Sections

Sections Keratosis Follicularis (Darier Disease)
Overview
Presentation
DDx
Workup
Treatment
Medication
Questions & Answers
Media Gallery
References

Presentation

History

Most patients with keratosis follicularis (Darier disease) have a family history of the disease. The pattern of inheritance is autosomal dominant. However, some patients, up to 47% in one series, have no clear family history. These cases may represent sporadic mutations, or these patients may have family members with mild disease that was not recognized.

The first skin lesions typically occur in the teenage years and are frequently associated with pruritus.

Heat, sweat, humidity, sunlight, UVB exposure,^[38]lithium, oral corticosteroids, and mechanical trauma have been reported to exacerbate keratosis follicularis (Darier disease). Some females report flares around menstruation.

Even though the severity of keratosis follicularis (Darier disease) fluctuates over time, keratosis follicularis (Darier disease) is a chronic, unremitting condition. In one study, one third of patients noted improvement of the condition with age; however, another one third of patients showed worsening of the disease with age.

Physical Examination

The lesions may first appear as skin-colored or yellow-brown papules with a greasy, warty texture. These lesions are especially common in seborrheic areas such as the forehead, scalp, margin of the scalp, nasolabial folds, ears, chest, and back (see the image below).

Typical distribution of keratotic papules in the seborrheic regions. Courtesy of Susan Mallory, MD, Director of Pediatric Dermatology, Washington University School of Medicine.

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Approximately 80% of patients have mild flexural involvement with scattered papules in the groin, axillae, or, in women, submammary skin. In less than 10% of patients, flexural disease predominates, with large, warty, vegetative plaques in the axillae, groin, or perineum. These large flexural lesions are especially bothersome to patients because of their malodor.

Involvement of the hands is very common (approximately 95%). Lesions on the palms include punctate keratoses (80%), palmar pits (80%), and hemorrhagic macules (< 10%). Acrokeratosis verruciformis–like lesions (warty flat-topped papules on the dorsal hands) are present in approximately half the patients. Interestingly, several patients with acrokeratosis verruciformis of Hopf (who have dorsal hand lesions only) have been found to harbor mutations in ATP2A2, suggesting this condition may actually be a localized form of keratosis follicularis (Darier disease).

Nail changes in keratosis follicularis (Darier disease) provide important diagnostic clues (see the image below). White and red longitudinal bands, longitudinal nail ridges, longitudinal splitting, and subungual hyperkeratosis are frequently found. A sandwich of red and white longitudinal bands, often with a V-shaped nick at the free margin of the nail, is the most pathognomonic nail finding in persons with keratosis follicularis (Darier disease). These changes on the hands can also occur on the feet, albeit less commonly.

Longitudinal ridges, red and white lines, and V-shaped nicks. Courtesy of Susan Mallory, MD, Director of Pediatric Dermatology, Washington University School of Medicine.

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Mucosal lesions are detected in approximately 15% of patients, and they appear as white papules with a central depression. These cobblestone lesions are most commonly found in the mouth, but they also may occur on the anogenital mucosa. At times, oral lesions may affect the salivary glands and cause obstruction.^[39]

Clinical variants of keratosis follicularis (Darier disease) include hypertrophic and vesicobullous types. Linear or segmental keratosis follicularis (Darier disease) has been shown in some cases to result from genetic mosaicism of ATP2A2. Guttate leukoderma has been noted in some families.^[40]

Complications

Renal disease has been reported.^[41]

