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Thymoma-associated multiorgan autoimmunity (TAMA) is a rare paraneoplastic disorder that presents similarly to graft versus host disease (GVHD). We report a unique case of TAMA presenting as a GVHD-like erythroderma in an elderly male with a history of benign thymoma. Cutaneous histopathological findings demonstrated vacuolar interface dermatitis with numerous dyskeratotic keratinocytes, exocytosis of lymphocytes, and a mildly acanthotic epidermis, which can be seen in several different disease processes. Thus, careful consideration of clinical presentation, historical information, laboratory testing, and histopathologic findings led to the correct diagnosis and treatment., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

An 89-year-old Japanese woman presented with erythroderma associated with significant scaling. A histological examination showed acanthosis with hyperkeratosis and hyperkeratinization of the hair follicles. Genetic analyses using DNA from the peripheral blood revealed heterozygous mutations in IL36RN (c.115+6T>C) and CARD14 c.2648G>A (p.Arg883His). Based on these findings, we diagnosed her with erythroderma attributable to autoinflammatory keratinization disease. She then developed more than 30 small, round, well-defined, spots on her back and extremities that appeared histologically normal. We suspected that these spots might be revertant mosaicism. Immunohistochemical staining with p65, which is a component of nuclear factor kappa-light-chain-enhancer of activated B cells (NF-kB), revealed nuclear staining in epidermal keratinocytes in erythematous lesions, but not in the normal-looking spots. However, mutations in IL36RN and CARD14 unexpectedly persisted in the epidermis and dermis of the normal-looking spots., (© 2024 The Author(s). The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.)

Erythroderma, an inflammatory skin condition characterized by widespread erythema with variable degrees of exfoliation, pustulation, or vesiculobullous formation, is associated with high morbidity and mortality. Determining the underlying cause of erythroderma frequently presents a diagnostic challenge, which may contribute to the condition's relatively poor prognosis. This review covers the clinical presentation, pathophysiology, diagnosis, and treatment of erythroderma. It discusses similarities and differences among the many underlying etiologies of the condition and differences between erythrodermic and non-erythrodermic presentations of the same dermatosis. Finally, this article explores current research that may provide future tools in the diagnosis and management of erythroderma., (© 2024. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Inborn errors of the CARD11-BCL10-MALT1 (CBM) signalosome have recently been shown to underlie severe combined immunodeficiency (SCID) and combined immunodeficiency (CID) with variable immunological and clinical phenotypes, and patients usually present with recurrent bacterial, viral, and fungal infections, periodontal disease, enteropathy, dermatitis, and failure to thrive. In the present study, we describe the clinical and immunological characteristics of an Egyptian patient with a mutation in the MALT1 gene. The patient suffered from an itchy exfoliative skin rash and eczematous lesions over his face and flexural surface of the limbs. He also had dental enamel erosion, repeated attacks of diarrhea, and pneumonia. He had elevated serum IgE and normal B- and T-lymphocyte subset counts, but there was an arrest in the B-cell maturation. DOCK8 expression on the lymphocytes by flow cytometry was normal. Next-generation sequencing revealed a novel homozygous variant in the MALT1 gene (c.762dup in exon 5 of 17; p.Ile255TyrfsTer10); this variant is likely pathogenic, thus supporting the genetic diagnosis of immunodeficiency-12 (IMD12). Although the presence of eczema, recurrent sinopulmonary, and staphylococcal infections are suggestive of DOCK8 deficiency, they are also a finding in CARD11 and MALT1 deficiency. Thus, whenever DOCK 8 has been excluded, the molecular diagnosis is mandatory as this could lead to discovering more patients hence better understanding and reporting of the phenotype and natural history of the disease especially since there are very few documented cases. Early diagnosis will also enable the proper patient management by hematopoietic stem cell transplantation (HSCT) prior to the establishment of infections and pulmonary damage leading to a better outcome., Competing Interests: Compliance with ethical standards Conflict of interest The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

