Síndrome de DRESS asociado al consumo de alopurinol: reporte de caso

El síndrome de DRESS (por sus siglas en inglés Drug Reaction with Eosinophilia and Systemic Symptoms) representa una farmacodermia grave con diferentes manifestaciones clínicas y paraclínicas secundarias a una reacción de hipersensibilidad farmacológica. Su incidencia exacta es desconocida pero se estima entre 1 a 1000 y 1 a 10000 casos de exposición a fármacos asociados. Se caracteriza por dermatosis generalizada extensa en conjunto con afección orgánica, linfadenopatia, eosinofilia y linfocitosis atípica. Entre los fármacos comúnmente asociados se encuentran anticomiciales aromáticos, carbamazepina, sulfonamidas y el alopurinol. Mediante el uso de la puntuación RegiSCAR es posible confirmar o descartar una sospecha de diagnóstico. El tratamiento depende de la severidad de presentación incluyendo esteroides tópicos hasta esteroide sistémico de duración variable dependiendo respuesta clínica y bioquímica. Se reporta tasas de mortalidad del 10 al 20% siendo la insuficiencia hepática la principal causa de muerte en estos pacientes. Se presenta el caso de un paciente femenino de 71 años de edad que, posterior a tratamiento con alopurinol, debuta con eritrodermia secundaria a Síndrome de DRESS.

DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) represents severe pharmacodermia with different clinical and paraclinical manifestations secondary to a drug hypersensitivity reaction. The exact incidence is unknown, but it is estimated to be between 1 in 1,000 and 1 in 10,000 cases of exposure to associated drugs. It is characterized by extensive generalized dermatosis, in conjunction with organic involvement, lymphadenopathy, eosinophilia, and atypical lymphocytosis. Commonly associated drugs include aromatic anticonvulsants, carbamazepine, sulfonamides, and allopurinol. By using the RegiSCAR score, it is possible to confirm or rule out a suspected diagnosis. Treatment depends on the severity of presentation, including topical steroids to systemic steroids of variable duration, depending on clinical and biochemical responses. Mortality rates of 10 - 20% have been reported, with liver failure being the main cause of death in these patients. We present the case of a 71-year-old female patient who, after treatment with allopurinol, developed erythroderma secondary to DRESS Syndrome.

Mutation analysis of the PNPLA1 gene in three families with congenital ichthyosiform erythroderma / 中华皮肤科杂志

Objective:To detect gene mutations in 3 Chinese families with congenital ichthyosiform erythroderma.Methods:Exome sequencing of peripheral blood DNA was performed for 3 probands clinically diagnosed with congenital ichthyosiform erythroderma by using a gene panel targeting hereditary skin diseases to identify mutation sites. Primers were designed according to the mutation sites for PCR amplification, and Sanger sequencing was performed to verify the mutations in probands and other family members in order to identify the cause of the disease.Results:The probands 1 and 2 presented with generalized skin dryness and scaling, and polygonal dark brown scales on the extensor aspect of the lower limbs; the proband 3 mainly presented with well-circumscribed erythema, papules and scales scattered on the trunk and extremities. All probands denied family history of similar diseases. Genetic testing showed that the proband 1 carried compound heterozygous mutations c.100G>A and c.377G>A in the PNPLA1 gene, which were inherited from her mother and father respectively; the proband 2 carried compound heterozygous mutations c.320T>A and c.434T>C in the PNPLA1 gene, which were inherited from her mother and father respectively; a homozygous mutation c.1300delG was identified in the PNPLA1 gene in the proband 3. The mutations co-segregated with the disease phenotypes in the two families with compound heterozygous mutations. Among the 5 identified mutations, the two missense mutations (c.377G>A and c.320T>A) were firstly reported.Conclusion:Biallelic mutations in the PNPLA1 gene are the causative mutations responsible for autosomal recessive congenital ichthyosis in the three probands, and the newly reported mutations expand the mutation spectrum in the disease.

