اطلاعات بیشتر ― فارسی

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.

دختر 6 ساله ای با چندین لکه تیره رنگ که ابتدا روی پوست سرش ظاهر شد و سپس در شش ماه گذشته به صورت و بدنش پخش شد وارد شد. او به احساس بلند شدن، قرمز شدن و خارش آنها هنگام اعمال فشار اشاره کرد. او گرگرفتگی، استفراغ، اسهال یا خس خس سینه را تجربه نکرد و سوابق پزشکی شخصی و خانوادگی او سرنخ مناسبی را ارائه نکرد. پس از معاینه، لکه‌های تیره متعددی را روی پوست سر، پیشانی، صورت و گردن او به همراه لکه‌های تیره کمی برجسته روی سینه و پشت او پیدا کردیم. مالش ملایم لکه ها باعث تورم و خارش آنها در عرض 2 دقیقه شد، اما علائم در عرض 15 تا 20 دقیقه محو شدند (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.