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    Glossary Keratosis Follicularis

    rare genetic skin disorder causing rough, warty papules and plaques

    Keratosis Follicularis, also known as Darier's Disease, is a rare genetic skin disorder characterized by the development of rough, warty papules and plaques, primarily in seborrheic areas such as the scalp, forehead, upper arms, chest, and back. It is caused by mutations in the ATP2A2 gene, which affects calcium regulation in skin cells, leading to abnormal keratinization and skin cell adhesion.

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    1. Overlap Between Ulerythema Ophryogenes and Keratosis Follicularis Spinulosa Decalvans: a Case Report 2015 · 1 citations
    2. Clinicopathologic and trichoscopic features of keratosis follicularis spinulosa decalvans: A case series study Journal of dermatology · 2023
    3. Oral Treatment of Keratinizing Disorders of Skin and Mucous Membranes With Etretinate Archives of dermatology · 1982 · 31 citations
    4. Hair Disorders Harper's Textbook of Pediatric Dermatology · 2019
    5. Disorders of Keratinization 2014
    6. Acquired and inheritedhair disorders 2023
    7. Clinical Findings, Cutaneous Pathology, and Response to Therapy in 21 Patients With Keratosis Pilaris Atrophicans Archives of dermatology · 1994 · 78 citations
    8. Follicular porokeratosis: four new cases Clinical and Experimental Dermatology · 2017 · 6 citations
    9. Keratosis Follicularis Spinulosa Decalvans. What Syndrome Is This? Pediatric dermatology · 2005 · 5 citations
    10. Mapping of a novel locus for keratosis follicularis squamosa on chromosome 7p14.3–7p12.1 Journal of Dermatological Science · 2010 · 4 citations
    11. Follicular keratotic diseases: a retrospective study of 50 cases in a tertiary care center of rural South India with dermoscopy and histopathology evaluation International Journal of Research in Dermatology · 2025
    12. Frontal fibrosing alopecia and ulerythema ophryogenes as two entites that can transist one into another Nasza Dermatologia Online · 2018
    13. Primary Cicatricial Alopecias Dermatologic Clinics · 2012 · 18 citations
    14. Primary cicatricial alopecias Expert Review of Dermatology · 2010 · 1 citations
    15. Monilethrix: A Novel Mutation (Glu402Lys) in the Helix Termination Motif and the First Causative Mutation (Asn114Asp) in the Helix Initiation Motif of the Type II Hair Keratin hHb6 Journal of Investigative Dermatology · 1999 · 29 citations
    16. Trichoscopic Findings of Erosive Pustular Dermatosis of the Scalp Associated with Gefitinib Case Reports in Dermatology · 2017 · 21 citations
    17. Mapping of monilethrix to the type II keratin gene cluster at chromosome 12q13 in three new families, including one with variable expressivity British Journal of Dermatology · 1997 · 21 citations
    18. Monilethrix: Beaded hair and hypotrichosis in a child Cosmoderma · 2026
    19. Keratosis follicularis spinulosa decalvans in a family Journal of the American Academy of Dermatology · 2008 · 32 citations
    20. Olmsted syndrome Journal of Dermatological Case Reports · 2013 · 2 citations
    21. Erythromelanosis Follicularis Faciei et Colli: A Case Report International Journal of Drug Delivery Technology · 2026
    22. Keratosis Follicularis Spinulosa Decalvans Associated with Acne Keloidalis Nuchae and Tufted Hair Folliculitis American Journal of Clinical Dermatology · 2008 · 35 citations
    23. Keratosis Follicularis Squamosa in Unilateral Buttock: A Case Report and Review of Literature in China Indian Journal of Dermatology · 2024
    24. Substance P in keratosis follicularis spinulosa decalvans JAAD case reports · 2015 · 4 citations
    25. Keratosis Follicularis The American Journal of the Medical Sciences · 1906
    26. Ichthyosis Follicularis, Alopecia, and Photophobia Syndrome in a Saudi Child: A Case Report Clinical Cosmetic and Investigational Dermatology · 2023
    27. Keratosis Follicularis Spinulosa Decalvans Archives of Dermatology · 1983 · 54 citations
    28. Optical coherence tomography-assisted diagnosis of follicular keratosis of the chin 2015 · 5 citations
    29. Dermoscopic features of follicular mycosis fungoides of nonscalp areas: Report of 11 cases and literature review JEADV Clinical Practice · 2023
    30. Pityriasis rubra pilaris International Journal of Dermatology · 1999 · 97 citations