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Keratosis pilaris atrophicans faciei: An observational, descriptive, retrospective clinical study
- Source :
- Exp Ther Med
- Publication Year :
- 2021
-
Abstract
- Keratosis pilaris atrophicans faciei (KPAF) is a hereditary follicular disorder, an atrophicans subtype of keratosis pilaris (KP) with a highly elusive diagnosis. Clinically, it presents with follicular, horny papules surrounded by an erythematous halo of the cheeks, forehead, chin and eyebrows, and it is followed by a gradual hair loss on the lateral margins of the eyebrows. The onset is as early as a few months after birth, but it is mainly diagnosed in children and adolescents and it can persist through adulthood. At present, the natural progression of the disease is poorly understood, which makes a correct diagnosis highly unlikely. The aim of the present study was to describe the clinical characteristics of KPAF in patients encountered in daily practice, in order to find common characteristics that may aid in the earlier recognition of the disease. An observational, descriptive, retrospective study was performed on 14 patients diagnosed with KPAF between January 2000 and December 2020. The mean age at diagnosis was 17.04 years and the onset of clinical symptoms appeared at a mean age of 4.85 years. The first clinical symptom was KP involving either the upper or lower limbs, or both. Then, erythema of the face appeared at a mean age of 7.21 years, keratotic papules on the face at a mean age of 8.35 years and, finally, loss of hair on the lateral margins of the eyebrows at a mean age of 14 years. The patients also had concomitant xerosis cutis, multiple mole syndrome, acne, contact dermatitis and Laugier-Hunziker syndrome. Evidence of disease progression, associations, as well as efficacious treatment measures are lacking. An earlier diagnosis potentially allows for a more efficacious, targeted treatment option. Either topical emollients, systemic retinoids or laser therapy may prove effective for each patient individually.
- Subjects :
- Keratosis pilaris atrophicans faciei
Cancer Research
medicine.medical_specialty
Erythema
business.industry
Cutis
Retrospective cohort study
General Medicine
Articles
medicine.disease
Dermatology
Keratosis Pilaris
medicine.anatomical_structure
Hair loss
Immunology and Microbiology (miscellaneous)
medicine
Forehead
medicine.symptom
business
Acne
Subjects
Details
- ISSN :
- 17921015
- Volume :
- 22
- Issue :
- 5
- Database :
- OpenAIRE
- Journal :
- Experimental and therapeutic medicine
- Accession number :
- edsair.doi.dedup.....2c2a1c95e3a9e99e6814d20de6c82499