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Print version ISSN 0365-0596
An. Bras. Dermatol. vol.86 no.4 supl.1 Rio de Janeiro July/Aug. 2011
Disabling acne fulminans*
Tiago Pina ZanelatoI; Gabriela Maria Abreu GontijoI; Célia Antônia Xavier de Moares AlvesII; Jacqueline Campoi Calvo Lopes PintoIII; Paulo Rowilson CunhaIV
IMedical resident of the Dermatology Service of the Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brasil
IIDermatologist - Assistant Teacher of the Dermatology Service of the Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brasil
IIIDermatologist. - Collaborating Teacher of the Dermatology Service of the Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brasil
IVFull Professor and Chief of the Dermatology Service of the Faculdade de Medicina de Jundiaí (FMJ). Associate Professor of the Faculdade de Medicina da Universidade de São Paulo (FMUSP) - São Paulo (SP), Brasil
Acne fulminans is a rare manifestation that may occur during the evolution of acne vulgaris primarily in male adolescents. Use of isotretinoin, testosterone, and exacerbated immune responses in the body are related triggers. Signs, symptoms and laboratory findings such as fever, hepatomegaly, polyarthralgia, leukocytosis, plaquetose, increased inflammatory markers and transaminases, are characteristic. A bone scan can detect osteolytic lesions in multiple skeletal sites. The treatment is performed with prednisolone, isotretinoin and antibiotics if secondary infection is present. This case describes a male patient with a diagnosis of grade III acne, who developed acne fulminans and bilateral sacroiliitis with inability to ambulate, after initiation of therapy with isotretinoin.
Keywords: Arthralgia; Isotretinoin; Prednisone
Acne fulminans is a very rare, severe manifestation that can develop during the course of acne vulgaris. Synonyms like acne maligna and acute febrile ulcerative acne conglobata were substituted by the term acne fulminans described in 1975 by Plewig and Kligman. 1 It develops mostly in male adolescents, aged 13 to 16, with mild to moderate acne. 2 Its ethiology is not yet fully understood. Hereditarity, exacerbated immune reactions, bacterial infections and use of some drugs such as isotretinoin, tetracycline and testosterone can be triggering factors. 2,3,4 It is postulated that some antigens found on the bones and skin are similar to bacterial antigens and, through a hypersensitivity immune reaction, cause the bone lesions. 5 There are cases described in association with Cohn's disease and after measles infection. 6,7
Acne fulminans manifests in patients previously diagnosed with mild to moderate acne, as an acute onset of multiple papules, plaques, hemorrhagic nodules, and ulcerations with necrotic floor that evolve to extensive scarring. 2 It develops on the face, trunk and rarely on the thighs. Signs and symptoms like fever, hepatosplenomegaly, polyarthralgias, polyarthritis (mostly in large joints), erythema nodosum, myalgia, anorexy, and weight loss might be present. 8,9
Laboratory tests usually show leukocytosis, thrombocytosis, anaemia, microscopic hematuria, increased hemossedimentation rate, reactive C protein and alteration of the hepatic enzymes. 9 Bone cintilography can detect inflammatory and lithic lesions with increased uptake of the radiotracer. 10
The treatment is based on the use of prednisolone with gradual reduction of the dose and concomitant or subsequent introduction of isotretinoin. 2,9,11 Systemic antibiotics can be associated to control secondary infections and local care of the lesions involves use of compresses and emollients.
The authors report a case of a male patient, 14 years old, with dark skin, with acne level III, in use of tetracycline for two months without improvement. After 12 days of beginning isotretinoin (0.5 mg per kilo per day) associated to prednisone (0,1 mg per kilo per day) there was worsening of the acne with sudden development of ulceronecrotic lesions on the face, back and trunk. (Figures 1 and 2). He also complained of fever, severe pain on the rib cage, hips and lower limbs, associated with inability to walk and in use of wheelchair. The laboratory tests showed; leukocytosis: 20490/mm3, (without deviation to the left); thrombocytosis: 849000/mm3; reactive C protein: 93,4mg/dl; hemossedimentation rate: 30mm; gamma glutamyl transferasis: 175Ul/l (normal < 30Ul/l); normal transaminases and bilirubins; negative HLA B27, ANF, and hemocultures. Imaging exams (simple x-ray, ultrasound and computerized tomography of the pelvis) were unaltered. Bone scintigraphy showed evidence of bilateral sacroiliitis and multiple osteoarticular processes with abnormal bone activity on the sternoclavicular, scapulohumeral, costochondral, mandible, ankles and lumbar spine joints. (Figure 3). The patient was admitted to hospital and the isotretinoin was suspended. He was treated with prednisone (0,5mg per kilo per day), acetaminofen 500mg four times daily, associated to sulfamethoxazole-trimethoprim (800/160mg) twice a day for secondary infection. The cutaneous lesions improved and the patient was able to walk again in 15 days. Isotretinoin was reintroduced (0,2mg per kilo per day) 30 days after being suspended. After two months the dose was increased to 0,5 mg per kilo per day with gradual reduction of the corticosteroid. (Figures 4 and 5).
The reported case represents a typical case of acne fulminans, a rare manifestation with few cases described on the literature. The development of ulceronecrotic lesions on the face, back and chest in male young patients with moderate acne associated with fever, polyarthalgia, laboratorial and scintigraphic abnormalities are characteristic of this disease. On the present case we regard the beginning of the therapy with isotretinoin as the triggering factor of the acne fulminans. 9,12 This could be explained by the increased fragility of the pilosebaceous ducts induced by isotretinoin and the extensive exposure to Propionibacterium acnes antigens. 2 The chemotaxis of the neutrofils and an exacerbated immune response to these antigens could have been a possible trigger. 13 Polyarthralgia, as seen on various involved sites through the scintigraphy, and especially the extreme sacroiliitis, reported in 21% of the patients with acne fulminans, lead to the patient's notable incapacity for walking. He had to use a wheelchair and later a walking aid to help him move around. 8
Osteolytic lesions, with increased radiotracer uptake at scintigraphy, on the sternum, clavicle, sacroiliac joints, humerus and ankles are described. 9,12 Usually they are sterile and have a good prognosis. Previous studies revealed growth of Propionibacterium acnes on culture of an osteolytic lesion in only one patient. 14
Hemocultures from the patient collected during febrile episodes were negative, in accordance to the literature review. Of the other 15 reported cases all hemocultures were negative. 9 The use of prednisone and sulfamethoxazole-trimethoprim as the treatment of choice led to the clinical improvement of the patient with return to his usual activities. The use of antibiotic was aimed at treating the secondary infection as well as at diminishing possible existent super antigens. The reintroduction of the isotretinoin was gradual due to the risk of worsening of the symptoms. Despite being a triggering factor it is indicated on treatment of acne fulminans in low doses with subsequent increase according to the patient's tolerance. 15
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Mailing address: Received on 05.10.2010. * Work performed at the Dermatology Department of the Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brasil.
Tiago Pina Zanelato
Rua Francisco Telles 250, Vila Arens
Jundiaí - SP, Brazil Cep: 13202-550
Approved by the Advisory Board and accepted for publication on 21.10.2010.
Conflict of interest: None
Financial funding: None
Received on 05.10.2010.
* Work performed at the Dermatology Department of the Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brasil.