Erythroderma in a Patient With Thymoma-Associated Myasthenia Gravis.

A woman in her 30s with myasthenia gravis diagnosed at age 27 years presented to the emergency department with severe erythroderma over the past 2 months. What is your diagnosis?

Crusted Scabies Presenting as Erythroderma in a Patient With Iatrogenic Immunosuppression for Treatment of Granulomatosis With Polyangiitis.

The diagnosis of scabies can be difficult when the infection presents as erythroderma. Crusted scabies is a severe form of scabies caused by cutaneous ectoparasitic infection by the mite Sarcoptes scabiei var hominis. Crusted scabies most commonly occurs in patients with underlying immunosuppression from acquired infection or subsequent to solid organ or bone marrow transplantation. We present a rare case of a patient with granulomatosis with polyangiitis (GPA) who developed azathioprine-induced myelosuppression and subsequent erythrodermic crusted scabies. It is critical to maintain a broad differential when patients present with erythroderma, especially in the setting of medication-induced immunosuppression for the treatment of autoimmune disease.

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.

Severe Erythroderma Due to Adult-onset Still's Disease-like Disease Related to Graft-versus-host Disease after Allogeneic Hematopoietic Stem Cell Transplantation.

A 35-year-old woman was treated with chemotherapy for leukemia. One year later, allogeneic hematopoietic stem cell transplantation (HSCT) was performed with umbilical cord blood. After nine months, she developed a spiking fever, sore throat, arthralgia, pleural effusion, hyperferritinemia, and persistent generalized pruritic erythema. A skin biopsy showed dyskeratotic cells in the epidermis, neutrophil infiltration in the epidermis and upper dermis, and neutrophils in the parakeratotic layer. Treatment with tocilizumab was effective. Adult-onset Still's disease (AOSD)-like disease related to graft versus-host disease (GVHD) after HSCT was suspected. Abnormal immune states related to GVHD may cause AOSD-like disease with more severe skin lesions than usual.

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.

Erythroderma with total scarring alopecia.

A woman in her 50s presented with total scarring ulcerated alopecia evolving for 10 years, and a recent budding tumour on the lower lip. Clinical examination revealed an associated exfoliated and infiltrated erythroderma with ulcerated cutaneous tumours of the legs, palmoplantar hyperkeratosis, hepatosplenomegaly and diffuse lymphadenopathies.Dermatoscopy of the scalp, leg tumours and infiltrated skin showed a typical yellowish background overlaid by arborescent vessels and whitish areas. Cutaneous biopsies of the same areas found sarcoidotic granulomas. Lip biopsy found an associated well-differentiated squamous cell carcinoma. Investigations confirmed the diagnosis of systemic sarcoidosis with an elevated converting enzyme level, mediastinal calcified lymphadenopathies in CT scan and deep localisations in positron emission tomography scan (thyroid, lymph nodes, mediastinum, liver, spleen and adrenal glands).The patient was treated with oral prednisone for sarcoidosis and intramuscular bleomycin followed by surgery and radiotherapy for squamous cell carcinoma. Sarcoidotic lesions healed, but a recurrence of her carcinoma led to death.

Las reacciones adversas a fármacos son la primera causa de eritrodermia. Estudio retrospectivo de 70 pacientes en un hospital universitario de Argentina / Adverse Drug Reactions Are the Main Causes of Erythroderma in an Argentinian Teaching Hospital: A Retrospective Study of 70 Patients

Antecedentes La eritrodermia es un cuadro caracterizado por un eritema y descamación que compromete al menos el 90% de la superficie corporal. Su etiología puede ser variable. La mayor parte de sus características clínicas y alteraciones del laboratorio son inespecíficas, lo que hace que el diagnóstico sea un desafío. Materiales y métodos Se realizó un estudio retrospectivo en el periodo comprendido entre el 1 de enero de 2010 y el 1 de junio de 2020. Se revisaron los antecedentes, las características clínicas, los hallazgos de laboratorio e histopatológicos, así como la evolución de todos los pacientes con una eritrodermia hospitalizados en el Hospital Italiano de Buenos Aires, con un seguimiento mínimo de 6 meses. Resultados Se incluyeron 70 pacientes. La edad media de aparición fue de 63 años con una relación hombre:mujer de 1,2:1. La principal causa de eritrodermia fueron las reacciones adversas a fármacos (48%), siendo la vancomicina el principal medicamento involucrado (30%), mientras que la segunda causa fueron las dermatosis preexistentes, dentro de las cuales la psoriasis fue la más común (42%). En el 40% de los pacientes se observó una adecuada correlación clínico-patológica con la primera biopsia, mientras que el rédito diagnóstico aumentó a un 76% con la segunda. El número máximo de biopsias requerido para efectuar el diagnóstico etiológico fue de 8 (2 pacientes). La evolución fue favorable en el 92% de los casos. Conclusión Las reacciones adversas a fármacos fueron la primera causa de eritrodermia y la vancomicina el fármaco responsable más frecuente. No se encontraron asociaciones estadísticamente significativas entre las variables estudiadas, ni se identificaron predictores de una determinada etiología, marcadores de mala evolución o factores de riesgo asociados a mortalidad (AU)

