June 2015 Dermatology Clinic
A man, aged 56 years, presents with a 3-month history of a rash on his face, scalp, and neck. The rash is slightly pruritic, and its cosmetic appearance is very bothersome to the patient. The patient reports that the rash was worsened with sun exposure. His primary care physician thought that the patient had acne and had treated the patient with topical tretinoin cream with no improvement. The physical examination was significant for numerous small erythematous papules on the face, scalp, and neck. The remainder of the skin examination was within normal limits.
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Granulomatous rosacea is a histological variant of rosacea distinguished by the presence of epithelioid cell granulomas in the dermis.1 First described in 1970 by Mullanax and Kierland, granulomatous rosacea presents as a papulonodular eruption distributed predominantly over the malar, perioral, and periocular surfaces of the face.2,3 The papules appear yellowish-brown to red in color and may be mistaken for acne.
Unlike classical rosacea, in which facial erythema is a typical feature, granulomatous rosacea may not be accompanied by marked redness.3,4 Although classified as a distinct variant, granulomatous rosacea may be found in association with any of the four clinical subtypes of rosacea: erythematotelangiectatic, papulopustular, phymatous, and ocular.4
Granulomatous rosacea has been reported primarily in middle-aged women and is believed to represent 10% of all cases of rosacea.2,3
The cause of granulomatous rosacea is unknown. The frequent finding of Demodex folliculorum within the granulomas suggests that these mites may have a role in pathogenesis by delayed-type hypersensitivity.5,6 Another common finding on histopathology is solar elastotic changes.4 Recently, granulomatous rosacea has been linked to infection with Helicobacter pylori, with several cases of resolution with clarithromycin, metronidazole, and pantoprazole.7,8 One study suggested that increased expression of matrix metalloproteinases in the dermis may participate in the formation of granulomas.9 Granulomatous rosacea is known to be triggered by certain environmental factors, including spicy foods, alcohol consumption, emotional stress, sun exposure, and vasodilator medications.1
The diagnosis of granulomatous rosacea is made by clinical features and histopathology. Typical features include non-caseating epithelioid granulomas, multinucleated giant cells, and lymphocytic infiltrates that are concentrated around pilosebaceous units in the superficial and deep dermis.1,4 The differential diagnosis includes perioral dermatitis that is characterized by lesions in the same stage of development and a distribution primarily around the mouth.1 Granulomatous periorificial dermatitis, the histology of which closely resembles that seen in granulomatous rosacea, is considered by some to be a variant seen in African American children.10 Lupus miliaris disseminatus faciei resembles granulomatous rosacea in clinical presentation but lupus miliaris disseminatus faciei exhibits caseation necrosis on histopathology and spontaneously resolves within 12 to 18 months.5,11 Sarcoidosis and cutaneous B-cell neoplasms should also be considered.12,13
Granulomatous rosacea has a chronic relapsing course and management can be very difficult.14 Patients should be instructed to avoid known exacerbating factors, which as mentioned above include spicy foods, alcohol consumption, emotional stress, sun exposure, and vasodilator medications. Systemic antibiotics such as erythromycin, minocycline, and doxycycline are the first-line in treatment of the condition and may be accompanied by topical metronidazole gel. For refractory cases, isotretinoin may be administered regularly at a low dose.3 There are numerous reports describing the use of pimecrolimus 1% cream to treat granulomatous rosacea that is unresponsive to antibiotics.1 In the event of unresponsiveness to medication, intense pulsed light may also be considered.15
The patient in this case began treatment with oral minocycline twice daily with significant improvement in the lesions at his 3-month follow-up appointment.
Lucette Liddell, BA, is a medical student and Maura Holcomb, MD, is a dermatology resident at Baylor College of Medicine in Houston.
- Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. 3rd ed. Philadelphia, Pa.: Elsevier Saunders; 2012.
- Mullanax MG, Kierland RR. Granulomatous rosacea. Arch Dermatol. 1970;101(2):206-211.
- Khokhar O, Khachemoune A. A case of granulomatous rosacea: Sorting granulomatous rosacea from other granulomatous diseases that affect the face. Dermatol Online J. 2004;10(1):6. Available at escholarship.org/uc/item/9773c559
- Sánchez JL, Berlingeri-Ramos AC, Dueño DV. Granulomatous rosacea. Am J Dermatopathol. 2008;30(1):6-9.
- Helm KF, Menz J, Gibson LE, Dicken CH. A clinical and histopathologic study of granulomatous rosacea. J Am Acad Dermatol. 1991;25 (6 Pt 1):1038-1043.
- Amichai B, Grunwald MH, Avinoach I, Halevy S. Granulomatous rosacea associated with Demodex folliculorum. Int J Dermatol. 1992;31(10):718-719.
- Mayr-Kanhäuser S, Kränke B, Kaddu S, Müllegger RR. Resolution of granulomatous rosacea after eradication of Helicobacter pylori with clarithromycin, metronidazole, and pantoprazole. Eur J Gastroenterol Hepatol. 2001;13(11):1379-1383.
- Millikan L. The proposed inflammatory pathophysiology of rosacea: Implications for treatment. Skinmed. 2003;2(1):43-47.
- Jang YH, Sim JH, Kang HY, et al. Immunohistochemical expression of matrix metalloproteinases in the granulomatous rosacea compared with the non-granulomatous rosacea. J Eur Acad Dermatol Venereol. 2011;25(5):544-548.
- Lucas CR, Korman NJ, Gilliam AC. Granulomatous periorificial dermatitis: A variant of granulomatous rosacea in children? J Cutan Med Surg. 2009;13(2):115-118. Available at cms.sagepub.com/content/13/2/115.long
- Shitara A. Lupus miliaris disseminatus faciei. Int J Dermatol. 1984;23(8):542-544.
- Di Meo N, Stinco G, Trevisan G. Cutaneous B-cell chronic lymphocytic leukaemia resembling a granulomatous rosacea. Dermatol Online J. 2013;19(10):20033. Available at escholarship.org/uc/item/8vm2m5gp
- Barzilai A, Feuerman H, Quaglino P, et al. Cutaneous B-cell neoplasms mimicking granulomatous rosacea or rhinophyma. Arch Dermatol. 2012;148(7):824-831. Available at archderm.jamanetwork.com/article.aspx?articleid=1148705
- Rallis E, Korfitis C. Isotretinoin for the treatment of granulomatous rosacea: Case report and review of the literature. J Cutan Med Surg. 2012;16(6):438-441.
- Lane JE, Khachemoune A. Use of intense pulsed light to treat refractory granulomatous rosacea. Dermatol Surg. 2010;36(4):571-573.
All electronic documents accessed on June 4, 2015.