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Rosacea: Pathogenesis, clinical features, and diagnosis

Author
Mark V Dahl, MD
Section Editor
Robert P Dellavalle, MD, PhD, MSPH
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Rosacea is a common, chronic skin disorder that presents with a variety of clinical manifestations primarily localized on the central face [1,2]. The disorder is divided into four main subtypes: erythematotelangiectatic, papulopustular, phymatous, and ocular rosacea.

Persistent centrofacial redness is a characteristic feature of the erythematotelangiectatic form of rosacea, while papulopustular disease presents with acne-like inflammatory papules and pustules in a similar distribution. The less common phymatous form of rosacea may demonstrate marked skin thickening and distortion of facial contours. Ocular rosacea, which often presents with eye redness, pruritus, or irritation and hordeola (styes), can occur in the presence or absence of cutaneous disease. (See "Ocular rosacea".)

The pathogenesis, clinical manifestations, and diagnosis of rosacea will be reviewed here. The treatment of rosacea is discussed separately. (See "Management of rosacea".)

EPIDEMIOLOGY

Rosacea is a common disorder that is most frequently observed in fair-skinned individuals (skin phototypes I and II) (table 1). People of Celtic and Northern European origin appear to have the greatest risk for this disorder [3,4]. The prevalence of rosacea is difficult to assess due to its variable clinical manifestations and the wide variety of skin disorders that exhibit similar clinical features (see 'Differential diagnosis' below). Estimates of the prevalence of rosacea in fair-skinned populations range from 1 to 10 percent [3,5,6].

Rosacea occurs in people with darker skin complexions, but is less frequently diagnosed in such populations (picture 1A-B) [7-12]. It is unknown whether factors such as masking of facial redness by abundant skin pigment, protective effects of melanin against ultraviolet radiation (an exacerbating factor for rosacea), or genetic differences in susceptibility to rosacea contribute to the lower rate of diagnosis in people with dark skin.

                             

