What is Erythrokeratodermia Variabilis?
Erythrokeratodermia variabilis (EKV) is a rare subtype of heterogeneous group of skin diseases called the erythrokeratodermi, and presents with migratory erythema and fixed hyperkeratotic plaques.  EKV lesions commonly occur in the early stage of life.
What is Erythrokeratoderma?
Erythrokeratoderma is an umbrella term for a group of rare genetic skin disorders characterized by well-demarcated plaques of reddened, dry and thickened skin. Typically, these lesions are distributed symmetrically on the body and tend to slowly expand and progress over time.
What is vohwinkel syndrome?
Vohwinkel syndrome is a disorder with classic and variant forms, both of which affect the skin. In the classic form of Vohwinkel syndrome, affected individuals have thick, honeycomb-like calluses on the palms of the hands and soles of the feet (palmoplantar keratoses) beginning in infancy or early childhood.
What is Bart Pumphrey syndrome?
Bart-Pumphrey syndrome (OMIM 149200), also known as knuckle pads, leukonychia, and sensorineural deafness, is a rare autosomal-dominant syndrome. It was described in 1967 by Bart and Pumphrey. 1. To our knowledge, only a few families with this syndrome have been reported worldwide.
What is a knuckle pad?
Discussion. Knuckle pads, also known as “Garrod’s nodes”, are benign fibrofatty subcutaneous pads located over the PIP joints that can be mistaken for arthritis(1). Rarely they affect the dorsal aspect of the MCP joints. Clinically they are painless and often affect both hands in an asymmetrical pattern.
What causes Papillon Lefevre syndrome?
Papillon-Lefèvre syndrome is caused by changes (alterations) in CTSC (Cathepsin C) gene. Genes provide instructions for creating proteins that play a critical role in many functions of the body. When a gene is altered, the protein product may be faulty, inefficient, or absent.
What is Olmsted syndrome?
Overview. Olmsted syndrome, also known as mutilating palmoplantar keratoderma (PPK) with periorificial keratotic plaques, is a very rare congenital (present from birth) disorder causing abnormal growth and thickening of skin.
Is there a cure for knuckle pads?
In general, treatment is not required for a knuckle pad. Avoidance of repetitive behaviour if possible may improve the situation. Moisturisers may be useful if the knuckle pads are hyperkeratotic. Surgery has been used, but can be complicated by the development of keloid scars.
Do knuckle pads go away on their own?
Juvenile knuckle pads may disappear, but they can last for indefinite periods. The pads most frequently cause no pain so no treatment is necessary. The pads should not be removed surgically because this can leave severe scarring, and the pads often recur.
Why do people get knuckle pads?
Knuckle pads may be idiopathic or inherited as part of autosomal dominant conditions such as Bart-Pumphrey syndrome and Dupuytren contracture. Similar to calluses, acquired forms are often a response to repetitive trauma and friction; many authors designate these lesions pseudo-knuckle pads.