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DOI: 10.1111/j.1468-3083.2010.03855.

x JEADV

REVIEW ARTICLE

Acne fulminans: explosive systemic form of acne
†, ‡ † † †
R Zaba, * RA Schwartz, S Jarmuda, M Czarnecka–Operacz, W Silny

Department of Dermatology, Poznan University School of Medical Sciences, Poznan, Poland

Dermatology, Pediatrics, Medicine, and Preventive Medicine and Community Health, New Jersey Medical School, Newark, NJ,
USA
*Correspondence: R Zaba. E-mail: zaba@dermatologia.pl

Abstract
Acne fulminans (AF) is a rare severe form of acne vulgaris associated with systemic symptoms. It primarily affects
male adolescents. Although the aetiology of AF remains unknown, many theories have been advanced to explain it.
There have been reported associations with increased androgens, autoimmune complex disease and genetic pre-
disposition. The disease is destructive, with the acute onset of painful, ulcerative nodules on the face, chest and
back. The associated systemic manifestations such as fever, weight loss and musculoskeletal pain are usually
present at the onset. The patients are febrile, with leucocytosis and an increased erythrocyte sedimentation rate.
They may require several weeks of hospitalization. The treatment of AF has been challenging; the response to
traditional acne therapies is poor. The recommended treatment is aggressive and consists of a combination of oral
steroids and isotretinoin. To avoid the relapses, duration of such treatment should not be less than 3–5 months.
Although the prognosis for patients treated appropriately is good, these acute inflammatory nodules often heal with
residual scarring.
Received: 15 February 2010; Accepted: 26 August 2010

Keywords
acne fulminans, acne maligna, acne with polyarthritis, acute febrile ulcerative conglobata, isotretinoin, prednisolone

Conflicts of interest
None declared.

Introduction (i) sudden onset; (ii) severe ulceration without
Acne fulminans (AF), also known as acne maligna, cyst formation;
is a severe, ulcerative form of acne with an acute (iii) fever and polyarthralgia; (iv) failure to
onset and systemic symp- toms. This serious disease respond to usual anti- biotic therapy; and (v) good
is rare and primarily affects Caucasian male response to debridement in combina- tion with
adolescents, aged 13–16 years, although it may steroid therapy. In 1975, Plewig and Kligman5
also occur in women.1,2 Its frequency is much lower separated this disease from acne conglobata and
in people of East Asian des- cent, such as in coined a term AF, which emphasized the
Japanese, who tend to have a milder course than characteristic sudden onset and severity of the
in people of Northern European lineage. AF was
illness. In 1977, Goldschmidt et al.6 reviewed thirteen
originally described in 1959 as acne conglobata
cases of AF
with septicaemia by Burns and Colville3, who
described a 16-year-old student with acute feb-
rile disease and acne conglobata. In 1971, Kelly
and Burns4 intro- duced a term ‘acute
febrile ulcerative conglobate acne with
polyarthralgia’ to name a syndrome with
following features:

ª 2010 The
JEADV 2011, 25, 501– Authors
507 Journal of the European Academy of Dermatology and Venereology ª 2010 European Academy of

symptoms (Table 1).11 AF has been evident at the onset. approximately 100 patients manifestation of the SAPHO syndrome. hidradenitis suppurativa. Systemic symp. psoriasis. The SAPHO with AF have been described. acne. hyperostosis and The primary clinical manifestations of this osteitis) syndrome was first described in 1987 disease include development of highly and has been predominately associated with inflammatory and haemorrhagic. upper chest and back. tender nodules on the back. They with AF develop erythema nodosum. acne weakness and musculoskeletal pain. chest about a year before the onset of their acute and face associated with various systemic signs.ance of highly Patients typically have mild acne vulgaris for inflammatory. tender. Less frequently. 25. chills.toms. hepatospleno.and also reported eight cases of their own.8 abnormalities such as palmoplantar pustulosis.megaly or myositis. 501– Authors 507 Journal of the European Academy of Dermatology and Venereology ª 2010 European Academy of . aseptic bone defined AF as a rare disorder of male teenagers osteolysis.10 characterized by sudden appear.7 (synovitis. pustulosis.9. crusted hyperostosis of the anterior chest and various skin nodules of the face. tends to be conglobata and acne fulminans. patients reported to develop at the ª 2010 The JEADV 2011. such as fever. AF can be the dermatological Since these reports.

