Malignancy-associated generalised exfoliative dermatitis: A retrospective study in a single-centre Asian cohort.

BACKGROUND: Erythroderma is an inflammatory skin condition that causes extensive erythema and skin scaling amounting ≥90% of the body surface area. This retrospective cohort study describes the prevalence of malignancy-associated erythroderma in a single centre where there was concerted effort to systematically offer malignancy screens to all adult erythroderma patients above the age of 65 years. METHODS: Clinical charts were reviewed for all adult inpatients and outpatients with erythroderma who attended the National University Hospital (NUH) from 1 July 2019 to 31 December 2021. Data collected included patient demographics, clinical findings, laboratory investigations, disease-specific investigations such as endoscopic procedures and biopsies, follow-up duration and mortality data. RESULTS: Seventy-four patients were analysed. The median age of the patients was 73 years old (interquartile range: 59-81 years old). An underlying dermatosis was the most common cause of erythroderma-63 patients having atopic dermatitis/asteatotic eczema or psoriasis. Three patients had erythroderma from drug eruptions, and 1 patient had chronic actinic dermatitis. Four patients had associated malignancies (5.4%). Half of our patients completed further evaluation for malignancy (52.7%). The rest had either declined or were eventually unable to complete the investigations. There was a higher prevalence of associated malignancy (7.8%) in elderly patients above 65 years old. CONCLUSION: When compared to existing literature, our cohort reflects a higher observed occurrence of malignancy in association with erythroderma. As delays in evaluation for underlying malignancy could result in potentially deleterious outcomes, it is prudent to consider systematic screening for malignancy in high-risk populations such as elderly erythroderma patients.

Prevalence of Metabolic Syndrome in Chinese Patients With Erythrodermic Psoriasis: A Case-Control Study.

Erythroderma psoriasis (EP) is a rare and severe form of psoriasis, which is a chronic inflammatory skin disease that usually occurs simultaneously with cardiovascular disease (CVD). Metabolic syndrome (MetS) is a significant precursor of CVD. This study was to investigate the association between EP and MetS in the Chinese population. We conducted a retrospective study on 86 consecutive patients with EP and 100 healthy controls from Huashan Hospital between 2013 and 2018. Demographic, biochemical parameters for MetS, and other relevant data including the severity of EP, family history of EP, age of onset, and treatment history involved in those individuals were recorded. The prevalence of MetS in erythrodermic psoriatic patients was 88.37%, which was significantly higher than that of controls (P < 0.0001). Erythrodermic psoriatic patients also had a higher prevalence of MetS components, including abdominal obesity, dyslipidemia and hypertension, whereas hyperglycemia was similar. Adjusted for confounding factors, MetS, abdominal obesity, hypertension, smoking and alcohol use were positive independent predictors of EP (odds ratio > 1, P < 0.05). The area under the receiver operating characteristic curve calculated from determined risk factors for predicting the EP's incidence was 0.934 (95% CI 0.902-0.966). There was no correlation between the severity of EP and the prevalence of MetS. Compared with patients with mild EP, patients with moderate-to-severe EP had higher body mass index, waist circumstance and blood pressure (P < 0.05). We concluded that the prevalence of MetS and its components was higher in patients with EP. MetS an independent predictor of EP, which can favor CVD and should be encouraged to correct these cardiovascular risk factors aggressively for managing EP.

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.

The rash that becomes an erythroderma.

Erythroderma is a dermatologic emergency with potentially serious consequences. Several diseases with different etiologies characteristically appear as erythroderma. Depending on the age groups, congenital ichthyosiform disorders, infections, preexisting dermatoses, drug eruptions, and internal malignancies commonly present with, or progress to, erythroderma. The course, prognosis, and management strategies also vary depending on the cause of erythroderma; hence, an accurate diagnosis is essential in minimizing associated morbidity and mortality. The generalized erythema and scaling often obscure the classic clinical features of the underlying skin diseases, posing a diagnostic challenge to dermatologists. Awareness and elicitation of subtle signs and clinical manifestations are crucial. A step-wise approach ensures completeness of clinical evaluation and avoids missing any relevant clinical data. The initial clinical presentation, cutaneous examination findings, and systemic clues reveal important information regarding the diagnosis, course, and prognosis of erythroderma. The age at onset, symptomatology, duration of illness, initial lesions, initial site of onset, clinical course, family history, types of scales, changes in cutaneous integuments and systemic clues will assist in delineating the nature of underlying disease.

