Elefantiasis verrugosa nostra asociada a linfedema crónico y acroangiodermatitis de mali (pseudo sarcoma de Kaposi). Reporte de caso / Elephantiasis nostra verrucosa associated with chronic lymphedema and acroangiodermatitis of mali (pseudo sarcoma of Kaposi). Case report

La elefantiasis verrugosa nostra es una patología poco frecuente secundaria a linfedema crónico no filariásico, con la consecuente deformación y aumento de volumen del miembro afecto acompañado de un engrosamiento excesivo de la piel. Presentamos el caso de un paciente de sexo masculino con hiperplasia verrugosa en miembros inferiores secundaria a linfedema crónico por trastornos de la circulación venolinfática.

The elephantiasis nostra verrucosa is a rare pathology secondary to chronic non-filarial lymphedema, with the consequent deformation and volume increase of the affected limb accompanied by excessive thickening of the skin. We present the case of a male patient with verrucous hyperplasia in the lower limbs secondary to chronic lymphedema due to disorders of the venolymphatic circulation.

The Concept of Chronic Edema-A Neglected Public Health Issue and an International Response: The LIMPRINT Study.

Lymphedema has always been a neglected global health care problem. A central requirement for the development of any chronic disease is the clear use of public health definitions that can be used internationally to define populations. The term "lymphedema" has historically been defined as either primary, resulting from failure of lymphatic development, or secondary, following damage to the lymphatics (e.g., cancer treatment, injury, or filariasis). Attempts to integrate causes of edema arising from damage to the venous system or the effects of gravity, immobility, and systemic disease have rarely been integrated. More recently, the prominent role of the lymphatics in tissue fluid homeostasis in all forms of chronic edema has been recognized. These advances led to the development of the term: "Chronic edema: a broad term used to describe edema, which has been present for more than three months." It can be considered an umbrella term that includes not only conventional "lymphedema" but also chronic swelling, which may have a more complex cause. This definition has been adapted in the international epidemiology study (LIMPRINT) that identified people throughout the health and social care systems in participating countries. Clearer definitions will allow for examination of this important public health problem that is likely to escalate given the projections of an aging population with multiple comorbidities. It will be possible to define both the hidden mortality and morbidity associated with complications, such as cellulitis and the impact on health-related quality of life. This evidence is urgently required to lobby for increased resource and effective health care in an increasingly competitive health care arena in which more established conditions have greater priority and funding.

The Development and Validation of the LIMPRINT Methodology.

The acronym Limprint stands for Lymphedema IMpact and PRevalence INTernational and was run under the auspices of the International Lymphedema Framework (ILF), a charity dedicated to improving provision of care globally. The primary aim was to identify the number of people with chronic edema (chronic edema present for >3 months and due to a range of underlying etiologies and associated risk factors) within diverse health services in nine participating countries and to determine its impact using validated methods. An international protocol and sampling framework, online data capture system, and standard operating procedures were adopted. An international consensus was used to create a core data tool that covered 13 domains. Specialist data on demographics and disability, details of swelling, wounds, cancer, and health-related quality of life were also available for sites. The study protocol was designed to allow flexibility in the types of studies undertaken within complex health care systems. All cases were confirmed using the modified pitting test. Sensitivity and specificity for this method were determined in Japanese and European populations. Following confirmation of a chronic edema case, Lymphologists defined whether it was a primary of a secondary form. The study was designed to provide robust evidence that chronic edema is an important and unrecognized public health problem in health services with significant morbidity. Without evidence of the size and complexity, it will remain considered a rare phenomenon and people affected will be denied access to appropriate treatment that would allow them to have fulfilled and productive lives.

Sepsis grave en paciente con elephantiasis verrucosa nostra colonizada / No disponible

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A Case of Elephantiasis Nostras Verrucosa

Elephantiasis nostras verrucosa (ENV) is a rare clinical condition associated with chronic non-filarial lymphedema caused by bacterial or non-infectious lymphatic obstruction. A variety of etiologies, including infection, tumor obstruction, trauma, radiation, chronic venous stasis, congestive heart failure, and obesity, can lead to chronic lymphatic obstruction and edema. Mossy papules, plaques, and cobblestone-like nodules are clinically impressive features of ENV, but biopsy reveals only moderately abnormal findings such as pseudoepitheliomatous hyperplasia, dilated lymphatic spaces, fibrous tissue hyperplasia, and chronic inflammation. We present a case of ENV in a 67-year-old man with a 10-year history of multiple nodules and verrucous plaques on both feet. Microbiology ruled out a filarial infection. Nodule biopsy revealed pseudoepitheliomatous hyperplasia, marked dermal fibrosis, and a chronic inflammatory infiltrate. No evidence of carcinoma was identified. Both venous stasis and recurrent cellulitis could contribute to the dermal fibrotic changes of the lesions. However, before the recurrent cellulitis, he did not have any nodular lesions on his feet despite a 10-year history of venous disease. Therefore, this case suggests that venous stasis alone cannot produce the fibrotic nodular lesions of ENV

A Case of Elephantiasis Nostras Verrucosa Caused by Recurrent Infection after Skin Grafting / 대한피부과학회지

Elephantiasis nostras verrucosa is an uncommon disorder that is characterized by dermal fibrosis, hyperkeratotic verrucous and papillomatous lesions and this is all caused by chronic non-filarial lymphedema secondary to infections, surgeries, tumor obstructions, radiations, congestive heart failure and obesity. We report here on a case of elephantiasis nostras verrucosa that occurred on the left lower leg of a 54-year-old woman who had a past history of extensive skin grafts and recurrent infection.