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A positive Darier sign - rubbing an area of mastocytosis has resulted in redness, swelling, and urtication in 5 minutes

Extensive infantile mastocytosis - the yellow-orange colour and positive Darier sign is diagnostic

Mastocytosis in a baby - red-brown patches are characteristic. The left lower back lesion has urticated after rubbing - a positive Darier sign

Red-brown maculopapules typical of adult-onset urticaria pigmentosa (CM-patient1)

Red-brown papules of adult-onset urticaria pigmentosa on the abdomen (CM-patient1)

A solitary mastocytoma on the wrist - it will urticate if rubbed

Telangiectasia macularis eruptiva perstans - the lesions are more red in this variant of mastocytosis

Adult-onset urticaria pigmentosa - the lesions are more red than usual, and are often brown in colour (CM-patient2)

Adult-onset urticaria pigmentosa - the lesions are more red than usual, and are often brown in colour (CM-patient2)

Yellow-brown pigmented macules in juvenile mastocytosis

Juvenile mastocytosis: red-brown macular lesions on the trunk. They are likley to resolve over the next few years (CM-patient3)

Juvenile mastocytosis: red-brown macular lesions on the trunk. They are likely to resolve over the next few years (CM-patient3)

Juvenile mastocytosis: red-brown macular lesions on the neck. They are likely to resolve over the next few years (CM-patient3)

Juvenile mastocytosis: red-brown macular lesions on the neck. They are likely to resolve over the next few years (CM-patient3)

A solitary mastocytoma on an infant's chest

Red-brown monomorphic maculopapules in adult-onset urticaria pigmentosa

Red-brown monomorphic maculopapules in adult-onset urticaria pigmentosa

Lesions of infantile mastocytosis on the face and lids (CM-patient4)

Infantile mastocytosis - some lesions have blistered after urticating due to rubbing (CM-patient4)

Infantile mastocytosis - crusting is evident where blistering has been present (CM-patient4)

Infantile mastocytosis - crusting is evident where blistering has been present (CM-patient4)

Urticated plaques on the arm due to infantile mastocytosis (CM-patient4)

Lesions of mastocytosis on the lid (CM-patient4)

Adult-onset urticaria pigmentosa in skin of colour (CM-patient5)

Adult-onset urticaria pigmentosa in skin of colour (CM-patient5)

Adult-onset urticaria pigmentosa in skin of colour (CM-patient5)

Red monomorphic maculopapules due to a variant of mastocytosis called telangiectasia macularis eruptiva perstans (CM-patient6)

Urticaria pigmentosa in an adult - dermoscopy showed telangiectasia; so called telangiectasia macularis eruptiva perstans (CM-patient6)

Red-brown maculopapules in adult onset urticaria pigmentosa

Diffuse cutaneous mastocytosis in an infant - the tan-orange colour is typical; blistering is more common in this variant