AF maybe treated successfully with isotretinoin or with isotretinoin combined with systemic steroids or other anti-inflammatory agents. two siblings were described.14 Isotretinoin has been suspected. Patients developing severe flares of disease may be showing an exaggeration of this response.tretinoin dose was 0. isotretinoin could also act by inhibiting collagenase and gelatinase activity in stimulated fibroblasts in vitro. increased ESR. Many theories have been advanced. acne fulminans. Table 1 The main features of AF Gender Male gender dominant Age 13–22 years Pathogenesis Unclear Onset Acute and sudden Localization Upper chest and back. genetic and immunological causes have been proposed. Another theory is that genetically determined change in neutrophil activity – hyperreactivity to chemoattrac. including neutrophil chemotaxis. acnes chemoattractants this association is unclear. osteolytic bone lesions. AF may commence during isotretinoin. 1–7 weeks). In some patients.12 with the immune system to occur. fatigue. anaemia. 40– 80 mg ⁄ day.2 In these patients. Paradoxically. Retinoids are known to increase skin fragility through a reduction in number of tono- filaments and desmosomal attachments with secondary accumu. tetracycline or erythromycin therapy. face Clinical picture Ulcerative lesions covered with haemorrhagical crusts Systemic signs Malaise.13 The massive contact of the immune system with P. Interestingly. the iso. acnes chemoattractants may occur after isotretinoin-induced fragility of the pilosebaceous duct epithelium. acnes antigens and ⁄ or P. fever. Propioni. splenomegaly.lation of epidermal amorphous material. one with Pathophysiology isotretinoin-induced eruptive pyogenic The aetiology of AF remains unknown. that is. erythrocyte sedimentation rate. acnes. acnes antigens and⁄ or P. arthralgias and myalgias. onset of Crohn’s disease. Moreover.ble for the early flares seen on treatment with isotretinoin. but infection. shoulders. One postulates that development of AF is related to an explosive immunologically mediated type III and ⁄ or type IV hypersensitivity reaction to Propionibacterium acnes antigens. which may be responsi.tants may result in decreased phagocytosis of P. Isotretinoin induces fragility of the pilosebaceous duct epithelium and allows massive contact of . microscopical haematuria Response to conventional antibiotic therapy Poor Treatment of choice Systemic corticosteroids combined with isotretinoin AF. ESR.502 Zaba et al. proteinuria. and the duration of treatment before AF origin averaged 3 weeks (range.bacterium acnes destruction is thought to result in mediator release.5–1 mg ⁄ kg ⁄ day. mild cystic acne rapidly becomes ulcerative and necrotic acne with systemic symptoms. but the significance of P. as it may precipitate acne fulminans. weight loss. hepatomegaly Laboratory findings Leucocytosis.

The increased increase in gamma globulins and the decrease in C3 amount of P.502 Zaba et al. It has been postulated of testosterone and anabolic steroids. Together with this increased of an autoimmune aetiology of AF are the rapid sebum excretion rate.gen excess in men. acnes or related antigen might. Immune reach. precipitate an immunological reaction in some patients that complexes are found predominately in patients with leads to an outbreak of AF. induced acne fulminans.16 Three patients were observed with AF leucocytosis. fulminans. . there is an increased response of the patient to systemic steroids. Degitz. but no levels were found to be within normal limits. Large doses reliable method for detecting soluble IgG of andro. Schmidt and Plewig20 described a Elevated blood levels of testosterone may play 15-year-old boy with 3-month history of an important role in the pathogenesis of acne deteriorating acne. the population density of P. The trigger for acne musculoskeletal complaints. circulating immune complexes were detected.9 Testosterone levels increase physiologically Employing the platelet aggregation test. bodybuilder who developed AF as a side-effect Another theory postulates that AF may be an after 4 weeks of self-medication with high doses autoimmune complex disease.15 Placzek. induction seemed to be testosterone therapy in a and the other who developed isotretinoin. an elevated erythrocyte sedimentation possibly induced by high doses of testosterone rate and arthralgias. In this case. patient with Marfan syndrome. AF was caused administrated for the treatment of excessively by androgen excess attributed to late-onset tall stature.17 Another example was a 21-year-old congenital adrenal hyperplasia. circulating immune complexes may be increase the sebum excretion rate in docu. who suddenly developed fever. after complement levels in several patients. a during puberty and may explain why this disease primarily affects male adolescents.ing a certain level. his serum testosterone AF is seen with erythema nodosum. taken to that an arthus reaction was taking place when increase the mass of his muscles.19 Acne may be the only clinical sign of andro.18 Despite testosterone intake.mented in some with AF. granulomas (PG) in the course of severe acne. acnes.genic or ⁄ and anabolic steroids complexes.21 Factors in favour postpubertal men.