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Literature review current through: Nov 2016. | This topic last updated: Thu Feb 04 00:00:00 GMT+00:00 2016.
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References
Top
  1. Webster GF. Rosacea. Med Clin North Am 2009; 93:1183.
  2. Powell FC. Clinical practice. Rosacea. N Engl J Med 2005; 352:793.
  3. Elewski BE, Draelos Z, Dréno B, et al. Rosacea - global diversity and optimized outcome: proposed international consensus from the Rosacea International Expert Group. J Eur Acad Dermatol Venereol 2011; 25:188.
  4. van Zuuren EJ, Kramer S, Carter B, et al. Interventions for rosacea. Cochrane Database Syst Rev 2011; :CD003262.
  5. Berg M. Epidemiological studies of the influence of sunlight on the skin. Photodermatol 1989; 6:80.
  6. McAleer MA, Fitzpatrick P, Powell FC. Papulopustular rosacea: prevalence and relationship to photodamage. J Am Acad Dermatol 2010; 63:33.
  7. Khaled A, Hammami H, Zeglaoui F, et al. Rosacea: 244 Tunisian cases. Tunis Med 2010; 88:597.
  8. Rosen T, Stone MS. Acne rosacea in blacks. J Am Acad Dermatol 1987; 17:70.
  9. Browning DJ, Rosenwasser G, Lugo M. Ocular rosacea in blacks. Am J Ophthalmol 1986; 101:441.
  10. Bae YI, Yun SJ, Lee JB, et al. Clinical evaluation of 168 korean patients with rosacea: the sun exposure correlates with the erythematotelangiectatic subtype. Ann Dermatol 2009; 21:243.
  11. Alexis AF. Rosacea in patients with skin of color: uncommon but not rare. Cutis 2010; 86:60.
  12. Sibenge S, Gawkrodger DJ. Rosacea: a study of clinical patterns, blood flow, and the role of Demodex folliculorum. J Am Acad Dermatol 1992; 26:590.
  13. Abram K, Silm H, Maaroos HI, Oona M. Risk factors associated with rosacea. J Eur Acad Dermatol Venereol 2010; 24:565.
  14. Chamaillard M, Mortemousque B, Boralevi F, et al. Cutaneous and ocular signs of childhood rosacea. Arch Dermatol 2008; 144:167.
  15. Kroshinsky D, Glick SA. Pediatric rosacea. Dermatol Ther 2006; 19:196.
  16. Lacz NL, Schwartz RA. Rosacea in the pediatric population. Cutis 2004; 74:99.
  17. Mc Aleer MA, Lacey N, Powell FC. The pathophysiology of rosacea. G Ital Dermatol Venereol 2009; 144:663.
  18. Dahl MV. Pathogenesis of rosacea. Adv Dermatol 2001; 17:29.
  19. Yamasaki K, Gallo RL. The molecular pathology of rosacea. J Dermatol Sci 2009; 55:77.
  20. Yamasaki K, Di Nardo A, Bardan A, et al. Increased serine protease activity and cathelicidin promotes skin inflammation in rosacea. Nat Med 2007; 13:975.
  21. Yamasaki K, Kanada K, Macleod DT, et al. TLR2 expression is increased in rosacea and stimulates enhanced serine protease production by keratinocytes. J Invest Dermatol 2011; 131:688.
  22. Zhao YE, Wu LP, Peng Y, Cheng H. Retrospective analysis of the association between Demodex infestation and rosacea. Arch Dermatol 2010; 146:896.
  23. Bonnar E, Eustace P, Powell FC. The Demodex mite population in rosacea. J Am Acad Dermatol 1993; 28:443.
  24. Forton F, Seys B. Density of Demodex folliculorum in rosacea: a case-control study using standardized skin-surface biopsy. Br J Dermatol 1993; 128:650.
  25. Erbağci Z, Ozgöztaşi O. The significance of Demodex folliculorum density in rosacea. Int J Dermatol 1998; 37:421.
  26. Lacey N, Delaney S, Kavanagh K, Powell FC. Mite-related bacterial antigens stimulate inflammatory cells in rosacea. Br J Dermatol 2007; 157:474.
  27. Tan J, Berg M. Rosacea: current state of epidemiology. J Am Acad Dermatol 2013; 69:S27.
  28. Whitfeld M, Gunasingam N, Leow LJ, et al. Staphylococcus epidermidis: a possible role in the pustules of rosacea. J Am Acad Dermatol 2011; 64:49.
  29. Dahl MV, Ross AJ, Schlievert PM. Temperature regulates bacterial protein production: possible role in rosacea. J Am Acad Dermatol 2004; 50:266.
