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What Is Pyoderma Faciale?

Pyoderma Faciale Is a Rare and Severe Form of Acne in Females

Last updated: May 14, 2019

Article Summary

Pyoderma faciale, also known as rosacea fulminans, is a rare, severe form of acne that occurs exclusively in females. Symptoms are always limited to the face and include intense redness of the skin and a mixture of nodules, cysts, papules, pustules, and draining sinuses. Treatment usually consists of oral prednisolone (an oral steroid to reduce inflammation) and isotretinoin, often known by its brand name Accutane® and takes at least three months.

Definition

Pyoderma faciale, also known as rosacea fulminans, is a rare, severe form of acne occurring exclusively in females. Pyoderma faciale falls under the umbrella term nodulocystic acne, which is used to refer to any type of acne that features, but is not necessarily limited to, large, red, painful acne lesions called nodules and/or cysts. The disease presents numerous merging nodules and/or cysts on the face accompanied by intense inflammatory redness of the skin. 

Incidence

The condition is rare. A typical patient would be a woman in her twenties, but cases in females from 15 to 59 years of age have been reported.1-3Males never develop pyoderma faciale.

Causes

Nothing is known about the causes of the disease. Research fails to identify any patterns in the medical history, unusual laboratory findings, or hormonal abnormalities. Today, the disease remains as mysterious as it was when it was discovered almost 80 years ago. 

Symptoms

The onset of pyoderma faciale can be compared to a torrential rain that hits suddenly. The disease may emerge from the healthy skin in patients who have never had a problem with acne. In some patients, pyoderma faciale resolves spontaneously within a year, while in others it may persist for over a year and alternate between becoming more or less severe over that time.4 Consequently, some degree of scarring likely will occur, even with adequate treatment. 

Pyoderma Faciale Types of Lesions

Features

Pyoderma faciale is a form of nodulocystic acne that gives rise to a mixture of nodules, cysts, and draining sinuses (several cysts fused together under the skin). Whiteheads, blackheadspapules, and pustules may be present as well, but they are never a dominant feature. Lesions are accompanied by intense redness of the skin and, often, swelling. Symptoms are always limited to the face, particularly the cheeks, nose, chin, forehead, and temples. The central portion of the cheeks is almost always involved.3

Nodules and cysts: Nodules and cysts in pyoderma faciale are plentiful. Both types of lesions are over five millimeters in diameter and are tender and painful. The difference is that nodules are fibrous lesions that do not contain pus, whereas cysts contain "foul-smelling" pus-like material. Healing of both nodules and cysts can be extremely slow, often resulting in massive, disfiguring scars. 

Nodule and Cysts

Draining sinuses: Draining sinuses arise when two or more nodules/cysts merge together under the skin to form a large, inflammatory lesion. Draining sinuses are a serious complication of chronic (persisting for a long time) pyoderma faciale. From the surface, a draining sinus is a large, two-to-five centimeter long red bump that periodically discharges pus. 

Draining Sinus

Scars: Scars in pyoderma faciale are often widespread and can be atrophic (indented) or hypertrophic (raised above the skin).

Pyoderma Faciale Scarring

Scarring from Pyoderma Faciale

Pyoderma faciale vs. other severe forms of acne 

One should be careful when differentiating pyoderma faciale from other severe forms of acne--acne conglobata and acne fulminans

Pyoderma Faciale Vs. Other Severe Form of Acne

Acne conglobata: Acne conglobata patients present with lesions of all kinds, most notably nodules, cysts, blackheads, abscesses, and draining sinuses. In acne conglobata, lesions are never limited to the face and cover large areas of the chest, back, neck, face, shoulders, limbs, and buttocks. The disease typically affects young males.

Acne fulminans: Acne fulminans is rare--only about 100 cases have been documented so far.5 The disease features numerous painful nodules and/or cysts on the upper chest, back, neck, and face and systemic symptoms (symptoms that affect the whole body) like fever, fatigue, and muscle pain. Notably, nodules and cysts in acne fulminans fill with blood and rapidly turn into open wounds that very often leave massive, disfiguring scars. The disease almost exclusively affects young white males. 

Characteristics of Nodulocystic Acne Types

Treatment

Because the condition is so rare, little clinical evidence is available for analysis. Studies with documented treatment regimens for pyoderma faciale, acne conglobata, and acne fulminans show that a combination of an oral corticosteroid (e.g., prednisolone) and isotretinoin is the preferred treatment for severe, nodulocystic acne.4-9 Isotretinoin is a highly controversial drug that must be taken only in close supervision by a doctor and is associated with numerous hazardous health effects. Only the severity of the disease justifies the prescribing of this treatment option. 

Pyoderma Faciale Treatment

Oral corticosteroids and isotretinoin

An oral corticosteroid taken in combination with isotretinoin is the mainstay of treatment. Usually, treatment starts with a corticosteroid for around six weeks. After two to four weeks from the start of the treatment with prednisolone, isotretinoin is incorporated. This means that a person takes both prednisolone and isotretinoin simultaneously for some time. The treatment course lasts until all lesions resolve, which usually takes over three months.4

Clinical trial on the effectiveness of an oral corticosteroid plus isotretinoin for pyoderma faciale

Archives of Dermatology

In a 1992 study published in the Archives of Dermatology, researchers started treatment of pyoderma faciale patients with 1mg/kg/day of prednisolone for one to two weeks and then added 0.2 - 1mg/kg/day of isotretinoin, with a slow tapering of prednisolone to 0mg/kg/day over the next two to three weeks.4 The participants of the study, 20 in total, continued to receive isotretinoin until all inflammatory lesions cleared, which took three to four months. 

