Urticaria pigmentosa
Urticaria pigmentosa est forma cutanei mastocytosis frequentissima. Rarus morbus est ex nimia multitudine cellularum mastocytarum (mast cells) in cute, quae urticaria (hives) vel laesiones in cute irritatae gignunt. Maculae rubrae vel fuscae saepe in cute visae sunt, typice circa pectus, frontem et dorsum. Hae cellularum mastocytarum (mast cells), cum irritatae (exemplo cute, expositio caloris attritu), nimium histaminum gignunt, excitat allergicam reactionem quae ducit ad urticaria (hives) locata ad locum irritationis, interdum ut "signum Darier".
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ReferencesMastocytosis est conditio ubi excessus cellulae mastocytarum est, saepe in variis partibus corporis sicut cutis, medulla ossis et systema digestivum invenitur. Secundum Organizatio Mundialis Salutis (WHO), mastocytosis cutanea in tria genera generari potest. Primum genus (mastocytomas) constat solitariis vel paucis (≤3) laesionibus. Alterum genus (urticaria pigmentosa (urticaria pigmentosa)) multas laesionibus involvit, plerumque a plus quam 10 ad minus quam 100. Ultimum genus implicationem per cutem late ostendit. Urticaria pigmentosa forma communissima est mastocytosis cutanea in pueris, sed etiam in adultis fieri potest. Solet innocens conditio quae in adolescentia saepe melioratur. Mastocytosis adulta dissimilis, raro viscera afficit. Unius notae urticaria pigmentosa proclivitas est ad parvas, pruriginosas, rubro‑brunneas, vel flavo‑brunneas, vel laesiones in cute, vulgo urticaria (hives). Hae maculae plerumque in pueris apparent et per totam vitam durare possunt.
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.
Puella VI annorum ingressa est cum pluribus maculis hyperpigmentatis (hyperpigmented macules), quae primo in capite apparuerunt, et deinde ad faciem et truncum per sex menses propagatae sunt. Ea referens elevationem, ruborem et pruritum in maculis ad pressionem applicatam. Non habuit ruborem, vomitum, alvi fluxionem (diarrhoea), aut sibilum, et historiae medicae personales et familiares non contributoriae sunt. In inspectione, multiplex maculae hyperpigmentatae (hyperpigmented macules) observatae sunt in capite, fronte, facie et collo, cum leviter elevatis plaque hyperpigmentatis (hyperpigmented plaques) in pectore et dorso. Levis frictio macularum elicuit urticam (urtication) et pruritum intra 2 minuta, quae resolutae sunt intra 15‑20 minuta, suggestiva signum Darier (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.
