ABSTRACT
Netherton syndrome is a rare autosomal recessive disease characterized by erythroderma, ichthyosis linearis circumflexa, atopy, failure to thrive and a specific hair shaft abnormality called trichorrhexis invaginata or bamboo hair, considered pathognomonic. We report the case of a 4-year-old boy with erythroderma since birth, growth deficit and chronic diarrhea. Trichoscopy was used to visualize typical bamboo and "golf tee" hair and of key importance to diagnose Netherton syndrome. We suggest the use of this procedure in all children diagnosed with erythroderma.
Subject(s)
Child, Preschool , Humans , Male , Dermatitis, Exfoliative/pathology , Dermoscopy/methods , Hair Diseases/pathology , Netherton Syndrome/pathology , Scalp/pathology , Diagnosis, Differential , Reproducibility of ResultsABSTRACT
A 24-year-old male patient, who underwent kidney transplant six years ago due to Lupus nephritis, for the last two years presented asymptomatic erythematous scaly plaques on the abdomen and areas exposed to light. Post-transplantation immunosuppressive medications included prednisone, mycophenolate sodium and sirolimus. The histopathologic features were typical for epidermodysplasia verruciformis. Epidermodysplasia verruciformis is a rare autosomal recessive genodermatosis with increased susceptibility to specific strains of cutaneous human papilloma virus. The term ''acquired epidermodysplasia verruciformis'' was recently introduced to the literature and describes epidermodysplasia verruciformis occurring in patients with impaired cell-mediated immunity. We report an additional case associated to immunosuppression after kidney transplantation.
Subject(s)
Humans , Male , Young Adult , Epidermodysplasia Verruciformis/pathology , Immunocompromised Host , Immunosuppression Therapy/adverse effects , Kidney Transplantation , Biopsy , Epidermodysplasia Verruciformis/immunology , Immunocompromised Host/immunology , Papillomavirus Infections/immunologyABSTRACT
Nevo melanocítico congênito gigante (NMCG) é uma entidade dermatológica rara, de etiologia desconhecida, fortemente relacionada com deformidades inestéticas, malignidade e alterações neurológicas. Este estudo é baseado no relato de caso de paciente de 10 anos com diagnóstico de NMCG. A lesão gigante exibia superfície espessa, ocupando grande parte do dorso, tipo "manta", com nódulos de consistência firme e tamanhos variados. Observaram-se, também, outras lesões melanocíticas menores, ditas satélites, em face, tronco e membros, além de palmas e plantas. O objetivo do estudo foi identificar o potencial de malignização das lesões e possibilidades terapêuticas, comparando com dados da literatura mundial consultada. Num período de acompanhamento de um ano, não foram observadas alterações clínicas sugestivas de malignização. A exérese completa da lesão foi considerada inviável, pois não haveria área de tecido sadio para enxertia de pele