Skin conditions can be a major health concern, with several common ailments taking their toll on your skin. But what about the rarer skin conditions? Skin wellness expert Jane McGrath outlines the most rare skin conditions in the world.
1. Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: According to McGrath, ‘Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are two forms of the same serious skin condition that causes skin and even the lining of internal organs to shed and fall off. The two forms are so closely associated that doctors often group them together, referring to them as SJS/TEN. Often starting with flu-like symptoms, SJS/TEN then causes skin to develop painful rashes and blisters. The top layer of skin then actually separates and falls off the body. If the patient’s body loses less than 10% body surface area, it is classified as SJS. Anywhere from 10 to 30% loss is considered overlapping SJS/TEN, and more than 30% is classified as TEN. People usually develop SJS/TEN as a result of a reaction to a drug. The drugs include non-steroid anti-inflammatory drugs, Allopurinol, Phenytoin, Carbamazepine, barbiturates, anticonvulsants and sulfa antibiotics. The reaction can start a week to two weeks after first taking the drug. But in about a quarter of all cases, doctors have been unable to pinpoint what causes the onset of SJS.’
2. Dermatographia: ‘People with dermatographia have extremely sensitive skin, where even the slightest scratch or rubbing can cause the skin to react with raised red lines,’ McGrath explains. ‘It affects perhaps up to 5% of the population. Slight scratches will cause skin to swell and perhaps develop hive-like welts. The skin will often begin to itch uncomfortably. Just to illustrate how severe it can be, people with this condition can literally write on their skin by lightly scratching it with their fingernails. Within minutes, distinct raised lines will appear, showing their writing with remarkable clarity. For this reason, the condition is also known as “skin writing.” Other things that can cause skin reactions are chafing from clothes as well as exposure to heat, cold or sunlight, or even experiencing emotion. Doctors aren’t sure what causes dermatographia, but some speculate that it is some kind of allergic reaction.’
3. Harlequin Ichthyosis: McGrath notes, ‘Harlequin ichthyosis affects newborns – they are born with thick, hard plates of skin covering their body. These plates are separated by deep cracks. This extremely rare condition makes it difficult for the affected babies to control water and fluid loss, resulting in severe dehydration. Babies with harlequin ichthyosis also have trouble moving, breathing, fighting off infection and regulating their body temperature. With such complications, it is very common for an infant to die within weeks after birth. Doctors believe that harlequin ichthyosis is a genetic disorder where a mutation in the ABCA12 gene affects the development of the epidermis. More than 100 cases have been reported around the world.’
4. Xeroderma Pigmentosum: ‘Most people know to wear sunscreen to avoid sunburn and to protect against skin cancer if they’re planning to be in the sun for any extended period of time,’ McGrath comments. ‘But can you imagine being so sensitive to ultraviolet (UV) light that you have to avoid sunlight through windows or even florescent lights? Such is life for those who have xeroderma pigmentosum (XP), which happens to be about only one in every 250,000 people in the United States. The dangers of sunlight to their skin are the same as for the rest of us – that is, damage from UV light – except that their skin is extremely sensitive to it. Symptoms include not only sunburn and blistering (after little sun exposure), but also light and dark skin patches. Skin can also become so thin that blood spots and vessels are visible. As you might have guessed, such sensitivity almost inevitably leads to the development of skin cancer.’
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