Role of flow cytometric analysis of peripheral blood in the diagnosis of lymphoma-associated erythroderma / 中华皮肤科杂志

Objective:To investigate the value of flow cytometric analysis of peripheral blood in the diagnosis of erythroderma.Methods:A total of 29 patients with erythroderma were collected from Hospital of Dermatology, Chinese Academy of Medical Sciences from September 2017 to December 2020, including 6 with erythrodermic mycosis fungoides (EMF) , 5 with Sézary syndrome (SS) , 18 with inflammatory erythroderma (IE) with different etiologies. Four healthy volunteers served as healthy controls. Flow cytometry was performed to detect peripheral blood lymphocyte subsets, immunophenotypes and clonality, and their differences were analyzed between inflammatory erythroderma and lymphoma-related erythroderma. One-way analysis of variance and least significant difference- t test were used for comparisons between groups. Results:The proportions of T cells, B cells, NK cells and CD4 -CD8 - cells significantly differed among the EMF group, SS group, IE group and control group (all P < 0.001) . The proportion of T cells was significantly higher in the SS group (93.8% ± 3.4%) than in the EMF group (42.7% ± 6.4%) and IE group (46.0% ± 6.8%, t = 12.8, 14.4, respectively, both P < 0.001) , and the proportion of CD4 -CD8 - cells was significantly lower in the IE group (0.37% ± 0.40%) than in the EMF group (2.93% ± 0.84%) and SS group (2.38% ± 0.74%, t = 9.2, 6.7, respectively, both P < 0.05) . The expression of clonal T-cell receptor β-chain variable region (TCR-vβ) was not detected in healthy controls or IE patients; the T cell subsets expressing clonal TCR-vβ were detected in 3 cases of EMF and all cases of SS, and they were all identified to be cells with a CD4 +CD7 -CD26 - phenotype. There were significant differences among the above 4 groups of subjects in the proportions of CD4 + T lymphocytes expressing chemokine receptors CCR4, CXCR3, CCR5, cutaneous lymphocyte antigen (CLA) or programmed death receptor-1 (PD-1) on the cell surface (all P < 0.001) . Compared with the SS group and EMF group, the IE group showed significant decreased proportions of CD4 + T lymphocytes expressing CCR4, CLA or PD-1 (all P < 0.001) , but significantly increased proportions of CD4 + T lymphocytes expressing CXCR3 or CCR5 (all P < 0.001) . Conclusion:Flow cytometric analysis of peripheral blood lymphocyte subsets, immunophenotypes and clonality can provide a reference for the etiological diagnosis of erythroderma, and is helpful for the differential diagnosis between lymphoma-associated erythroderma and inflammatory erythroderma.

Chanarin-Dorfman syndrome: the first case reported in China / 中华皮肤科杂志

Objective:To detect gene mutations and make a diagnosis in a family with ichthyosis accompanied by liver injury.Methods:Clinical data were collected from the proband, and genomic DNA was extracted from peripheral blood samples from the proband and his parents. Exome sequencing was performed in the proband by using a gene panel targeting hereditary skin diseases to identify mutation sites, and then the candidate mutation site was verified by PCR and Sanger sequencing in the family members. Results of peripheral blood smear examination and other auxiliary examinations were collected from the proband and his parents and analyzed.Results:The proband presented with generalized dry skin and tiny white scales on the lower limbs, accompanied by elevated transaminase levels, mild sensorineural hearing loss in both ears and fatty liver. Exome sequencing revealed a homozygous mutation c.933dupA in exon 6 of the ABHD5 gene encoding CGI-58 protein in the peripheral blood genomic DNA of the proband, resulting in a frameshift mutation p.R312Tfs*45 in the amino acid sequence. Heterozygous mutations at this site were identified in his father and mother. The mutation cosegregated with the disease phenotype in the family. The peripheral blood smear examination of the proband showed lipid vacuoles in neutrophils, which were called Jordan anomaly. Conclusion:The diagnosis of Chanarin-Dorfman syndrome was made in the proband based on the presentation of ichthyosis-like skin lesions and abnormal liver function, as well as the homozygous mutation in the ABHD5 gene and Jordan anomaly in peripheral blood smears.

