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.

Facial flush in a pregnant woman

Presented by Henry Foong

Ipoh, Malaysia

A 37 year old restaurant waitress had these rashes on the face for several years, but worse recently since her pregnancy. She is G2P1 at the end of her first trimester. The rash was described as itching, burning. She had seen a dermatologist in Japan and was diagnosed as rosacea. There was no fever or polyarthralgia. Family history was insignificant. Drug history nil.

She feels very uncomfortable. Examination was unremarkable except facial flushing with for bilateral and symmetrical erythematous papules on both cheeks with a mild involvement of the bridge of nose. There was no comedones. Her scalp was normal.
What do you think of the diagnosis? Do you think this is rosacea? What other differentials would you consider - lupus erythematosus, seborrheic dermatitis? How would you manage her remembering that she 3 months pregnant? Would you use topical metrondazole?

Neurotic Excoriations

Abstract: 37 yo woman with few year history of excoriations

HPI: The patient is a disabled 37 yo woman who has suffered with painful sores on face, arms, buttocks, upper back for a few years. She was a trainer of race horses till a few years ago. She has a history of alcoholism. A few years back, she was diagnosed with alcoholic hepatitis and hemochromatosis (she gets regular phlebotomies). There is a history of sexual abuse starting at age 14 or 15 which continued for ten years. Thereafter she was in a physically abusive relationship. She is on a Fentanyl patch.

O/E: Fresh and healing excoriations on face, upper back, left earlobe, buttocks.


Diagnosis: Neurotic Excotiations (NE).

Discussion: In my experience, most women who excoriate their faces and bodies in this way have experienced sexual or physical abuse. This is similar to "cutting behavior." NE may be a minor varient of cutting. Adverse Childhood Experiences can manifest themselves in this kind of self-destructive behavior. Her alcoholism may be another expression.

Treatment: These patients are very diffuclt to reach. I started with clobetasol ointment and mupirocin ointment -- these are sometime helpful although one has to be careful not to use for too long on the face. Psychotrophic medications may be helpful. Cognitive behavioral therapy can help but is rarely available for poor patients. The ones who need it the most are those least likely to find a therapist who will help. Patients with NE like this woman are very needy. It can take months to reach them.