Estudio clínico-histopatológico de las eritrodermias en un hospital universitario: 14 años de experiencia / Clinical-histopathological study of erythroderma in a university hospital: 14 years of experience

INTRODUCCIÓN: La eritrodermia es un síndrome inflamatorio cutáneo infrecuente caracterizado por compromiso eritematoso generalizado y descamación, de más del 90% de superficie cutánea total. OBJETIVO: Caracterizar clínica e histopatológicamente a los pacientes con eritrodermia en un hospital universitario chileno. METODOLOGÍA: Estudio retrospectivo, realizado en el Hospital Clínico Universidad de Chile, basado en revisión de fichas clínicas e informes histopatológicos de pacientes con eritrodermia, entre 2005 y 2018. Se evaluó edad, sexo y variables clínicas (co-morbilidades, síntomas, días de evolución, ingreso hospitalario, informe histopatológico, diagnóstico y evolución). RESULTADOS: Total de 28 pacientes, 18 hombres (64%), edad promedio 59 años. Causa más frecuente de eritrodermia fue dermatosis pre-exis-tentes, con 15 casos (54%), que incluyen: psoriasis 9 (32%), dermatitis de contacto 3 (11%), PRP 2 (7%), dermatitis atópica 1 (4%). A estas le siguen: reacción adversa medicamentosa 6 (21%), idiopática 6 (21%) y Síndrome de Sezary 1 (4%). CONCLUSIÓN: El presente estudio corresponde a la primera serie de eritrodermias realizada en Chile. Destacan las dermatosis preexistentes como la principal causa, lo que se correlaciona con la literatura.

INTRODUCTION: Erythroderma is an infrequent cutaneous inflammatory disorder characterized by generalized erythematous compromise and desquamation, of more than 90% of total cutaneous surface. OBJECTIVE: Clinical and histopathological cha-racterization of patients with erythroderma in a Chilean university hospital. METHODOLOGY: Retrospective study, performed at the University of Chile Clinical Hospital, based on review of clinical records and histopatho-logical reports of patients with erythroderma, between 2005 and 2018. Age, sex and clinical variables were evaluated (co-morbidities, symp-toms, days of evolution, hospital admission, histopathological report, diagnosis and evolu-tion). RESULTS: A total of 28 patients, 18 were men (64%), average age 59 years. Most frequent cause of erythroderma was pre-existing dermatosis, with 13 cases (52%), which included: psoriasis 9 (32%), contact dermatitis 3 (11%), PRP 2 (7%), atopic dermatitis 1 (4%). These are followed by adverse drug eruption 6 (21%), idiopathic 6 (21%) and Sezary syndrome 1 (4%). CONCLUSION: The present study corresponds to the first series of erythrodermas performed in Chile. The pre-existing dermatoses were the main cause of erythroderma, which coincides with other reports.

Clinical analysis of 84 cases of erythrodermic psoriasis and 121 cases of other types of erythroderma from 2010-2015.

