Chronic Recalcitrant Lesions of an Enlarged Limb: Consider Parkes Weber Syndrome
Journal Title: Journal of Dermatology Research - Year 2022, Vol 3, Issue 1
Abstract
In this case, we diagnosed the patient with Parkes Weber syndrome. This syndrome is considered by some experts as a sub-form of the Klippel-Trenaunay syndrome. Both syndromes are defined by congenital vascular malformations, which can affect the capillary system as well as the venous or lymphatic system [2]. PWS is a congenital angiodysplastic syndrome combining capillary, venous, lymphatic and arteriovenous shunts in the overgrown limb. It can affect the upper or lower limbs. It can affect the upper or lower limbs, including the pelvic vessels. Most of these patients are already diagnosed in childhood or at puberty [3]. In contrast, in our patient’s case, the disease did not present until he was 36 years old. The abnormal increase in flow in the venous vasculature resulted in venous hypertension. As a result, the patient developed chronic venous insufficiency with increasing age. Simultaneously, the areas located distally around the AV shunts suffered from arterial under excitation. The combination of these two mechanisms resulted in decompensation of the microcirculation, which then led to local hypoxia that subsequently led to chronic leg ulcers that were difficult to treat. Hyperpigmentation, such as yellow ochre purpura, and hypopigmentation, such as white atrophy, developed as typical clinical signs [3,4].
Authors and Affiliations
Ryme Dassouli1*, Hanane BayBay1, Kenza Tahiri Joutei1, Zakia Douhi1, Sara Elloudi1, Fatima Zahra Mernissi1
Keywords
Related Articles
- EP ID EP703200
- DOI https://doi.org/10.46889/JDR.2022.3105
- Views 35
- Downloads 0
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