Urticaria pigmentosa
La Urticaria pigmentosa es la forma más común de mastocitosis cutánea. Es una enfermedad rara causada por un número excesivo de mastocitos en la piel que producen urticaria o lesiones en la piel cuando se irrita. A menudo se ven manchas rojas o marrones en la piel, generalmente alrededor del pecho, la frente y la espalda. Estos mastocitos, cuando se irritan (por ejemplo, frotando la piel, exposición al calor), producen demasiada histamina, lo que desencadena una reacción alérgica que provoca urticaria localizada en el área de irritación, a veces denominada "signo de Darier".
☆ En los resultados de 2022 del Stiftung Warentest de Alemania, la satisfacción del consumidor con ModelDerm fue sólo ligeramente inferior que con las consultas de telemedicina pagadas.
Suele aparecer en el torso de los niños pequeños.
References
Mastocytosis es una afección en la que hay un exceso de mastocitos, que a menudo se encuentran en varias partes del cuerpo, como la piel, la médula ósea y el sistema digestivo. Según la Organización Mundial de la Salud (OMS) , el cutaneous mastocytosis se puede clasificar en tres tipos principales. El primer tipo (mastocytomas) consiste en lesiones solitarias o unas pocas (≤3) . El segundo tipo (urticaria pigmentosa) implica múltiples lesiones, que generalmente varían entre más de 10 y menos de 100. El último tipo muestra una afectación generalizada en toda la piel. Urticaria pigmentosa es la forma más común de mastocitosis cutánea en niños, pero también puede ocurrir en adultos. Suele ser una afección inofensiva que suele mejorar durante la adolescencia. A diferencia de la mastocitosis en adultos, urticaria pigmentosa rara vez afecta los órganos internos. Una característica distintiva de urticaria pigmentosa es su tendencia a causar pequeñas manchas o lesiones en la piel, de color marrón rojizo o marrón amarillento, que pican, comúnmente conocidas como urticaria. Estas manchas suelen aparecer en la infancia y pueden durar toda 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 niña de 6 años llegó con varias manchas de color oscuro que aparecieron primero en su cuero cabelludo y luego se extendieron a su cara y cuerpo durante los últimos seis meses. Mencionó sentir que se elevaban, se enrojecían y picaban cuando se aplicaba presión. No experimentó sofocos, vómitos, diarrea ni sibilancias, y sus antecedentes médicos personales y familiares no proporcionaron pistas relevantes. Tras el examen, encontramos múltiples manchas oscuras en el cuero cabelludo, la frente, la cara y el cuello, junto con manchas oscuras ligeramente elevadas en el pecho y la espalda. Frotar ligeramente las manchas provocó que se hincharan y picaran en 2 minutos, pero los síntomas desaparecieron en 15 a 20 minutos (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.