Erythroderma with complicated etiology is one of the severe skin diseases and has high mortality, of which the incidence was 0.5‰-1.5‰ in skin diseases. Erythrodermic psoriasis (EP) is the commonest type of erythroderma. In addition, there are drug-induced erythroderma, erythroderma secondary to preexisting dermatoses, malignancy-related erythroderma, and idiopathic erythroderma of unknown etiology. Erythroderma of different etiologies has various clinical manifestations, resulting in relevant curative effects and outcomes. In this article, we retrospectively investigated 205 erythroderma patients about clinical symptoms, auxiliary examination and treatments, and evaluated the efficacy and prognosis. There were 84 cases of EP among 205 patients, 10 cases of erythroderma caused by specific drugs, 77 cases of erythroderma secondary to preexisting dermatoses (excluding psoriasis), 7 cases of erythroderma patients suffering from malignancy and 27 cases with unknown causes. We concluded that the etiology of male patients in different age groups had significant difference. The incidence of EP was the highest among all types. The EP was commonly accompanied with hypoproteinemia, and changed into psoriasis vulgaris after treatment. Drug-induced erythroderma was commonly accompanied with fever, and mostly cured by systematic steroid therapy. For erythroderma secondary to preexisting dermatoses, the original dermatoses must be actively treated to achieve a satisfying prognosis. Erythroderma with malignancy or unknown causes had long-term duration, poor response to the treatment, and high potential to relapse. Therefore, clarifying the etiology, providing an appropiate and individual regimen, and regular follow-up are crucial for the successful treatment of erythroderma with unknown causes.

Exfoliative dermatitis: patterns of clinical presentation in a tropical rural and suburban dermatology practice in Nigeria.

BACKGROUND: Exfoliative dermatitis (ED) refers to a skin condition in which the skin is diffusely red and inflamed, with varying degrees and types of scaling. It is a serious medical condition and may require hospitalization. OBJECTIVES: This study was conducted to establish the demographics, etiology, and clinical outcomes of patients presenting with ED at the dermatology outpatient clinic at Irrua Specialist Teaching Hospital (ISTH), Irrua, Edo State, Nigeria. METHODS: The study used a retrospective, cross-sectional, descriptive design. The records of all dermatology patients attending the ISTH outpatient clinic were scrutinized for patients in whom ED was diagnosed and managed between March 1, 2004, and November 30, 2010. Relevant records were extracted and details subjected to statistical analysis. Main outcome measures were discharge and death. RESULTS: A total of 1422 dermatology patients were seen at the dermatology outpatient clinic during the study period. Of these, 39 were diagnosed with ED (hospital prevalence rate: 2.7%). The mean age of the ED patients was 60.0 ± 19.5 years (range: 21-90 years). Patients included 22 men and 17 women, giving a male:female ratio of 1.3:1. Of patients aged <40 years, 70% were HIV-positive, and antiretroviral medications accounted for 5% of cases of ED, whereas 50% of elderly patients had a suspected underlying malignancy. CONCLUSIONS: Erythroderma is an uncommon dermatologic presentation. Its recognized association with various internal malignancies makes it a diagnosis of significant clinical importance, particularly in resource-poor rural and suburban centers across Africa. Its recognition and prompt treatment can prevent early mortality or reduce morbidity in elderly patients who present with this condition in the early stages, especially if it is the first harbinger of internal malignancy.

Peeling skin diseases: 21 cases from Turkey and a review of the literature.

BACKGROUND: Peeling skin diseases (PSD) refer to a group of rare autosomal recessive dermatosis which are characterized by spontaneous, continual peeling of the skin. Three different clinical pictures can be distinguished: Inflammatory PSD also referred to as peeling skin syndrome (PSS) type B, non-inflammatory PSD also referred to as PSS type A, and localized forms i.e. acral type PSS. OBJECTIVE: To characterize the clinical and histopathological features of PSD in Turkey. METHODS: We retrospectively reviewed the medical records and clinical photographs of patients who were given diagnosis of PSD and conducted histopathological evaluation of skin biopsies to identify the site of cleavage. Also we evaluated the cases including age, gender, age onset, clinical and histological findings, family history, associated disorders and PSD type. RESULTS: Twenty-one patients with PSD were seen at Gulhane School of Medicine in Ankara between the years 1994 and 2010 in this retrospective study. All patients were men. Their ages were between 20 and 26 years (22.44±2.30, Mean age±SD). Of the patients, eight cases (40%) were type A, eight cases (40%) were type B, and five cases (20%) were acral type PSS. Eleven cases (52%) had parental consanguinity. Keratoderma, cheilitis, keratosis pilaris, melanonichia, clubbing, hyperhidrosis, onychodystrophy were observed in eight cases as an accompanying disorder. CONCLUSIONS: In this case series, PSD occurred rarely and also showed generally mild course of disease in Turkey and most likely related to consanguineous of marriages. Future investigations on PSD will contribute to our progressing alternative targets for pathogenesis-based therapy.

