Photomicrographs by Jag Bhawan
Abstract: 10 yo girl with 4 month history of a dermatosis on the neck
HPI: The patient is a pleasant 10 year old who presents for evaluation of a symmetrical papular eruption on the sides of the neck which has been present for about 4-5 months. She has been treated with a number of different topicals by her pediatrician without relief. The patient lives with a grandmother and there is no pertinent family history.
O/E: The examination show somewhat rough, 1 - 2 mm in diameter, keratotic micropapules on the lower folds of the neck. The remainder of the cutaneous examination is unremarkable.
Clinical Photos:
Biopsy: Focal acantholysis, multiple dyskeratotic cells, corps ronds and grains consistent with Darier's disease.
10 x and 20 x
40 x
Diagnosis: Keratosis follicularis (Darier's disease). It's unusual to see keratosis follicularis when it first appears. This is a sweet 10 year old and it's sad to contemplate what this may turn into. After "reviewing the literature" I decided to try pimecrolimus cream as there have been some reports of success.
Questions: How would you treat this child? Have you seen forme-frustes of keratosis follicularis?
References:
1. Good Overview: Darier's Disease eMedicine
2. Pérez-Carmona L, et. al. Successful treatment of Darier's disease with topical pimecrolimus. Eur J Dermatol. 2011 Mar-Apr;21(2):301-2.
3. (supplied by Yoon Cohen) Rubegni P, Poggiali S, Sbano P, Risulo M, Fimiani M. A case of Darier's disease successfully treated with topical tacrolimus. J Eur Acad Dermatol Venereol. 2006 Jan;20(1):84-7.
Abstract: Tacrolimus is a macrolide that inhibits T-cell activation. The most extensive experience with topical tacrolimus has been in treating atopic dermatitis but it has been used in various skin diseases, including Hailey-Hailey disease, with encouraging results. We report a case of extensive Darier's disease successfully treated with topical tacrolimus, after suspension of oral isotretrinoin due to major depression.
