Robert W. Martin, III
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. 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.
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, myopathy[18,19] and synovitis, may occur in association with acne fulminans. Although alopecia, erythema,[21,22] ulceration of the lower extremities and Crohn’s disease have also been reported with this disease process, they are thought to be less commonly associated.
Acne fulminans typically affects white males, although three cases involving females[8,12,23] and one case involving a West Indian patient 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).
Elevated ESR and leukocytosis are characteristic laboratory findings, 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.
The thrombocytosis in the illustrative case is unusual, but this finding has been previously reported in a 17-year-old woman with acne fulminans. 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.
The pathogenesis of acne fulminans is unknown. An immune system abnormality is suggested by reports of circulating immune complexes, splinter hemorrhages, microscopic hematuria,[12,19] myositis,[18,19] low C3 (complement) levels, immunoglobulin elevation,[8,19,22] erythemanodosum[21,22] and an exaggerated response to intradermal P. acnes antigen in patients with acne fulminans. Interestingly, the simultaneous occurence of the disorder in identical twins 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. The onset of acne conglobata is less explosive than that of acne fulminans and is characterized by more nodules, cysts and polyporous comedones.
Oral corticosteroids have been the treatment of choice for acne fulminans for many years, 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. 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.
REFERENCES[1.] Goldschmidt H, Leyden JJ, Stein KH. Acne
fulminans: investigation of acute febrile ulcerative
acne. Arch Dermatol 1977;113:444-9. [2.] Stern RS. The epidemiology of cutaneous disease.
In: Fitzpatrick TB, ed. Dermatology in
general medicine: textbook and atlas. 3d ed.
New York: McGraw-Hill, 1987:6-10. [3.] Stern RS, Johnson ML, DeLozier J. Utilization
of physician services for dermatologic complaints.
The United States, 1974. Arch Dermatol
1977;113:1062-6. [4.] Darley CR, Currey HL, Baker H. Acne
fulminans with arthritis in identical twins
treated with isotretinoin. J R SocMed 1984;77:
328-30. [5.] Davis DE, Viozzi FJ, Miller OF, Blodgett RC.
The musculoskeletal manifestations of acne
fulminans. J Rheumatol 1981;8:317-20. [6.] Hunter LY, Hensinger RN. Destructive arthritis
associated with acne fulminans: a case report.
Ann Rheum Dis 1980;39:403-5. [7.] Moschella SL. Report of a case of acne conglobata
and arthritis. Bull Assoc Milit Dermatol
1964;13:15. [8.] Statham BN, Holt PJ, Pritchard MH. Acne
fulminans – report of a case with polyarthritis.
Clin Exp Dermatol 1983;8:401-4. [9.] Windom RW, Sanford JP, Ziff M. Acne conglobata
and arthritis. Arthritis Rheum 1961;
4:632-6. [10.] Burns RE, Colville JM. Acne conglobata with
septicemia. Arch Dermatol 1959;79:361-3. [11.] Engber PB, Marino CT. Acne fulminans with
prolonged polyarthralgia. Int J Dermatol 1980;
19:567-9. [12.] Strom S, Thyresson N, Bostrom H. Acute febrile
ulcerative conglobate acne with leukemoid
reaction. Acta Derm Venereol [Stockh] 1973;
53:306-12. [13.] Cros D, Gamby T, Serratrice G. Acne rheumatism.
Report of a case. J Rheumatol 1981;
8:336-9. [14.] Lane JM, Leyden JJ, Spiegel RJ. Acne arthralgia.
J Bone Joint Surg [Am] 1976;58:673-5. [15.] Nault P, Lassonde M, St. Antoine P. Acne
fulminans with osteolytic lesions. Arch Dermatol
1985;121:662-4. [16.] O’Malley BP, Anderson I, Rosenthal FD. Bone
lesions in systemic acne (acne fulminans). Br J
Dermatol 1979;100:703-5. [17.] Siegel D, Strosberg JM, Wiese F, Chen J. Acne
fulminans with a lytic bone lesion responsive to
dapsone [Letter]. J Rheumatol 1982;9:344-6. [18.] Leiferman KM, Groover RV, Dicken CH. Acne
fulminans and myositis. Cutis 1984;34:249-51. [19.] Noseworthy JH, Heffernan LP, Ross JB, Sangalang
VE. Acne fulminans with inflammatory
myopathy. Ann Neurol 1980;8:67-9. [20.] Marino CT. Acne fulminans with polyarthralgia
[Letter]. J Rheumatol 1981;8:866-8. [21.] Kellet JK, Beck MH, Chalmers RJ. Erythema
nodosum and circulating immune complexes in
acne fulminans after treatment with isotretinoin.
Br Med J [Clin Res] 1985;290(6471):820. [22.] Williamson DM, Cunliffe WJ, Gatecliff M,
Scott DG. Acute ulcerative acne conglobata
(acne fulminans) with erythema nodosum. Clin
Exp Dermatol 1977;2:351-4. [23.] Wolf R, David M, Feuerman EJ. Acne with
acute systemic reaction (acne fulminans?).
Report of a case. Cutis 1981;28:210-11,215-6. [24.] McAuley D, Miller RA. Acne fulminans associated
with inflammatory bowel disease. Report
of a case. Arch Dermatol 1985;121:91-3. [25.] Marks VJ, Briggaman RA. Pyoderma faciale:
successful treatment with isotretinoin [Letter]. J
Am Acad Dermatol 1987;17:1062-3. [26.] Strauss JS. Sebaceous glands. In: Fitzpatrick
TB, ed. Dermatology in general medicine: textbook
and atlas. 3d ed. New York: McGraw-Hill,
1987:666-85. [27.] Plewig G, Wagner A. Anti-inflammatory effects
of 13-cis-retinoic acid. An in vivo study. Arch
Dermatol Res 1981;270:89-94. [28.] Langner A, Wolska H, Fraczykowska M, Jablonska
S, Majewski S. 13-cis-Retinoic acid and
tetracycline versus 13-cis-retinoic acid alone in
the treatment of nodulocystic acne. Dermatologica
1985;170:185-8. [29.] Verbov J. Acne fulminans in a 14-year-old. Practitioner
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.
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.
COPYRIGHT 1989 American Academy of Family Physicians
COPYRIGHT 2004 Gale Group