Differential Diagnoses

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Ronan A, Ingrey A, Murray N, Chee P. Recurrent ATP2A2 p.(Pro602Leu) mutation differentiates Acrokeratosis verruciformis of Hopf from the allelic condition Darier disease. Am J Med Genet A. 2017 May 12. [QxMD MEDLINE Link].
Onozuka T, Sawamura D, Yokota K, Shimizu H. Mutational analysis of the ATP2A2 gene in two Darier disease families with intrafamilial variability. Br J Dermatol. 2004 Apr. 150(4):652-7. [QxMD MEDLINE Link].
Bchetnia M, Charfeddine C, Kassar S, Zribi H, Guettiti HT, Ellouze F. Clinical and mutational heterogeneity of Darier disease in Tunisian families. Arch Dermatol. 2009 Jun. 145(6):654-6. [QxMD MEDLINE Link].
Shi HJ, Li M, Zhang GL, et al. Novel splice-site and frameshift ATP2A2 mutations in Chinese patients with Darier disease. Clin Exp Dermatol. 2012. [QxMD MEDLINE Link].
Folster-Holst R, Nellen RG, Jensen JM, et al. Molecular genetic support for the rule of dichotomy in type 2 segmental Darier disease. Br J Dermatol. Feb 2012. 166:464-6. [QxMD MEDLINE Link].
Wang Y, Bruce AT, Tu C, et al. Protein aggregation of SERCA2 mutants associated with Darier disease elicits ER stress and apoptosis in keratinocytes. J Cell Sci. Nov 2011. 124:3568-80. [QxMD MEDLINE Link].
Harboe TL, Willems P, Jespersgaard C, et al. Mosaicism in segmental Darier disease: an in-depth molecular analysis quantifying proportions of mutated alleles in various tissues. Dermatology. Jul 2011. 222:292-6. [QxMD MEDLINE Link].
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Klausegger A, Nischler E, Wagner RN, Pletschacher F, Hintner H, Bauer JW. Seven novel mutations in the ATP2A2 gene of Austrian patients with Darier's disease. Arch Dermatol Res. 2011 Jul. 303(5):371-4. [QxMD MEDLINE Link].
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Li CR, Cui PG, Jia H, et al. Identification of a novel mutation in a Chinese patient with mild Darier’s disease. Int J Dermatol. Nov 2010. 49:1286-8. [QxMD MEDLINE Link].
Huo J, Liu Y, Ma J, Xiao S. A novel splice-site mutation of ATP2A2 gene in a Chinese family with Darier disease. Arch Dermatol Res. Dec 2010. 302:769-72. [QxMD MEDLINE Link].
Lu FY, Xu L, Yin XG, Wan P, Zhang XD, Chen WW, et al. Identification of mutation c.632G>A (p.G211D) in the ATP2A2 gene and genotype-phenotype correlation in a large Chinese family with Darier's disease. Int J Dermatol. 2011 Nov. 50(11):1366-70. [QxMD MEDLINE Link].
Shi HJ, Li M, Zhang GL, Xu SX, Shao MH, Gu Y, et al. Novel splice-site and frameshift ATP2A2 mutations in Chinese patients with Darier disease. Clin Exp Dermatol. 2012 Aug. 37(6):677-9. [QxMD MEDLINE Link].
Shi BJ, Xue M, Zhong GS, Jiang Y, Chen DY, Feng J, et al. The ATP2A2 gene in patients with Darier's disease: one novel splicing mutation. Int J Dermatol. 2012 Sep. 51(9):1074-7. [QxMD MEDLINE Link].
Ueo D, Hamada T, Hashimoto T, Hatano Y, Okamoto O, Fujiwara S. Late-onset Darier's disease due to a novel missense mutation in the ATP2A2 gene: a different missense mutation affecting the same codon has been previously reported in acrokeratosis verruciformis. J Dermatol. 2013 Apr. 40(4):280-1. [QxMD MEDLINE Link].
Green EK, Gordon-Smith K, Burge SM, Grozeva D, Munro CS, Tavadia S, et al. Novel ATP2A2 mutations in a large sample of individuals with Darier disease. J Dermatol. 2013 Apr. 40(4):259-66. [QxMD MEDLINE Link].
