RESUMEN
Lichen myxedematosus (LM) is a chronic cutaneous mucinosis that can present as a localized skin lesion or as a generalized systemic disease termed scleromyxedema. The differential diagnosis is determined by a combination of clinical presentation, serological studies, and histopathological examination. Currently, well-established and accepted histopathological features to distinguish localized LM from scleromyxedema have not been elucidated. Our recent publication, together with a retrospective literature review, suggests that the presence of groups of light chain-restricted plasma cells represents a distinct histopathological clue for the diagnosis of localized LM. In this report, we provide two additional cases of localized LM with lambda light chain-restricted plasma cells, together with clinical and histopathological findings that are similar to our previous publication. These cases support our theory that the light chain-restricted plasmacytic microenvironment is primarily attributed to the pathogenesis of localized LM. Therefore, we consider these cases to constitute a clinically and pathologically new variant of localized LM and name it primary localized cutaneous LM with light chain-restricted plasma cells.
Asunto(s)
Células Plasmáticas , Escleromixedema , Humanos , Células Plasmáticas/patología , Células Plasmáticas/inmunología , Escleromixedema/patología , Escleromixedema/diagnóstico , Femenino , Masculino , Persona de Mediana Edad , Diagnóstico Diferencial , Adulto , Cadenas lambda de Inmunoglobulina , AncianoRESUMEN
We describe a 3-month-old infant who presented to our institution with interleukin (IL)-1 receptor antagonist deficiency (DIRA), which consists of neutrophilic pustular dermatosis, periostitis, aseptic multifocal osteomyelitis, and persistently high acute-phase reactants. Skin findings promptly improved upon initiation of treatment with anakinra (recombinant human IL-1 receptor antagonist), and the bony lesions and systemic inflammation resolved with continued therapy.
Asunto(s)
Enfermedades Autoinflamatorias Hereditarias/tratamiento farmacológico , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Osteomielitis/tratamiento farmacológico , Periostitis/tratamiento farmacológico , Enfermedades Cutáneas Vesiculoampollosas/tratamiento farmacológico , Antirreumáticos/uso terapéutico , Femenino , Enfermedades Autoinflamatorias Hereditarias/complicaciones , Humanos , Lactante , Osteomielitis/etiología , Periostitis/etiología , Enfermedades Cutáneas Vesiculoampollosas/etiologíaRESUMEN
Addison's disease, or chronic adrenocortical insufficiency, is the overproduction of adrenocorticotropic hormone by the pituitary gland as a compensatory mechanism for decreased cortisol production by the adrenal glands. Classically, patients affected with Addison's disease develop weakness, anorexia, electrolyte imbalances: decreased sodium and chloride with increased serum potassium resulting in hypotension, and hyperpigmentation of the skin and mucous membranes. Herein this case report, we focus on the subtle findings of diffuse hyperpigmentation and intermittent but repetitive "flu-like" symptoms in two patients to correctly identify the diagnosis of Addison's disease effectively and efficiently.