Acne fulminans

Acne fulminans

Robert W. Martin, III

Acne Fulminans

Acne fulminans is a unique type of acne that presents acutely and dramatically with fever, weight loss and papulopustular lesions that are highly inflamed, tender and, eventually, ulcerative. These lesions occur on the face, torso and upper extemeties. Musculoskeletal symptoms and hematologic manifestations frequently accompany this disorder. Acne fulminans in a 13-year-old boy was effectively treated with isotretinoin, prednisone and minocycline.

Acne fulminans is a rare skin disorder with multisystem involvement. The most severe presentation of acne, the condition most commonly occurs in white adolescent males.[1] Clinically, nodulocystic acneiform lesions develop on the face, torso and upper extremeties. The lesions are highly inflamed and tender, and eventually ulcerate. Systemic manifestations include fever, weight loss and musculoskeletal complaints. Leukocytosis, mild anemia and elevated erythrocyte sedimentation rate (ESR) are typical laboratory findings. Since family physicians and other primary care physicians evaluate and treat more skin conditions than do dermatologists,[2,3] it is essential that this potentially catastrophic form of acne vulgaris be generally recognized and promptly treated.

Illustrative Case

A 13-year-old boy with mild to moderate acne vulgaris of one year’s duration developed fever, malaise, anorexia (with a weight loss of 12 lb [5.5kg]) and dramatic worsening of cystic acne two weeks before seeking medical consultation. On physical

The initial leukocyte count was 21,900 per [mm.sup.3] (21.9 x [10.sup.9] per L), with 43 percent (0.43) segmented neutrophils, 25 percent (0.25) band cells, 23 percent (0.23) lymphocytes, phocytes, 5 percent (0.05) monocytes, 3 percent (0.03) eosinophils and 1 percent (0.01) reactive lymphocytes. The hemoglobin level was 11.8 g per dL (118 g per L), and the ESR was 128 mm per hour (128 mm per h). The platelet count was initially 458 x [10.sup.3] per [mm.sup.3] (458 x [10.sup.3] per L) and subsequently rose to 535 x [10.sup.3] per [mm.sup.3] (535 x [10.sup.9] per L). Cultures obtained from facial pustules were positive for Staphylococcus epidermidis and Propionibacterium acnes. Nasal cultures grew only normal flora, while blood, urine and throat cultures were negative. Antinuclear antibody test, spot test for infectious mononucleosis, blood chemistries and chest radiograph were negative or within normal range.

Before the culture results were available a presumptive diagnosis of gram-negative folliculitis was made, and the patient was given clindamycin (Cleocin), 150 mg four times daily. However, when the culture results were known and the patient had shown no improvement after seven days of therapy, the clindamycin was discontinued and the diagnosis of acne fulminans was made.

A daily regimen consisting of prednisone, 60 mg, isotretinoin (Accutane), 80 mg, and minocycline (Minocin), 100 mg, was started. Prednisone theraphy was tapered and discontinued after ten weeks. Isotretinoin was reduced to 40 mg daily after nine weeks and discontinued 11 weeks later. The minocycline was continued for a total of 29 weeks.

Constitutional symptoms of fever, anorexia and malaise resolved within two weeks of therapy. The patient’s platelet count, hemoglobin level, ESR and white blood cell count returned to normal by the fourth, sixth, ninth and tenth weeks, respectively. An isotretinoin-induced rise in triglycerides, from a pretreatment level of 89 mg per dL (1.00 mmol per L) to a level of 488 mg per dL (5.50 mmol per L) during treatment, responded to a low-sugar and low-cholesterol diet.

At the end of 20 weeks of theraphy, the skin lesions had improved dramatically (Figure 2). Two years later, the skin remained clear, but residual keloids were present on the torso (Figure 3).

Clinical Picture and Epidemiology

Acne fulminans is an uncommon form of nodulocystic acne associated with fever and weight loss, and characterized by inflamed, tender and ulcerated lesions on the face, torso and upper extremities. Musculoskeletal abnormalities, including arthritis,[4-9] arthralgias,[1,10-12] lytic bone lesions,[6,13-17] myalgias,[5] myopathy[18,19] and synovitis,[20] may occur in association with acne fulminans. Although alopecia,[14] erythema,[21,22] ulceration of the lower extremities[13] and Crohn’s disease[13] have also been reported with this disease process, they are thought to be less commonly associated.