Pengjun Z, Jianwen R, Biao Y. A novel missense mutation of ATP2A2 gene in a Chinese family with Darier disease. Int J Dermatol. 2014 Mar. 53(3):e204-5. [QxMD MEDLINE Link].
Miyabe C, Mitsuhashi Y, Saito M, Tsuboi R. Novel mutation in the ATP2A2 gene in a Japanese Darier's disease patient with extremely hyperkeratotic lesions. J Dermatol. 2012 Apr. 39(4):401-3. [QxMD MEDLINE Link].
Shi BJ, Xue M, Zhu YJ, Wang SP, Du Y, Chen DY, et al. Exon 12 of the ATP2A2 gene in patients with Darier disease: one novel mutation and one previously described. J Eur Acad Dermatol Venereol. 2014 Feb 19. [QxMD MEDLINE Link].
Miyauchi Y, Daiho T, Yamasaki K, Takahashi H, Ishida-Yamamoto A, Danko S. Comprehensive analysis of expression and function of 51 sarco(endo)plasmic reticulum Ca2+-ATPase mutants associated with Darier disease. J Biol Chem. 2006 Aug 11. 281(32):22882-95. [QxMD MEDLINE Link].
Müller EJ, Caldelari R, Kolly C, Williamson L, Baumann D, Richard G, et al. Consequences of depleted SERCA2-gated calcium stores in the skin. J Invest Dermatol. 2006 Apr. 126(4):721-31. [QxMD MEDLINE Link].
Kassar S, Tounsi-Kettiti H, Charfeddine C, Zribi H, Bchetnia M, Jerbi E, et al. Histological characterization of Darier's disease in Tunisian families. J Eur Acad Dermatol Venereol. 2009 Oct. 23(10):1178-83. [QxMD MEDLINE Link].
Dhitavat J, Fairclough RJ, Hovnanian A, Burge SM. Calcium pumps and keratinocytes: lessons from Darier's disease and Hailey-Hailey disease. Br J Dermatol. 2004 May. 150(5):821-8. [QxMD MEDLINE Link].
Dhitavat J, Cobbold C, Leslie N, Burge S, Hovnanian A. Impaired trafficking of the desmoplakins in cultured Darier's disease keratinocytes. J Invest Dermatol. 2003 Dec. 121(6):1349-55. [QxMD MEDLINE Link].
Leinonen PT, Hägg PM, Peltonen S, Jouhilahti EM, Melkko J, Korkiamäki T, et al. Reevaluation of the normal epidermal calcium gradient, and analysis of calcium levels and ATP receptors in Hailey-Hailey and Darier epidermis. J Invest Dermatol. 2009 Jun. 129(6):1379-87. [QxMD MEDLINE Link].
Celli A, Mackenzie DS, Zhai Y, Tu CL, Bikle DD, Holleran WM, et al. SERCA2-controlled Ca²+-dependent keratinocyte adhesion and differentiation is mediated via the sphingolipid pathway: a therapeutic target for Darier's disease. J Invest Dermatol. 2012 Apr. 132(4):1188-95. [QxMD MEDLINE Link]. [Full Text].
Savignac M, Simon M, Edir A, Guibbal L, Hovnanian A. SERCA2 Dysfunction in Darier Disease Causes Endoplasmic Reticulum Stress and Impaired Cell-to-Cell Adhesion Strength: Rescue by Miglustat. J Invest Dermatol. 2014 Jul. 134(7):1961-70. [QxMD MEDLINE Link].
Fong G, Capaldi L, Sweeney SM, Wiss K, Mahalingam M. Congenital Darier disease. J Am Acad Dermatol. 2008 Aug. 59(2 Suppl 1):S50-1. [QxMD MEDLINE Link].
Matsumoto A, Gregory N, Rady PL, Tyring SK, Carlson JA. Brief Report: HPV-17 Infection in Darier Disease With Acrokeratosis Verrucosis of Hopf. Am J Dermatopathol. 2017 May. 39 (5):370-373. [QxMD MEDLINE Link].
Okada E, Nagai Y, Motegi S, Tamura A, Ishikawa O. Fatal case of Darier's disease with recurrent severe infections. Acta Derm Venereol. 2009. 89(4):408-9. [QxMD MEDLINE Link].
Woo SM, Won CH, Cho S. Darier's disease: variable clinical presentation and a fatal outcome. Clin Exp Dermatol. 2009 Jul. 34(5):628-9. [QxMD MEDLINE Link].
Tang C, Chan M, Lee J, Hariram J. Darier's disease and schizophrenia. East Asian Arch Psychiatry. 2010 Dec. 20(4):190-2. [QxMD MEDLINE Link].
