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Simple cases
Case 41
3-year-old girl presents with increasing number of lesions on her back since the first month of life
History
Does anyone else in the family have similar lesions?
Not that I know. Her grandfather has lots of brown warty things, but he got them when he was old.
This disease is usually not inherited. The grandfather probably has seborrheic keratoses.
Do you (the parents) have eczema, asthma or hay fever?
The mother had atopic dermatitis as a child and still has hay fever in the spring.
The search for atopy is usually wise, but irrelevant in this case.
Do the skin lesions change with rubbing or bathing?
Yes, spots under areas that are rubbed, by clothing for example, sometimes swell up. She also complains of itching. We never noticed anything when bathing her.
The lesions respond to mechanical pressure by release of chemical mediators (Darier sign).
Has she ever has blisters?
Yes, about a year ago a few spots in the diaper area got really swollen and then blistered. They were hard to get to heal.
When the swelling is extreme, then blistering occurs. This is almost limited to the first 3 years of life.
Does anyone have psoriasis? Did she have cradle cap?
No, not that we know.
Untreated psoriasis in children is always scaly, and after 3 years, she should have some larger plaques.
Did she have blisters at birth?
No, everything starts a few months after her birth.
While this disease can present at birth, congenital appearance would also suggest incontinentia pigmenti, congenital lues and TORCH syndrome. None of these should have persisted so long.
How is she doing in general? Did she meet the milestones as soon as other children?
If anything, she's ahead of her siblings in everything.
These cutaneous disease is not associated with any psychomotor problems.
Lesion
Choose the right efflorescences:
The red-brown macules are typical; they are often oval and follow skin lines.
When a lesion is rubbed, a hive is produced.
Rarely lesions can swell so much that they blister.
No persistent palpable lesions, such as nodules or papules, are seen.
There is no scale, as this disease does not involve the epidermis.
Diagnosis
Choose the right diagnosis:
There is no dermatitis; the epidermis is not involved.
The classic target lesions of erythema multiforme are not seen. In addition, erythema multiforme is an acute illness.
Café-au-lait macules do not swell with rubbing and are fewer in number.
Very good! The red-brown color, age of child, and the positive Darier sign are all classic.
The lesions are not granulomatous and sarcoidosis does not have a positive Darier sign. In addition, such extensive cutaneous sarcoidosis in small children is very rare.
Therapy
Choose the right therapy(ies):
There are not melanocytic lesions, but collections of benign mast cells. They go away on their own and have zero malignant potential.
If the lesions itch, this is the way to go.
PUVA is not indicated in childhood for a self-resolving disease. In adults, it may be useful for other forms of mast cell disease.
Such children can have massive degranulation of mast cells with environmental challenge (jumping into cold water), following a bee or wasp sting, or when exposed to medications that destabilize mast cells. They should carry an emergency set with corticosteroids and antihistamines.
Test
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