SIAIC Toscana-Emilia Romagna San Marino Allergia e mastocitosi in età pediatrica Elio Novembre Dipartimento di Scienze della Salute AOU Meyer, Firenze
Cosa fare in questo bambino? Ha 8 mesi, viene per una sospetta AA : a 6 mesi vomito e orticaria dopo ingestione di una minestra con zucchina, carota, finocchio semolino e 1 cucchiaio di parmigiano. Portato al DEA e trattato con antiH1. Ha anche maculo papule sul tronco e gli arti, da sempre dicono i genitori, non prudono, ma talvolta si arrossano, senza particolari fattori scatenanti. Lo visitiamo = ndn, in particolare non organomegalia, solo alcune macule rossastre su tronco e arti
Cosa fare in questo bambino? Fa il prick: latte 0, uovo 2, merluzzo 0, grano 3, soia 0, patata 0 , carota 0, mais 0, riso 0, Pbyp zucchino 0 Vediamo se c’è il segno di Darier= positivo. Visita dermatologica= Mastocitosi cutanea (orticaria pigmentosa)
Prevalenza e insorgenza della mastocitosi - Prevalenza sconosciuta (circa 1/100000) - Insorgenza • 55% dei casi nei primi 2 aa • 10% in bambini 2- 15 aa • 35% dopo i 15 aa - M=F
Clinical classification of cutaneous mastocytosis in children* I U rticaria pigmentosa (70–90%) II Mastocytoma (10–30%) III Diffuse cutaneous mastocytosis (1–3%) • Darier's sign positive in all forms.
Orticaria pigmentosa Di solito lesioni multiple; sintomi lievi • Macule, placche or noduli • Interessamento viscerale e osseo raro and benigno • Prurito, arrrossamento, diarrea occasionale • Prognosi: buona, con risoluzione spontanea nell’ 80% dei casi entro la pubertà
Mastocitoma Una o due lesioni: noduli, placche o macule con frequente vescicolazione • • Prurito, flushing e diarrea rari • Non interessamento viscerale o osseo • Prognosi: molto buona con risoluzione spontanea nella grande maggioranza di casi
Mastocitosi cutanea diffusa Diffusa infiltrazione cutanea (pelle coriacea) , eritema, vescicole • • Frequenti arrossamenti, prurito, diarrea cronica, e complicanze come lo shock ipovolemico, sanguinamento. Interessamento viscerale e osseo frequente e benigno • • Prognosi: discreta. Le bolle tendono a scomparire. Persistenza di orticaria, iperpigmentazione e cute coriacea. La insorgenza neonatale può essere correlata a esito fatale.
Cosa fare in questo bambino? - Allergia alimentare (grano, uovo?) - Mastocitosi (Orticaria pigmentosa)
§ § A skin biopsy is recommended unless the exam is unambiguous Fried AJ Curr Asthma Report 2013
Cosa fare in questo bambino? Prescriviamo gli esami per sospetta mastocitosi cutanea - emocromo completo con formula, - test di funzionalità epatica, - sideremia, - dosaggio plasmatico della triptasi - facciamo lo SCORMA - I genitori rifiutano la biopsia + Unicap per gli allergeni sospetti
A Estensione= 1% (Mastocitoma solitario) 100%( Mastocitosi diffusa) B Intensità= 1 lesione tipica valutata in base a pigmentazione/ eritema, vescicolazione segno di Darier (0-3) 8 C Segni soggettivi = 0-10 1 VALORI FRA 5.2 E 100 1 3 17.7 Heide R et al Clin Exp Dermatol 2008
Serum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis R. : Heide et al Clinical and Experimental Dermatology Volume 34, Issue 4, pages 462–468, June 2009 METHODS: The SCORMA Index in 64 patients (31 children and 33 adults) was compared with serum tryptase levels. The results of the first visit at which SCORMA and tryptase were evaluated were analysed. RESULTS: There was a positive correlation between the SCORMA Index and serum tryptase levels, indicating the value of the SCORMA Index in the assessment of mastocytosis with skin involvement. CONCLUSION: The results of this study showed that the SCORMA Index is a useful tool for evaluating the severity of cutaneous mastocytosis. The correlation between the SCORMA Index and serum tryptase levels underlines the benefit of the SCORMA Index as a clinical tool. Repeated SCORMA Index measurements can provide a rapid impression of changes in the clinical state of mastocytosis. This is particularly relevant in children, because taking blood samples from this group is much more difficult.
