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Published on: December 15, 2011
[Urticaria pigmentosa: two different clinical presentations in pediatric patients]
Julieta Spada1, Mariana Lequio, María de los Ángeles Pyke
1Centro de Investigaciones Dermatológicas, Buenos Aires, Argentina. julieta@spada.com.ar
Insights
Urticaria pigmentosa (UP), a skin condition, presents in early childhood or adulthood. Recognizing both typical and unusual presentations is key for pediatricians managing this mast cell disorder.
Area of Science:
- Dermatology
- Pediatrics
- Pathology
Background:
- Urticaria pigmentosa (UP) is the most common manifestation of cutaneous mastocytosis.
- UP exhibits a bimodal incidence, with peaks in early childhood and adulthood.
- Darier's sign is a characteristic clinical finding in affected skin.
Observation:
- Early-onset UP generally has a favorable prognosis, often resolving by adolescence.
- Late-onset UP is frequently linked to persistent disease or systemic involvement.
- This report details two UP cases: one classic and one with an atypical presentation.
Findings:
- The study highlights the diverse clinical spectrum of urticaria pigmentosa.
- It emphasizes the importance of considering both typical and unusual UP presentations in pediatric cases.
- The findings suggest a unified approach to managing UP, regardless of onset age or presentation.
Implications:
- Pediatricians should be aware of the varied clinical presentations of urticaria pigmentosa.
- Early recognition and diagnosis of UP can lead to timely management and improved outcomes.
- Understanding the prognostic differences between early and late-onset UP is crucial for patient care.
Abstract:
Urticaria pigmentosa (UP) is the most frequent clinical feature of cutaneous mastocytosis. It usually begins in a bimodal way: a peak of incidence from birth to the age of 3 and the other one between 2(nd) and 6(th) decades of life. Darier's sign is constant over the affected skin without affecting the surrounding skin. When UP starts early, it has a good prognosis disappearing into adolescence, while late onset is often associated with persistent or systemic involvement. This article reports two cases of UP, one with the classic description of the disease and the other with an unusual clinical presentation, prompting the pediatrician to incorporate both forms as different manifestations of the same entity.
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