RESUMO
BACKGROUND: Drug hypersensitivity syndrome is among the most severe drug hypersensitivity reactions and in rare cases it may progress to hemophagocytic lymphohistiocytosis. Herein, we report a case of allopurinol-induced drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis. CASE REPORT: An 18-year-old girl presented with maculopapular rash associated with cervical lymphadenopahy appearing 3 weeks after treatment with allopurinol. Her hemodynamic status at admission was unstable. Cutaneous examination revealed an itchy maculopapular rash, which was purpuric at certain sites, together with facial edema. The diagnosis of drug hypersensitivity was suggested and was confirmed by histological examination of a skin biopsy. Allopurinol was stopped. Two weeks later, however, eosinophilia was noted. Further, four days after discontinuation of allopurinol, in view of the laboratory signs of bicytopenia, hyponatremia, hypertriglyceridemia and hyperferritinaemia, as well as the presence of hemophagocytosis in bone marrow, a diagnosis was made of lymphohistiocytosis hemophagocytic syndrome complicating a drug reaction with eosinophilia and systemic symptoms. Moreover, viral serology tests were negative. The patient was given intravenous immunoglobulin and the outcome was good. DISCUSSION: The literature contains only very few reports of drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis. The incriminated drugs were vancomycin, lamotrigine and phenobarbital. To our knowledge, there has only been one report of allopurinol-induced drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis.
Assuntos
Alopurinol/efeitos adversos , Síndrome de Hipersensibilidade a Medicamentos/complicações , Síndrome de Hipersensibilidade a Medicamentos/etiologia , Linfo-Histiocitose Hemofagocítica/etiologia , Adolescente , Feminino , HumanosRESUMO
BACKGROUND: Acute localized exanthematous pustulosis (ALEP) is a rare and localized variant of acute generalized exanthematous pustulosis (AGEP). Only 15 cases of ALEP have been reported to date in the literature, with all cases following drug administration. We report 6 paediatric cases of ALEP occurring in springtime, with no associated drug administration in any case. PATIENTS AND METHODS: Over the last three years (2011, 2012 and 2013), we observed 6 cases of ALEP in 6 Tunisian children aged between 9 and 14 years. All cases were observed during the spring months. Diagnosis of ALEP was based in all cases on the EuroSCAR criteria and on the definition of ALEP proposed by Prange et al. A drug-related aetiology was ruled out in all cases, with exposure to a specific planned plant (Thapsia garganica) being retained as an aetiological factor in one case. DISCUSSION: Drug administration is the most frequent though not the sole cause of ALEP. The seasonal nature of this dermatosis may suggest other causes, mainly viral infection, plant contact or airborne allergens.
Assuntos
Pustulose Exantematosa Aguda Generalizada/diagnóstico , Dermatoses Faciais/diagnóstico , Pustulose Exantematosa Aguda Generalizada/tratamento farmacológico , Pustulose Exantematosa Aguda Generalizada/epidemiologia , Pustulose Exantematosa Aguda Generalizada/etiologia , Pustulose Exantematosa Aguda Generalizada/patologia , Adolescente , Corticosteroides/uso terapêutico , Antibacterianos/uso terapêutico , Biópsia , Criança , Dermatoses Faciais/tratamento farmacológico , Dermatoses Faciais/epidemiologia , Dermatoses Faciais/etiologia , Dermatoses Faciais/patologia , Feminino , Flores/efeitos adversos , Humanos , Leucócitos/patologia , Masculino , Estações do Ano , Thapsia/efeitos adversos , Tunísia/epidemiologiaAssuntos
Dermatoses Faciais/diagnóstico , Hiperpigmentação/diagnóstico , Ceratose Seborreica/diagnóstico , Acantólise , Idoso de 80 Anos ou mais , Biópsia , Carcinoma/diagnóstico , Diagnóstico Diferencial , Dermatoses Faciais/patologia , Dermatoses Faciais/cirurgia , Humanos , Hiperpigmentação/patologia , Ceratose Seborreica/patologia , Ceratose Seborreica/cirurgia , Masculino , Melanoma/diagnóstico , Neoplasias Cutâneas/diagnóstico , Verrugas/diagnósticoAssuntos
Orelha Externa/patologia , Lúpus Eritematoso Discoide/diagnóstico , Adulto , Atrofia , Carcinoma de Células Escamosas/diagnóstico , Diagnóstico Diferencial , Neoplasias da Orelha/diagnóstico , Feminino , Humanos , Hiperplasia , Líquen Plano/diagnóstico , Lúpus Eritematoso Discoide/patologiaRESUMO
The classification of cutaneous T-cell lymphomas (CTCL) is based on clinical, histological and immunohistochemical findings. Diagnosis may be difficult given the significant heterogeneity of these tumours. We describe herein an original case of primary cutaneous aggressive epidermotropic CD4/CD8 double-negative cytotoxic T-cell lymphoma that illustrates the difficulty in classification.
Assuntos
Epiderme/patologia , Linfoma de Células T/patologia , Neoplasias Cutâneas/patologia , Linfócitos T Citotóxicos/patologia , Idoso , Linfócitos T CD4-Positivos/patologia , Linfócitos T CD8-Positivos/patologia , Epiderme/imunologia , Feminino , Humanos , Linfoma de Células T/imunologia , Neoplasias Cutâneas/imunologiaRESUMO
Xeroderma pigmentosum (XP) is a rare genodermatosis predisposing to skin cancers. The disease is classified into eight groups. Among them, XP group A (XP-A) is characterized by the presence of neurological abnormalities in addition to cutaneous symptoms. In the present study, we report a particular family with XP-A in which some members showed an atypical clinical presentation, i.e. unexplained neurological abnormalities with discrete skin manifestations. Molecular investigation allowed identification of a novel XPA mutation and complete phenotype-genotype correlation for this new phenotypic expression of XP-A.