Extensive Darier's Disease

This 55 yo man presents with a flare of Darier’s disease. In his own words, he suffers from “mental illness” and has been on lithium for decades. He very embarassed about his skin and feels that he looks “hideous” because of this. Two yeas ago he had squamous cell carcinoma of the base of the tongue that was treated with surgery, radiation and chemothreapy.  This is in remission presently.

O/E: The examination shows widespread discrete and confluent reddish-brown greasy papules on the the chest and back. 

We have treated him successfully with isotretinoin in the past and were concerned about the possible interaction between lithium and isotretinoin, but the patient is at his wits end with his disease.

PLAN: He was requalified for iPledge today. He will be started on 40 mg of isotretinoin a day. In a month, we will do biochemistry survey, CBC, lipid profile, and lithium level.  We will obtain his last lithium level, in addition.

Have you managed similar patients and if so, what are your recommendations?

The patient was treated with 40 mg of isotretinoin daily.  After a few months, he is completely clear and his dosage is being tapered.

 

References

Isotretinoin treatment of Darier's disease.
J Am Acad Dermatol. 1982 Apr;6(4 Pt 2 Suppl):721-6.
Dicken CH, et, al.

Keratosis Follicularis

Presented by DJ Elpern
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.