Drug-induced erythroderma in patients with acquired immunodeficiency syndrome / 世界急诊医学杂志（英文）

@#BACKGROUND: To explore the clinical manifestations, diagnosis, and treatment of patients with acquired immunodeficiency syndrome (AIDS) complicated with drug-induced erythroderma. METHODS: The clinical data of 12 AIDS patients with drug-induced erythroderma in our hospital were retrospectively analyzed. The general information, offending medications, complications, modified severity-of-illness score for toxic epidermal necrolysis (SCORTEN) scores, and disease outcome spectrums were analyzed. RESULTS: Drug-induced erythroderma was mostly caused by antiviral drugs, antituberculosis drugs, antibiotics, traditional Chinese medicine, and immune checkpoint inhibitors. The spectrum of sensitizing drugs was broad, the clinical situation was complex, and infections were common. The affected areas were greater than 40% body surface area in all patients. The modified SCOTERN score averaged 3.01±0.99. All patients were treated with glucocorticoids, and nine patients were treated with intravenous immunoglobulin (IVIG) pulse therapy at the same time. The average time to effectiveness was 7.08±2.23 days, and the average hospital stay was 17.92±8.46 days. Eleven patients were cured, and one patient died of secondary multiple infections, who had a modified SCORTEN score of 5 points. The mortality rate in this study was 8.3%. CONCLUSIONS: The clinical situation of AIDS patients with drug-induced erythroderma in hospitalized patients is complex and the co-infection rate is high. The use of modified SCORTEN score may objectively and accurately assess the conditions, and the use of glucocorticoid combined with IVIG therapy may improve the prognosis.

Eritrodermia y Linfomas Cutáneo de Células T / Erythroderma and Cutanous T-Cell Lymphomas / Linfomas de células T e erâmulas

RESUMEN La eritrodermia es una erupción eritematosa más o menos escamosa que afecte a más del 90% de la superficie corporal. Se trata de una enfermedad capaz de comprometer el pronóstico vital y que puede complicarse con desequilibrios hidroelectrolíticos, trastornos de la termorregulación, infecciones, así como con fallo cardiovascular. En la presente publicación referimos el caso de un paciente masculino de 72 años con antecedentes de hipertensión arterial y de Lepra que recibió poliquimioterapia, es ingresado en el servicio de dermatología en el período de observación, pues hace alrededor de un año comenzó a presentar enrojecimiento de la piel y múltiples escamas. Al momento del examen se observa paciente eritrodérmico, ectropión, distrofia ungueal importante, con adenopatías palpables axilares e inguinales, asociado un prurito intenso generalizado. Se realizan estudios para el diagnostico incluyendo biopsia de ganglios inguinal y de piel, esta última compatible con Micosis Fungoide. Actualmente se reconocen tres variantes eritrodérmicas en el linfoma cutáneo de células T: el síndrome de Sézary, la Eritrodermia en micosis fungoide y la Eritrodermia en linfomas cutáneos de células T:No Especificada. Si bien se entiende al síndrome de Sézary como una expresión leucémica del linfoma cutáneo de células T eritrodérmico, con numerosas células de Sézary en piel, sangre y otros tejidos, la Eritrodermia en micosis fungoide se determinaría ante la ausencia de estos mismos hallazgos hematológicos e histológico ante un cuadro clínico eritrodérmico. Se hace necesaria la publicación del artículo por la forma de presentación de la micosis fungoide, teniendo en cuenta que esta dermatosis tiene un sin número de diagnósticos diferenciales que la hace la gran simuladora en la Dermatología.

ABSTRACT Erythroderma is a more or less scaly erythematous rash that affects more than 90% of the body surface area. It is a disease capable of compromising the vital prognosis and that can be complicated by hydroelectrolytic imbalances, thermoregulation disorders, infections, as well as cardiovascular failure. In this publication we refer to the case of a 72-year-old male patient with a history of high blood pressure and leprosy who received polychemotherapy, is admitted to the dermatology service in the observation period, as about a year ago he began to develop redness of the skin and multiple scales. At the time of the examination, an erythrothermal patient, ectropion, important ungueal dystrophy, with axillary and inguinal palpable adenopathies, associated with a widespread intense itching, is observed. Studies are conducted for diagnosis including inguinal and skin node biopsy, the latter compatible with Mycosis Fungoide. Three erythrodermal variants are currently recognized in skin T-cell lymphoma: Sézary syndrome, Erythroderma in fungoid mycosis, and Erythroderma in skin T-cell lymphomas: Undepede. While Sézary syndrome is understood as a leukemia expression of erythrodermal T-cell skin lymphoma, with numerous Sézary cells in skin, blood and other tissues, Erythroderma in fungoid mycosis would be determined in the absence of these same haematological and histological findings before an erythrodermal clinical picture. It is necessary to publish the article by the form of presentation of fungoid mycosis, taking into account that this dermatosis has a number of differential diagnoses that makes it the great simulator in Dermatology.

