Urticaria pigmentosa
Urticaria pigmentosa ni aina ya kawaida ya mastocytosis ya ngozi. Ni ugonjwa adimu unaosababishwa na idadi kubwa ya seli za mlingoti kwenye ngozi, ambazo hutoa mizinga au vidonda unapowashwa. Madoa nyekundu au kahawia mara nyingi huonekana kwenye ngozi, hasa karibu na kifua, paji la uso, na mgongo. Seli hizi za mlingoti, zinapowashwa (km. kwa kusugua ngozi, mfiduo wa joto), hutoa histamini nyingi na kusababisha athari ya mzio inayowekwa ndani ya eneo la mwasho, ambayo wakati mwingine hujulikana kama “ishara ya Darier”.
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ReferencesMastocytosis ni hali ambapo kuna ziada ya seli za mlingoti, mara nyingi hupatikana katika sehemu mbalimbali za mwili kama vile ngozi, uboho, na mfumo wa usagaji chakula. Kulingana na Shirika la Afya Duniani (WHO), cutaneous mastocytosis inaweza kugawanywa katika aina kuu tatu. Aina ya kwanza (mastocytomas) inajumuisha vidonda vya pekee au vichache (≤3). Aina ya pili (urticaria pigmentosa) inahusisha vidonda vingi, kwa kawaida kuanzia zaidi ya 10 hadi chini ya 100. Aina ya mwisho inaonyesha ushiriki ulioenea kwenye ngozi. Urticaria pigmentosa ndiyo aina ya kawaida ya mastocytosis ya ngozi kwa watoto, lakini pia inaweza kutokea kwa watu wazima. Kawaida ni hali isiyo na madhara ambayo mara nyingi huboresha wakati wa ujana. Tofauti na mastocytosis ya watu wazima, urticaria pigmentosa mara chache huathiri viungo vya ndani. Kipengele kimoja muhimu cha urticaria pigmentosa ni tabia yake ya kusababisha madoa madogo, kuwasha, nyekundu‑kahawia au manjano‑kahawia kwenye ngozi, ambayo hujulikana kama mizinga. Dalili hizi kawaida huonekana katika utoto na yanaweza kudumu maishani mwa mgonjwa.
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.
Msichana mwenye umri wa miaka 6 aliingia akiwa na madoa kadhaa ya rangi nyeusi ambayo yalionekana kwa mara ya kwanza kwenye kichwa chake, kisha yakawa yameenea usoni na mwili wake katika kipindi cha miezi sita iliyopita. Aliripoti kuwa madoa yanainuka, yanakuwa nyekundu, na yanawaka wakati shinikizo linapowekwa. Hakupata dalili za kutokwa na maji mwilini, kutapika, kuhara, au shida ya kupumua, na historia yake ya matibabu ya kibinafsi na ya familia haikutoa vidokezo muhimu. Baada ya uchunguzi, tuligundua madoa mengi meusi kwenye ngozi ya kichwa, paji la uso, uso, na shingo, pamoja na mabaka meusi yaliyoinuliwa kidogo kwenye kifua na mgongo wake. Kusugua kidogo madoa kulisababisha kuvimba na kuwaka ndani ya dakika 2, lakini dalili zilififia ndani ya dakika 15–20 (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.