Dodiuk-Gad RP, Cohen-Barak E, Khayat M, Milo H, Amariglio-Diskin L, Danial-Faran N, et al. Darier disease in Israel: combined evaluation of genetic and neuropsychiatric aspects. Br J Dermatol. 2016 Mar. 174 (3):562-8. [QxMD MEDLINE Link].
Cederlöf M, Bergen SE, Långström N, Larsson H, Boman M, Craddock N, et al. The association between Darier disease, bipolar disorder, and schizophrenia revisited: a population-based family study. Bipolar Disord. 2015 May. 17 (3):340-4. [QxMD MEDLINE Link].
Cederlöf M, Karlsson R, Larsson H, Almqvist C, Magnusson PK, Nordlind K, et al. Intellectual disability and cognitive ability in Darier disease: Swedish nation-wide study. Br J Dermatol. 2015 Jul. 173 (1):155-8. [QxMD MEDLINE Link].
Otley CC, Momtaz K. Induction of Darier-White disease with UVB radiation in a clinically photo-insensitive patient. J Am Acad Dermatol. 1996 May. 34(5 Pt 2):931-4. [QxMD MEDLINE Link].
Bernabé DG, Kawata LT, Beneti IM, Crivelini MM, Biasoli ER. Multiple white papules in the palate: oral manifestation of Darier's disease. Clin Exp Dermatol. 2009 Oct. 34(7):e270-1. [QxMD MEDLINE Link].
Kansal NK, Hazarika N, Rao S. Familial Case of Darier Disease with Guttate Leukoderma: A Case Series from India. Indian Dermatol Online J. 2018 Jan-Feb. 9 (1):62-63. [QxMD MEDLINE Link].
Matsuoka LY, Wortsman J. Renal involvement in Darier disease. J Am Acad Dermatol. 2016 Dec. 75 (6):e235. [QxMD MEDLINE Link].
Mansura A, Maly A, Ramot Y, Zlotogorski A. Acantholytic dermatosis of the vulva. Dermatol Online J. 2015 May 18. 21 (5):[QxMD MEDLINE Link].
Ashok Kumar P, Paulraj S, Dutta S. Debilitating Darier's Disease and Its Impact on the Quality of Life. Cureus. 2020 May 15. 12 (5):e8133. [QxMD MEDLINE Link].
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Bachar-Wikstrom E, Curman P, Ahanian T, Leong IUS, Larsson H, Cederlöf M, et al. Darier disease is associated with heart failure: a cross-sectional case-control and population based study. Sci Rep. 2020 Apr 23. 10 (1):6886. [QxMD MEDLINE Link].
Casals M, Campoy A, Aspiolea F, Carrasco MA, Camps A. Successful treatment of linear Darier's disease with topical adapalene. J Eur Acad Dermatol Venereol. 2009 Feb. 23(2):237-8. [QxMD MEDLINE Link].
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Burkhart CG, Burkhart CN. Tazarotene gel for Darier's disease. J Am Acad Dermatol. 1998 Jun. 38(6 Pt 1):1001-2. [QxMD MEDLINE Link].
Oster-Schmidt C. The treatment of Darier's disease with topical tazarotene. Br J Dermatol. 1999 Sep. 141(3):603-4. [QxMD MEDLINE Link].
Dicken CH, Bauer EA, Hazen PG, Krueger GG, Marks JG Jr, McGuire JS, et al. Isotretinoin treatment of Darier's disease. J Am Acad Dermatol. 1982 Apr. 6(4 Pt 2 Suppl):721-6. [QxMD MEDLINE Link].
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Yoon TY, Kim JW, Kim MK. Successful treatment of Darier disease with topical 5-fluorouracil. Br J Dermatol. 2006 Jun. 154(6):1210-2. [QxMD MEDLINE Link].
Abe M, Yasuda M, Yokoyama Y, Ishikawa O. Successful treatment of combination therapy with tacalcitol lotion associated with sunscreen for localized Darier's disease. J Dermatol. 2010 Aug. 37(8):718-21. [QxMD MEDLINE Link].
Pérez-Carmona L, Fleta-Asín B, Moreno-García-Del-Real C, Jaén-Olasolo P. Successful treatment of Darier's disease with topical pimecrolimus. Eur J Dermatol. 2011 Mar-Apr. 21(2):301-2. [QxMD MEDLINE Link].