Background Erythroderma, characterized by erythema and scaling that affects at least 90% of the body, has diverse causes. Most of the clinical manifestations and laboratory findings are nonspecific, making diagnosis challenging. Material and methods Retrospective study of patients treated between January 1, 2010, and June 1, 2020. We reviewed the records to identify all patients with erythroderma who were hospitalized in Hospital Italiano de Buenos Aires and followed for at least 6 months. We extracted information on clinical histories, the characteristics of the episodes, laboratory and histopathologic findings, and clinical course. Results Seventy patients were studied. The mean age at onset was 63 years, and the ratio of men to women was 1.2:1. Adverse drug reactions caused the largest proportion of the rashes (48%), and vancomycin was the most common culprit (involved in 30% of the cases). The next most frequent cause was a preexisting skin disease, psoriasis being the most common (in 42%). The clinicopathologic correlation was adequate for diagnosis after the first biopsy in 40% of patients, but the diagnostic yield increased to 76% with the second biopsy. The largest number of biopsies required was 8, in 2 patients. The outcome was favorable in 92% of the cases. Conclusion Adverse reactions to medications accounted for the largest proportion of erythroderma cases in this series, and vancomycin was the main culprit. We found no statistically significant associations among the variables studied. Nor did we identify potential predictors of causes, poor outcomes, or mortality (AU)

[Translated article] Adverse Drug Reactions Are the Main Causes of Erythroderma in an Argentinian Teaching Hospital: A Retrospective Study of 70 Patients / Las reacciones adversas a fármacos son la primera causa de eritrodermia. Estudio retrospectivo de 70 pacientes en un hospital universitario de Argentina

Background Erythroderma, characterized by erythema and scaling that affects at least 90% of the body, has diverse causes. Most of the clinical manifestations and laboratory findings are nonspecific, making diagnosis challenging. Material and methods Retrospective study of patients treated between January 1, 2010, and June 1, 2020. We reviewed the records to identify all patients with erythroderma who were hospitalized in Hospital Italiano de Buenos Aires and followed for at least 6 months. We extracted information on clinical histories, the characteristics of the episodes, laboratory and histopathologic findings, and clinical course. Results Seventy patients were studied. The mean age at onset was 63 years, and the ratio of men to women was 1.2:1. Adverse drug reactions caused the largest proportion of the rashes (48%), and vancomycin was the most common culprit (involved in 30% of the cases). The next most frequent cause was a preexisting skin disease, psoriasis being the most common (in 42%). The clinicopathologic correlation was adequate for diagnosis after the first biopsy in 40% of patients, but the diagnostic yield increased to 76% with the second biopsy. The largest number of biopsies required was 8, in 2 patients. The outcome was favorable in 92% of the cases. Conclusion Adverse reactions to medications accounted for the largest proportion of erythroderma cases in this series, and vancomycin was the main culprit. We found no statistically significant associations among the variables studied. Nor did we identify potential predictors of causes, poor outcomes, or mortality (AU)

