RESUMO
Pigmented purpuric eruptions comprise a group of benign dermatoses that are characterized clinically by pinpoint petechiae and purpura on a hyperpigmented base and histologically by capillaritis. The etiology of this group of disorders is unknown, although aberrant cell-mediated immunity has been proposed. Pigmented purpuric eruptions are well characterized in the pediatric population. In this case series we present three children with these disorders and review the clinical subtypes of pigmented purpuric eruptions that have been described in the literature.
Assuntos
Transtornos da Pigmentação/patologia , Púrpura/patologia , Biópsia por Agulha , Criança , Feminino , Humanos , Imuno-Histoquímica , Lactente , Masculino , Transtornos da Pigmentação/diagnóstico , Prognóstico , Púrpura/diagnóstico , Índice de Gravidade de DoençaAssuntos
Hiperoxalúria Primária/diagnóstico , Necrose , Dermatopatias Vasculares/diagnóstico , Adulto , Cardiomiopatias/diagnóstico , Cardiomiopatias/etiologia , Evolução Fatal , Feminino , Humanos , Hiperoxalúria Primária/classificação , Hiperoxalúria Primária/complicações , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/etiologia , Nefrocalcinose/diagnóstico , Nefrocalcinose/etiologia , Doenças Vasculares Periféricas/diagnóstico , Doenças Vasculares Periféricas/etiologia , Dermatopatias Vasculares/complicaçõesRESUMO
A 1-year-old girl presented for evaluation of a linear plaque on her forehead. She was born at 36 weeks' gestation following an uncomplicated pregnancy and delivery. At birth, she was noted to have an enlarged right cheek. She had no seizure history, but developed grand mal seizures 1 year later. On examination, she had a yellow plaque on her forehead which extended onto her nose. Under her right jaw, extending onto her anterior neck, there was a café-au-lait macule within which there was a yellow plaque which followed the lines of Blaschko. Her right cheek was enlarged and was erythematous (Fig. 1). Magnetic resonance imaging (MRI) of her face showed a mass in the right cheek deep to the subcutaneous fat tissue layer. The signal from the mass was identical to that from the fat, indicating that the mass represented a lipoma. This was later excised surgically and was histologically a lipoma. MRI of the brain demonstrated enlargement of the right lateral ventricle in addition to enlargement of the right cerebral hemisphere. There was also evidence of abnormal gyral architecture. Computerized tomography (CT) three-dimensional reconstruction of the skull demonstrated overgrowth of the right maxilla, right mandible, and right orbit (Fig. 2).
Assuntos
Neoplasias Faciais/patologia , Lipoma/patologia , Nevo/patologia , Neoplasias Cutâneas/patologia , Feminino , Humanos , Lactente , Síndromes Neurocutâneas/patologia , SíndromeRESUMO
Acute generalized exanthematous pustulosis (AGEP) is characterized by acute onset of a widespread pustular eruption in association with fever. It is usually seen as a medication reaction. We describe a 17-month-old boy with AGEP secondary to exposure to amoxicillin. This is an uncommon condition in children.
Assuntos
Amoxicilina/efeitos adversos , Toxidermias/diagnóstico , Exantema/induzido quimicamente , Penicilinas/efeitos adversos , Dermatopatias Vesiculobolhosas/induzido quimicamente , Diagnóstico Diferencial , Toxidermias/patologia , Exantema/patologia , Humanos , Lactente , Masculino , Indução de Remissão , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/patologiaRESUMO
BACKGROUND: Bullous pemphigoid is an immunobullous disease affecting predominantly older patients. In severe cases, high-dose corticosteroids and/or other immunosuppressants are often needed long term to control the disease. These can be associated with serious side-effects in this patient population. Objective To evaluate the benefit of plasmapheresis as a steroid saving agent in a cohort of 10 patients. RESULTS: Plasmapheresis was effective as a steroid saving therapy. All patients went into remission with a lower daily dosage of oral prednisone at 3 and 6 months postplasmapheresis. Two patients had side-effects from therapy that, while significant, did not interfere with long-term improvement in their disease. Eight patients had circulating immunoglobulin G (IgG) antibodies reactive with bullous pemphigoid antigen 1, and three of these had circulating antibodies reactive with bullous pemphigoid antigen 2 on Western immunoblot. CONCLUSIONS: Plasmapheresis was an effective steroid sparing therapy in these patients. Due to its high cost and potential morbidity, plasmapheresis should not be recommended as routine therapy for bullous pemphigoid, but it is a useful adjunct in resistant cases.