RESUMO Eritrodermia é uma erupção cutânea mais ou menos erethema que afeta mais de 90% da superfície do corpo. É uma doença capaz de comprometer o prognóstico vital e que pode ser complicada por desequilíbrios hidroelegóticos, distúrbios termoreguladores, infecções, bem como insuficiência cardiovascular. Nesta publicação, refere-se ao caso de um paciente do sexo masculino de 72 anos com histórico de pressão alta e hanseníase que recebeu poliquimoterapia, é internado no serviço de dermatologia no período de observação, pois há cerca de um ano começou a desenvolver vermelhidão da pele e múltiplas escamas. No momento do exame, observa-se um paciente eritrótermal, ectropion, importante distrofia ungueal, com adenopatias palpáveis axilares e inguinais, associadas a uma coceira intensa generalizada. São realizados estudos para diagnóstico, incluindo biópsia inguinal e nódulo da pele, este último compatível com Mycosis Fungoide. Três variantes eritromicais são atualmente reconhecidas em linfoma de células T da pele: síndrome de Sézary, Erythroderma na micose fungoide, e Erythroderma em linfomas de células T da pele: Undepede. Enquanto a síndrome de Sézary é entendida como uma expressão de leucemia de linfoma de pele de células T eritorodérmica, com numerosas células Sézary na pele, sangue e outros tecidos, eritrorgema em micose fungoide seria determinada na ausência desses mesmos achados haematológicos e histológicos diante de um quadro clínico eritroxermal. É necessário publicar o artigo sob a forma de apresentação da micose fungoide, tendo em vista que essa dermatose possui uma série de diagnósticos diferenciais que o torna o grande simulador em Dermatologia.

Annular epidermolytic ichthyosis: a case report and literature review

Abstract Annular epidermolytic ichthyosis is a rare subtype of epidermolytic ichthyosis that is characterized by erythematous, polycyclic, and migratory scaly plaques accompanied by palmoplantar keratoderma. This report presents the case of an 8-year-old girl who developed migratory, erythematous, scaly plaques associated with palmoplantar keratoderma. The initial hypothesis was erythrokeratodermia variabilis et progressiva; however, the finding of epidermolytic hyperkeratosis in histopathological examination led to the diagnosis of annular epidermolytic ichthyosis.

Epidemiology of exfoliative dermatitis at the University of Santo Tomas Hospital (Department of Dermatology) from 2008-2012: A five-year review

@#INTRODUCTION: Exfoliative dermatitis is a potentially life- threatening inflammatory reaction that poses a significant risk for morbidity and mortality. Several underlying etiologies of this dermatologic condition include pre-existing dermatoses, drugs and malignancy. Although it is a common disease entity, local studies on exfoliative dermatitis published in literature are very limited. OBJECTIVE: The primary objective of this study is to determine the epidemiological profile of patients with exfoliative dermatitis diagnosed at University of Santo Tomas Hospital Dermatology department from January 2008 to December 2012. METHODS: Inpatient and outpatient clinical records of patients diagnosed and treated as exfoliative dermatitis were retrieved. The prevalence, clinical presentation, history of previous dermatoses or use of any drugs/topical medications, family history and accompanying systemic symptoms were reviewed and analyzed. RESULTS: A total of 67 patients were included in this retrospective study. The prevalence among patients with exfoliative dermatitis in this study was computed at 1 per 1000 dermatologic patients. The highest number of cases belonged to the group aged seventy-one to seventy-nine (25.4%) with a mean age of 56.62 years. There was a male predilection (65.7%). Clinical presentation of patients included pruritus, generalized scaling and erythema, accompanied by bipedal edema (41.8%), chills (22.4%), fever (T ≥ 38 °C), lymphadenopathies (6%) and joint pains (4.5%). Several etiologic factors of exfoliative dermatitis recorded were: pre-existing dermatosis (67.2%), idiopathic or undetermined causes (19.4%), drug-induced (10.4%) and malignancy (3%). CONCLUSION: Exfoliative dermatitis is a condition more commonly found in the older age group. Pre-existing dermatoses, drugs and malignancy are etiologic factors. The most common pre-existing dermatosis causing exfoliative dermatitis in this study is psoriasis while the most implicated drug is allopurinol.

