Cutaneous polyarteritis nodosa: An atypical simulator. Report of 3 cases and a narrative review of the literature

Objectives: To report the clinical manifestations and therapeutic schemes established in three patients with cutaneous polyarteritis nodosa, as well as to describe the pathophysiology, clinical features, diagnostic criteria, and therapeutic options. Methods: A literature review was performed using Google scholar and PubMed and MeSH terms. There was no limit on the publication date or language for the selection of the articles. Results: Cutaneous polyarteritis nodosa is an uncommon small and medium-sized vessel vasculitis, and is rarely reported in Colombia. Although its pathophysiology is unknown, it is considered to be mediated by the deposition of immunocomplexes in the walls of blood vessels. It can be triggered by infectious agents and be associated with autoimmune diseases. The cutaneous manifestations mainly include subcutaneous painful nodules, livedo reticularis, and ulcers. Myalgia, arthralgia, peripheral neuropathy, and fever can also be present. It represents a diagnostic challenge. Treatment is not standardized and is guided according to the severity. Conclusions: Cutaneous polyarteritis nodosa is a rare entity, with a wide variety of cutaneous manifestations. There is still no specific diagnostic test. Its diagnosis represents a challenge for the dermatologist, and requires multidisciplinary management, in which the rheumatologist plays a fundamental role.

Objetivos: Reportar las diferentes manifestaciones clínicas y los esquemas terapéuticos instaurados en 3 pacientes con poliarteritis nudosa cutánea. Así mismo, describir la fisiopatología, el cuadro clínico, los criterios diagnósticos y las opciones terapéuticas. Métodos: Se realizó una búsqueda de la literatura en Google Académico y PubMed utilizando términos MeSH. En la selección de la bibliografía, la búsqueda no se limitó por fecha de publicación ni por idioma, debido al escaso número de reportes. Resultados: La poliarteritis nudosa cutánea es una vasculitis de pequeño y mediano vaso, infrecuente, poco reportada en Colombia. Su fisiopatología no es del todo conocida, se considera mediada por el depósito de inmunocomplejos en las paredes de vasos sanguíneos; puede ser desencadenada por agentes infecciosos y asociarse con enfermedades autoinmunes. Las manifestaciones cutáneas incluyen principalmente nódulos subcutáneos dolorosos, livedo reticular y úlceras. A nivel extracutáneo podrían encontrarse mialgias, artralgias, neuropatía periférica y fiebre. El diagnóstico representa un desafío. El tratamiento no está estandarizado y es guiado según la severidad de la patología. Conclusión: La poliarteritis nudosa cutánea es una entidad poco frecuente, con una amplia variedad de manifestaciones cutáneas. Hasta el momento, no contamos con una prueba diagnóstica específica. Su diagnóstico representa un reto para el dermatólogo y requiere un manejo multidisciplinario, en el cual el reumatólogo desempeña un papel fundamental.

Palmar and Plantar Pits, More than Just Viral Warts.

A 40-year-old woman with a family history of gastric, pulmonary, and endometrial cancers (parents and grandparents) was referred to our institution with a 3-year history of progressive pruritus with palmar and plantar papules. On physical examination, there were keratotic yellowish pinpoint papules on the hands (Figure 1) and yellowish keratotic plaques and papules on the plantar pressure points (Figure 2). The diagnosis of punctate palmoplantar keratoderma was confirmed histopathologically (Figure 3), and treatment was initiated with 30% urea cream, which proved helpful. Molecular studies were requested, which documented a variant in the AAGAB gene. After treatment, the patient slightly improved and clinical symptoms were better tolerated.

Liquen escleroso y atrófico extragenital en hombre con cáncer de próstata: ¿manifestación cutánea paraneoplásica? / Extragenital lichen sclerosus et atrophicus in man with prostate cancer: paraneoplastic skin manifestation?

Resumen Introducción: los síndromes esclerodermiformes se definen por la presencia de esclerosis cutánea, induración y pérdida de la elasticidad de la piel. Su diagnóstico diferencial incluye casos de escleroderma localizada, dentro de los cuales se describe el liquen escleroso y atrófico que, hasta la fecha, no se ha reconocido en el escenario de síndromes cutáneos paraneoplásicos. Resultados: se presenta el caso de un paciente con adenocarcinoma de próstata avanzado, quién desarrolló lesiones compatibles con liquen escleroso y atrófico extragenital durante el curso de su enfermedad oncológica, confirmadas por histopatología. Conclusiones: Se cuestiona la naturaleza paraneoplásica de esta entidad cutánea en este contexto clínico particular

Abstract Introduction: Scleroderma syndromes are defined by the presence of skin sclerosis, induration and loss of skin elasticity. Their differential diagnosis includes cases of localized scleroderma, within which lichen sclerosus et atrophicus are described, which, to date, have not been recognized in the setting of paraneoplastic skin syndromes. Results: We present the case of a patient with advanced adenocarcinoma of the prostate, who developed cutaneous lesions compatible with extragenital lichen sclerosus et atrophicus during the course of the cancer, confirmed by histopathology. Conclusions: The paraneoplastic nature of this skin entity is questioned in this particular clinical context.

Phototherapy in sclerosing and pseudo-sclerosing skin diseases Fototerapia en enfermedades cutáneas esclerosantes y pseudoesclerosantes.

Sclerosing and pseudo-sclerosing skin diseases are a therapeutic challenge. Ultraviolet radiation, depending on its wavelength, penetrates into different layers of the skin and acts on cells that promote tissue remodeling and differentiation, such as keratinocytes and fibroblasts. Furthermore, it modulates the inflammatory processes in dendritic cells, endothelial cells, and leukocytes by intervening in the production of cytokines and profibrotic molecules. For these reasons ultraviolet light is a useful option in the treatment of these conditions. Las enfermedades esclerosantes y pseudoesclerosantes de la piel son un grupo de dermatosis que suponen un reto terapéutico para el clínico. La radiación ultravioleta, de acuerdo con su longitud de onda, penetra en las diferentes capas de la piel y actúa sobre aquellas células que favorecen la diferenciación y remodelación tisular como queratinocitos y fibroblastos. Además, modula los procesos inflamatorios en células dendríticas, endoteliales y leucocitos al intervenir en la producción de citoquinas y moléculas profibróticas, volviéndose una alternativa útil en el tratamiento de estas condiciones.

Hallopeau type of pemphigus vegetans, an unusual clinical presentation of an infrequent disease.

Pemphigus vegetans is a rare variant of pemphigus vulgaris, characterized by vegetative lesions that develop mainly in flexural areas. Herein, we present a middle-aged woman who presented to the emergency department with a rare cutaneous finding of bilateral axillary pustules that progressed over months to vegetant and malodorous plaques associated with subsequent painful erosions.

Linear lesions on the arm of a child: a diagnostic challenge.

There are a number of conditions that follow the lines of Blaschko. Linear discoid lupus erythematosus is a rare variant of chronic lupus erythematosus with less than 20 cases reported in children. It can be misdiagnosed as lichen striatus or linear morphea. We describe a 15-year-old boy with a confirmed histologic diagnosis of linear chronic discoid lupus erythematosus following the lines of Blaschko, with no signs of systemic involvement.