Immunopathologic study of erythema dyschromicum perstans (ashy dermatosis).

Erythema dyschromicum perstans (EDP) is a pigmentary disease of unknown etiology in which damage to basal cells is thought to be mediated by adhesion molecules. The aim of this study was to characterize the histopathology and immunopathology of EDP. Forty-three patients from Medellín, Colombia, with the diagnosis of EDP were evaluated. Skin biopsy specimens were obtained for histopathology and immunohistochemistry, using monoclonal antibodies directed against the following markers: CD4, CD8, CD56, CD1a, CD68, CLA, HLA-DR, ICAM-1 and LFA-1alpha. A dermal lymphocytic infiltrate was observed in all cases, with a perivascular location in 86%. Other histologic features included melanophages in all specimens, vacuolization of the basement membrane zone (BMZ) 58% and exocytosis of lymphocytes (53.5%). The mean number of total leukocytes was 1510 cells mm-2 of tissue. There was a predominance of CD8+ T lymphocytes in the dermis and HLA-DR+, ICAM-1+ keratinocytes in the epidermis. Exocytosis of cutaneous lymphocyte antigen (CLA)+cells was observed in areas of BMZ damage, suggesting that response to antigenic stimulation may play a role in the development of EDP.

Immunolocalization of HLA-DR and metallothionein on amalgam tattoos.

Despite studies concerning toxic reactions related to amalgam components in the literature, few studies have been devoted to evaluate local noxious effects of amalgam tattoos (AT) on biological tissues. In addition, little is known about activation of inflammatory cells by mucosa-implanted amalgam debris. Tissue reaction to AT depends on the particle size. Human leukocyte antigen DR (HLA-DR) is an activation marker of inflammatory cells associated with antigen presentation. Metallothioneins (MT) are proteins involved with metal detoxication, including mercury and silver. The purpose of the present study was to investigate the immunolocalization of HLA-DR and MT in AT with large or powdered particles. Paraffin-embedded AT tissue blocks were sectioned and subjected to immunohistochemistry for HLA-DR and MT localization. The results demonstrated a dense mononuclear inflammatory infiltrate associated with large and powdered debris and positivity for HLA-DR and MT in inflammatory cells. While blood vessel walls and connective fibers impregnated with powdered particles were negative for HLA-DR, they were positive for MT. In addition, wherever epithelial basement membrane impregnation by powdered amalgam particles was observed, a strong positivity for MT was detected. These findings demonstrate that residual elements of AT still have noxious local effects over tissues.

Eritema discrómico perstans (dermatosis cenicienta) / Erythema dyschromicum perstans (ashy dermatosis)

El Eritema Discrómico Perstans (EDP) o Dermatosis Cenicienta es una condición poco común y se caracteriza por máculas grisáceas bien definidas y de contornos policíclicos. En la histopatología los hallazgos son característicos, pero no patognomónicos, y corresponden a una dermatitis liquenoide. La causa del EDP continúa siendo un misterio; se han propuesto causas infecciosas, alteraciones del sistema inmune, e incluso, factores ambientales. Para el diagnóstico, la mayoría de las veces el cuadro clínico es muy típico. Aún no existe un tratamiento efectivo probado para el EDP, sin embargo, la clofazimina parece ser la mejor alternativa.

Doença de Schamberg: capilarite linfocítica pigmentar purpúrica / Schamberg's disease: lymphocitic pigmentar purpuric capilaritis

Objetivos: Revisäo da doença de Schamberg enfocando os aspectos imunológicos e terapêuticos. Métodos: A doença de Schamberg, o líquen aureus, e as purpúras de Majocchi, Gougerot-Blum, e ectematosa de Doucas-Kapetanakis, pertencem ao espectro das chamads púrpuras simples. As lesöes purpúricas pigmentadas säo geralmente assintomáticas e näo têm etiopatogenia estabelecida. Resultados: A púrpura de Schamberg apresenta um quadro histopatológico de inflamaçäo com hemorragia, sem necrose fibrinóide (capilarite ou pervasculite). A agressäo vascular, com estravazamentode hemácias e conseqüente deposiçäo de hemossiderina, é secundária a reaçöes imunológicas mediadas por linfócitos T na regiäo capilar dérmica. As citocinas e oa umento da expressäo das moléculas de adesäo endoteliais promovem a aderência células T -queratinócitos. A ativaçäo precoce destes receptores de adesäo endoteliais, determina a organizaçäo do infiltrado inflamatório pericapilar. Alteraçöes sutis no sistema coagulaçäo-fibrinólise foram descritas. Näo há terapia definida. A pentoxifilina, diminuindo a adesäo de células T às células endoteliais e aos queratinócitos, foi útil no controle das lesöes de alguns casos. Há um potencial futuro para a utilizaçäo de outras estratégias terapêuticas antiinflamatórias. Conclusöes: A púrpura de Schamberg é uma capilarite com a participaçäo de linfócitos T, queratinócitos, citocinas e moléculas de adesäo. Requer terapia antiinflamatória potente.

Involvement of cell adhesion and activation molecules in the pathogenesis of erythema dyschromicum perstans (ashy dermatitis). The effect of clofazimine therapy.

OBJECTIVES: To assess the expression of several cell adhesion and lymphocyte activation molecules in erythema dyschromicum perstans lesions, and to evaluate the effect of clofazimine therapy on the expression of these molecules. DESIGN AND METHODS: A prospective study. Skin biopsy samples were obtained from patients before and after 3 months of clofazimine therapy, and the expression of cell adhesion and activation molecules was assessed by an immunohistochemical technique. SETTING: This study was performed in a clinical referral center and an immunology research laboratory. PATIENTS: We studied 6 patients with erythema dyschromicum perstans. A diagnosis was made on the basis of clinical and histological criteria. Two patients discontinued participation in the study: one because of adverse effects and the other for unknown reasons. INTERVENTIONS: Patients were treated with clofazimine, 100 mg/d, for 3 months. MAIN OUTCOME MEASURES: Expression of cell adhesion and lymphocyte activation molecules in skin biopsy specimens before and after clofazimine therapy. RESULTS: Before clofazimine therapy, we detected a noticeable expression of intercellular adhesion molecule 1 and major histocompatibility complex class II molecules (HLA-DR) in the keratinocyte basal cell layer. In addition, CD36, a thrombospondin receptor that is not expressed by normal skin, was detected in the strata spinosum and granulosum. The dermal cell infiltrate expressed the activation molecule AIM/CD69 and the cytotoxic cell marker CD94. After clofazimine therapy, the expression of intercellular adhesion molecule 1 and HLA-DR disappeared, as well as the mononuclear cell infiltrate. CONCLUSIONS: Our results suggest that some cell adhesion and activation molecules are involved in the pathogenesis of erythema dyschromicum perstans. Clofazimine appears to have an important effect on the inflammatory phenomenon of erythema dyschromicum perstans.

Schimke immuno-osseous dysplasia: a newly recognized multisystem disease.

On the basis of five cases personally observed and one previously reported, we describe a disorder characterized by skeletal dysplasia, rapidly progressive nephropathy, episodes of lymphopenia, and pigmentary skin changes. Defects of T-cell function were compatible with an autoimmune process. The disorder is probably of genetic origin and inherited as an autosomal recessive trait.