Collodion baby-congenital ichthyosis: clinical review

Collodion baby is a rare form of congenital ichthyosis in which the entire body is covered by a parchment-like membrane. These neonates are at the risk of dehydration, sepsis, electrolyte disturbances, and temperature instability. It is inherited in autosomal recessive manner. We report a case of Collodion baby, born of a consanguineous marriage. Here, we present a short review of this condition and the various methods available for the prenatal diagnosis. A literature search was done using PubMed, Medline, and Google Scholar databases using the mesh terms 'Ichthyosis', 'collodion baby', 'collodion membrane', 'Congenital ichthyosiform erythroderma', and 'Lamellar ichthyosis'.

Collodion Baby With Congenital Bilateral Ectropion And Eclabion- Rare Presentation

Congenital Ichthyosiform Erythroderma- an extremely rare dermatological condition with an estimated incidenceof 1 in 50,000 to 100,000 birth. There is defective Stratum corneum barrier associated with collodion membranewhen the baby moves from an amniotic fluid to external dry environment during parturition. The membranedessicates and peels off. This condition is due to mutations in Transglutaminase1, ALOX genes, ABCA12, Ichthyin,ABHD5.

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 95 cases with erythroderma / 医学研究生学报

Objective Erythroderma is a very serious disease that affects nearly the entire cutaneous surface and are highly subjected to secondary hypoalbuminemia, infection, cardiovascular diseases, complex causes and high death rates. The article aimed to explore the etiology, comorbidities and complicated infection of erythroderma.Methods Retrospective analysis was conducted on clinical data of 95 cases of erythroderma in our department from January 2009 to August 2016. Observations were made on the patients' clinical characteristics, etiology and inducement, lab examination, complications and complicated infection.Results There were 73 first-episode and 22 recurrent patients, among which 14 cases are psoriasis as the basic disease. As to etiological factors, there were 57 cases secondary to other skin diseases (60%) and 25 cases by drug reactions (26%). As to inducing factors, there were 6 cases by upper respiratory tract infection, 38 cases by irrational application of glucocorticoids, and 7 cases by external stimulants (traditional Chinese medicine scrubbing and external medicinal liquor). The main complications were 38 cases of cardiovascular diseases (40%). The complicated infection rates of plasma albumin in patients <35g/L and ≥35g/L were 65.78% and 12.28%(P<0.01). The complicated infection rates of the patients with hypoalbuminemia and electrolyte disturbance were 44.2% and 25% respectively (P<0.05).Conclusion The erythroderma is mainly secondary to previous skin diseases, mostly psoriasis, with cardiovascular diseases as the main comorbidities. In clinical practice, importance should be attached to monitoring decreased plasma albumin level and electrolyte disturbances in order to reduce the risk of infection.

Analysis of keratin 1 gene mutation and phenotypes in a pair of twins with bullous congenital ichthyosiform erythroderma / 中华皮肤科杂志