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.

Eritrodermia en 3160 pacientes internados en clínica médica / Erythroderma in 3160 in-patients at clinical department

La eritrodermia es una enfermedad inflamatoria cutánea generalizada. Generalmente se presenta en la edad media de la vida y en la mayoría de los casos es debida a exacerbación de dermatosis preexistentes. Puede asociarse a manifestaciones sistémicas como poliadenopatías, hepatomegalia, eritrosedimentación acelerada, leucocitosis y/o eosinofilia, entre otros. Con el fin de conocer la incidencia y la etiología de esta patología en nuestro hospital realizamos un estudio retrospectivo y observacional sobre 3160 historias clínicas de pacientes internados en el servicio de clínica médica. Detectamos 25 casos de eritrodermia lo cual implica una incidencia del 0.79%. La edad media de presentación fue 48 años. La etiología más frecuente fue la secundaria a medicamentos, siendo los β lactámicos los más involucrados. La alteración sistémica mayormente observada fue la eosinofilia (62%). El comienzo de la enfermedad en la mayoría de los casos fue agudo.

Erythroderma is an inflammatory and generalized skin disorder (more than 90% of the body surface area) accompanied by a variably degree of scaling. Often affects middle ages of life and it is usually caused by the exacerbation of a preexisting dermatoses. It could be associated to systemic manifestations like lymphadenopathy, liver enlargement, leukocytosis and elevated erythrocyte sedimentation. The aim of this study was to determinate the incidence and also the etiology of this pathology in our environment. We made a retrospective and observable study based on the research of 3160 clinic stories of patients that were in the hospital at the Clinical Department. We found that 25 patients were diagnosed with erithroderma (incidence 0.79%). The mean age at diagnosis was 48 years. The most common causative factor was drug reactions been the β lactams the most involved drug. Eosinophilia was the most important systemic manifestation (62%). The onset of erithroderma was usually abrupt and florid.

Bronchoplastic lobectomy with wide wedge resection for lung cancer with long-term steroid medication.

A 57-year-old man with erythrodermia, who was given 5-10 mg/day of prednisolone for 2.5 years, was admitted to our hospital for squamous cell lung carcinoma of the right upper lobe. A bronchoscopy revealed a tumor nearly obstructing the right upper lobe bronchus. A bronchoplastic lobectomy was performed with wide wedge resection of the main bronchus and truncus intermedius. A postoperative bronchoscopy revealed good healing of the anastomosis and a 3-dimensional construction of the bronchus with chest computed tomography demonstrated no stenotic change and no kinking change in the anastomosis. One year and 6 months after surgery, no local recurrence was seen in the region of bronchoplasty. Bronchoplastic lobectomy with wide wedge resection is a useful procedure in cases with risk factors of anastomotic dehiscence, such as after induction therapy or during long-term administration of adrenal cortical steroids.

Erythroderma: a clinical study of 97 cases.

BACKGROUND: Erythroderma is a rare skin disorder that may be caused by a variety of underlying dermatoses, infections, systemic diseases and drugs. METHODS: We reviewed the clinical, laboratory and biopsy material of 97 patients diagnosed with erythroderma who were treated in our department over a 6-year period (1996 through 2002). RESULTS: The male-female ratio was 1.85:1. The mean age at diagnosis was 46.2 years. The most common causative factors were dermatoses (59.7%), followed by drug reactions (21.6%), malignancies (11.3%) and idiopathic causes (7.2%). Carbamazepine was the most common drug (57.1%). The best clinicopathologic correlation was found in cutaneous T-cell lymphoma and pityriasis rubra pilaris related erythroderma. Apart from scaling and erythema that were present in all patients, pruritus was the most common finding (97.5%), followed by fever (33.6%), lymphadenopathy (21.3%), edema (14.4%) and hyperkeratosis (7.2%). CONCLUSION: This study outlines that underlying etiologic factors of erythroderma may show geographic variations. Our series had a high percentage of erythroderma secondary to preexisting dermatoses and a low percentage of idiopathic cases. There was no HIV-infected patient among our series based on multiple serum antibody tests. The clinical features of erythroderma were identical, irrespective of the etiology. The onset of the disease was usually insidious except in drug-induced erythroderma, where it was acute. The group associated with the best prognosis was that related to drugs.

