What is diffuse cutaneous mastocytosis?

Background: Diffuse cutaneous mastocytosis (DCM) is an extremely rare disease characterized by mast cell (MCs) infiltration of the entire skin. Little is known about the natural course of DCM.

Is cutaneous mastocytosis fatal?

The long-term outlook ( prognosis ) for people affected by cutaneous mastocytosis varies depending on the age at diagnosis. The prognosis is typically excellent in children who develop symptoms before age 2 as approximately 80% of these cases resolve before puberty.

Does cutaneous mastocytosis itch?

You might have a red and itchy rash if there are too many mast cells in your skin. You could get hives or have a rash that looks like freckles. If you rub the rash, it can get red and swollen.

Is urticaria pigmentosa the same as mastocytosis?

Urticaria pigmentosa is a type of mastocytosis. This is when the mast cells that are part of your immune system build up. Mastocytosis can occur in your skin, bones, intestines, or other organs. Urticaria pigmentosa is when the build-up of mast cells only happens in the skin.

How long does mastocytosis rash last?

Some people with systemic mastocytosis may experience episodes of severe symptoms that last 15-30 minutes, often with specific triggers such as physical exertion or stress. Many people do not have any problems. During an episode you may have: skin reactions – such as itching and flushing.

What do mastocytosis lesions look like?

These lesions are typically tan to red-brown macules that appear on the trunk and spread symmetrically. Patients with mastocytosis often have a long history of chronic and acute symptoms that were unrecognized as mastocytosis. Skin lesions may or may not accompany systemic mastocytosis.

What are the treatment options for cutaneous mastocytosis?

Treatment is usually symptomatic and may include oral antihistamines, topical steroids, and/or photochemotherapy. [1] [5] Cutaneous mastocytosis is a form of mastocytosis that primarily affects the skin.

Where can I find information about mastocytosis in New Zealand?

DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information on maculopapular cutaneous mastocytosis and mastocytoma, which are two specific types of cutaneous mastocytosis.

When does maculopapular cutaneous mastocytosis go away?

In young children, the patches may form a blister if itched or rubbed. Itching may worsen with changes in temperature, strenuous activity, emotional stress, and/or certain medications. Maculopapular cutaneous mastocytosis is most commonly seen in infants and young children and often fades by the teenaged years.

What is the prognosis for systemic mastocytosis?

Prognosis is favorable with regression of disease in adolescence or young adulthood Can appear at birth or early infancy; may persist into adulthood, possibly as well differentiated systemic mastocytosis (WDSM) 5 Blisters, some of which are hemorrhagic, and bullae may be present and dermatographism may be prominent