  30. Fernandez-Obregon A, Patton DL. The role of Chlamydia pneumoniae in the etiology of acne rosacea: response to the use of oral azithromycin. Cutis 2007; 79:163.
  31. Parodi A, Paolino S, Greco A, et al. Small intestinal bacterial overgrowth in rosacea: clinical effectiveness of its eradication. Clin Gastroenterol Hepatol 2008; 6:759.
  32. Brauchle M, Funk JO, Kind P, Werner S. Ultraviolet B and H2O2 are potent inducers of vascular endothelial growth factor expression in cultured keratinocytes. J Biol Chem 1996; 271:21793.
  33. Bielenberg DR, Bucana CD, Sanchez R, et al. Molecular regulation of UVB-induced cutaneous angiogenesis. J Invest Dermatol 1998; 111:864.
  34. Longuet-Perret I, Schmitt D, Viac J. Tumour necrosis factor-alpha is involved in the contrasting effects of ultraviolet B and ultraviolet A1 radiation on the release by normal human keratinocytes of vascular permeability factor. Br J Dermatol 1998; 138:221.
  35. Crawford GH, Pelle MT, James WD. Rosacea: I. Etiology, pathogenesis, and subtype classification. J Am Acad Dermatol 2004; 51:327.
  36. Guzman-Sanchez DA, Ishiuji Y, Patel T, et al. Enhanced skin blood flow and sensitivity to noxious heat stimuli in papulopustular rosacea. J Am Acad Dermatol 2007; 57:800.
  37. Aubdool AA, Brain SD. Neurovascular aspects of skin neurogenic inflammation. J Investig Dermatol Symp Proc 2011; 15:33.
  38. Spoendlin J, Voegel JJ, Jick SS, Meier CR. Migraine, triptans, and the risk of developing rosacea: a population-based study within the United Kingdom. J Am Acad Dermatol 2013; 69:399.
  39. Berg M, Lidén S. An epidemiological study of rosacea. Acta Derm Venereol 1989; 69:419.
  40. Berg M, Lidén S. Postmenopausal female rosacea patients are more disposed to react with migraine. Dermatology 1996; 193:73.
  41. Ramelet AA. Rosacea: a reaction pattern associated with ocular lesions and migraine? Arch Dermatol 1994; 130:1448.
  42. Tan SG, Cunliffe WJ. Rosacea and migraine. Br Med J 1976; 1:21.
  43. Wilkin J, Dahl M, Detmar M, et al. Standard classification of rosacea: Report of the National Rosacea Society Expert Committee on the Classification and Staging of Rosacea. J Am Acad Dermatol 2002; 46:584.
  44. Tan J, Blume-Peytavi U, Ortonne JP, et al. An observational cross-sectional survey of rosacea: clinical associations and progression between subtypes. Br J Dermatol 2013; 169:555.
  45. Levin J, Miller R. A Guide to the Ingredients and Potential Benefits of Over-the-Counter Cleansers and Moisturizers for Rosacea Patients. J Clin Aesthet Dermatol 2011; 4:31.
  46. Ghanem VC, Mehra N, Wong S, Mannis MJ. The prevalence of ocular signs in acne rosacea: comparing patients from ophthalmology and dermatology clinics. Cornea 2003; 22:230.
  47. Nazir SA, Murphy S, Siatkowski RM, et al. Ocular rosacea in childhood. Am J Ophthalmol 2004; 137:138.
  48. Drolet B, Paller AS. Childhood rosacea. Pediatr Dermatol 1992; 9:22.
  49. Scharschmidt TC, Yost JM, Truong SV, et al. Neurogenic rosacea: a distinct clinical subtype requiring a modified approach to treatment. Arch Dermatol 2011; 147:123.
  50. Schram AM, James WD. Neurogenic rosacea treated with endoscopic thoracic sympathectomy. Arch Dermatol 2012; 148:270.
  51. Hua TC, Chung PI, Chen YJ, et al. Cardiovascular comorbidities in patients with rosacea: A nationwide case-control study from Taiwan. J Am Acad Dermatol 2015; 73:249.
  52. Rainer BM, Fischer AH, Felipe da Silva DM. Rosacea is associated with chronic systemic diseases in a skin severity-dependent manner: Results of a case-control study. J Am Acad Dermatol 2015.
  53. Li WQ, Zhang M, Danby FW, et al. Personal history of rosacea and risk of incident cancer among women in the US. Br J Cancer 2015; 113:520.
