Phakomatosis Pigmentovascularis

We report a patient with phakomatosis pigmentovascularis IIb and numerous iris hamartomas. Phakomatosis pigmentovascularis IIb is characterized by the simultaneous occurrence of a nevus flammeus, a mongolian spot, and sometimes a nevus anemicus in the same individual, with systemic involvement. To our knowledge, the association with multiple iris hamartomas has been reported only once. This second patient suggests that the association might be more common. Additional reports will indicate if such an association is more frequent than is now assumed

Value of urinary N-methylhistamine measurements m childhood mastocytosis

Background: Histamine is an indicator of mast cell activation. N- methylhistamine (NMH) is a metabolite of histamine that can be measured in urine. Objective: Our purpose was to assess the usefulness of determining urinary NMH levels for the diagnosis and follow-up of patients with mastocytosis. Methods: Urinary NMH levels were determined in 44 patients and were correlated with disease activity and extension. The control group consisted of 24 children without mastocytosis or any other skin disease. Results: A significant negative correlation was found between NMH and age in patients with active mastocytosis and in the control group. Adjusted for age, NMH values were significantly higher in patients with active mastocytosis. There was a significant difference in NMH values between patients with diffuse cutaneous mastocytosis, patients with active urticaria pigmentosa, and patients with active mastocytomas. However, there was a substantial overlap of NMH values in the different subgroups. Conclusion: Urinary NMH values tend to decrease with age. Urinary NMH values correlated with the extent and the activity of the disease. High NMH values suggest more extensive involvement

Food-induced contact urticaria syndrome (CUS) in atopic dermatitis: Reproducibility of repeated and duplicate testing with a skin provocation test,the skin application food test (SAFT)

IgE-mediated contact urticaria syndrome (CUS) is one of the manifestations of allergy in childhood atopic dermatitis (AD). Allergens such as foods and animal products penetrate the skin easily. They can then cause urticarial reactions in sensitized individuals. A provocation test system for foods, called the skin application food test (SAFT), has been developed. Over more than 5 years, a group of 175 patients with AD was built-up and investigated in a prospective follow-up study with SAFT. SAFT was more frequently positive in AD children aged 6–2 years than in older children. In several children of this population (Group 1), we repeated SAFT within a period of 1 year. In another unrelated group of children (Group 2–1), we compared the results of ‘original’ SAFT and SAFT using square chambers (Van der Bend) or Silver patches. In the 3rd group (Group 2–2) we compared‘original' SAFT with SAFT using big Finn Chambers. The agreement between the tests was high: in Group 1, we observed 88 to 93% concordant scores, and in Group 2, the scores were 96% to 100%. Statistically, the K coefficient ranged from 0.71–0.87 in Group 1, and from 0.83–1.00 in Group 2. SAFT is therefore highly reproducible. Agreement was at least geqslant R: gt-or-equal, slanted88% between the scores (the lowest K value observed was at least 0.71)