Urticaria pigmentosa - Urticària Pigmentosa
Urticària Pigmentosa (Urticaria pigmentosa) és la forma més freqüent de mastocitosi cutània. És una malaltia rara causada per un nombre excessiu de mastòcits a la pell que produeix urticària o lesions a la pell quan s'irrita. Sovint es veuen taques vermelles o marrons a la pell, normalment al voltant del pit, el front i l'esquena. Aquests mastòcits, quan s'irriten (per exemple, fregant la pell, exposició a la calor), produeixen massa histamina, provocant una reacció al·lèrgica que provoca urticària localitzada a la zona d'irritació, de vegades denominada "signe de Darier".
☆ En els resultats de la Stiftung Warentest d'Alemanya de 2022, la satisfacció dels consumidors amb ModelDerm va ser només lleugerament inferior a la de les consultes de telemedicina de pagament.
References
Mastocytosis és una afecció on hi ha un excés de mastòcits, que sovint es troben en diverses parts del cos com la pell, la medul·la òssia i el sistema digestiu. Segons l'Organització Mundial de la Salut (OMS) , cutaneous mastocytosis es pot classificar en tres tipus principals. El primer tipus (mastocytomas) consisteix en lesions solitàries o poques (≤3) . El segon tipus (urticaria pigmentosa) implica múltiples lesions, que normalment van des de més de 10 fins a menys de 100. L'últim tipus mostra una afectació generalitzada a tota la pell. Urticaria pigmentosa és la forma més comuna de mastocitosi cutània en nens, però també pot ocórrer en adults. Normalment és una condició inofensiva que sovint millora durant l'adolescència. A diferència de la mastocitosi adulta, urticaria pigmentosa rarament afecta els òrgans interns. Una característica distintiva de urticaria pigmentosa és la seva tendència a causar petites taques o lesions a la pell, amb picor, de color marró vermellós o marró groguenc, conegudes comunament com a urticària. Aquestes taques solen aparèixer durant la infància i poden durar tota la vida.
Mastocytosis is a disorder characterized by mast cell accumulation, commonly in the skin, bone marrow, gastrointestinal (GI) tract, liver, spleen, and lymphatic tissues. The World Health Organization (WHO) divides cutaneous mastocytosis into 3 main presentations. The first has solitary or few (≤3) lesions called mastocytomas. The second, urticaria pigmentosa (UP), involves multiple lesions ranging from >10 to <100 lesions. The last presentation involves diffuse cutaneous involvement. UP is the most common cutaneous mastocytosis in children, but it can form in adults as well. It is considered a benign, self-resolving condition that often remits in adolescence. Unlike adult forms of mastocytosis, there is rarely any internal organ involvement in UP. What makes UP particularly distinctive is its tendency to manifest as small, itchy, reddish-brown, or yellowish-brown spots or lesions on the skin, commonly referred to as urticaria or hives. These spots typically appear in childhood and can persist throughout a person's life.
Mastocytosis is a disorder characterized by mast cell accumulation, commonly in the skin, bone marrow, gastrointestinal (GI) tract, liver, spleen, and lymphatic tissues. The World Health Organization (WHO) divides cutaneous mastocytosis into 3 main presentations. The first has solitary or few (≤3) lesions called mastocytomas. The second, urticaria pigmentosa (UP), involves multiple lesions ranging from >10 to <100 lesions. The last presentation involves diffuse cutaneous involvement. UP is the most common cutaneous mastocytosis in children, but it can form in adults as well. It is considered a benign, self-resolving condition that often remits in adolescence. Unlike adult forms of mastocytosis, there is rarely any internal organ involvement in UP. What makes UP particularly distinctive is its tendency to manifest as small, itchy, reddish-brown, or yellowish-brown spots or lesions on the skin, commonly referred to as urticaria or hives. These spots typically appear in childhood and can persist throughout a person's life.
Una nena de 6 anys va entrar amb diverses taques de color fosc que van aparèixer primer al cuir cabellut i després es van estendre a la cara i al cos durant els últims sis mesos. Va esmentar sentir-los pujar, posar-se vermells i picar quan s'aplicava pressió. No va experimentar rubor, vòmits, diarrea o sibilàncies, i els seus antecedents mèdics personals i familiars no van proporcionar pistes rellevants. Després de l'examen, vam trobar múltiples taques fosques al cuir cabellut, el front, la cara i el coll, juntament amb taques fosques lleugerament elevades al pit i a l'esquena. Fregar lleugerament les taques va provocar que s'inflessin i piquessin en 2 minuts, però els símptomes es van esvair en 15-20 minuts (Darier's sign) .
A 6-year-old female, presented with multiple dark-colored lesions, which started over the scalp and further progressed to involve the face and trunk since past six months. She gave a history of elevation, redness, and itching on the lesions on application of pressure. There was no associated flushing, vomiting, diarrhoea, or wheeze. The personal and family history was not contributory. On examination, there were multiple hyperpigmented macules over the scalp, forehead, face, and neck in addition to minimally elevated hyperpigmented plaques over the chest and the back. Gentle rubbing of the lesions elicited urtication and itching within 2 min and it resolved within 15–20 minutes, suggestive of the Darier's sign.
A 6-year-old female, presented with multiple dark-colored lesions, which started over the scalp and further progressed to involve the face and trunk since past six months. She gave a history of elevation, redness, and itching on the lesions on application of pressure. There was no associated flushing, vomiting, diarrhoea, or wheeze. The personal and family history was not contributory. On examination, there were multiple hyperpigmented macules over the scalp, forehead, face, and neck in addition to minimally elevated hyperpigmented plaques over the chest and the back. Gentle rubbing of the lesions elicited urtication and itching within 2 min and it resolved within 15–20 minutes, suggestive of the Darier's sign.
