Introduction and definition
Urticaria pigmentosa can be defined as an uncommon rash that generally affects the necks, arms, legs and trunks of children and young adults. The rashes consist of reddish-brown spots those turn into hives when they get rubbed hard or being itched. This disease is also found in infants and the first patch appears when the child is few months old. In the beginning, it might be confused for some insect bite but slowly rashes develop for several months or years.
This condition can strike on any part of the body including face, extremities, head and trunk. In the case of young children, the patch gets blistered when rubbed. If many patches occur at the same time, the infant may become irritable but it is very rare that severe symptoms come out. Within the next few years, the severity turn less irritable and eventually the patches go off. When a person enters the teenage, most of the patches might have gone. In case of the adults, the condition persists for quite a longer period and is also linked with some internal signs.
General information
Urticaria pigmentosa is considered to be very common form of cutaneous mastocytosis. It is a rare condition caused because of excessive amounts of mast cells within the skin those produce hives or lesions onto the skin when get irritated. Some clinicians propose that urticaria pigmentosa is the childhood class of mastocytosis. Mast cells are nothing but some special cells of connective tissue those release substances like histamine (a chemical essential for the inflammatory process) and heparin when the body's alarming mechanism is activated.
When mast cells cluster and multiply severely, the histamine and heparin are freed into the skin. The characteristic skin lesion of urticaria pigmentosa appears in those areas. Urticaria pigmentosa is usually benign and is generally self-limiting. Urticaria pigmentosa is a condition that develops skin lesions and severe itching. If the lesions are rubbed, hives might appear onto the site. They are seated most commonly onto the trunk, but can also be seen on the extremities, heads, and necks.
Causes of Urticaria pigmentosa
Urticaria pigmentosa is one of several classes of mastocytosis that is caused due to excessive numbers of inflammatory cells (mast cells) within the skin. Other forms include solitary mastocytoma (which is just one lesion) and systemic mastocytosis (occurring in other organs and not only skin). Urticaria pigmentosa is most commonly seen in children, but they can strike adults as well.
Rubbing of the lesions produces a speedy wheal (like hive-like bumps). Younger children might develop fluid-filled blisters over lesions if they are scratched. A large amount of histamine gets released from these extra mast cells might cause flushing, headaches, diarrheas, a rapid heartbeat (medically termed as tachycardia), and even fainting. This is not common with urticaria pigmentosa but might be seen in other forms of mastocytosis.
Signs and Symptoms of Urticaria pigmentosa:
Some of the common signs and symptoms may include the following:
• There might be facial flushing
• There would be blister formation over lesion when it is rubbed.
• Common sign also includes appearance of brownish lesions on skin.
• There might be red or brown spots.
• Emotional Stress is also quite common in person suffering from Urticaria pigmentosa.
Complications of Urticaria pigmentosa
There might be discomfort because of itching, and possible self-consciousness about those ugly spots. Other problems like diarrheas and fainting are rare but seen in some of the bad cases. Some particular medications might also trigger flares of urticaria pigmentosa. However, one has to discuss these things with the dermatologists.
Bee stings might also cause severe allergic reactions in some people suffering from urticaria pigmentosa. A doctor might advise one to carry an injection of epinephrine kit (many kits are readily available in the market) to be used in case of a bee-sting.
The treatment
The most common treatment for this condition is using some potent antihistamines. Since antihistamines may relieve some of the histamine-induced features such as itching and flushing. However, one can discuss the choice for antihistamine with any personal healthcare provider or family doctor. With more intense and unusual forms of urticaria pigmentosa, systemic symptoms are to be taken under controlled with other medicines.
However, there is no satisfactory treatment for urticaria pigmentosa. But certain points to be remembered if you are a patient of Urticaria pigmentosa like patients with urticaria pigmentosa should avoid certain drugs and other things such as aspirins, codeines, opiates, procaine, any sort of alcohol. polymyxin B etc. They are also advised to give up hot baths and vigorous rubbing after having bathed or shower. This is because such things can release great amount of histamine that can cause itching, flushing and hives.
