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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. Mapping of a novel locus for keratosis follicularis squamosa on chromosome 7p14.3–7p12.1 Journal of Dermatological Science · 2010 · 4 citations
    2. A toddler case of keratosis follicularis squamosa (Dohi) successfully treated with salicylic acid ointment European Journal of Dermatology · 2019 · 1 citations
    3. Keratosis Follicularis Squamosa in Unilateral Buttock: A Case Report and Review of Literature in China Indian Journal of Dermatology · 2024
    4. Obesity and the skin British Journal of Dermatology · 2011 · 128 citations
    5. Floral Eponyms in Dermatology: A Blooming Collection of Patterns, Signs, and Appearances Indian Dermatology Online Journal · 2025
    6. Keratosis Follicularis Spinulosa Decalvans Archives of Dermatology · 1983 · 54 citations
    7. Keratosis follicularis spinulosa decalvans: a family study of seven male cases and six female carriers. Journal of medical genetics · 1992 · 41 citations
    8. Keratosis follicularis spinulosa decalvans and acne keloidalis nuchae Australasian Journal of Dermatology · 2005 · 37 citations
    9. Keratosis Follicularis Spinulosa Decalvans Associated with Acne Keloidalis Nuchae and Tufted Hair Folliculitis American Journal of Clinical Dermatology · 2008 · 35 citations
    10. Keratosis follicularis spinulosa decalvans in a family Journal of the American Academy of Dermatology · 2008 · 32 citations
    11. Keratosis follicularis spinulosa decalvans in a female Indian journal of dermatology, venereology, and leprology · 2011 · 17 citations
    12. Cystatin M/E Variant Causes Autosomal Dominant Keratosis Follicularis Spinulosa Decalvans by Dysregulating Cathepsins L and V Frontiers in Genetics · 2021 · 6 citations
    13. KERATOSIS FOLLICULARIS WITH UNUSUAL INVOLVEMENT OF THE SCALP Archives of Dermatology and Syphilology · 1943 · 5 citations
    14. Keratosis Follicularis Spinulosa Decalvans. What Syndrome Is This? Pediatric dermatology · 2005 · 5 citations
    15. Substance P in keratosis follicularis spinulosa decalvans JAAD case reports · 2015 · 4 citations
    16. Unilateral keratosis pilaris atrophicans faciei mimicking follicular mucinosis Journal of the American Academy of Dermatology · 2011 · 3 citations
    17. Overlap Between Ulerythema Ophryogenes and Keratosis Follicularis Spinulosa Decalvans: a Case Report 2015 · 1 citations
    18. Keratosis Follicularis Spinulosa Decalvans: Diagnosis and Therapeutic Evaluation. Journal of clinical case reports · 2015 · 1 citations
    19. Keratosis Follicularis The American Journal of the Medical Sciences · 1906
    20. Clinicopathologic and trichoscopic features of keratosis follicularis spinulosa decalvans: A case series study Journal of dermatology · 2023
    21. A Rare Case of Keratosis Follicularis Spinulosa Decalvans Affecting a Female Child Clinical dermatology review · 2022
    22. (16) The Graham-Little syndrome Follicular keratosis with cicatricial alopecia 1975 · 15 citations
    23. Follicular Keratosis of the Chin Treated with 1.24R‐Dihydroxyvitamin D3 Ointment Pediatric dermatology · 2007 · 13 citations
    24. Optical coherence tomography-assisted diagnosis of follicular keratosis of the chin 2015 · 5 citations
    25. 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
    26. Inverted Follicular Keratosis in Young Adult: A Rare Entity in the Differential Diagnosis of Skin Tumors Clinical Cosmetic and Investigational Dermatology · 2026
    27. Inverted follicular keratosis of the ear lobe masquerading as malignancy The Sri Lanka Journal of Dermatology · 2025
    28. Inverted Follicular Keratosis of the Lower Eyelid—A Diagnostic Conundrum Annals of Plastic Surgery · 2025
    29. An observational analysis of erythromelanosis follicularis faciei et colli Clinical and experimental dermatology · 2008 · 25 citations
    30. Erythromelanosis Follicularis Faciei: First Case Report from Saudi Arabia 2003 · 1 citations