Objective To identify mutations in keratin genes (KRT1 and KRT10) in a pair of twins with bullous congenital ichthyosiform erythroderma (BCIE),and to explore the relationship between the causative genes and phenotypes.Methods Clinical data were collected from a pair of twins with BCIE and their family members.Peripheral blood samples were obtained from the twins,their old brother and parents,and DNA was extracted from these blood samples.Polymerase chain reaction (PCR)was performed to amplify all the coding exons and their flanking sequences of the KRT1 and KRT10 genes,and 100 unrelated healthy persons served as controls.Results The 11-year-old male proband presented with recurrent blisters,hypertrophy and desquamation all over the body for 11 years.His twin brother had similar skin lesions.Skin examination of the proband showed diffuse erythema covered with thick scaly crusts on the trunk and extremities.Blisters,bullae and erosions due to ruptured blisters were observed locally with tenderness on palpation.There were obvious hyperkeratotic and hard lesions on the big joints of the extremities.Diffuse hyperkeratosis could be seen on the palms and soles.A mutation c.591 + 1G ＞ A was identified at position 1 in intron 1 of the KRT1 gene in the twins,but not in the 3 healthy family members or the 100 unrelated healthy controls.Conclusion The mutation c.591 + 1G ＞ A at position 1 in intron 1 of the KRT1 gene may contribute to the clinical phenotype of the twins with BCIE.

Eritrodermia psoriática en un hombre joven: sospechar infección por VIH / Erythrodermic psoriasis in young man: suspect HIV infection

Resumen La infección por VIH puede presentarse con distintas manifestaciones cutáneas, que en algunas ocasiones son consideradas marcadores de infección. Una posible manifestación es la eritrodermia psoriática, que corresponde a una forma generalizada de psoriasis. Presentamos un caso clínico de un hombre joven en que se sospechó una infección por VIH por un cuadro de eritrodermia psoriática confirmada por biopsia, asociado a un sarcoma de Kaposi. Posteriormente, la infección por VIH fue confirmada por serología. Se manejó con terapia antirretroviral, con buena respuesta al mes de tratamiento. La eritrodermia psoriática se puede considerar un marcador cutáneo de infección por VIH cuando ocurre en pacientes previamente sanos o con psoriasis recalcitrante.

HIV infection can be manifested with different skin symptoms, which are sometimes considered infection markers. Erythrodermic psoriasis is a possible manifestation, which is a widespread form of psoriasis. We report a clinical case of a young man suspected of HIV infection due to a psoriatic erythroderma confirmed by biopsies, associated with Kaposi sarcoma. Afterwards, HIV infection was confirmed by serological tests. Antiretroviral therapy was started, with positive response at one month of treatment. Erythrodermic psoriasis can be considered a skin marker of HIV infection when occurs in previously healthy patients or in recalcitrant psoriasis.

Cisto ductal de glândula lacrimal em paciente com eritrodermia ictiosiforme congênita / Ductal cyst of lacrimal gland in patient with congenital ichthyosiform erythroderma

Resumo A ictiose lamelar é uma doença congênita de herança autossômica recessiva que se caracteriza clinicamente por descamação de toda a superfície corporal devido hiperceratose mucocutânea que pode levar a comprometimento oftalmológico. Neste estudo relatamos um caso de cisto ductal de glândula lacrimal em paciente portador de ictiose lamelar atendido no Hospital Oftalmológico de Anápolis.

Abstract Lamellar ichthyosis is a congenital disease autosomal recessive which is characterized clinically by peeling of all the body surface due hyperkeratosis mucocutaneous that can cause ocular involvement. We reported a case of ductal cyst of the lacrimal gland in patient with lamellar ichthyosis attended in the Ophthalmological Hospital of Anápolis.

Exfoliative Dermatitis: A Clinico-etiological Study

Background: Exfoliative dermatitis or erythroderma is a symptom complex characterized by universal desquamation and erythema of the skin in response to various internal or external, known or unknown factors. Largely it is a secondary process and therefore it is mandatory to establish its etiopathology to facilitate the precise management. We attempted to establish the different causes of exfoliative dermatitis. Methods: We reviewed the clinical, laboratory and histopathological findings of 50 patients diagnosed with exfoliative dermatitis. Results: The frequency of exfoliative dermatitis was found to be 0.1 percent. The male-female ratio was 2.5:1 and majority of patients were in their 5th & 6th decade of life (mean age at diagnosis being 45.2 years). The total duration of disease ranged from 2 months to 1 year. The common causative factors were preexisting dermatomes (64%), followed by idiopathic (18%), drug induced (16%) and malignancy (2%). The most common dermatoses were psoriasis (24%) and eczema (24%). Carbamazepine (6%) and antitubercular drugs (6%) were the most frequent drugs which induced exfoliative dermatitis. Apart from scaling with erythema, pruritus and thickening of skin were found in all patients. Anemia was the most common finding (90%), followed by fever (42%), lymphadenopathy (38%) and edema (32%). The best Clinicopathological correlation was found in psoriasis and pemphigus foliaceous. Conclusions: This study outlined that the underlying etiologic factors of exfoliative dermatitis may show geographic variations. In this study preexisting dermatoses was most common cause of exfoliative dermatitis followed by idiopathic causes. Clinical features were identical irrespective of the etiology.