Serum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis Heide et al Clinical and Experimental Dermatology 2008
Cosa fare in questo bambino? Risultato esami: - RAST grano 10, uovo 0.6 - Triptasi sierica = 5 Eseguiamo TPO uovo=ndn Dimettiamo il pz solo con dieta di esclusione per grano Programmiamo un controllo dopo 6 mesi
Quale follow-up in questo bambino? Allergia alimentare : monitoraggio clinico e allergologico e individuazione tempi per TPO grano Orticaria pigmentosa : monitoraggio clinico e della triptasi
Storia naturale della mastocitosi cutanea del bambino La storia naturale della mastocitosi cutanea è benigna. Nella maggior parte dei bambini le lesioni cutanee tendono a scomparire con la pubertà. Hartmann K., et al. Int Arch Allergy Immunol 2002. Brockow K. Immunol Allergy Clin North Am 2004.
Follow-up of paediatric mastocytosis:a review of 180 patients Ris. Com Ris. Parz. Nessuna mod. Mastocitoma (27) 20 (74%) 5 (18%) 2 (7.5%) Durata media (aa) 7.4 5.6 2.4 Orticaria pigmentosa (62) 35 (56%) 15 (24%) 12 (19.4%) Durata media (aa) 10.2 7.1 2.8 Ben-Amitai D et al IMAJ 2005
Fattori di rischio nel bambino • Elevata estensione delle lesioni cutanee • Aumento valori triptasi basale • Blistering diffuso Brockow K. Immunol Allergy Clin North Am 2014
Allergia e mastocitosi nella età pediatrica
Differences between Mast Cell Activation in Mastocytosis and during IgE-mediat ed Allergic Hypersensitivity Mastocytosis IgE-me diated Alle rgic Hyper sensitivity Key cell Mast cell Mast cell and basophil Recep tor C-kit receptor (D816V mutation) Fc RI Mechanism Non IgE-mediated IgE-cross-linking by aller gen Triggers Nonspecific (psychological, Specific (IgE antib ody) pha rm acological, mechanica l factors and t em perature changes) Serum tryptase after t he Incre ased Incre ased clinical reaction Basal serum try ptase Usually incre ased (>20 µg/ l) in Not incre ased SM Less than 20 µg/ l in CM Skin te sts Negative Positive
Mastocytosis and atopy: a study of 33 patients with urticaria pigmentosa. Thirty-three patients with histologically verified urticaria pigmentosa were studied for coexisting atopic disease by means of history, skin prick testing with five common inhalants and serological investigation for total IgE and specific IgE antibodies to five common inhalants. The prevalence of atopy in urticaria pigmentosa was similar to that observed in the normal Swiss population, both on the basis of history (7/33 = 21%) and of positive skin prick tests to common inhalants (12/33 = 36%). However, total serum IgE levels were significantly lower (geometric mean value 16.8 kU/l) than in a control group of 52 Swiss blood donors of comparable age and sex distribution (geometric mean value 43.0 kU/l, t = 2.93, P less than 0.005). Specific IgE antibodies to common inhalants were also observed less frequently in urticaria pigmentosa patients than in controls, although this difference was not statistically significant. Low total and specific IgE values in patients with urticaria pigmentosa may be explained by increased absorption of circulating IgE to abundant tissue mast cells. Muller U et al, Allergy. 1990.
Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in Spain: a study of the Spanish network on mastocytosis (REMA). -A questionnaire was given to 210 patients with mastocytosis (cutaneous mastocytosis (CM), n =8; indolent -systemic mastocytosis (ISM), n =140 (125 with skin involvement); well-differentiated systemic mastocytosis (WDSM), n =5; Isolated BM mastocytosis (BMM), n =3 and mastocytoma, n =1) to evaluate the history of asthma, rhinitis, conjunctivitis,atopic dermatitis, urticaria and anaphylaxis. Patients underwent total IgE, Phadiatop infant (aeroallergens and food allergens), specific IgE to latex and to Anisakis simplex determinations. Skin tests were done to 72 patients. RESULTS: The prevalence of allergy, as defined by clinical symptoms associated to specific IgE, was 23.9%. Allergic diseases coexist in patients with mastocytosis with similar frequency as compared with the general population. The coexistence of atopy does not influence mastocytosis-associated symptoms- Gonzales de Olano D et al, Clin Exp Allergy. 2007
Mastocitosi e rischio di anafilassi nella età pediatrica: sono necessarie particolari misure preventive?
Clinical classification of cutaneous mastocytosis in children* I U rticaria pigmentosa (70–90%) II Mastocytoma (10–30%) III Diffuse cutaneous mastocytosis (1–3%) • Darier's sign positive in all forms.
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