The incidence of erythroderma: a survey among all dermatologists in The Netherlands.

BACKGROUND: Erythroderma is a rare skin disorder, and studies on its incidence and causes are lacking. The annual incidence has been estimated to be 1 to 2 patients per 100,000 inhabitants. OBJECTIVE: We investigated the incidence and cause of erythroderma in an unselected population and evaluated the referral pattern of erythrodermic patients by nonacademic dermatologists. METHODS: A survey was performed among all nonacademic dermatologists in The Netherlands, using a mailed questionnaire. Questions dealt with the number of patients diagnosed with erythroderma in the year 1997, the cause of the erythroderma, and whether these patients were referred to a university hospital. A questionnaire was also sent to all university hospitals. RESULTS: Seventy-eight percent of the nonacademic dermatologists answered the questionnaire, and all 8 university hospitals responded. In the year 1997, 141 patients were diagnosed with erythroderma in The Netherlands. The annual incidence of erythroderma based on these figures is 0.9 patients per 100,000 inhabitants. Compared with the university hospitals, erythroderma was more often diagnosed as an exacerbation of preexisting dermatoses (61% vs 51%; P =.37) and less often as idiopathic (14% vs 31%; P =.04) among the nonacademic dermatologists. Cutaneous T-cell lymphoma was rarely the cause, occurring in only 1% of patients diagnosed by the nonacademic dermatologists but in 6% of patients at the university hospitals. Overall, only 37% of nonacademic dermatologists referred patients with erythroderma to a university hospital. CONCLUSIONS: The incidence of erythroderma in The Netherlands is slightly lower than the earlier estimate in the literature. Moreover, the cause of erythroderma is different among patients diagnosed in an academic and a nonacademic setting. The majority of dermatologists in The Netherlands treat patients with erythroderma themselves and do not refer these patients to university hospitals.

Erythroderma in Thai patients.

Erythroderma is a clinical manifestation of dermatoses from different causes. Our objective was to determine its incidence, causes and clinicopathological features. Clinical, laboratory, and biopsy materials of 49 patients diagnosed as having erythroderma were reviewed. They were treated in our department over a 10-year period (1985 through 1994). The male-female ratio was 2:1. The mean age at diagnosis was 51.7 years. The most common causative factors were drugs (38.77%) and preexisting dermatoses (26.5%). Hepatomegaly, jaundice and abnormal liver function tests were found more commonly in the drug allergy group, while in cases with preexisting dermatoses nail involvement was a common finding. Clinicopathologic correlation in our study did not inform the etiology because it showed chronic nonspecific dermatitis or psoriasiform dermatitis, without any clue as to its origin. Drug-induced-erythroderma had an acute onset and a good prognosis with rapid resolution when the causative drug was withdrawn, while histopathology and laboratory findings were largely unrewarding.

Localization of the gene causing keratolytic winter erythema to chromosome 8p22-p23, and evidence for a founder effect in South African Afrikaans-speakers.

