Anetodermia secundaria a sífilis / Anetoderma secondary to syphilis. Case report and literature review

La anetodermia es una entidad cutánea benigna, rara e infrecuente, cuya característica es la pérdida localizada de fibras elásticas a nivel de la dermis. Suele observarse en pacientes con síndrome antifosfolípidico, lupus eritematoso sistémico, acné y varicela y de manera inusual como manifestación de una sífilis secundaria. Comunicamos el caso de una mujer con secundario o sifilítico que desarrolló anetodermia posterior a este proceso infeccioso.

Anetoderma is a benign, rare and infrequent cutaneous entity whose characteristic is the localized loss of elastic fibers at the level of the dermis. It is usually observed in patients with antiphospholipid syndrome, systemic lupus erythematosus, acne and varicella and an unusual way as a manifestation of secondary syphilis. We report a case of a woman with syphilitic secondary disease who developed an anetoderma after this infectious process.

Anetoderma due to secondary syphilis: Report of two cases and discussion of the histopathological findings.

Anetoderma is a rare benign condition of diverse etiology whose characteristic is the diminution or absence of the dermal elastic fibers. Classified as primary and secondary, the latter associated with tumors, inflammatory, and infectious diseases. Although the etiology of the lesions is well described in literature, the pathogenesis is still poorly determined. Anetoderma in syphilis is rare, and occurs even in the most uncommon cutaneous manifestations of the disease, such as the nodular form. In order to better understand the changes that lead to elastolysis, we propose a better correlation with the histopathological findings of the lesions that precede it. We present two cases of anetoderma secondary to syphilis, whose clinical aspects resembled the pattern of their initial secondary syphilis rash.

Ultrastructural aspects of primary anetoderma.

Anetoderma is a rare cutaneous disorder characterized by focal loss of dermal elastic tissue due to unknown mechanisms. Primary anetoderma develops on clinical normal skin, without any preceding dermatosis and it can be associated with autoimmune conditions. Secondary anetoderma develops on the same area of a previous disorder, such as infectious, neoplastic or inflammatory diseases. A 37-year-old female patient noticed for 4 years circumscribed, normochromic, asymptomatic herniated plaques on the trunk and upper limbs. Family history was negative. Only a positive antinuclear factor (ANF) test, with titer of 1:160 and nuclear homogeneous pattern was found. Light microscopy with Weigert staining showed a lessening of elastic fibers with fragmentation; the oxytalanic fibers were also affected or absent. Transmission electron microscopy showed fragmentation and granular degeneration of elastic fibers. With greater magnification, fragments similar to those seen with optical microscopy were identified. The collagen fibers did not present any alteration. The examination of the dermis with scanning electron microscopy also identified fragmentation and significant fissures of the elastic tissue, granular degeneration was also observed. With greater magnification fragmented elastic fibers were seen.

Anetodermia primaria asociada a lupus eritematoso sistémico. A propósito de un caso / Associated primary anemia to systemic lupus erythematosus About a case

La anetodermia es un trastorno elastolítico infrecuente, caracterizado clínicamente por áreas de piel laxa y pérdida o disminución de las fibras elásticas en la histología. Este hallazgo sin enfermedad cutánea previa es conocido como anetodermia primaria y se suele asociar a enfermedades autoinmunes; dentro de éstas el lupus eritematoso sistémico (LES), con anticuerpos antifosfolipídicos, y el síndrome antifosfolipídico (SAF) son las más frecuentes. Presentamos una paciente con anetodermia primaria, LES y anticuerpos antifosfolipídicos positivos sin clínica de SAF (AU)

Anetoderma is an infrequent elastolytic disorder, clinically characterized by areas with lack of the skin and decreased elastic fibers at histology. This finding without previous skin disease is known as primary anetoderma and is often associated with autoimmune diseases such as systemic lupus erythematosus (SLE), with antiphospholipid antibodies, and antiphospholipid syndrome (APS). A female patient with primary anetoderma, SLE and positive antiphospholipid antibodies without clinical APS is reported (AU)

Elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico secundarias y tratamiento con D- penicilamina en paciente con enfermedad de Wilson / Elastosis perforans serpiginosa and pseudo-pseudoxanthomaelasticum secondary to D-penicillamine in a patient with Wilson’s disease

La D-penicilamina es la opción terapéutica más utilizada en la enfermedad de Wilson, rara enfermedad genética, autosómica recesiva, en la cual existe una alteración en el metabolismo del cobre que se deposita en los tejidos (hígado, encéfalo y córnea). Presenta numerosos efectos adversos, la mayoría cutáneos, que se observan cuando la droga es utilizada en altas dosis y por largo tiempo; entre ellos se encuentran las dermatosis degenerativas, que incluyen elastosis perforante serpiginosa, cutis laxa, anetodermia y pseudo-pseudoxantoma elástico (también llamado pseudoxantoma elástico símil o pseudoxantoma elástico like). Se presenta una paciente de 29 años con antecedentes de enfermedad de Wilson asociada a elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico, ambas secundarias al tratamiento con D-penicilamina.

Penicillamineis the most commonly used therapeutic option in Wilson's disease.This is a rare, genetic, autosomal recessive diseasein which there is an alteration inthe metabolism of copper that is deposited in the tissues (liver, brain and cornea).It has numerous adverse effects, most of them affecting skin, but they are onlyobserved when the drug is used in high doses and for a long time, such as perforatingelastosis serpiginosa, cutis laxa, anetodermia and pseudo-pseudoxantomaelasticum (also called elasticum pseudoxantoma simil or elasticum pseudoxantomalike). We present the case of a29 year-old woman with a history of Wilson's diseaseand two concomitant degenerative dermatoses: elastosis perforans serpiginosa andpseudo pseudoxanthoma elasticum, both of them, secondary to treatment with Dpenicillamine.

Anetoderma primaria: revisión de la literatura a propósito de un caso / Primary anetoderma: a case report and literature review

La Anetoderma es una enfermedad elastolítica rara que se caracteriza por la pérdida focal de tejido elástico en la dermis. La patogénesis de esta enfermedad es desconocida y ha sido descrita en asociación con enfermedades autoinmunes, como lupus eritematoso y síndrome antifosfolípidos, y con enfermedades infecciosas, principalmente VIH, sífilis y borreliosis. Su diagnóstico diferencial es amplio e incluye otras enfermedades elastolíticas, liquen escleroatrófico extragenital y morfea, entre otros. Se reporta el caso de una paciente que consultó por un cuadro de larga data de múltiples parches blanquecinos atróficos, cuya biopsia cutánea fue compatible con Anetoderma. Se realiza una revisión de la literatura dada la necesidad de un adecuado estudio y seguimiento de estos pacientes en busca de una pesquisa temprana de enfermedades autoinmunes asociadas.

Anetoderma is a rare elastolytic disease characterized by focal loss of elastic tissue in the dermis. Pathogenesis is unknown and has been described in association with autoimmune diseases such as lupus erythematosus and antiphospholipid syndrome, and infectious diseases, especially HIV, syphilis and Lyme disease. Differential diagnosis is broad and includes other elastolytic disease, extragenital lichen sclerosus and morphea. We report the case of a patient with long-standing history of multiple atrophic white patches on the trunk and extremities, with diagnostic skin biopsy of Anetoderma. We conducted a literature review given the importance of good study and monitoring of these patients for an early investigation of associated autoimmune diseases.

Primary anetoderma: a cutaneous marker of antiphospholipid antibodies.

Primary anetoderma is a rare idiopathic disease of the skin characterized by circumscribed areas of slack skin and loss of elastic fibers found on histopathologic examination. It has been related to systemic lupus erythematosus and other immune diseases. In recent years, however, its association with antiphospholipid antibodies has been highlighted, and it should be considered a clinical manifestation of these antibodies.