ABSTRACT
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.
Subject(s)
Humans , Female , Pemphigus, Benign Familial/pathology , Dermatitis, Exfoliative/pathology , Acantholysis/pathology , Pemphigus, Benign Familial/complications , Pemphigus, Benign Familial/drug therapy , Dermatitis, Exfoliative/complications , Dermatitis, Exfoliative/drug therapy , Fatal Outcome , Catheter-Related Infections , Middle AgedABSTRACT
Abstract: Wet wrap dressings provide an ancillary treatment option for erythrodermic patients. Wet wrap therapy consists of the application of topical corticosteroids on all affected surfaces, followed by the application of a warm moist cotton cloth with a dry cotton cloth on top. The advantages of this procedure include its safety, as well as the prompt and marked improvement of the erythroderma. For erythrodermic psoriasis, wet wrap dressings can serve as an important rescue therapy option. The purpose of this study is to report a case series of seven patients with erythrodermic psoriasis that responded promptly to wet wrap therapy prior to the initiation of systemic treatment.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Young Adult , Bandages , Adrenal Cortex Hormones/administration & dosage , Dermatitis, Exfoliative/drug therapy , Administration, Topical , Treatment OutcomeABSTRACT
A síndrome da pele escaldada estafilocócica é uma dermatose esfoliativa rara no adulto, que acomete cerca de 0,09 a 0,56 por milhão de habitantes. É causada por exotoxinas produzidas por Staphylococcus aureus e manifesta-se com a formação de lesões bolhosas difusas pelo corpo. O diagnóstico é feito por meio de exame físico, hemocultura, cultura de sítio infectado e análise histopatológica. As medidas de suporte e a antibioticoterapia são a base do tratamento. Este relato descreve o raro acometimento de paciente idoso e evidencia a associação da doença com a imunossupressão. A presença de lesões em mucosa oral é um achado atípico e similar a um dos principais diagnósticos diferenciais da síndrome da pele escaldada estafilocócica: a necrólise epidérmica tóxica. É de suma importância diferenciá-las por meio de análise histopatológica das lesões de pele devido às formas inerentes de tratamento e à gravidade. O relato alerta para a importância do rápido reconhecimento do diagnóstico, a fim de introduzir tratamento adequado precoce, evitando os riscos da terapia inadequada e das complicações naturais da doença. Desta forma, é possível alcançar um desfecho positivo em uma doença de baixa prevalência e alta mortalidade no adulto.(AU)
The Staphylococcal Scalded Skin Syndrome is a rare exfoliative dermatitis in adults, affecting about 0.09 to 0.56 per million inhabitants. It is caused by exotoxins produced by Staphylococcus aureus and is manifested by the formation of diffuse bullous body lesions. The diagnosis is made through physical examination, blood culture, infected site culture, and histopathology. Supportive measures and antibiotics are the mainstay of the treatment. This report describes the rare involvement of an elderly patient, and highlights the association of the disease with immunosuppression. The presence of lesions in the oral mucosa is an atypical finding, being similar to one of the main differential diagnosis of the Staphylococcal Scalded Skin Syndrome: the Toxic Epidermal Necrolysis. It is very important to differentiate them through histopathology of the skin lesions due to the peculiar forms of treatment and the severity of the diseases. The report highlights the importance of rapid recognition of diagnosis in order to introduce early appropriate treatment, avoiding the risks of inappropriate therapy, and natural complications of the disease. This way, it is possible to achieve a positive outcome in a disease of low prevalence and high mortality in adults.(AU)
Subject(s)
Humans , Male , Aged, 80 and over , Anti-Bacterial Agents/therapeutic use , Dermatitis, Exfoliative/diagnosis , Staphylococcal Scalded Skin Syndrome/diagnosis , Staphylococcal Scalded Skin Syndrome/drug therapy , Dermatitis, Exfoliative/drug therapySubject(s)
Humans , Male , Middle Aged , Arthritis, Psoriatic/drug therapy , Dermatitis, Exfoliative/drug therapy , Dermatologic Agents/therapeutic use , Infliximab/therapeutic use , Time Factors , Arthritis, Psoriatic/complications , Arthritis, Psoriatic/pathology , Dermatitis, Exfoliative/complications , Dermatitis, Exfoliative/pathologyABSTRACT
A psoríase acomete 0,12 por cento a 0,71 por cento da população infantil, sendo que a forma eritrodérmica, grave e rara, ocorre em menos de 1,5 por cento dos casos. Os antagonistas do Fator de Necrose Tumoral-α (TNFα) constituem nova classe de drogas, utilizada para tratamento da psoríase grave a moderada, refratária às terapias convencionais. O Etanercepte é uma proteína de fusão do receptor do TNF-α, aprovada pelo Food and Drug Administration para tratamento da artrite reumatoide juvenil no grupo infantil. Apresentamos um caso de criança com 7 anos de idade, com psoríase em placa desde 8 meses de vida, que evoluiu para eritrodermia refratária a ciclosporina e metotrexato, com excelente resposta ao etanercepte, sem feitos adversos.
