A different approach for identifying hypomelanotic macules in tuberous sclerosis complex
L Gualandri1, D Boccardi, S Menni
1Dermatologic Clinic, University of the Study of Milan San Paolo Hospital, Milan, Italy - lorenzo.gualandri@ao-sanpaolo.it.
Aim:
Tuberous sclerosis complex is a neurocutaneous syndrome with an autosomal dominant pattern of inheritance and a high percentage of sporadic cases. One of the most interesting thing about tuberous sclerosis complex is the earliest skin manifestation represented by the hypomelanotic patches. These lesions may be difficult to diagnose. In our article we provide a different diagnostic tool in order to increase the likely of right identification of these lesions.
Methods:
We used a modified starch-iodine, applied on the hypomelanotic patches of the patient and on the normal skin. It allowed to show a decreased sweat production by eccrine glands in hypomelanotic macules in TSC. This test has not been standardized yet, but it is simple and inexpensive.
Results:
Three patients of pediatric age and 1 adult patient affected by tuberous sclerosis complex were enrolled. The test put in evidence a decreased activity of the sweat glands.
Conclusion:
The hypomelanotic patches are very common in tuberous sclerosis complex, and sometimes are the only earliest sign with few elements of this syndrome. There are different diagnostic tools like skin biopsies, but they are invasive and not always easy to perform. The opportunity of a simple and unexpensive test for helping in the right diagnosis of this syndrome should be considered.
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