Mastocytosis
| Figure 2.15
A: Dermoid cyst
(Reprint from Laxer RM, ed.
The Hospital for Sick Children:
Atlas of Pediatrics. Philadelphia,
PA: Current Medicine; 2005)
B: Urticaria pigmentosa
(Courtesy of Dr. Michelle
B. Bain)
C: Urticaria pigmentosa
(Courtesy of Dr. Paul Getz) |
- Childhood mastocytosis – onset before puberty (50% before age 2), c-kit alteration (proto-oncogene, tyrosine kinase subfamily); several forms in children:
| | | | | | | | Solitary Mastocytoma | | – Tan to brown, minimally infiltrated plaque or nodule; spontaneous resolution over months
– Positive Darier sign | | | Urticaria Pigmentosa (UP) | | – Onset early childhood, may occur in adults
– Hyperpigmented to pink pruritic macules or papules on trunk; positive Darier sign
– Variant: bullous UP | | | Diffuse Cutaneous Mastocytosis | | – Doughy or boggy skin texture with lichenification and yellow hue
– Extreme pruritus, friction may cause bullae
– Systemic symptoms: bronchospasm, diarrhea | | | Telangiectasia Macularis Eruptiva Perstans (TMEP) | | – Persistent eruption of macules and papules with red-brown hue
– Rare in childhood |
| | | | | |
|
- Avoid mast cell degranulators: aspirin, alcohol, opiates, quinine, polymyxin B sulfate, amphotericin B, tubocuraine, scopolamine
|
