Secondary lower limbs lymphedema in patients with Chikungunya fever.

Chikungunya fever (CHIKF) is an arbovirus characterized by acute fever, myalgia and polyarthralgia. Lymphedema in the lower limbs (LL) was observed in several patients during an outbreak of CHIKF in the state of Pernambuco (Brazil) in 2016. No reports on lymphatic vessels disease due to CHIKF have been described. The aim of the study was to follow lymphatic abnormalities in the LL of 16 patients with CHIKF, using lymphoscintigraphy.An observational, prospective study with patients in the acute phase of CHIKF (confirmed serological diagnosis) with LL edema submitted to clinical evaluation and lymphoscintigraphy at baseline and after 90 days.Sixteen patients (81% females) participated in this study. All patients presented with lower limb lymphedema, being 15 (94%) bilateral. Of the 31 limbs affected by lymphedema, 24 (77%) presented abnormalities in lymphatic drainage by lymphoscintigraphy. The delay to visualize pelvic lymph nodes was the most frequent lymphoscintigraphic abnormality, observed in 16 (51,6%) LL. Nine (56%) patients were clinically reevaluated after 90 days, and all 18 LL remained with lymphedema. A second lymphoscintigraphy showed persistent abnormalities in 13 (72%) of the 18 LL.CHIKF can lead to lymphedema, and lymphedema may persist or progress after 3 months of the acute phases of the disease.

Kaposi sarcoma following postmastectomy lymphedema.

Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle-shaped cells that delimitated slit-like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV-8) latent nuclear antigen-1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV-8 is useful for the distinction between KS and AS or BLAP.

Opportunistic localization of skin lesions on vulnerable areas.

Genetic, developmental, and immune defects can make certain anatomic areas of the body more prone than others to harbor skin lesions. Cutaneous areas with skin barrier dysfunction (eg, atopic dermatitis) are the clearest example of vulnerable sites where opportunistic diseases, mainly infections (eg, herpes simplex), can easily occur. Somatic mosaicism, by giving rise to mutated cell clones with a bandlike arrangement, may form tissue segments prone to developing congenital or acquired skin disorders. Cutaneous districts that have been infected by herpes viruses become sites permissive for a subsequent onset of heterogeneous skin disorders, mainly tumors, further infections, and disimmune reactions (Wolf isotopic response). Regional lymphedema, by impairing lymph circulation and consequently the local immune control, favors the location of immunity-related lesions in the involved district. A vast series of skin injuries, such as ionizing or ultraviolet radiation, burns, traumas, and even vaccinations, can render the affected areas vulnerable to subsequent cutaneous disorders. Lack of immune control, ensuing from locally altered neuroimmune interaction, may be the basic defect responsible for the opportunistic location of skin lesions in herpes-infected, lymphedematous, or otherwise damaged areas, together featuring the novel concept of "immunocompromised district."