Millán-Parrilla F, Rodrigo-Nicolás B, Molés-Poveda P, Armengot-Carbó M, Quecedo-Estébanez E, Gimeno-Carpio E. Improvement of Darier disease with diclofenac sodium 3% gel. J Am Acad Dermatol. 2014 Apr. 70(4):e89-90. [QxMD MEDLINE Link].
Kontochristopoulos G, Katsavou AN, Kalogirou O, Agelidis S, Zakopoulou N. Letter: Botulinum toxin type A: an alternative symptomatic management of Darier's disease. Dermatol Surg. 2007 Jul. 33(7):882-3. [QxMD MEDLINE Link].
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Zamiri M, Munro CS. Successful treatment with oral alitretinoin in women of childbearing potential with Darier's disease. Br J Dermatol. 2013 Sep. 169(3):709-10. [QxMD MEDLINE Link].
Farb RM, Lazarus GS, Chiaramonti A, Goldsmith LA, Gilgor RS, Balakrishnan CV. The effect of 13-cis retinoic acid on epidermal lysosomal hydrolase activity in Darier's disease and pityriasis rubra pilaris. J Invest Dermatol. 1980 Aug. 75(2):133-5. [QxMD MEDLINE Link].
Veit JGS, De Glas V, Balau B, Liu H, Bourlond F, Paller AS, et al. Characterization of CYP26B1-Selective Inhibitor, DX314, as a Potential Therapeutic for Keratinization Disorders. J Invest Dermatol. 2020 Jun 4. [QxMD MEDLINE Link].
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Kamijo M, Nishiyama C, Takagi A, Nakano N, Hara M, Ikeda S, et al. Cyclooxygenase-2 inhibition restores ultraviolet B-induced downregulation of ATP2A2/SERCA2 in keratinocytes: possible therapeutic approach of cyclooxygenase-2 inhibition for treatment of Darier disease. Br J Dermatol. 2012 May. 166(5):1017-22. [QxMD MEDLINE Link].
Wheeland RG, Gilmore WA. The surgical treatment of hypertrophic Darier's disease. J Dermatol Surg Oncol. 1985 Apr. 11(4):420-3. [QxMD MEDLINE Link].
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Brown VL, Kelly SE, Burge SM, Walker NP. Extensive recalcitrant Darier disease successfully treated with laser ablation. Br J Dermatol. 2010 Jan. 162(1):227-9. [QxMD MEDLINE Link].
McElroy JA, Mehregan DA, Roenigk RK. Carbon dioxide laser vaporization of recalcitrant symptomatic plaques of Hailey-Hailey disease and Darier's disease. J Am Acad Dermatol. 1990 Nov. 23(5 Pt 1):893-7. [QxMD MEDLINE Link].
Minsue Chen T, Wanitphakdeedecha R, Nguyen TH. Carbon dioxide laser ablation and adjunctive destruction for Darier-White disease (keratosis follicularis). Dermatol Surg. 2008 Oct. 34(10):1431-4. [QxMD MEDLINE Link].
Beier C, Kaufmann R. Efficacy of erbium:YAG laser ablation in Darier disease and Hailey-Hailey disease. Arch Dermatol. 1999 Apr. 135(4):423-7. [QxMD MEDLINE Link].
Roos S, Karsai S, Ockenfel HM, Raulin C. Successful treatment of Darier disease with the flashlamp-pumped pulsed-dye laser. Arch Dermatol. 2008 Aug. 144(8):1073-5. [QxMD MEDLINE Link].
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Krakowski AC, Nguyen TA, Eichenfield LF. Treatment of segmental keratosis follicularis (Darier disease) using ablative fractional laser resurfacing. Dermatol Surg. 2015 Apr. 41 (4):516-8. [QxMD MEDLINE Link].
Minsue Chen T, Wanitphakdeedecha R, Nguyen TH. Carbon dioxide laser ablation and adjunctive destruction for Darier-White disease (keratosis follicularis). Dermatol Surg. 2008 Oct. 34(10):1431-4. [QxMD MEDLINE Link].
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Media Gallery