Acne fulminans typically affects white males,[1] although three cases involving females[8,12,23] and one case involving a West Indian patient[16] have been reported. The reported age range has been 13 to 29 years of age.[5,24] Mild to moderate acne vulgaris may precede the fulminant flare by one to 96 months (mean: 10.5 months).[5]

Elevated ESR and leukocytosis are characteristic laboratory findings,[1] and anemia is frequently present.[5,14] Cultures of the skin lesions yield the usual acne flora. Blood, synovial fluid and osteolytic bone lesion cultures are usually negative. However, P. acnes has been obtained from the subculture of an osteolytic bone lesion.[14]

The thrombocytosis in the illustrative case is unusual, but this finding has been previously reported in a 17-year-old woman with acne fulminans.[23] Her platelet count ranged from 420 x [10.sup.3] per [mm.sup.3] (420 x [10.sup.9 per L) to 670 x [10.sup.3] per [mm.sup.3] (670 x [10.sup.9] per L), and both the disease and the thrombocytosis responded to corticosteroid therapy. There was no evidence of blood loss, bacterial infection, carcinoma, rheumatoid arthritis, inflammatory bowel disease or vasculitis in either case, which suggests that the thrombocytosis was a nonspecific reaction to the inflammatory nature of acne fulminans.

Pathogenesis

The pathogenesis of acne fulminans is unknown. An immune system abnormality is suggested by reports of circulating immune complexes,[21] splinter hemorrhages,[19] microscopic hematuria,[12,19] myositis,[18,19] low C3 (complement) levels,[14] immunoglobulin elevation,[8,19,22] erythemanodosum[21,22] and an exaggerated response to intradermal P. acnes antigen[22] in patients with acne fulminans. Interestingly, the simultaneous occurence of the disorder in identical twins[4] suggests a possible hereditary or environmental predisposition.

Acne fulminans must be differentiated from pyoderma faciale and acne conglobata. The presence of systemic symptoms distinguishes acne fulminans from both pyoderma faciale and acne conglobata. Pyoderma faciale is an abrupt acneiform eruption consisting of painful nodulocystic acne that typically resolves slowly over several years with significant residual scarring.[25] The onset of acne conglobata is less explosive than that of acne fulminans and is characterized by more nodules, cysts and polyporous comedones.[26]

Management

Oral corticosteroids have been the treatment of choice for acne fulminans for many years,[1] with some authors suggesting the simultaneous use of antibiotics.[1,26] More recently, isotretinoin has been used effectively,[4,27] although treatment failures have occured without the concomitant use of corticosteroids.[28] The simultaneous use of corticosteroids and isotretinoin appears to be the accepted treatment at the present time.[4,26,28,29] The role of antibiotic therapy remains uncertain.[1,14] Serial measurements of the ESR and white blood cell count provide an objective means of monitoring therapeutic response, which should correlate with the patient’s appearance.

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PHOTO : FIGURE 1. Lesions (nodulocystic and ulcerative) of the face and torso at onset of disease.

PHOTO : FIGURE 2. Appearance of lesions immediately after therapy with isotretinoin, prednisone and minocycline.

PHOTO : FIGURE 3. Appearance of skin two years after treatment. Some residual keloids remain.

The Authors

ROBERT W. MARTIN III, M.D. was formerly a clinical associate professor of family medicine at Southern Illinois University School of Medicine, Springfield. A graduate of Southern Illinois University School of Medicine, Dr. Martin is currently serving a residency in dermatology at Texas Tech University Health Sciences Center, Lubbock, Texas.

W. GERALD KLINGLER, M.D. is a clinical assistant professor of dermatology at Southern Illinois University School of Medicine. Dr. Klingler graduated from the University of Michigan Medical School, Ann Arbor. He completed a residency in dermatology at the University of North Carolina, Chapel Hill.

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