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.

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.

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.

[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.

A review of the diagnosis and management of erythroderma (generalized red skin).

Erythroderma is a condition caused by several etiologies that result in red inflamed skin on 90% or more of the body surface. To optimize the diagnosis and management of the erythrodermic patient, healthcare professionals should be familiar with the underlying etiologies and treatment modalities. Patients with erythroderma require immediate attention as they may face a variety of medical complications. Early detection and effective management of these complications significantly reduce mortality and morbidity of this potential dermatologic emergency. This review highlights the underlying common diagnoses, assessment, and management of the patient with erythroderma.

Adult-onset acral peeling skin syndrome in a non-identical twin: a case report in South Africa.

BACKGROUND: Acral peeling skin syndrome is a rare autosomal recessive disorder in which skin exfoliation is limited to the hands and feet. While it typically manifests from early childhood, in this first reported case from South Africa, the patient did not manifest clinically until the fourth decade of life. CASE REPORT: A 44-year-old woman of African descent, 1 of a set of non-identical twins, presented with recurrent episodes of skin peeling of the upper and lower limbs. The first episode occurred 4 years prior, followed by perennial skin peeling during the spring seasons. She was not on treatment for any chronic disease and reported no exposure to chemicals or other irritants. The family, including the non-identical twin sister, has no history of skin disorders and the patient's HIV antibody test was negative. At presentation, physical examination revealed ongoing exfoliation with new skin formation on the palms and soles. The mucous membranes and nails were spared. Other systems were normal. Skin biopsy taken from the palms confirmed peeling skin syndrome. The patient was managed with topical aqueous cream and analgesics. She was briefly counseled on the nature and prognosis of the disease, and referred for genetic testing and counseling. On follow-up, she continues to have skin peeling once or twice a year. CONCLUSIONS: This first reported case of this rare disease in South Africa contributes to the growing body of literature on the disease and highlights the need for clinicians to be aware of its variable clinical onset.

Diagnóstico diferencial em eritrodermias / Differential diagnosis in erythrodermias

Eritrodermia, doença com baixa incidência, caracterizada por eritema em mais de 80% da pele, com limitação importante da qualidade de vida do paciente. Pode apresentar diversas causas, sendo as mais prevalentes dermatoses pré-existentes e uso de fármacos. Frente ao quadro de eritrodermia, torna-se obrigatório a exclusão de neoplasia como causa.

Erythroderma, is an uncommon skin disorder, characterized by erythema in more than 80% of the skin and significant limitation the quality of life. The disease has various causes, the most prevalent are pre-existing dermatoses and drug use. In the moment of diagnose of erythroderma, is mandatory to exclude malignancy as the cause.

Neonatal erythroderma.

PURPOSE OF REVIEW: Neonatal erythroderma is a potentially life-threatening condition in neonates less than 1 month old. During the first month of life, erythroderma is generally a presentation of genodermatosis, primary immune deficiency, or, more exceptionally, severe psoriasis, metabolic disease or infection. Atopic erythroderma is observed later in life, usually after the age of 1 month. Rapid determination of the underlying cause is crucial for better management. However, the diagnosis is often a challenge for the clinician and is frequently delayed due to the nonspecific nature of the clinical signs. We summarize the different causes of neonatal erythrodermas and list their clinical, biological, histological, and sometimes genetic characteristics. RECENT FINDINGS: Severe erythroderma, typified by early onset, skin induration, severe alopecia and failure to thrive, is immediately suggestive of immunodeficiency or Netherton syndrome. In such cases, an early skin biopsy may be particularly of use in allowing accurate differentiation between these two disorders. SUMMARY: This review outlines the clinical and histological features of these disorders and suggests an approach to their differential diagnosis and management.

Neonatal dermatological emergencies.

The neonates are unique in several ways in comparison with older children and adults which render them highly susceptible to severe, sometimes life threatening dermatological disorders. The neonatal dermatological emergencies are a diagnostic and therapeutic challenge. A wide range of dermatoses such as infections, genodermatoses, metabolic disorders and vascular tumors may require emergency care. The clinical presentation also varies from generalized involvement of skin to localized disease with or without systemic symptoms. Irrespective of the etiology and clinical presentation, these disorders are associated with significant morbidity and mortality. With the availability of effective drugs and monitoring facilities, and awareness of need for immediate care, there has been a significant decline in the fatality rate associated with neonatal dermatological emergencies. Knowledge of clinical presentations, rapid diagnostic methods, emergency care and monitoring of progress of the disease helps in comprehensive multidisciplinary care of neonates with these disorders.

