Cutaneous mastocytosis of childhood Q82.2

For aetiopathogenesis, therapy and literature see below Urticaria pigmentosa (overview)

10% of juvenile forms of Urticaria pigmentosa develop between the 12th and 15th year of life.

trunk, upper and lower extremities, rarely face, capillitium, palms of hands and soles

The skin changes of the Urticaria pigmentosa of childhood differ significantly from those of adulthood. Preferably on the trunk, also on the upper and lower extremities, there are disseminated, more in light than close arrangement (as in the adult form), 0.2-1.0 - 2.0 cm large, symptomless, light brown, also yellow-brown moderately sharp but irregularly limited, scaleless spots and plaques.

The surface of the lesions always and continuously shows the structure of the surrounding field skin.

The Darier's sign is always triggerable, an important diagnostic phenomenon.

If a lesion is highly irritated, lesional (subepithelial, bulging) blistering may occur (maximized Darier's phenomenon). However, the blisters disappear within a week without leaving residuals (no milia formation).

Note: No blisters are formed on unmodified skin.

Multiple leiomyomas can lead to a clinically similar picture and the activation of the smooth muscles can result in a pseudo-Darier sign, but lacking erythema.

Due to the high spontaneous emission rate "wait and see". Gfls. antipruritic effective lotions (e.g. 5% Polidocanol lotion)

Verl: all forms of mastocytosis of childhood are characterized by a high tendency of regression. In 70-80% of the cases a clear spontaneous regression tendency until puberty. Persistence of the herd is possible even in adulthood. In these cases system participation is possible (in 15-30% of cases).