Antecedentes La eritrodermia es un cuadro caracterizado por un eritema y descamación que compromete al menos el 90% de la superficie corporal. Su etiología puede ser variable. La mayor parte de sus características clínicas y alteraciones del laboratorio son inespecíficas, lo que hace que el diagnóstico sea un desafío. Materiales y métodos Se realizó un estudio retrospectivo en el periodo comprendido entre el 1 de enero de 2010 y el 1 de junio de 2020. Se revisaron los antecedentes, las características clínicas, los hallazgos de laboratorio e histopatológicos, así como la evolución de todos los pacientes con una eritrodermia hospitalizados en el Hospital Italiano de Buenos Aires, con un seguimiento mínimo de 6 meses. Resultados Se incluyeron 70 pacientes. La edad media de aparición fue de 63 años con una relación hombre:mujer de 1,2:1. La principal causa de eritrodermia fueron las reacciones adversas a fármacos (48%), siendo la vancomicina el principal medicamento involucrado (30%), mientras que la segunda causa fueron las dermatosis preexistentes, dentro de las cuales la psoriasis fue la más común (42%). En el 40% de los pacientes se observó una adecuada correlación clínico-patológica con la primera biopsia, mientras que el rédito diagnóstico aumentó a un 76% con la segunda. El número máximo de biopsias requerido para efectuar el diagnóstico etiológico fue de 8 (2 pacientes). La evolución fue favorable en el 92% de los casos. Conclusión Las reacciones adversas a fármacos fueron la primera causa de eritrodermia y la vancomicina el fármaco responsable más frecuente. No se encontraron asociaciones estadísticamente significativas entre las variables estudiadas, ni se identificaron predictores de una determinada etiología, marcadores de mala evolución o factores de riesgo asociados a mortalidad (AU)

[A patient with erythroderma and pruritus: Sézary syndrome]. / Een man met jeuk en roodheid van de huid.

BACKGROUND: Erythroderma could be the first sign of a cutaneous T-cell lymphoma (CTCL), such as Sézary syndrome. Causes of erythroderma include inflammatory dermatosis, toxicoderma, paraneoplastic erytroderma, and CTCL. Hence, diagnosing Sézary syndrome can be difficult. Sézary syndrome is a rare, aggressive disease characterized by erythroderma, generalized lymphadenopathy and the presence of clonally related neoplastic T-cells in skin, peripheral blood, and lymph nodes. Treatment consists of photochemotherapy (PUVA), radiotherapy, immunomodulatory agents, low dose cytotoxic agents, and intensive chemotherapy. Immunotherapy directed against CCR4 and PD1 are new, promising developments. CASE DESCRIPTION: A 51-year-old man presented with a 1-year history of progressive, itchy erythroderma and lymphocytosis. After extensive cytomorphological, histopathological and molecular examination the diagnosis of Sézary syndrome could be established. Combination treatment of interferon and photochemotherapy (PUVA) was started. CONCLUSION: Diagnostic delay in Sézary syndrome is common. Integrated cytomorphological, immunological, and molecular evaluation of peripheral blood in patients with unexplained erythroderma non-responsive to (topical) treatment is warranted.

Proposal for a 6-step approach for differential diagnosis of neonatal erythroderma.

The broad differential diagnosis of neonatal erythroderma often poses a diagnostic challenge. Mortality of neonatal erythroderma is high due to complications of the erythroderma itself and the occasionally severe and life-threatening underlying disease. Early correct recognition of the underlying cause leads to better treatment and prognosis. Currently, neonatal erythroderma is approached on a case-by-case basis. The purpose of this scoping review was to develop a diagnostic approach in neonatal erythroderma. After a systematic literature search in Embase (January 1990 - May 2020, 74 cases of neonatal erythroderma were identified, and 50+ diagnoses could be extracted. Main causes were the ichthyoses (40%) and primary immunodeficiencies (35%). Congenital erythroderma was present in 64% (47/74) of the cases, predominantly with congenital ichthyosis (11/11; 100%), Netherton syndrome (12/14, 86%) and Omenn syndrome (11/23, 48%). Time until diagnosis ranged from 102 days to 116 days for cases of non-congenital erythroderma and congenital erythroderma respectively. Among the 74 identified cases a total of 17 patients (23%) died within a mean of 158 days and were related to Omenn syndrome (35%), graft-versus-host disease (67%) and Netherton syndrome (18%). Disease history and physical examination are summarized in this paper. Age of onset and a collodion membrane can help to narrow the differential diagnoses. Investigations of blood, histology, hair analysis, genetic analysis and clinical imaging are summarized and discussed. A standard blood investigation is proposed, and the need for skin biopsies with lympho-epithelial Kazal-type related Inhibitor staining is highlighted. Overall, this review shows that diagnostic procedures narrow the differential diagnosis in neonatal erythroderma. A 6-step flowchart for the diagnostic approach for neonatal erythroderma during the first month of life is proposed. The approach was made with the support of expert leaders from international multidisciplinary collaborations in the European Reference Network Skin-subthematic group Ichthyosis.