Eritrodermia y artritis psoriática tratadas exitosamente con etanercept en un paciente coinfectado con HIV y hepatitis C / Erythroderma and psoriatic arthritis treated with etanercept in a paitient coinfected with HIV and Hepatitis C

El tratamiento de pacientes inmunosuprimidos con agentes anti-factor de necrosis tumoral alfa (TNF-alfa), representa un desafío en tanto se trata de fármacos con un efecto inmonomodulador que presuponen un aumento en el riesgo de infecciones oportunistas. Presentamos un paciente de 42 años, coinfectado con el virus de inmunodeficiencia humana (HIV) y hepatitis C (HCV), con eritrodermia y artritis psoriática severa en respuesta al tratamiento con metotrexato, tratado con etanercept. Durante el primer año de tratamiento no se reportaron efectos adversos ni infecciones oportunistas. Si bien ha habido reportes de eritrodermia y de artritis psoriásica tratados con etanercept, asociados o no a infección por HIV y/o HCV, en nuestro conocimiento, este es el primer caso reportado con una presentación concomitante de eritrodermia psoriásica y artritis psoriásica en un paciente coinfectado con HIV y HCV tratado existosamente con un agente anti-TNF-alfa.

The use of anti tumor necrosis factor alfa (TNF-alfa) agents in immunosuppressed patients, represents a challenge in as much as this drugs with an immune modulator effect, pose a raise in the risk for opportunistic infections. We present a 42 year old patient, co-infected with human immunodeficiency virus (HIV) and hepatitis C virus (HCV), with erythroderma and severe psoriatic arthritis, without response to methotrexate, treated with etanercept. No adverse effects, or opportunistic infections were reported during the first year of treatment. This it is the first report of a successful treatment with an anti-TNF alfa agent in a patient with a concomitant presentation of psoriatic erythroderma, severe psoriatic arthritis, co-infected with HIV and HCV.

Aetiopathological and clinical study of erythroderma.

Erythoderma first described by Hebra in 1868 is a reaction pattern, characterised by generalised and confluent erythema with desquamation affecting more than 90% of body surface and is usually accompanied by systemic manifestation including some metabolic changes. Tile study was envisaged to assess their profile of aetiology, clinical features and to correlate with histopathological findings. Twenty-four patients (18 males and 6 females) with erythroderma were considered for the study. All patients were hospitalised, detailed history was taken and were subjected to relevant investigations--haemogram, serum proteins, liver and renal function tests, routine and microscopic urine tests, HIV screening, x-ray chest, ultrasound abdomen, FNAC of enlarged lymph nodes and skin biopsy. The age group affected ranged between 7 and 70 years, peak incidence between 50 and 60 years. The male to female ratio was 3:1. Scaling, erythema and pruritus were predominant clinical manifestations. In 12 clinically suspected psoriatic erythroderma, only in 6 cases the histopathological features were correlated to psoriasis and remaining showed non-specific chronic dermatitis. Two patients were found to be HIV-positive. There is good clinicopathological correlation in erythroderma caused by lichen planus, atopic dermatitis, seborroeic dermatitis, non-bullous congenital ichthyosiform erythroderma. Drugs were found to be aetiological factor in 3 cases. The study was conducted to assess variations in clinical, aetiological and histopathological profile of erythroderma. The present series had high percentage of erythroderma secondary to preexisting dermatoses. The onset of disease was insidious except in drug-induced erythroderma where it was acute.

Papuloerythroderma associated with monoclonal gammopathy of undetermined significance.

We describe a 73-year-old Japanese man with papuloerythroderma overlapped with monoclonal gammopathy of undetermined significance (MGUS). Clinically, prominent erythroderma was associated with disseminated pruriginous papules, which were characteristically spared on the axillary and inguinal regions, the cubital and popliteal fossae as well as abdominal and small positional folds. Histopathologically, there was a significant perivascular infiltrate of lymphohistiocytic cells intermingled with eosinophils in the upper dermis. A biochemical profile revealed the presence of immunoglobulin G kappa chain type monoclonal protein in the serum but the absence of hematological neoplasms. We diagnosed the patient as papuloerythroderma with MGUS, and treated him with narrow-band ultraviolet B and topical steroid. His skin changes were improved, but the sharp gamma-globulin peak remained in the electrophoresis of serum protein. This case suggests an association between papuloerythroderma and MGUS.