Despite being highly effective for severe acne, isotretinoin often comes with a long list of side effects and, sometimes, even severe, toxic reactions. This is why isotretinoin is approved for use only for nodulocystic acne.

Side Effects

The most frequent side effects include:

  • Inflammation of lips (90%) 
  • Dryness of skin and mucous membranes (80%) 
  • Pink eye (40%) 
  • Itching (40%) 
  • Gastrointestinal symptoms, such as nausea, vomiting, and abdominal pain (20%) 
  • Muscle and/or joint pain (16%)
  • Increased sensitivity to light (10%) 

Oral Corticosteroid Isotretinoin Treatment Side Effects

Toxic reactions: Possible toxic reactions associated with the administration of isotretinoin might include inflammatory bowel disease and increased intracranial (in the head) pressure that causes moderate to severe headaches and visual impairments, such as blurred and double vision, difficulty seeing to the side, and brief episodes of blindness.10 The incidence of these events is unknown, but they are uncommon. 

Adverse effect on the fetus: EXTREME CAUTION! Isotretinoin is contraindicated in pregnancy due to its severe adverse effect on the fetus.10 In fact, it is the No.1 birth defect - causing medication on the market. Birth defects are often severe,including death. That is the reason that it is strongly recommended for females to obtain two negative pregnancy tests prior to starting therapy. Additionally, women must use two forms of contraception for 30 days both before and after treatment with isotretinoin.

Expand to see side effects of isotretinoin and oral corticosteroids

Side Effects of Isotretinoin Accutane

Oral Corticosteroid Side Effects

Pyoderma Faciale Treatment Oral Antibiotics

Oral antibiotics 

Evidence shows that an oral antibiotic might be able to replace the oral corticosteroid/isotretinoin combination in the treatment of severe acne, including pyoderma faciale.3 They do clear nodules and cysts, but the lesions usually come back when treatment is discontinued. 

In a 1982 study carried out by the Mayo Clinic, 28 patients were treated with an oral antibiotic: 18 subjects received tetracycline; 2 were given erythromycin, and 8 were administered various agents, including erythromycin, sulfasalazine, trimethoprim-sulfamethoxazole, penicillin, and oxacillin. The patients also received some combination of topical treatments. At a follow-up after one year, complete remission (returning to the predisease state, with or without the emergence of a few new lesions) was achieved in 23 patients (79%) and in 16 patients (55%) after six months.3) However, "[a]lthough remissionwas achieved in most patients…, [15]patients[(51.7%)  required treatment, usually oral antibiotics inconjunction with…benzoyl peroxide or topical antibiotics for longer than [one] year."3

It is important to keep in mind that in the study on the effectiveness of an oral corticosteroid plus isotretinoin, the combination took no more than six months to clear acne completely in all patients but in about only 50 - 60% of the patients taking oral antibiotics after six months. So while both therapies are effective, the oral corticosteroid/isotretinoin approach produces faster results in more patients. 

Expand to see side effects of oral antibiotics

Side Effects of Oral Antibiotics

Summary

Pyoderma faciale is a rare, severe form of acne that occurs exclusively in females. Symptoms are always limited to the face and include intense redness of the skin and a mixture of inflammatory acne lesions, most notably nodules, cysts, and draining sinuses. Clinical evidence suggests that a combination of oral prednisolone and isotretinoin is the most effective treatment option.

The Experts at Acne.org

Our team of medical doctors, biology & chemistry PhDs, and acne experts work hand-in-hand with Dan (Acne.org founder) to provide the most complete information on all things acne. If you find any errors in this article, kindly use this Feedback Form and let us know.

References:

  1. Habif, T. Clinical Dermatology: A Color Guide to Diagnosis and Therapy (6th Edition). 231 - 233 (Saunders, 2015). 
  2. O'Leary, P. Pyoderma Faciale. Arch Dermatol 41, 451 (1940).
  3. Massa, M. & Su, W. Pyoderma faciale: A clinical study of twenty-nine patients. J Am Acad Dermatol 6, 84 - 91 (1982).
  4. Plewig, G. Pyoderma faciale. A review and report of 20 additional cases: is it rosacea? Arch Dermatol 128, 1611 - 1617 (1992).
  5. Zaba, R. Acne Fulminans. Emedicine.medscape.com (2016). at http://emedicine.medscape.com/article/1072815-overview#showall
  6. Schwartz, R. "Acne Conglobata." Emedicine.medscape.com. 2017. Web. 10 June 2017.
  7. Karvonen, S. Acne fulminans: Report of clinical findings and treatment of twenty-four patients. J Am Acad Dermatol 28, 572 - 579 (1993).
  8. Seukeran,D. C. & Cunliffe, W. J. The treatment of acne fulminans: a review of 25 cases. Br J Dermatol 141, 307 - 309 (1999).
  9. Kunynetz, R. A Review of Systemic Retinoid Therapy for Acne and Related. Medscape (2004). at http://www.medscape.com/viewarticle/472395
  10. Hodgson, B. & Kizior, R. Saunders nursing drug handbook 2012. (W. B. Saunders Co., 2014).

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