Erythroderma combined with deeper dermal dermatophytosis due to Trichophyton rubrum in a patient with myasthenia gravis: first case report and literature review.

BACKGROUND: Dermatophytes are the most common causative pathogens of mycoses worldwide and usually cause superficial infections. However, they can enter deep into the dermis lead to invasive dermatophytosis such as deeper dermal dermatophytosis on rare occasions. Erythroderma is a severe dermatological manifestation of various diseases resulting in generalized skin redness, but erythroderma due to fungi infections is barely reported. In this article, we reported the first case of erythroderma combined with deeper dermal dermatophytosis due to Trichophyton rubrum (T. rubrum) in a patient with myasthenia gravis. CASE PRESENTATION: A 48-year-old man was hospitalized because of erythema with scaling and nodules covering his body for a month. The patient had a history of myasthenia gravis controlled by regularly taking prednisolone for > 10 years and accompanied by onychomycosis and tinea pedis lasting > 8 years. Based on histopathological examinations, fungal cultures, and DNA sequencing results, the patient was finally diagnosed with dermatophyte-induced erythroderma combined with deeper dermal dermatophytosis caused by T. rubrum. After 2 weeks of antifungal treatment, the patient had recovered well. CONCLUSIONS: This case report shows that immunosuppressed patients with long histories of superficial mycoses tend to have a higher risk of developing invasive dermatophytic infections or disseminated fungal infections. Dermatologists should be alert to this condition and promptly treat the superficial dermatophytosis.

When Body Art Goes Awry-Severe Systemic Allergic Reaction to Red Ink Tattoo Requiring Surgical Treatment.

The aim of this report is to present a case of a patient who developed unusual systemic hypersensitivity reaction to a red-pigmented tattoo and to discuss diagnostic difficulties in case of systemic reactions to tattoo ink. The patient reported erythroderma on his arms and chest accompanied by plaque elevation of red parts of his most recently performed forearm tattoo as his primary symptoms. His health condition entailed a prolonged topical and intravenous immunosuppressive therapy, which proved ineffective. Over a year after emergence of initial symptoms, he presented to the Plastic Surgery Clinic with generalized erythroderma, systemic lymphadenopathy, elevation and granuloma formation in red tattoos on his forearm and complaints of fatigue and inability to participate fully in work-related and social activities. The patient underwent six staged excisions with direct closures, flap plasties and full-thickness skin grafts. Following completion of each surgical resection, the patient's symptoms gradually subsided. We find this case illustrative of a clinical challenge that delayed hypersensitivity reactions to red tattoos may pose. Furthermore, we provide insights on management of hypersensitivity reactions. This report underlines the importance of social awareness of and public health approach to tattoo complications as key to successful prevention, identification and treatment of adverse reactions to tattoos.

Omenn Syndrome due to RAG1 Mutation Presenting With Nonimmune Hydrops Fetalis in Two Siblings.

Omenn syndrome (OS) is a rare variant of severe combined immunodeficiency characterized by susceptibility to severe opportunistic infections and peculiar manifestations, such as protein-losing erythroderma, alopecia, hepatosplenomegaly, lymphadenopathies, and severe diarrhea. The typical form of the disease is caused by hypomorphic mutation of the recombination-activating genes (RAG1 and RAG2), which are critical in initiating the molecular processes leading to lymphocyte and immunoglobulin receptor formation. Affected patients lack B cells, whereas autoreactive oligoclonal T cells infiltrate the skin, gut, spleen, and liver. In the absence of hematopoietic stem cell transplantation, patients with OS usually succumb early in life because of opportunistic infections. The incidence of OS is estimated to be <1 per 1 000 000; however, the actual frequency is difficult to ascertain. We report 2 siblings affected by OS due to a homozygous frameshift mutation (NM_000448.3:c.519delT, p.E174Sfs*26) in the RAG1 gene presenting with nonimmune hydrops fetalis (NIHF). To the best of our knowledge, this is the first reported association between OS and NIHF. NIHF specifically refers to the presence of ≥2 abnormal fluid collections in the fetus, without red blood cell alloimmunization. A broad spectrum of pathologies is associated with NIHF; however, in ∼20% of the cases, the primary cause remains unclear. Understanding the etiology of NIHF is essential for guiding clinical management, determining prognosis, and informing parents regarding recurrence risk. Our case contributes to expanding the spectrum of OS presentation and highlights the importance of a complete immunologic and genetic workup in otherwise unexplained cases of NIHF.