Typical distribution of keratotic papules in the seborrheic regions. Courtesy of Susan Mallory, MD, Director of Pediatric Dermatology, Washington University School of Medicine.
Longitudinal ridges, red and white lines, and V-shaped nicks. Courtesy of Susan Mallory, MD, Director of Pediatric Dermatology, Washington University School of Medicine.
Acantholysis and dyskeratosis (abnormal keratinization) are the 2 main features of Darier disease. Loss of epidermal adhesion with acantholysis frequently results in the formation of suprabasal clefts (lacunae).

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Author

Pui-Yan Kwok, MD, PhD Henry Bachrach Distinguished Professor, Department of Dermatology and Cardiovascular Research Institute, University of California, San Francisco, School of Medicine

Pui-Yan Kwok, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American Society of Human Genetics, Dermatology Foundation, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Jillian Wong Millsop, MD, MS Resident Physician, Department of Dermatology, University of California, Davis, School of Medicine

Jillian Wong Millsop, MD, MS is a member of the following medical societies: American College of Physicians, American Medical Association, American Medical Women's Association

Disclosure: Nothing to disclose.

Tina Bhutani, MD Clinical Research Fellow, Department of Dermatology, University of California School of Medicine, San Francisco

Tina Bhutani, MD is a member of the following medical societies: American Medical Association, American Medical Women's Association, American Association of Physicians of Indian Origin, National Psoriasis Foundation, Indian Medical Association

Disclosure: Nothing to disclose.

Wilson Liao, MD Associate Professor, Department of Dermatology, University of California, San Francisco, School of Medicine

Wilson Liao, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology, National Psoriasis Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steven R Feldman, MD, PhD Professor, Departments of Dermatology, Pathology and Public Health Sciences, and Molecular Medicine and Translational Science, Wake Forest Baptist Health; Director, Center for Dermatology Research, Director of Industry Relations, Department of Dermatology, Wake Forest University School of Medicine

Steven R Feldman, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, North Carolina Medical Society, Society for Investigative Dermatology

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbvie for consulting; Received honoraria from Galderma for speaking and teaching; Received consulting fee from Lilly for consulting; Received ownership interest from www.DrScore.com for management position; Received ownership interest from Causa Reseasrch for management position; Received grant/research funds from Janssen for consulting; Received honoraria from Pfizer for speaking and teaching; Received consulting fee from No.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Arash Taheri, MD Research Fellow, Center for Dermatology Research, Department of Dermatology, Wake Forest University School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Sarah Fitzmaurice, MD, Jillian Wong Millsop, MD, MS, Tina Bhutani, MD, and Wilson Liao, MD, to the development and writing of this article.

Sections Keratosis Follicularis (Darier Disease)
Overview
Presentation
DDx
Workup
Treatment
Medication
Questions & Answers
Media Gallery
References

Keratosis Follicularis (Darier Disease) Clinical Presentation

History

Physical Examination

Complications

References

Tables

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