  54. Duman N, Ersoy Evans S, Atakan N. Rosacea and cardiovascular risk factors: a case control study. J Eur Acad Dermatol Venereol 2014; 28:1165.
  55. Egeberg A, Hansen PR, Gislason GH, Thyssen JP. Association of Rosacea With Risk for Glioma in a Danish Nationwide Cohort Study. JAMA Dermatol 2016; 152:541.
  56. Egeberg A, Hansen PR, Gislason GH, Thyssen JP. Clustering of autoimmune diseases in patients with rosacea. J Am Acad Dermatol 2016; 74:667.
  57. Rainer BM, Fischer AH, Luz Felipe da Silva D, et al. Rosacea is associated with chronic systemic diseases in a skin severity-dependent manner: results of a case-control study. J Am Acad Dermatol 2015; 73:604.
  58. Marks R, Harcourt-Webster JN. Histopathology of rosacea. Arch Dermatol 1969; 100:683.
  59. Tope WD, Sangueza OP. Rhinophyma's fibrous variant. Histopathology and immunohistochemistry. Am J Dermatopathol 1994; 16:307.
  60. Aloi F, Tomasini C, Soro E, Pippione M. The clinicopathologic spectrum of rhinophyma. J Am Acad Dermatol 2000; 42:468.
  61. Ioffreda MD. Inflammatory diseases of the hair follicles, sweat glands, and cartilage. In: Lever's Histopathology of the Skin, 9th ed, Elder D, Elenitsas R, Johnson BL, et al.. (Eds), Lippincott Williams and Wilkins, Philadelphia 2005. p.469.
  62. Brown TT, Choi EY, Thomas DG, et al. Comparative analysis of rosacea and cutaneous lupus erythematosus: histopathologic features, T-cell subsets, and plasmacytoid dendritic cells. J Am Acad Dermatol 2014; 71:100.
  63. Hsu CK, Hsu MM, Lee JY. Demodicosis: a clinicopathological study. J Am Acad Dermatol 2009; 60:453.
  64. Baima B, Sticherling M. Demodicidosis revisited. Acta Derm Venereol 2002; 82:3.
  65. Karincaoglu Y, Miman O, Kalayci B, et al. A demodicosis case which responded to systemic ivermectin. Eur J Dermatol 2009; 19:189.
  66. Forstinger C, Kittler H, Binder M. Treatment of rosacea-like demodicidosis with oral ivermectin and topical permethrin cream. J Am Acad Dermatol 1999; 41:775.
  67. Aquilina C, Viraben R, Sire S. Ivermectin-responsive Demodex infestation during human immunodeficiency virus infection. A case report and literature review. Dermatology 2002; 205:394.
  68. Al-Mutairi N. Nosology and therapeutic options for lupus miliaris disseminatus faciei. J Dermatol 2011; 38:864.
  69. Esteves T, Faria A, Alves R, et al. Lupus miliaris disseminatus faciei: a case report. Dermatol Online J 2010; 16:10.
  70. van de Scheur MR, van der Waal RI, Starink TM. Lupus miliaris disseminatus faciei: a distinctive rosacea-like syndrome and not a granulomatous form of rosacea. Dermatology 2003; 206:120.
  71. Barzilai A, Feuerman H, Quaglino P, et al. Cutaneous B-cell neoplasms mimicking granulomatous rosacea or rhinophyma. Arch Dermatol 2012; 148:824.
  72. Lucas CR, Korman NJ, Gilliam AC. Granulomatous periorificial dermatitis: a variant of granulomatous rosacea in children? J Cutan Med Surg 2009; 13:115.
  73. Zalaudek I, Di Stefani A, Ferrara G, Argenziano G. Childhood granulomatous periorificial dermatitis: a controversial disease. J Dtsch Dermatol Ges 2005; 3:252.
  74. Knautz MA, Lesher JL Jr. Childhood granulomatous periorificial dermatitis. Pediatr Dermatol 1996; 13:131.
  75. Williams HC, Ashworth J, Pembroke AC, Breathnach SM. FACE--facial Afro-Caribbean childhood eruption. Clin Exp Dermatol 1990; 15:163.
  76. Cribier B, Lieber-Mbomeyo A, Lipsker D. [Clinical and histological study of a case of facial Afro-Caribbean childhood eruption (FACE)]. Ann Dermatol Venereol 2008; 135:663.
  77. Plewig G, Jansen T, Kligman AM. Pyoderma faciale. A review and report of 20 additional cases: is it rosacea? Arch Dermatol 1992; 128:1611.
  78. Ormond P, Rogers S. Case 3. Pyoderma faciale (PF) (rosacea fulminans). Clin Exp Dermatol 2003; 28:107.