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.
The clinical pictures and the histopath slides are superb. I think the skin biopsy was the clue to the diagnosis and it's important that dermatologists should do more biopsies when there is a doubt in the diagnosis. I notice too often dermatologists in Ipoh and in Malaysia go by the gut and make too much clinical diagnosis without a histological confirmation. We have dermatologists who do not do skin biopsies and also dermatologists who do only a biopsy or two a month. Aren't they missing out great opportunities to understand and make a in depth observation of the disease??
ReplyDeleteBased on the clinical history and the histopath, i agree this is Darier's. Wonder if topical retinoids would help? Topical pimecrolimus is a novel treatment for limited Darier's. would topical tacrolimus be more potent and effective??
I agree the clinical and histopathological images are just so outstanding! I found an article on this topic and will ask David to post the article. I thought the following comment was interesting in the article. More to follow up.
ReplyDeleteIn the present case, tacrolimus ointment was chosen because it has recently been used with success in all the immune-mediated pathologies mentioned above, including Hailey–Hailey’s disease, which is clinically and pathogenetically similar to Darier’s disease because of a mutation in the ATP2C1 gene on chromosome 3q, encoding a calcium pump. Although the skin of patients with acantholytic dyskeratotic diseases is characterized by defective cell adhesion, it has been suggested that symptomatic lesions only occur when external (trigger) factors such as infection, maceration and friction induce a cascade of inflammatory mediators and T lymphocyte recruitment.