Skin is one of the finest organs of our body. It is easily prone to get damaged. Skin provides an aesthetic appeal to our bodies. Imagine our body only covered with flesh and bone. Besides acting as a covering it also acts as a first-line defense to foreign entrants thereby protecting our bodies.
However, due to some reason if skin loses its sheen and luster it is no less than a social stigma for the person. It is a sign of good health to have soft and supple skin.
Sometimes manifestation of the disease is difficult to predict and at such times the only source of relief is seeking immediate treatment.
Here are a few rarest of the rare skin diseases that are needed to be focused on to gain deeper insights about them
1. Inverse Psoriasis
Inverse psoriasis aka intertriginous psoriasis. The location and appearance of lesions set apart inverse psoriasis from other types of psoriasis. It is a skin condition that causes red and inflamed lesions in the skin folds of the body. They do not appear flaky and silver scales akin to psoriasis rather they are smooth in their appearance because of the moisture present in the folds of the skin.
Common sites affecting Inverse Psoriasis :
- Under breast
- Another region of the body where there are folds in the skin
Complications of Inverse Psoriasis
- Chafing and irritation
- Secondary infection particularly thrush
- Skin atrophy (thinning of the skin)
It mostly includes topical applications for the short surface area while for larger surface area and severe injuries systemic medications are prescribed. The physician may also use a combination of drugs to determine the severity of the disease. Sometime they may prescribe powder to keep the skin folds dry and also to avoid any secondary fungal infections
A rare inherited premature aging syndrome also is known as “Gottron syndrome”. It is characterized by unusually fragile, thin skin of distal extremities( hands and feet). The thinning of skin is observed due to the loss of fatty tissue under the subcutaneous region. Other characteristics that may later develop in life included premature senility, endocrine disturbances, and cataracts.
It is thought to be an autosomal recessive genetic trait and can be described as a mild, nonprogressive, and congenital form of skin atrophy
Only 40 cases have been reported so far in the medical journal
Signs and symptoms
Children since their infancy appear older than their actual age. This skin is unusually thin, tout with parchment-like on their limbs. The facial features have hollow cheeks, a beaked nose, and owl-like eyes with a bluish tint. Rapid aging and short stature is also one of the sign to distinguish
Apparently, there is no treatment available for Acrogeria
Argyria results from prolonged exposure to or ingestion of silver salts. It is characterized by gray to gray-black staining of the skin produced by silver deposition. It is a rare form of the disease caused by chronic absorption of high silver salts products. The skin pigmentation changes because silver salts surpass the body’s renal and hepatic excretory capacities. The cases of Argyria are mostly found in people with constant industrial contact or result of medications containing silver salts.
Sign and symptoms
Argyria usually begins with gray-brown staining of gums which later progresses to involve a large area of skin and mucosa acquiring blue-grey pigmentation.
The degree of hyperpigmentation is correlated with the amount of silver exposed and ingested
The pigmentation is mostly pronounced in sun-exposed areas such as the face, neck, arms, and hands. The fingernails, conjunctival membranes, and mucus membrane may become slate- blue-grey color
Hair turns metallic in color
Skin pigmentation is not just a matter of silver deposition in the dermis but also by stimulating melanin synthesis. It is believed that silver when comes in contact with skin attains brownish-black tone, through chemical reduction reaction, thus giving skin blue- greyish color.
Cases of mortality/ morbidity are quite rare.
- Depigmentation and chelation have been unsuccessful
- Selenium and sulfur form a complex with silver. However, they are not stable hence
- So far the new discovery of QS Nd: YAG laser has been proven safe and effective treatment with skin color returning to normal.
Scleroderma is an autoimmune chronic disease mainly affecting the skin. The main findings are thickening and hardening of the skin. The term Scleroderma means Scleo- hard and derma – skin.
It occurs when the immune system causes your body to make an excess of a protein called collagen, an essential part of the skin.
Therefore, the skin hardens and becomes tout, sometimes scar forms on the lungs and kidneys. The disease is not defined only to the skin but also affects internal organs.
There are 2 types of Scleroderma
- Localized Scleroderma- – generally affecting only skin
- Systemic Scleroderma- generalized in nature i.e can involve many body parts
Signs and Symptoms
The symptoms may vary depending upon the area of the body. The tight patches are formed on the skin, these patches could be oval, linear, or may cover a wide surface area of the trunk and limbs.
Skin tends to appear shiny. And the movement is restricted.
One of the early signs of scleroderma is Raynaud’s disease which causes small blood vessels in the fingers and toes to contract.
Anyone can get affected by Scleroderma but generally, women outnumber men and they are of 30-50 years of age.
The exact reason is not known but the immune system has a major role to play in scleroderma. It means our body’s immune system begins to attack the connective tissue of the skin.
Currently, there is no cure for scleroderma but alternative treatments are available to reduce the impact of the disease.
5. Harlequin Ichthyosis
It is a severe form of autosomal recessive congenital ichthyosis. It is characterized by a profound thickening of the keratin layer also known as hyperkeratosis in fetal skin. Imagine a barren land with cracks and fissures. The thick skin can pull apart and distort facial features.
Harlequin ichthyosis is caused by a mutation in the ABCA12 gene. This gene is responsible for making a protein that is essential for the normal development of skin cells. The protein transports fats in the outermost layer of the skin(epidermis). Some mutation in the ABCA12 gene hampers its protein production therefore the lipids are not transported to the epidermis where the hard, thick scales appear.
This rare form of the disease is inherited in an autosomal recessive pattern which means both copies of genes in each cell have the mutation.
Signs and symptoms
There are thick plates that crack and split apart. The tightness of the skin pulls around the eyes and mouth coercing the eyelids and lips to turn inside out. Revealing the inner linings. It also makes eating and breathing difficult. Harlequin ichthyosis babies required immediate neonatal intensive care because it is the most severe form of skin disease.
The thick skin gradually peels off. Antibiotics and oral administration of etretinate are given to accelerate the shedding of thick plates like scales. The kids who have survived harlequin appear to have red skin syndrome and dry skin which may be covered with thin scales and sparse hair. Harlequin ichthyosis requires a proper skincare regime to keep the skin hydrated and moisturized all time and prevent any cracks or fissures to protect it from secondary infection.
Above mentioned skin diseases make our hair stand on end. It is advisable to take proper skincare and follow a skin regime. If you have any doubt do not self assist the wound, instead seek advice from your dermatologist.