Dermatology Online Journal

A red plaque on the cheek
Taylor DeFelice MD MPH¹, Maxwell A Fung MD², Jashin J Wu MD³
Dermatology Online Journal 16 (3): 7

1. Department of Dermatology, New York University School of Medicine, New York, New York
2. Departments of Dermatology and Pathology, University of California Davis, Sacramento, California
3. Department of Dermatology, Kaiser Permanente Los Angeles Medical Center, Los Angeles, California. jashinwu@hotmail.com

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Report of a case

Figure 1

A 25-year-old woman presented complaining of a red, tender, and pruritic rash on her right cheek, which developed rapidly over one month. The patient denied trauma or exposures to new medications, cosmetics, or other products. She denied cough, fever, chills, or other systemic symptoms. Her past medical history included an episode of bloody diarrhea diagnosed by sigmoidoscopy and biopsy as proctitis. The rash was unrelated to sun exposure and was confined to her cheek. Physical examination revealed a 3.5 x 4.0 cm erythematous, circular granulomatous plaque on the right cheek (Figure 1). A 4 mm punch biopsy was taken of the lesion at the first visit (Figures 2 and 3). A diagnosis of pyoderma faciale was made.

Figure 2	Figure 3

Microscopic findings and clinical course

Biopsy from a plaque on the right cheek showed perifollicular neutrophilic granulomatous dermatitis with plasma cells and collections of neutrophils within plugged, dilated, and distorted folliculo-sebaceous units. Special stains were negative for bacteria, mycobacteria, and fungi. No foreign material was identified under polarized light.

The patient was treated with erythromycin-benzoyl peroxide 3/5 percent topical gel every morning, metronidazole 0.75 percent topical gel every evening, and minocycline 100 mg orally twice a day. On return visit two months later, the plaque showed improvement; 5-10 mm red granulomatous papules within a 4 cm pink patch remained. Treatment was continued as above for an additional month and on follow up at that time she had completely cleared. After clearing, she was continued on minocycline 50 mg orally twice a day for one month. Seven months after stopping the minocycline the patient had a recurrence on the opposite cheek. We restarted her on the original regimen three months ago but she hasn’t returned for follow-up yet. Prior to the initial outbreak the patient had not had signs of rosacea, nor was she using topical steroids.

Discussion

Pyoderma faciale is a relatively rare dermatosis originally described in 1940 by O’Leary and Kierland [1]. It occurs most commonly in women 20-40 years old and has been considered a severe form of rosacea by Plewig et al. [2], who suggested the term rosacea fulminans in 1992. Localized eruptions of the central face often appear rapidly and involve papulo-pustular lesions coalescing into nodules and plaques, often exhibiting edema and confluent draining sinuses on a background of intense reddish to cyanotic erythema. Seborrhea may precede onset and many patients are flushers and blushers. Included in the clinical differential are acute lupus erythematous and angioedema given the livid erythema and edema. Sarcoidosis, granuloma faciale, and acne vulgaris must also be considered. However, the condition can be differentiated from severe acne by its fulminating course and the absence of comedones or lesions on the chest or back.

Etiological factors are still unknown. Hormonal and immunologic factors have been suggested given the predominance in women. The condition has been described in association with pregnancy [3], thyroid disease [4], and intake of high doses of B vitamins [5]. There are two patients who were on pegylated interferon and ribavirin therapy for hepatitis C [6] and one case with concomitant erythema nodosum [7]. Interestingly, several cases have been reported in patients with inflammatory bowel disease [4, 8], which also may have played a role in our patient.

Histopathologic features include a neutrophilic and granulomatous dermatitis with folliculo-sebaceous involvement. Histiocytes are present often in early lesions. Additional features include neutrophilic abscess formation, edema, and extension into the subcutis. Clinical correlation is required to address the histological differential diagnosis of granulomatous rosacea (includes lupus miliaris disseminatum facei), granulomatous perioral dermatitis, and acne fulminans.

With early diagnosis and intervention scarring may be avoided. Several authors have hypothesized that if adequate treatment is not initiated promptly, the localized form may spread widely and there is slower recovery with severe scarring [4, 9]. The condition is most commonly treated with a brief course of systemic and/or high-potency topical steroids, followed by oral isotretinoin for 3-5 months [2], although some cases have responded to dapsone, tetracycline, or 0.75 percent metronidazole topical cream [3, 6]. Our case illustrates good response with an oral antibiotic and topical acne therapy. Recurrence is uncommon.

References

1. O’Leary, PA, Kierland RR. Pyoderma Faciale. Arch Derm Syphilol. 1940; 41: 451-62.

2. Plewig G, Jansen T, Kligman AM: Pyoderma faciale. A review and report of 20 additional cases: is it rosacea? Arch.Dermatol. 1992 Dec;128(12):1611-7. [PubMed]

3. Ferahbas A, Utas S, Mistik S, Uksal U, Peker D: Rosacea fulminans in pregnancy: case report and review of the literature. Am.J.Clin.Dermatol. 2006;7(2):141-4. [PubMed]

4. Massa MC, Su WP: Pyoderma faciale: a clinical study of twenty-nine patients. J.Am.Acad.Dermatol. 1982 Jan;6(1):84-91. [PubMed]

5. Jansen T, Romiti R, Kreuter A, Altmeyer P: Rosacea fulminans triggered by high-dose vitamins B6 and B12. J.Eur.Acad.Dermatol.Venereol. 2001 Sep;15(5):484-5. [PubMed]

6. Bettoli V, Mantovani L, Boccia S, Virgili A: Rosacea fulminans related to pegylated interferon alpha-2b and ribavirin therapy. Acta Derm.Venereol. 2006;86(3):258-9. [PubMed]

7. Akhyani M, Daneshpazhooh M, Ghandi N: The association of pyoderma faciale and erythema nodosum. Clin.Exp.Dermatol. 2007 May;32(3):275-7. [PubMed]

8. Romiti R, Jansen T, Heldwein W, Plewig G: Rosacea fulminans in a patient with Crohn's disease: a case report and review of the literature. Acta Derm.Venereol. 2000 Mar-Apr;80(2):127-9. [PubMed]

9. Fender AB, Ignatovich Y, Mercurio MG. Pyoderma faciale. Cutis. 2008 Jun;81(6):488-90. [PubMed]

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