This case report presents the diagnostic journey of a man in his mid-70s who experienced shortness of breath, cough, recurrent episodes of fever, weight loss, pruritic erythroderma, uveitis and macrocytic anaemia. The initial diagnosis of cryptogenic organising pneumonia was made based on antibiotic refractory infiltrates seen in the lung CT scan. The patient initially responded favourably to immunosuppression but experienced a recurrence of symptoms when the corticosteroid dose was tapered. Despite ongoing systemic inflammation and refractory symptoms, it took nearly a year to establish the diagnosis of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic) syndrome. This case highlights the challenges in diagnosing and managing VEXAS syndrome due to its recent discovery and limited awareness in the medical community, as well as the need to consider this syndrome as a rare differential diagnosis of therapy-refractory pulmonary infiltrates., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Erythroderma is a dermatological emergency presenting with generalized erythema and scaling involving <90% of the body surface. 1 There are various causes of erythroderma, such as psoriasis, atopic dermatitis, phytophotodermatitis, pityriasis rubra pilaris, pemphigus foliaceus, cutaneous T-cell lymphoma, and drug eruptions. 2 However, erythroderma induced by dermatophytosis is encountered rarely. We, here, describe a case of erythroderma developed secondary to extensive dermatophytosis., (© Journal of the Association of Physicians of India 2024.)

Erythroderma is a dermatological emergency presenting with generalized erythema and scaling involving >90% of the body surface. 1 There are various causes of erythroderma, such as psoriasis, atopic dermatitis, phytophotodermatitis, pityriasis rubra pilaris, pemphigus foliaceus, cutaneous T-cell lymphoma, and drug eruptions. 2 However, erythroderma induced by dermatophytosis is encountered rarely. We, here, describe a case of erythroderma developed secondary to extensive dermatophytosis., (© Journal of the Association of Physicians of India 2024.)

A wide range of inherited and acquired conditions can manifest as infantile erythroderma, among which CARD14 -associated papulosquamous eruption (CAPE) is a rare cause. An infant boy presented with a psoriasiform rash that progressed to erythroderma and was unresponsive to topical steroids and cyclosporine. The early onset of the disease, its severity and resistance to conventional treatment were suggestive of a genetic cause. Genetic evaluation revealed a homozygous CARD14 variant of uncertain significance establishing the diagnosis of CAPE, and his parents were heterozygous carriers. There was only minimal improvement in the condition with supportive management and treatment with acitretin. Unfortunately, the child succumbed to sepsis and metabolic complications following a sudden worsening of skin disease. This case highlights the significance of genetic studies in diagnosing treatment-refractory cases of infantile erythroderma and emphasises the importance of early recognition of this rare condition., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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.

CARD14-associated papulosquamous eruption (CAPE) was proposed in 2018 to describe the clinical features of psoriasis and pityriasis rubra pilaris with CARD 14 mutations. We report a 5-month-old female infant who developed CAPE-associated erythroderma. Although she did not respond to conventional therapies, she responded well to ustekinumab treatment at the age of 4 years., (© 2022 Wiley Periodicals LLC.)

Erythroderma is challenging to diagnose. The aim of this single-centre retrospective study was to identify factors that can be used to improve the diagnosis of erythroderma. Among 91 patients with erythroderma, 21 were diagnosed with eczema, 17 with psoriasis, 20 with drug-induced erythroderma, 13 with erythrodermic mycosis fungoides and 20 with Sézary syndrome. Nail alterations, ear involvement, and severe scaling were significantly associated with psoriasis (p = 0.044). Fever and hypereosinophilia were associated with drug-induced erythroderma. Expression of programmed cell death protein 1 was observed in all skin biopsies. However, with Sézary syndrome, programmed cell death protein 1 expression was significantly higher than with other aetiologies. A programmed cell death protein 1 hormone receptor score (H-score) >50 was associated with Sézary syndrome (p < 0.001, sensitivity 75%, specificity 92%) as well as CXCL13 expression (p < 0.044). CD7 loss was more frequent with erythrodermic mycosis fungoides and Sézary syndrome (p = 0.022). This study reports the importance of programmed cell death protein 1 expression for the differential diagnosis of Sézary syndrome and other aetiologies, including erythrodermic mycosis fungoides.

