It’s a day for the skin and rare diseases, so prepare for some really interesting terms. Our skin(s) can be quite sensitive, which results in any one of different unexplainable diseases. This is why it is important to take note of your skin and its level of exposure to any agents. Without going too far, here are four rare skin diseases you probably haven’t heard of:
Pyoderma Gangrenosum (PG)
Pyoderma gangrenosum is an ulcerative skin disease that is not infectious. It is an extremely rare disease that affects about 1 person in every 100,000 and usually affects people in the young to middle ages. It also affects more women than men.
PG is an inflammatory skin disorder that is characterized by small, red bumps or blisters that eventually grow to form swollen open sores. There are up to 5 different types pf PG, but the main types are:
- The typical form (also called classic pyoderma gangrenosum) which can an occur on any skin surface, but is most commonly seen on the legs
- The atypical form (also called Peristomal pyoderma gangrenosum) which comprises about 15% of all cases of pyoderma and can occur on other parts of the body
While the cause is not fully understood, PG is thought to occur due to dysfunction in the immune system, hence it is usually found in people who have certain conditions such as: Inflammatory bowel disease and rheumatoid arthritis.
The diagnosis of PG is based mainly on clinical findings because biopsies usually fail to correctly identify it. Patients hovwever have leukocytosis and and dense neutrophilic infiltrate in the biopsy.
PG does not have any specific treatment and management. In mild cases, anti-bacterial ointments and dressings can be applied to the skin and affected body parts should be elevated. In more severe cases, clinicians usually resort to immune suppressing medications to manage the condition (because the condition is due to an over-active immune system). Medications like ciclosporin and corticosteroids form the mainstay of treatment.
Generalized pustular psoriasis
Pustular psoriasis is an uncommon form of psoriasis. Pustular psoriasis appears as clearly defined, raised bumps that are filled with a white, thick fluid composed of white blood cells, commonly called pus. The skin under and around these bumps is red.
Puastular psoriasis is classified into Acute (sudden and severe), Chronic (long term) or in-between (sub-acute). Pustular psoriasis affects everybody and affects men and women equally.
Generally , the skin is very red and painful to touch. This is then followed in most cases by the formation of pus containing vesicles (pustules). Pustular psoriasis is usually found more in the anal and genital region.
Pustular psooriais can be caused and worsened by:
- emotional stress
- skin injury
- exposure to certain metals or chemicals
- overexposure to UV light
- Drugs e.g. steroids
It is usually treated by a combibation of various treatment methods; which include topical agents and medications containing Cyclosporine, methotrexate, Acitretin, Psolralen. Additionally, ultraviolet light (both natural and artificial is used) as a complement addition to these medications.
Necrotising Fasciitis (flesh eating bacteria)
Necrotising Fasciitis is a rare and aggressive skin disease with about 10000 cases recorded per year in Nigeria . It is a very serious condtion that affects the body’s soft tissues and results in the death of the tissues at the site of infection.
Necrotising Fasciitis occurs when certain bacteria enter the skin through cuts or bites on the skin. The common bacteria responsible for it is the Methicillin Resistant Staphylococcus aureus (MRSA), usually contracted through municipal water waste treatment). A large percentage of Necrotising Fasciitis is recorded in patients who are diabetic and have malignancies .
The symptoms of Necrotising Fasciitis can start manifesting from as early as 1 day after exposure. These symptoms differ in severity and could include:
- Pain (usually increasing and seemingly worse than normal) at the site of the injury or
- Flu-like symptoms such as diarrhea, nausea, fever, dizziness, weakness, generally coupled with intense dehydration.
In advanced conditions, symptoms could include all of the above listed and
- Swelling, possibly accompanied by a purplish rash.
- Large, violet-colored marks that transform into blisters filled with dark, foul-smelling fluid.
Critical symptoms, which often occur within four to five days of infection, include:
- severe drop in blood pressure
- toxic shock
Necrotising Fasciitis is a potentially life threatening occurrence and requires urgent attention to prevent rapid deterioration of the patient. Surgical excision of the affected tissues is one of the most important steps in management. In severe cases, the surgical excision is repeated more than once to ensure total removal of diseased tissues and in more severe cases, amputation may be needed. Antibiotics should be given immediately there is any suspicion of Necrotising Fasciitis.
Vitiligo is a skin condition characterized by the gradual loss of pigments (color in the skin), which usually results in a gradual and often worsening discoloration of parts of the skin. The extent and rate of color loss from vitiligo is unpredictable and can affect the skin on any part of the body. It may also affect hair and the inside of the mouth. Vitiligo is an important skin disease having a major impact on the quality of life of the patient suffering from it and is more common in Africans (generally people with dark skins) than other races.
Vitiligo usually occurs when cells in the skin that normally produce melanin (the natural skin pigment) die off and cease to produce melanin. Different reasons have been suggested as its causes which include immune disorders, heredity and/or exposure to certain chemical compounds as well as sunburns. The prevailing belief is that vitiligo is caused by the combination of the above factors.
Patients with vitiligo usually present patches of de-pigmented skin that grow progressively worse over time. In most cases, the loss of pigmentation is symmetrical (i.e. it occurs in both sides of the body at the same time), while in a number of other cases, they are not symmetrical. The severity of vitiligo varies from mild (covering a tiny percentage of body surface area) to severe (covering almost all the body).
Currently, there is no treatment for vitiligo. However, Management techniques aim to reduce the speed at which it is spread. Cosmetics are important in the management of mild vitiligo as they help to conceal areas of discoloration and achieve a fairly even tone. Creams that lighten other areas of the skin (to even out skin tone) could also be used with appropriate precautions.
Immune suppressing agents such as corticosteroids, tacrolimus, etc. have found uses in vitiligo management because they help suppress immune responses, thus delaying the worsening of the discoloration. Additionally, phototherapy with the use of ultra violet light (UV- type B) is a second line alternative in management of vitiligo, as it stimulates the few remaining melanocytes to produce more melanin .
Do you know of any other rare skin diseases? Share with us!