Psoriasis affects 0.12 percent to 0.71 percent of all children. Erythrodermic psoriasis is an uncommon but serious disorder, occurring in less than 1.5 percent of cases. Tumor necrosis factor-alpha blockers (TNF-α) are a new class of drugs used to treat moderate to severe psoriasis refractory to conventional therapies. Etanercept is a TNFα receptor fusion protein, approved by the FDA for treating juvenile rheumatoid arthritis. We present the case of a 7-year-old suffering from plaque psoriasis since 8 months old which evolved into erythroderma refractory to cyclosporine and methotrexate. Patient responded excellently to etanercept, with no adverse side effects.
Subject(s)
Child , Female , Humans , Dermatitis, Exfoliative/drug therapy , Immunoglobulin G/therapeutic use , Immunosuppressive Agents/therapeutic use , Methotrexate/therapeutic use , Psoriasis/drug therapy , Receptors, Tumor Necrosis Factor/therapeutic use , Drug Therapy, Combination/methodsABSTRACT
We report a HTLV-I positive infant, whose infection was confirmed by polymerase chain reaction. The infant presented with an acute, severe, generalized eczema, exfoliation and severe erythroderma that yielded to an acute proteic malnutrition and frequent staphyloccocal infections, unresponsive to treatment, since the second month of life. Immunodeficiencies from other origin and other causes of erythroderma were ruled out. The histopathology studies and clinical course yielded to the diagnosis of infective dermatitis associated to HTLV-I. A review of the literature is performed.
Se presenta un niño infectado por virus HTLV-I por vía vertical, confirmado por reacción de polimerasa en cadena, quien, a partir del segundo mes de vida, presentó un cuadro de eccema agudo severo generalizado, que llegó a la eritrodermia y exfoliación masiva, provocando una desnutrición proteica aguda e infecciones repetidas por Staphylococcus aureus, de difícil manejo. Se descartaron inmunodeficiencias de otro origen, así como otras causas de eritrodermia. Posteriormente, de acuerdo con la evolución clínica y con las biopsias, se interpretó el cuadro como una dermatitis infecciosa asociada a HTLV-I. Se revisa la literatura en relación a la infección por HTLV-I.
Subject(s)
Humans , Infant , Male , Dermatitis, Exfoliative/virology , HTLV-I Infections/complications , Staphylococcal Skin Infections/etiology , Dermatitis, Exfoliative/diagnosis , Dermatitis, Exfoliative/drug therapy , HTLV-I Infections/diagnosis , HTLV-I Infections/drug therapy , Severity of Illness Index , Staphylococcal Skin Infections/diagnosis , Staphylococcal Skin Infections/drug therapy , Staphylococcus aureus/isolation & purificationABSTRACT
Se presentan dos formas graves de psoriasis infantil, el primero para remarcar los factores precipitantes tales como el uso de corticoides orales y PUVA en la eritrodermia psoriásica y el segundo por las escasas comunicaciones de psoriasis pustulosa generalizada en niños menores de un año de edad; se comenta el tratamiento exitoso con el etretinato en ambos casos.
Subject(s)
Humans , Male , Child , Dermatitis, Exfoliative/complications , Etretinate/therapeutic use , Psoriasis/complications , Adrenal Cortex Hormones/adverse effects , Dermatitis, Exfoliative/diagnosis , Dermatitis, Exfoliative/drug therapy , Etretinate/administration & dosage , Psoriasis/diagnosis , Psoriasis/drug therapy , PUVA Therapy/adverse effectsSubject(s)
Humans , Skin Diseases/complications , Dermatitis, Exfoliative/diagnosis , Dermatitis, Exfoliative/drug therapy , Erythema Multiforme/diagnosis , Erythema Multiforme/drug therapy , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy , Pemphigus/diagnosis , Pemphigus/drug therapy , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/drug therapy , Sunburn/diagnosis , Blister/diagnosis , Blister/therapyABSTRACT
La ciclosporina A es un agente inmunosupresor selectivamente sobre los linfocitos T helper. Ha sido usada primariamente en los transplantes de órganos y también ha sido beneficiosa en el tratamiento de la psoriasis severa resistente a tratamientos convencionales. Hemos tratado a 12 pacientes con ciclosporina A a dosis inicialmente baja (3mg/kg/día), 9 presentaban eritrodermia generalizada y 3 psoriasis en placa extensiva. Los 12 pacientes respondieron rápidamente y con completo aclaramiento de la psoriasis a las 3 semanas de iniciado el tratamiento. Un paciente con eritrodermia presentó recurrencia a la forma inicial de psoriasis durante el tratamiento. No se hallaron efectos adversos clínicos o bioquímicos de importancia. La nefrotoxicidad parecía no ser un problema importante con bajas dosis usadas. Las evidencias sugieren que la ciclosporina A podría ser efectiva en el tratamiento de la psoriasis severas. A los 5 y 8 meses de suspendido el tratamiento con ciclosporina, 2 pacientes presentaron cáncer de laringe y próstata respectivamente