RESUMO
Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis, characterized by shiny, atrophic, hypochromic papules with a predilection for the genital and perineal skin. Extragenital involvement may occur, but is rare in the isolated form. LSA more commonly affects prepubertal and postmenopausal women. We describe an unusual case of isolated extragenital LSA, restricted to the wrists and mimicking lichen planus.
Assuntos
Líquen Plano/patologia , Líquen Escleroso e Atrófico/patologia , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Pele/patologia , Punho/patologiaAssuntos
Dermatologia/métodos , Dermatopatias/complicações , Dermatopatias/diagnóstico , Dermatopatias/epidemiologia , Dermatopatias/tratamento farmacológico , Dermatopatias/genética , Dermatopatias/imunologia , Dermatopatias/patologia , Dermatopatias/terapia , Pele/anatomia & histologia , Pele/embriologia , Pele/imunologiaRESUMO
Dermatologic diseases are classified most commonly by morphology, by pathogenesis, or by etiology. Nontraditional classifications may be useful in terms of providing a reassessment of traditional views about disease interrelationships. This review of dermatoses characterized by neutrophilic infiltrates and dermal vessel changes reveals evidence suggesting that these dermatoses result from immune complex-mediated, neutrophil-induced dermal vessel damage. Therapeutic approaches to these heretofore unlinked dermatoses are remarkably similar.
Assuntos
Humanos , Derivação Jejunoileal , Dermatopatias , Neutrófilos , Pele , Pioderma , Síndrome de Behçet , VasculiteRESUMO
Pustular vasculitis is a new disease concept that links cutaneous, and possibly systemic, aspects of Behçet's, bowel bypass, bowel-associated dermatosis-arthritis, and disseminated gonorrhea syndromes. The pathomechanism of pustular vasculitic lesion generation may relate to circulating immune complex (CIC)-mediated vessel damage and serum enhancement of neutrophil migration. Thalidomide, an oral pharmaceutical available on strict protocol, has therapeutic effects based on proposed modulation of CIC- and neutrophil-mediated cytotoxicity. Thalidomide therapy was started for four patients with significant morbidity from Behçet's syndrome and for one patient with bowel-associated dermatosis-arthritis syndrome. Clinical benefit was dramatic in all patients who completed sequential four-week "on" and "off" thalidomide therapeutic cycles. In three of four patients, in vivo testing for CIC after histamine injection immunopathology converted from positive (immunoreactant deposition in dermal vasculature [four hours after histamine] and CIC-mediated vasculitis [24 hours after histamine]) to negative during therapy. No effects were noted on neutrophil migration or on the LFA-1/Mac-1/p150,95 family of glycoproteins associated with neutrophil adherence as assessed qualitatively by tritium labelling of neutrophil cell surfaces. In this small patient group, thalidomide was a clinically effective, safe (with rigid monitoring) therapy whose mechanism of action may relate more to inhibitory effects on CIC-induced vasculitis than to effects on neutrophil-mediated cytotoxicity.
Assuntos
Complexo Antígeno-Anticorpo/análise , Glicoproteínas/análise , Inibição de Migração Celular , Linhagem Celular , Neutrófilos/imunologia , Pele/imunologia , Pele/patologia , Síndrome de Behçet/complicações , Síndrome de Behçet/imunologia , Síndrome de Behçet/tratamento farmacológico , Talidomida/efeitos adversos , Talidomida/uso terapêutico , Vasculite/etiologia , Vasculite/imunologia , Vasculite/tratamento farmacológicoRESUMO
Dermatologists, while becoming increasingly involved in the diagnosis and management of patients with connective tissue diseases, have left rheumatoid arthritis relatively unexplored. An increased awareness of possible pathomechanisms of rheumatoid arthritis may allow for generalizations that lead to increased understanding of other connective tissue disorders. The types of cutaneous disorders that occur in association with rheumatoid arthritis include: vasoreactive dermatoses (e.g., various forms of vasculitis), which may occur secondary to the circulating immune complexes present in rheumatoid arthritis; autoimmune bullous disorders, which may occur in the setting of a suppressor T cell defect in rheumatoid arthritis; and various miscellaneous cutaneous associations. Hopefully, this review will lead to an increased understanding of both rheumatoid arthritis and the wide array of cutaneous associations of rheumatoid arthritis.