Scleredema is a rare,
self-limiting skin
Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation.
Other animal coverings, such as the arthropod exoskeleton, have different ...
condition defined by progressive thickening and hardening of the skin, usually on the areas of the upper
back
The human back, also called the dorsum (: dorsa), is the large posterior area of the human body, rising from the top of the buttocks to the back of the neck. It is the surface of the body opposite from the chest and the abdomen. The vertebral c ...
,
neck
The neck is the part of the body in many vertebrates that connects the head to the torso. It supports the weight of the head and protects the nerves that transmit sensory and motor information between the brain and the rest of the body. Addition ...
,
shoulders and
face
The face is the front of the head that features the eyes, nose and mouth, and through which animals express many of their emotions. The face is crucial for human identity, and damage such as scarring or developmental deformities may affect th ...
.
The skin may also change color to
red
Red is the color at the long wavelength end of the visible spectrum of light, next to orange and opposite violet. It has a dominant wavelength of approximately 625–750 nanometres. It is a primary color in the RGB color model and a seconda ...
or
orange. The disease was discovered by
Abraham Buschke. Although the cause of scleredema is unknown, it is usually associated with a disease, usually
diabetes
Diabetes mellitus, commonly known as diabetes, is a group of common endocrine diseases characterized by sustained high blood sugar levels. Diabetes is due to either the pancreas not producing enough of the hormone insulin, or the cells of th ...
,
a
viral illness or
strep throat
Streptococcal pharyngitis, also known as streptococcal sore throat (strep throat), is pharyngitis (an infection of the pharynx, the back of the throat) caused by ''Streptococcus pyogenes'', a Gram-positive bacteria, gram-positive, group A str ...
.
It is usually not fatal, but it may cause death if the disease spreads to the
internal organs
In a multicellular organism, an organ is a collection of tissues joined in a structural unit to serve a common function. In the hierarchy of life, an organ lies between tissue and an organ system. Tissues are formed from same type cells to a ...
.
It may also cause an infection.
Diagnosis
The scleredema is usually proposed as a diagnosis based on the appearance of the skin and the patient's medical history. To confirm the diagnosis, the doctor performs a
skin biopsy
Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 day ...
, in which hematoxylin and eosin staining will show a thick
reticular dermis with thick
collagen
Collagen () is the main structural protein in the extracellular matrix of the connective tissues of many animals. It is the most abundant protein in mammals, making up 25% to 35% of protein content. Amino acids are bound together to form a trip ...
bundles separated by clear spaces.
The patient's
blood
Blood is a body fluid in the circulatory system of humans and other vertebrates that delivers necessary substances such as nutrients and oxygen to the cells, and transports metabolic waste products away from those same cells.
Blood is com ...
may be examined for diseases that may appear after the onset of symptoms, such as
multiple myeloma
Multiple myeloma (MM), also known as plasma cell myeloma and simply myeloma, is a cancer of plasma cells, a type of white blood cell that normally produces antibody, antibodies. Often, no symptoms are noticed initially. As it progresses, bone ...
.
Treatment
Although many types of medications have been tried as treatments, none of them have been proven effective in treating scleredema. Those treatments, such as
corticosteroids
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...
, may benefit the patient, but will not
cure
A cure is a substance or procedure that resolves a medical condition. This may include a medication, a surgery, surgical operation, a lifestyle change, or even a philosophical shift that alleviates a person's suffering or achieves a state of heali ...
their condition. If the affected area is infected, it is usually treated immediately. The symptoms of the condition usually resolve within six months to two years after onset. However, patients whose condition was associated to diabetes may suffer for longer periods of time.
Myocarditis
Myocarditis is inflammation of the cardiac muscle. Myocarditis can progress to inflammatory cardiomyopathy when there is associated ventricular remodeling and cardiac dysfunction due to chronic inflammation. Symptoms can include shortness of bre ...
resulting as a complication from the disease has been successfully treated with
penicillin
Penicillins (P, PCN or PEN) are a group of beta-lactam antibiotic, β-lactam antibiotics originally obtained from ''Penicillium'' Mold (fungus), moulds, principally ''Penicillium chrysogenum, P. chrysogenum'' and ''Penicillium rubens, P. ru ...
and
steroids
A steroid is an organic compound with four fused rings (designated A, B, C, and D) arranged in a specific molecular configuration.
Steroids have two principal biological functions: as important components of cell membranes that alter mem ...
.
See also
*
Necrobiosis lipoidica
References
External links
{{Medicine
Connective tissue diseases