Dermatology

Rosacea Fulminans (Pyoderma Faciale)

The condition was initially termed pyoderma faciale. The term rosacea fulminans was recommended by Plewig in 1992 after determining that the condition represented the most severe variant of rosacea.

Are You Confident of the Diagnosis?

  • What you should be alert for in the history

Rosacea fulminans may be preceded by seborrhea and may occur more frequently in patients with a history of "flushing and blushing."

  • Characteristic findings on physical examination

There is rapid onset of intense reddish or cyanotic erythema, combined with papulopustules and fluctuant draining nodules and sinus tracts on the face (Figure 1). It is usually localized on the chin, cheeks, and forehead. Firm edema of the face and scarring can occur. Constitutional symptoms are not typically seen.

Figure 1.

Pyoderma faciale in a young woman.

  • Expected results of diagnostic studies

Histology shows deep and superficial inflammatory infiltrate around hair follicles and sebaceous glands; biopsies are not typically recommended if the clinical exam and history is suggestive. There are no specific lab abnormalities. Cultures of drainage are usually negative for pathogenic bacteria.

  • Diagnosis confirmation

Differentiate from acne based on absence of comedones, rapid onset, fulminant course, and absence of acne on chest and back.

Who is at Risk for Developing this Disease?

  • Women in their 20s to 40s, who may have a history of recent onset seborrhea and flushing and blushing. Very rarely can affect men.

What is the Cause of the Disease?

  • Etiology and Pathophysiology

Unknown. There are multiple case reports of associations with pregnancy and also oral contraceptive use. Inflammatory bowel disease has also been associated on multiple occasions. Single cases have been associated with erythema nodosum, infection with Helicobacter pylori, thyroid and liver disease. Pegylated interferon alpha-2b and ribavirin as well as high doses of vitamin B6 and B12 have also been implicated as inciting this condition.

Systemic Implications and Complications

Ocular involvement including keratitis, conjunctivitis, and ocular perforation have been observed.

Treatment Options

Severe scarring may result; treatment should be aggressive to avoid further scarring.

Medical options

Topicals

  • Topical mid to high potency steroids once to twice daily (e.g., fluocinonide 0.05% cream, desoximetasone 0.25% cream, clobetasol 0.05% cream)

Systemics

  • Oral steroids beginning at 1 mg/kg daily

  • Oral isotretinoin beginning at 10-20 mg daily and increasing to 0.5-1 mg/kg/day

If oral steroids or oral isotretinoin are contraindicated or not tolerated, dapsone 100 mg daily or an oral antibiotic (tetracyclines such as minocycline 100 mg twice a day or macrolides such as erythromycin 500 mg four times daily or azithromycin) could be tried but with limited expectations of improvement in comparison to the above choices.

Optimal Therapeutic Approach for this Disease

Initiate therapy with oral corticosteroids for 2-4 weeks and then initiate isotretinoin at a low dose (10 mg daily); topical steroids can be used for the first 2 weeks as an adjuvant therapy. Steroids should be begun first to avoid explosive worsening of disease with isotretinoin initiation

Once the acute inflammatory stage is controlled, isotretinoin can be slowly increased to 0.5-1 g/kg/day and corticosteroids can be tapered. A full course of isotretinoin (120-150 mg/kg) should be completed.

Patient Management

Monitor for side effects of therapy. Follow patient closely during the first few weeks of therapy until the acute inflammatory stage is controlled, then monthly for isotretinoin therapy. Educate the patient on the risk of scarring.

Unusual Clinical Scenarios to Consider in Patient Management

Pregnant and postpartum patients are a therapeutic challenge. Under consultation with the patient’s obstetrician, oral steroids or antibiotics (erythromycin or amoxicillin) may be viable options. Barring these, topical antibiotics, such as erythromycin and clindamycin, may be used with limited expectation of improvement.

What is the Evidence?

Plewig, G, Jansen, T, Kligman, AM. "Pyodermafaciale. A review and report of 20 additional cases: is it rosacea?". Arch Dermatol.. vol. 128. 1992 Dec. pp. 1611-7.

(A summary and report of cases of pyoderma faciale. This is an excellent reference summarizing prior case reports and their findings. The clinical and histology photographs are clear.)

Bettoli, V, Mantovani, L, Boccia, S, Virgili, A. "Rosacea fulminans related to pegylated interferon alpha-2b and ribavirin therapy". Acta Derm Venereol.. vol. 86. 2006. pp. 258-9.

(The second reported case of rosacea fulminans caused by pegylated interferon alpha-2b and ribavirin therapy.)

Bormann, G, Gaber, G, Fischer, M, Marsch, WC. "Dapsone in rosacea fulminans". J Eur Acad Dermatol Venereol.. vol. 15. 2001 Sep. pp. 465-7.

(Dapsone as a successful treatment for rosacea fulminans.)

de Morais e Silva, FA, Bonassi, M, Steiner, D, da Cunha, TV. "Rosacea fulminans in pregnancy with ocular perforation". J Dtsch Dermatol Ges.. vol. 9. 2011 Jul. pp. 542-3.

(A case report of ocular perforation in a pregnant woman with rosacea fulminans.)

Fuentelsaz, V, Ara, M, Corredera, C, Lezcano, V, Juberias, P, Carapeto, FJ. "Rosacea fulminans in pregnancy: successful treatment with azithromycin". Clin Exp Dermatol.. vol. 36. 2011 Aug. pp. 674-6.

(A case report of rosacea fulminans in pregnancy treated with azithromycin. There is a table summarizing other cases of rosacea fulminans in pregnancy and treatments that were used.)

Ribeiro, LB, Ramos-e-Silva, M. "Rosacea fulminans". Cutis.. vol. 92. 2013 Jul. pp. 29-32.

(An excellent review of rosacea fulminans including the history, clinical presentation, and treatment options.)
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