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The characteristic skin lesions of urticaria pigmentosa appear in these areas.
Most treatments for mastocytosis can be used to treat urticaria pigmentosa.
If hives appear, it most likely signifies the presence of urticaria pigmentosa.
Some clinicians suggest that urticaria pigmentosa is the childhood form of mastocytosis.
Urticaria pigmentosa is a rare disease, affecting fewer than 200,000 people in the United States.
Urticaria pigmentosa was first described in 1869.
Urticaria pigmentosa is characterized by excessive amounts of mast cells in the skin.
There are no permanent cures for urticaria pigmentosa.
Urticaria pigmentosa is generally benign and is usually self-limited.
Several factors can worsen the symptoms of urticaria pigmentosa:
However, nifedipine has never been approved by the FDA for treatment of urticaria pigmentosa.
Darier's sign is a change observed after stroking the skin of a person with systemic mastocytosis or urticaria pigmentosa.
Generalized eruption of cutaneous mastocytosis (childhood type) (Urticaria pigmentosa)
The most common cutaneous mastocytosis is urticaria pigmentosa (UP), more common in children, although also seen in adults.
The majority of urticaria pigmentosa cases are caused by a point mutation at amino acid 816 of the proto-oncogene c-kit.
Doctors can diagnose urticaria pigmentosa (cutaneous mastocytosis, see above) by seeing the characteristic lesions that are dark-brown and fixed.
A 1984 study by Fairly et al. included a patient with symptomatic urticaria pigmentosa who responded to nifedipine at dose of 10 mg po tid.
At least one clinical study suggested nifedipine, one of the dihydropyridines, may reduce mast cell degranulation in patients who exhibit urticaria pigmentosa.
Prior to specializing in ophthalmology, Nettleship studied veterinary medicine and dermatology, and in 1869 provided an early description of urticaria pigmentosa.
The typical cutaneous mast cell infiltrates of urticaria pigmentosa are usually not present before, during, or after diagnosis in patients who have mast cell leukemia.
At least one clinical study suggested that nifedipine, a calcium channel blocker used to treat high blood pressure, may reduce mast cell degranulation in patients with urticaria pigmentosa.
For example, urticaria pigmentosa is a condition that arises from mast cells and is occasionally associated with mast cell leukemia; cutaneous T-cell lymphoma is often confined to the skin throughout its course.
Localized Infantile Mastocytosis Mastocytosis, Infantile Nettleship's, E. Disease Type I Urticaria, Perstans Hemorrhagica Xanthelasmoidea None Urticaria pigmentosa is a rare skin disorder that is a localized (cutaneous) form of mastocytosis.