring especially on signs and symptoms suggestive of acute skin failure induced complications. Early diagnosis and referral of erythroderma to centres with dermatological services is crucial and will directly affect the outcome of the patients.

[Diagnostic approach of erythroderma in the adult]. / Abordaje diagnóstico de la eritrodermia en el adulto.

Erythroderma consists on erythema and scaling that covers more than 90% of the cutaneous surface. It has multiple causes and for the most common we can use the acronym PALM (psoriasis, atopic dermatitis, lymphoproliferative disease and medication). It is important to diagnose the cause in order to get a prognosis and a targeted treatment. In 30% of the cases the cause is not evident and the patient requires periodic assessment to rule out evolution to a lymphoproliferative disease. In many cases, erythroderma is a chronic disease but it can also be a life-threatening dermatologic urgency that requires hospitalization.

La eritrodermia se caracteriza por eritema y escama en más del 90% de la superficie cutánea. Tiene muchas causas y es un reto para el dermatólogo identificarlas. Las más comunes se engloban en el acrónimo derivado de sus siglas en inglés PALM (psoriasis, dermatitis atópica, proceso linfoproliferativo y medicamentos). La importancia del diagnóstico certero es el tratamiento dirigido y el pronóstico. En los casos idiopáticos que llegan a ser de hasta el 30%, el paciente requiere de valoraciones periódicas por probable evolución a un proceso linfoproliferativo. Aunque en muchas ocasiones la eritrodermia es un cuadro crónico, también puede presentarse como una urgencia dermatológica que pone en riesgo la vida del paciente y requiere hospitalización.

Thymoma-associated multi-organ autoimmunity: A case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy.

Thymoma-associated multi-organ autoimmunity disease (TAMA) is a rare paraneoplastic disorder, clinicopathologically similar to graft-versus-host disease (GVHD). Many reported cases follow a difficult course; half of them die from serious infectious diseases subsequent to immunosuppression induced by chemotherapy for unresectable thymoma, or intensive therapies including systemic steroids for complicating autoimmune diseases and GVHD-like symptoms. We report a patient whose skin symptoms were improved subsequently to total thymectomy. The patient also presented with hypogammaglobulinemia, which led to the diagnosis of complicated Good syndrome. Taking account of her immunodeficient condition, antibiotics and i.v. immunoglobulin were administrated promptly on onset of bacterial pneumonia, which was successfully treated. According to a review of the published work, treatments with systemic steroids for skin symptoms have limited effects and may contribute to serious infection. Our case indicates that successful treatment of thymoma itself may lead to the amelioration of the disease. The management priority should be given to the treatment of thymoma and the control of subsequent immune abnormality other than GVHD-like erythroderma.