Keratolytic winter erythema (KWE), also known as "Oudtshoorn skin disease," or "erythrokeratolysis hiemalis," is an autosomal dominant skin disorder of unknown etiology characterized by a cyclical erythema, hyperkeratosis, and recurrent and intermittent peeling of the palms and soles, particularly during winter. Initially KWE was believed to be unique to South Africa, but recently a large pedigree of German origin has been identified. The disorder occurs with a prevalence of 1/7,000 in the South African Afrikaans-speaking Caucasoid population, and this high frequency has been attributed to founder effect. After a number of candidate regions were excluded from linkage to KWE in both the German family and several South African families, a genomewide analysis was embarked on. Linkage to the microsatellite marker D8S550 on chromosome 8p22-p23 was initially observed, with a maximum LOD score (Z(max)) of 9.2 at a maximum recombination fraction (theta(max)) of .0 in the German family. Linkage was also demonstrated in five of the larger South African families, with Z(max) = 7.4 at theta(max) = .02. When haplotypes were constructed, 11 of 14 South African KWE families had the complete "ancestral" haplotype, and 3 demonstrated conservation of parts of this haplotype, supporting the hypothesis of founder effect. The chromosome segregating with the disease in the German family demonstrated a different haplotype, suggesting that these chromosomes do not have a common origin. Recombination events place the KWE gene in a 6-cM interval between D8S550 and D8S552. If it is assumed that there was a single South African founder, a proposed ancestral recombinant suggests that the gene is most likely in a 1-cM interval between D8S550 and D8S265.

Erythroderma: analysis of 247 cases

Foi analisado o perfil de 247 doentes com eritrodermia em um período de 23 anos, de janeiro de 1962 a março de 1985, com o período de seguimento variando de 1 a 26 anos. Os doentes se apresentavam com eritema universal, descamaçäo e prurido com mais de 2 meses de duraçäo e a idade variava de 16 a 60 anos. A psoríase foi a doença associada mais freqüente, com uma proporçäo estimada de 44,9 por cento, as reaçöes cutâneas ao uso de drogas contribuíram com 7,3 por cento do total de casos e a associaçäo com reticuloses mostrou uma proporçäo de 4,1 por cento. A eritrodermia permaneceu como de causa desconhecida em 29,2 por cento dos casos. Näo foram observadas diferenças entre os sexos no que diz respeito à doença associada. Um ou mais resultados anátomo patológicos das biópsias de pele, em conjunto com o quadro clínico, foi diagnóstico ou sugestivo do diagnóstico da doença associada em 63,6 por cento dos casos. Recomendam-se biópsias de pele seriadas como o melhor método para a elucidaçäo diagnóstica da eritrodermia. Ao nível de significância P=0,05, foi encontrada uma proporçäo homem/mulher em 2 : 1. Especula-se se o agente causal da eritrodermia estaria relacionado à exposiçäo diferenciada entre os sexos a antígenos do meio ambiente

Erythroderma. A clinicopathological study of 56 cases.

BACKGROUND AND DESIGN: Erythroderma may be the result of many different causes. There are several publications on this subject, most of them from England, the United States, and the Scandinavian countries reporting a different incidence of each etiologic group. Our objective has been to determine the frequency of erythroderma in our environment, its cause, and patient evolution. We reviewed the clinical, laboratory, and biopsy material of 56 patients diagnosed with erythroderma who were treated in our department in the last 8 years (1984 through 1991). Patients were followed up to know the evolution of the erythroderma. RESULTS: The male-female ratio was 4:1. The mean age at diagnosis was 57 years. Dermatoses were the most frequent cause of erythroderma (62.5%), followed by topical or systemic drug reactions (16%), and cutaneous T-cell lymphomas (12.5%). Follow-up information was obtained from 42 patients (66%). Eight patients died (19%), but only in three cases was death directly related to erythroderma (7.14%). The group associated with the best prognosis was that related to drugs. The best clinicohistologic correlation was found in cutaneous T-cell lymphoma-related erythroderma. CONCLUSIONS: Erythroderma of unknown cause and protracted course may be secondary to senile atopic dermatitis, intake of drugs overlooked by the patients, and patients who are in slow progression to cutaneous T-cell lymphoma. Close follow-up of erythrodermas of unknown cause by repeating cutaneous biopsies will in time allow an early diagnosis in patients in the latter group.