Background: Psoriasis and lymphoma risk is widely debated, but few is known about misdiagnosis risk between erythrodermic psoriasis and lymphoma. In fact, erythroderma might represent a clinical presentation of psoriasis, cutaneous T-cell lymphomas and skin dissemination of systemic lymphomas., Methods: All patients referred to psoriasis outpatient service with a diagnosis of erythrodermic psoriasis were re-examined. Among them, all the patients with a subsequent lymphoma diagnosis were included. For each patient data concerning age, gender, age at erythroderma onset, age at lymphoma diagnosis, immune-suppressive therapy, type of lymphoma and relative stage, lymphoma treatment and outcome were obtained., Results: Twenty-five patients (15 females and 10 males) with a diagnosis of erythrodermic psoriasis were retrieved. Among them, 9 patients (5 males and 4 females) were affected by erythrodermic lymphoma, including 4 patients with Sèzary Syndrome, 3 with mycosis fungoides, and 2 with peripheral T-cell lymphoma not otherwise specified. Prior to lymphoma diagnosis all the patients (9/9) received cyclosporine, two (2/9) of them methotrexate, one (1/9) azatioprine, and two (2/9) systemic corticosteroids. The prognosis of our patients was poor, due to immune-suppressive drugs administration in patients with undiagnosed lymphoma. The only exception was one (1/9) patient with Sèzary Syndrome still alive with disease after 120 months of follow-up., Conclusions: In case of patients with erythroderma, multiple skin biopsies and specific peripheral blood studies like flow cytometry and T-cell receptor gene rearrangement analysis are required in order to avoid misdiagnosis risk between psoriasis and lymphoma.

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.

There is a paucity of data concerning the usefulness of trichoscopy in patients with erythroderma. The aim of the study was to compare the trichoscopic features in erythroderma of various aetiologies. In total, 49 patients with a determined cause of erythroderma [including atopic dermatitis (AD), mycosis fungoides (MF), allergic contact eczema (ACE), psoriasis (Pso), Sézary syndrome (SS), drug reaction, pityriasis rubra pilaris (PRP), dermatomyositis (DM), actinic reticuloid (AR), crusted scabies (CS) and pemphigus foliaceus (PF)] were included in the study. Dotted vessels were present in patients with AD, PRP, MF, SS and Pso, and absent in DM, CS and PF (χ², P < 0.02). Spermatozoon-like vessels were observed only in MF and SS (P = 0.001). Whitish-pinkish structureless areas were described in all patients with DM, AR and CS (P < 0.03). The type of vessel and the presence of whitish-pinkish structureless areas under trichoscopy may indicate the cause of erythroderma., (© 2021 British Association of Dermatologists.)

With the onset of the COVID-19 pandemic, healthcare providers have made increasing use of inpatient teledermatology; however, few studies have analysed the impact of teledermatology on patient outcomes. In this study, we investigated the diagnostic concordance between the primary team and teledermatologist, and we analysed the impact of this technology on the diagnosis and management of erythroderma, a condition with high morbidity and mortality. Overall, out of 2987 inpatient teledermatology encounters reviewed, we found 33 cases of erythroderma, and, of these, 78.8% had a change in diagnosis after teledermatology consult, 81.8% were recommended biopsy and all patients had a change in topical/systemic therapy. We hope to promote further study of the efficacy of teledermatology as it may begin to address large gaps in dermatological access to care particularly in regional and community hospitals., (© 2021 British Association of Dermatologists.)

Competing Interests: Les auteurs déclarent n'avoir aucun liens d’intérêts.

Competing Interests: The authors declare no conflict of interest.

We report a 52-year-old man who presented with erythroderma and nodular lesions on face manifesting as "Leonine facies". He had impaired sensation over the face and was initially diagnosed to have lepromatous leprosy and was treated with antileprosy drugs. Investigations showed a total Leukocyte count of 550 X 10 9 /l with 90% atypical lymphoid cells with prominent central nucleolus suggestive of prolymphocytes. On flow cytometry, these cells were positive for cytoplasmic CD3, CD2, CD5, CD7, CD4, and CD38 (dim) and were negative for CD1a and TdT and diagnosis of T-prolymphocytic leukemia was made.

Competing Interests: Les auteurs déclarent n'avoir aucun liens d’intérêts.

Competing Interests: AUTHOR DISCLOSUREDrs Sánchez-Espino, Gil-Téllez, Cantú-Reyna, and Martínez-Longoria have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

A 6-year-old boy with Down's syndrome presented with recurrent episodes of asymptomatic peeling of the skin from the trunk, palms, soles, and face since he was 2 years old. He was the first child of a non-consanguineous marriage. There was no history of any seasonal aggravation or variation. The peeling occurred once or twice a month. Several pediatricians and dermatologists diagnosed it to be either a staphylococcal scalded skin syndrome or a drug reaction. A temporal correlation could not be established with any drugs, including cefixime, amoxicillin, and paracetamol.

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., (© 2021 British Association of Dermatologists.)