Table 2.
Treatment of Pediatric Mastocytosis
| A. Avoidance of Triggers |
| Specific foods, medications, allergens; and general triggers (Table 3) |
| Physical measures |
| Avoid sudden changes of temperature. |
| Avoid extreme temperatures in bath/shower, swimming pool, air conditioned. |
| Avoid dryness of skin |
| Avoid rubbing |
| Local care of skin |
| Take steps to avoid drying of skin and use skin moisturizer |
| Water-soluble sodium cromolyn cream |
| Apply two to four times a day for urticaria, pruritus, vesicles or bullae. Do not use on denudated lesions (consider topical antibiotics) |
| Topical corticosteroid cream |
| In diffuse lesions apply bath or sterile gauze with zinc sulfate |
| 1. Solitary mastocytoma |
| Water-soluble sodium cromolyn cream: |
| Corticosteroid cream. |
| Avoid friction and pressure |
| Consider surgical excision (flexures, soles, palms, scalp) |
| 2. UP and other forms |
| Trigger (s)-related symptoms |
| Avoidance of triggers |
| H1 antihistamines |
| H2 antihistamines |
| Continuous moderate symptoms |
| Scheduled non-sedating H1 antihistamines; if necessary add sedating H1 antihistamines at demand |
| Scheduled or at demand H2 antihistamines |
| Oral disodium cromolyn in case of persistence of symptoms |
| Severe symptoms |
| Scheduled non-sedating H1 antihistamines |
| Scheduled sedating H1 antihistamines |
| Scheduled H2 antihistamines |
| Oral disodium cromolyn |
| Add antileukotrienes in refractory cases |
| 3. Diffuse forms with life-threatening mast cell-mediator related symptoms, bullae and blistering |
| Treatment may require hospitalization and, in some cases, at the Pediatric Intensive Care Unit. |
| Local therapy |
| Sterile conditions |
| Topic sodium cromolyn |
| Topic corticosteroids |
| Zinc sulfate |
| Antibiotics in denude areas |
| Systemic therapy |
| Epinephrine if necessary |
| Adequate sedation if necessary |
| Scheduled non-sedating H1 antihistamines |
| Scheduled sedating H1 antihistamines |
| Scheduled H2 antihistamines |
| Oral disodium cromolyn |
| Corticosteroids mainly in cases with associated angioedema or abdominal pain (with or without diarrhea) unresponsive to sodium cromolyn |
| Add antileukotrienes in refractory cases |
| Consider PUVA as an exceptional alternative in cases with persistent episodes of diffuse bullae and blistering unresponsive to anti-mediator therapy |