Diffuse cutaneous leishmaniasis presenting as erythroderma

Introduction: Exfoliative Erythroderma syndrome is a serious, at times life-threatening reaction pattern of the skin characterized by generalized and uniform redness and scaling involving the entire skin and often associated with systemic toxicity, lymphadenopathy and fever. Diffuse Cutaneous Leishmaniasisis observed in anergic patients with a low immune response. Case report: We are presenting a case of a 17-year-old patient diagnosed with Diffuse Cutaneous and Visceral recidivial Leishmaniasis, which clinically presented Erythroderma and skin exfoliation. The most common causes of Erythroderma are Psoriasis, Allergic Dermatitis, Drug Reactions, Lymphoma and Pityriasis Rubra Pilaris. Conversely, Diffuse Cutaneous Leishmaniasis is characterized by the presence of non-ulcerative nodules which resemble Lepromatous Leprosy, scattered in in every part of the body.To the best of our knowledge, the present clinical manifestation of Leishmaniasis has not yet been reported in the literature. Conclusion: In the correct clinical and epidemiological scenario, diffuse Cutaneous Leishmaniasis should be suspected in patients presenting Exfoliative Erythroderma and diffuse nodular lesions.

A case series of dermatological emergencies – Erythroderma

Erythroderma can be life-threatening, primarily because of its metabolic burden and complications. It is mandatory to establish its etiopathology in order to facilitate precise and definitive management. This disorder may be the morphologic presentation of a variety of cutaneous and systemic diseases. Detailed history and thorough work-up is therefore essential. Management of erythroderma involves multi-disciplines with progress monitoring especially on signs and symptoms suggestive of acute skin failure induced complications. Early diagnosis and referral of erythroderma to centres with dermatological services is crucial and will directly affect the outcome of the patients.

Clinical Analysis of 84 Cases of Erythrodermic Psoriasis and 121 Cases of Other Types of Erythroderma from 2010-2015 / 华中科技大学学报（医学）（英德文版）

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 der matoses,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.

Tumor Necrosis Factor Alpha Blocker-Induced Erythrodermic Sarcoidosis in with Juvenile Rheumatoid Arthritis: A Case Report and Review of the Literature

The development of cutaneous sarcoidosis as a paradoxical adverse event of tumor necrosis factor alpha (TNF-α) blockers has been reported in the literature; however, an erythrodermic form of cutaneous sarcoidosis during anti-TNF-α therapy has not yet been reported. Herein, we report the first case of an erythrodermic form of cutaneous sarcoidosis during anti-TNF-α therapy and review previous studies of cutaneous sarcoidosis. A 6-year-old Korean girl who had been suffering from juvenile rheumatoid arthritis presented with generalized erythematous skin eruption involving more than about 90% of her body surface area. After 14 months of etanercept treatment, the new erythematous skin eruption had developed and progressed into generalized erythroderma. Exclusion of suspected co-medication had been performed based on medication history. She had no other systemic symptoms, and ophthalmologic and neurologic examinations were normal. Histopathologic findings of the skin lesion revealed diffuse non-caseating granulomatous infiltrates composed of epithelioid histiocytes with sparse lymphocytes involving the entire dermis. Periodic-acid-Schiff and acid-fast stains were negative, and acid-fast bacilli was not detected by polymerase chain reaction of the skin biopsy. Based on clinicopathologic findings, she was diagnosed with etanercept-induced sarcoidal granuloma. After discontinuation of the suspected agent, the lesions spontaneously disappeared.