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.

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.

Cutaneous T-cell lymphoma in erythrodermic cases may be suspected on the basis of scalp examination with dermoscopy.

Erythrodermic variants of cutaneous T-cell lymphoma (CTLC) are one of the case of erythroderma. The aim of the study was to assess the value of scalp dermoscopy in differentiation between erythrodermic CTCL, psoriasis, and atopic dermatitis. A total of 76 patients were included into the study (16 patients with erythrodermic CTCL, 20 patients with psoriatic erythroderma, 20 with erythrodermic atopic dermatitis, and 20 healthy volunteers). The most common trichoscopic features of erythrodermic CTCL were: numerous pili torti, numerous broken hairs, white thick interfollicular bands, and patchy hyperpigmentation of the background. They were observed in 81% (13/16), 75% (12/16), 56% (9/16), and 37.5% (6/16) of patients with CTCL, respectively (p < 0.001). Other specific features of erythrodermic CTCL were 8-shaped hairs (19%; 3/16) and visible anagen bulbs (12.5%; 2/16) (p < 0.05 and p = 0.052, respectively). The most common vascular pattern of erythrodermic CTCL was perifollicular arrangement of glomerular (50%; 8/16; p < 0.001) or linear vessels (31%; 5/16; p < 0.05). Follicular spicules-like scaling was pathognomonic for erythrodermic CTCL (12%, 2/16) although its presence did not reach statistical significance (p = 0.052). In conclusion, the characteristic trichoscopic findings of erythrodermic CTCL are numerous pili torti, eight-shaped hairs, thick white interfollicular bands, color heterogeneity of the background and perifollicular arrangement of vessels.

A systematic review of treatment strategies for erythrodermic psoriasis.

Background: Erythrodermic psoriasis (EP) is a severe, rare form of psoriasis that can be life threatening. Treatment of EP is usually based on anecdotal evidence or past clinical experience, which is in part due to the rarity and often emergent nature of this psoriasis subtype.Methods: In December 2018, a keyword search for 'erythrodermic psoriasis' and 'treatment' was conducted. All studies investigating treatment strategies for EP in at least 2 patients were included in this review.Results: The database search yielded 921 results, and 23 studies comprising over 200 patients with EP were included in the final analysis. Biologics including tumor necrosis factor inhibitors (infliximab [n = 4], etanercept [n = 2], and adalimumab [n = 1]), interleukin-17 inhibitors (secukinumab [n = 3], ixekizumab [n = 2], and brodalumab [n = 1]), ustekinumab (n = 4), and guselkumab (n = 1) have been shown to rapidly achieve clinical improvement in patients with EP. The included studies also demonstrated efficacy of systemic agents cyclosporine (n = 4), etretinate (n = 3), and methotrexate (n = 1).Conclusions: A fair number of poor-quality studies support the use of various biologic and systemic therapies in the treatment of EP. Treatment of EP should be based on the severity of the clinical scenario as well as patient comorbidities.

Síndrome de DRESS asociado al uso de anticonvulsivantes / DRESS syndrome associated to the use of anti-convulsiveness

Introducción: El síndrome Drug Reaction with Eosinophilia and Systemic Symptoms - DRESS, constituye una grave reacción adversa a medicamentos, principalmente a fármacos anticonvulsivantes. Objetivo: Describir la evolución clínica de un síndrome de DRESS en una paciente atendida en el Hospital Militar Central "Dr. Luis Díaz Soto". Caso Clínico: Paciente femenina de 27 años de edad con antecedentes patológicos personales de epilepsia. Tres meses luego de iniciada terapia con difenilhidantoína aparece fiebre, exantema maculopapular que progresa a eritrodermia exfoliativa, signos de daño hepático, adenopatías cervicales y eosinofilia. Se diagnosticó síndrome de DRESS secundaria al uso de anticonvulsivantes. Conclusiones: La evolución clínica resultó favorable, luego de la retirada del fármaco y la aplicación de esteroides por vía oral(AU)

Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms - DRESS, syndrome constitutes a serious adverse reaction to medications, mainly anticonvulsant drugs. Objective: To describe the clinical evolution of DRESS syndrome in a patient treated at the Hospital Militar Central "Dr. Luis Díaz Soto". Case Report: 27-year-old female patient with a personal pathological history of epilepsy. Three months after initiation of diphenylhydantoin therapy, fever appeared maculopapular rash that progressed to exfoliative erythroderma, signs of liver damage, cervical adenopathies and eosinophilia. DRESS syndrome was diagnosed secondary to the use of anticonvulsants. Conclusions: The clinical evolution was favorable, after the withdrawal of the drug and the application of steroids orally(AU)

Phenotypic suppression of acral peeling skin syndrome in a patient with autosomal recessive congenital ichthyosis.

Autosomal recessive congenital ichthyosis (ARCI) manifests with generalized scaling often associated with generalized erythema. Mutations in at least 13 different genes have been reported to cause ARCI. Acral peeling skin syndrome (APSS) is a rare autosomal recessive disorder manifesting with peeling over the distal limbs and dorsal surfaces of hands and feet. APSS is mostly due to mutations in TGM5, encoding transglutaminase 5. Both ARCI and APSS are fully penetrant genetic traits. Here, we describe a consanguineous family in which one patient with mild ARCI was found to carry a homozygous mutation in ALOXE3 (c.1238G > A; p.Gly413Asp). The patient was also found to carry a known pathogenic homozygous mutation in TGM5 (c.1335G > C; p.Lys445Asn) but did not display acral peeling skin. Her uncle carried the same homozygous mutation in TGM5 but carried the ALOXE3 mutation in a heterozygous state and showed clinical features typical of APSS. Taken collectively, these observations suggested that the ALOXE3 mutation suppresses the clinical expression of the TGM5 variant. We hypothesized that ALOXE3 deficiency may affect the expression of a protein capable of compensating for the lack of TGM5 expression. Downregulation of ALOXE3 in primary human keratinocytes resulted in increased levels of corneodesmosin, which plays a critical role in the maintenance of cell-cell adhesion in the upper epidermal layers. Accordingly, ectopic corneodesmosin expression rescued the cell-cell adhesion defect caused by TGM5 deficiency in keratinocytes as ascertained by the dispase dissociation assay. The present data thus provide evidence for phenotypic suppression in a human hereditary skin disorder.

Treatment of erythrodermic psoriasis with biologics: A systematic review.

BACKGROUND: Biologic medications for plaque psoriasis have been used to treat erythrodermic psoriasis (EP). Since the guidelines for management of EP were published, new biologic medications have been approved for the treatment of plaque psoriasis. OBJECTIVE: To analyze the evidence of biologic medications in the treatment of EP based on response and tolerability. METHODS: A comprehensive search was conducted with the PubMed, Cochrane Library, Embase, and Scopus databases through December 31, 2018. Studies reporting 1 or more cases of EP, defined as >75% body surface area involvement, in patients aged ≥18 years treated with biologics were included. Baseline Psoriasis Area and Severity Index score, score improvement, and adverse events were documented. Adequate response to treatment was defined as Psoriasis Area and Severity Index ≥50. RESULTS: Included were 43 articles, yielding a total of 179 patients. Most patients responded at some point during treatment, with a higher level of evidence for infliximab, ustekinumab, ixekizumab, and guselkumab. Infection was the most common adverse event (n = 35). LIMITATIONS: Data are limited to case reports, case series, and uncontrolled studies. CONCLUSION: Patients with EP treated with biologics demonstrated positive responses and treatment was well-tolerated, with a weak recommendation and limited quality of evidence in favor of infliximab, ustekinumab, ixekizumab, and guselkumab.

Familial "benign" pemphigus? Erythroderma and fatal outcome

Abstract Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

Familial "benign" pemphigus? Erythroderma and fatal outcome.

Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.