RESUMO
Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain.
Assuntos
Amiloidose/patologia , Dermatopatias Metabólicas/patologia , Dermatopatias/patologia , Adulto , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina , Imuno-Histoquímica , MasculinoRESUMO
Macular amyloidosis is a form of cutaneous amyloidosis characterized by dusky-brown lesions usually located on the upper back between the shoulder blades. This report describes the case of a 45 year-old female presenting with hyperpigmented macules and lace-like, non-pruritic erythema in the sacral and cervical region as well as on both arms and legs. Histology revealed amyloid deposits in the papillary dermis which exhibited apple-green birefringence after Congo red staining. There were no systemic findings. This is a case of macular amyloidosis with an unusual presentation. The literature on the subject is also reviewed.
Assuntos
Amiloidose/patologia , Dermatopatias Metabólicas/patologia , Corantes , Vermelho Congo , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
Diffuse plane normolipemic xanthomatosis (DPNX) is a rare, non-inherited disease that is often associated with systemic diseases, mainly malignant hematological (especially multiple myeloma) or lymph proliferative disorders. The DPNX can precede the appearance of such conditions by several years, so careful follow-up and periodic laboratory examinations are recommended even for patients that seemed to have no underlying disease. We describe a case associated with monoclonal gammopathy. This case shows that dermatological lesions can be the first manifestation of important hematological diseases and so physicians should be familiarized with this entity.
Assuntos
Gamopatia Monoclonal de Significância Indeterminada/complicações , Dermatopatias Metabólicas/complicações , Xantomatose/complicações , Idoso de 80 Anos ou mais , Humanos , Masculino , Gamopatia Monoclonal de Significância Indeterminada/patologia , Dermatopatias Metabólicas/patologia , Xantomatose/patologiaRESUMO
A amiloidose resulta da deposição de proteína amiloide fibrosa e insolúvel em espaços extracelulares de órgãos e tecidos. O depósito da substância amiloide pode ser localizado ou sistêmico e pode ser de natureza primária ou secundária. Relataremos um caso de amiloidose localizada cutânea primária nodular, manifesta por lesões pápulo-nodulares, eritêmato-acastanhadas, acometendo a região nasal, sem evidência de acometimento sistêmico. O estudo imunoistoquímico demonstrou presença de imunoglobulinas de cadeia leve kappa.
Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain.
Assuntos
Adulto , Humanos , Masculino , Amiloidose/patologia , Dermatopatias Metabólicas/patologia , Dermatopatias/patologia , Imuno-HistoquímicaRESUMO
A amiloidose macular é uma forma cutânea primária de amiloidose, caracterizada tipicamente por máculas acastanhadas, acometendo predominantemente a região interescapular. O presente caso é o de uma mulher de 45 anos, há um ano com manchas hipercrômicas, rendilhadas e não pruriginosas, em membros superiores, região cervical, sacral e membros inferiores. No estudo histopatológico da biópsia de pele, foi evidenciada substância amiloide amorfa no topo das papilas dérmicas, que se tornou mais evidente após a coloração pelo vermelho Congo. Não havia alterações sistêmicas associadas. Além da localização atípica e extensa da amiloidose cutânea macular, o presente caso procura acrescentar dados para esclarecimento do tema.
Macular amyloidosis is a form of cutaneous amyloidosis characterized by dusky-brown lesions usually located on the upper back between the shoulder blades. This report describes the case of a 45year-old female presenting with hyperpigmented macules and lace-like, non-pruritic erythema in the sacral and cervical region as well as on both arms and legs. Histology revealed amyloid deposits in the papillary dermis which exhibited apple-green birefringence after Congo red staining. There were no systemic findings. This is a case of macular amyloidosis with an unusual presentation. The literature on the subject is also reviewed.
Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Amiloidose/patologia , Dermatopatias Metabólicas/patologia , Corantes , Vermelho CongoRESUMO
Diffuse plane normolipemic xanthomatosis (DPNX) is a rare, non-inherited disease that is often associated with systemic diseases, mainly malignant hematological (especially multiple myeloma) or lymph proliferative disorders. The DPNX can precede the appearance of such conditions by several years, so careful follow-up and periodic laboratory examinations are recommended even for patients that seemed to have no underlying disease. We describe a case associated with monoclonal gammopathy. This case shows that dermatological lesions can be the first manifestation of important hematological diseases and so physicians should be familiarized with this entity.
A xantomatose plana difusa normolipêmica (XPDN) é uma dermatose adquirida rara, muitas vezes associada a doenças sistêmicas, nomeadamente neoplasias hematológicas(sobretudo o mieloma múltiplo) ou a processos linfoproliferativos. A XPDN pode preceder o aparecimento dessas doenças em vários anos, sendo por isso recomendada uma vigilância clínica e laboratorial periódica, mesmo para os doentes que aparentemente não apresentam uma doença associada. Descrevemos um caso associado à gamopatia monoclonal. Este caso demonstra a importância das manifestações cutâneas como primeira manifestação de doenças hematológicas importantes e por isso os clínicos devem estar familiarizados com esta entidade.
Assuntos
Idoso de 80 Anos ou mais , Humanos , Masculino , Gamopatia Monoclonal de Significância Indeterminada/complicações , Dermatopatias Metabólicas/complicações , Xantomatose/complicações , Gamopatia Monoclonal de Significância Indeterminada/patologia , Dermatopatias Metabólicas/patologia , Xantomatose/patologiaRESUMO
The cutaneous deposition disorders are a group of unrelated conditions characterized by the presence of either endogenous or exogenous substances within the dermis or the subcutis. Part I of this two-part series will focus on metabolic processes involved in the endogenous deposition in the various forms of amyloidosis, porphyria, colloid milium, and lipoid proteinosis. We will also review the clinical, histologic, biochemical, and ultrastructural findings relevant to each disorder. Basic mechanisms of pathogenesis, diagnostic modalities, and treatment options are also discussed.