Assuntos
Anti-Inflamatórios/uso terapêutico , Proteínas de Transporte , Proteínas do Citoesqueleto , Proteínas do Tecido Nervoso , Colágenos não Fibrilares , Penfigoide Bolhoso/terapia , Plasmaferese , Prednisona/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Autoantígenos/imunologia , Azatioprina/uso terapêutico , Colágeno/imunologia , Terapia Combinada , Ciclofosfamida/uso terapêutico , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Distonina , Estudos de Avaliação como Assunto , Feminino , Humanos , Imunoglobulina G/sangue , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Penfigoide Bolhoso/patologia , Indução de Remissão , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Colágeno Tipo XVIIRESUMO
Henoch-Schönlein purpura is a systemic vasculitis of unknown cause. It is frequently triggered by a streptococcal upper respiratory tract infection. Other bacteria have been implicated as triggering agents. We report a recurring case of Henoch-Schönlein purpura in a patient with Pseudomonas pyelonephritis. The Henoch-Schönlein purpura remitted only when the infection was eradicated. Pseudomonas infection should be added to the list of bacteria that can trigger Henoch-Schönlein purpura.
Assuntos
Vasculite por IgA/etiologia , Infecções por Pseudomonas/complicações , Pielonefrite/complicações , Adulto , Bacteriúria/microbiologia , Biópsia , Doença Crônica , Humanos , Vasculite por IgA/microbiologia , Vasculite por IgA/patologia , Perna (Membro) , Masculino , Infecções por Pseudomonas/microbiologia , Infecções por Pseudomonas/patologia , Pseudomonas aeruginosa/isolamento & purificação , Pielonefrite/microbiologia , Pielonefrite/patologia , Recidiva , Pele/patologiaRESUMO
Carcinoma erysipelatodes, also known as inflammatory metastatic carcinoma, is a type of cutaneous metastatic disease. We describe a 64-year-old woman with metastatic breast carcinoma who presented with a blistering erythematous eruption resembling erysipelas with formation of vesicles and bullae. She was found to have carcinoma erysipelatodes with a formation of vesicles and bullae.
Assuntos
Neoplasias da Mama/patologia , Carcinoma/secundário , Neoplasias Cutâneas/secundário , Vesícula/patologia , Carcinoma/patologia , Celulite (Flegmão)/diagnóstico , Diagnóstico Diferencial , Erisipela/diagnóstico , Evolução Fatal , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologiaRESUMO
We describe 5 patients with severe pompholyx who did not respond to conventional therapy or who had debilitating side effects from corticosteroids. Low-dose methotrexate was added to their treatment regimens and led to significant improvement or clearing with a favorable side-effect profile. In all 5 patients the need for oral corticosteroid therapy was substantially decreased or eliminated, thus decreasing potential corticosteroid-induced morbidity. In this uncontrolled series of patients with recalcitrant palmoplantar pompholyx, methotrexate was an effective treatment and acted as a steroid-sparing agent.
Assuntos
Eczema Disidrótico/tratamento farmacológico , Metotrexato/administração & dosagem , Administração Oral , Adulto , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/efeitos adversos , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Eczema Disidrótico/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , EsteroidesAssuntos
Antiasmáticos/efeitos adversos , Beclometasona/efeitos adversos , Rosácea/induzido quimicamente , Administração Intranasal , Adulto , Antiasmáticos/uso terapêutico , Beclometasona/uso terapêutico , Feminino , Humanos , Pessoa de Meia-Idade , Rinite/tratamento farmacológico , Rosácea/patologia , Pele/efeitos dos fármacos , Pele/patologiaRESUMO
BACKGROUND: Molluscum contagiosum virus (MCV) causes cutaneous skin growths that mainly affect children, sexually active adults, and immunocompromised individuals. Lesions of MCV in patients infected with human immunodeficiency virus can be large and numerous, and response to available treatments is often unsatisfactory. OBSERVATIONS: We describe 3 men infected with human immunodeficiency virus who presented with extensive MCV lesions that were not responsive to various treatments. Patient 1 demonstrated dramatic clearing of his MCV lesions when intravenous cidofovir therapy was started for his treatment-resistant bilateral CMV retinitis and because of cidofovir's possible activity against MCV. In case 2, cidofovir was compounded as a 3% cream in a combination vehicle (Dermovan) for extensive facial involvement, and complete resolution of MCV was seen after 1 month of therapy. In case 3, intravenous cidofovir therapy was started both for CMV retinitis and in an attempt to clear 90% facial MCV involvement; after 1 month of treatment, all clinical evidence of MCV had resolved. All 3 patients remain clear of recurrence. CONCLUSIONS: Cidofovir, a nucleotide analog of deoxycytidine monophosphate, appears to have contributed to clearing of advanced MCV lesions in these 3 patients, thus providing suggestive evidence of clinical activity against MCV. Controlled trials of cidofovir therapy for MCV in persons infected with human immunodeficiency virus are warranted.