Over the past several years.25 In each case the age of presentation and severity of the disease was similar. Before the sudden onset of the disease. its incidence appears to be decreasing.27 At the onset. severe and often ulcerating acne.Acne fulminans 503 antibacterial therapy and good response to oral steroids after 4–6 weeks. tical human leucocyte antigen (HLA) phenotypes of two siblings.nous. responsible.TNF-a agent. Clinical The term AF is appropriate to describe the course of this entity.cline or isotretinoin therapy. in some cases there may be a genetic predisposition.28 They are so painful that the that the anti.5–5). An association of suppurative degeneration. necrotic the onset of clinical symptoms 2 weeks after a debris.23 Genetic and hereditary factors may play an important role in some patients. implying that the measles can trigger a transient release of inflammatory cytokines.2 It is a unique disease. most individuals have mild to moderate acne. with the addition of oral isotretinoin. resembles acne conglobata with the presence of the associa. shows patient is unable to lie on his back. including ulcerative nodules covered with haemorrhagic tumour necrosis factor-a (TNF-a). distributed mainly on the upper chest. the disease Although the pathogenesis of AF remains unknown. This is strengthened by the finding back and shoulders. The patient may seek initial care from an internist rather a dermatologist.tion with SAPHO syndrome suggests numer. possibly because of earlier and better treatment of acne.2 Age Acne fulminans predominantly affects young men aged 13– 22 years with a history of acne.24. highly inflammatory tender and that systemic inflammatory cytokinaemia. failure to respond to . infliximab. AF has been reported in identical monozygotic twins. Frequency Acne fulminans is a very rare systemic disease. The mean duration of acne before the onset of AF is 2 years (range 0.22 usually less severely involved than the trunk Infectious factors have been considered to be (Figs 1 and 2).26 Although the tissue typing has not shown these patients to have HLA- B27. Open and closed comedones are uncommon measles infection. The primary features of AF include the following: sudden onset. fever and polyarthritis. AF occurs usually after institution of tetracy. might be crusts. perhaps associated with the HLAcw6 gene. The lesions undergo swift important in acne fulminans.2 The disease in most patients begins as papulopustular or mild cystic acne and rapidly becomes AF. the similar clinical picture and iden. A hereditary nature has been suggested by the identical age of presentation. resulting in AF in predisposed individ- uals. leaving ulcerations viral infection with AF has been sug.gested by whose base is filled with gelati. The face is beneficial effects on AF.ous.

In contrast with acne vulgaris. early. early. back. in male adolescent.Acne fulminans 503 Figure 2 Acne fulminans. in male adolescent. polyporous . and inconspicuous. Figure 1 Acne fulminans.

large non-inflamed secondary cysts and polyporous comedones are absent.trast to acne conglobata. The most common systemic usually have been negative.coccal and anti- conglobata. there is a subset of patients with acne of severity comparable with that of AF but with the absence of systemic involvement.tion and fibrin deposition around the sebaceous glands. arthralgias. The sternum and clavicles are the most common sites. sternum and long bones of the extremities. At this stage. In con. Rarely.ated acanthotic epidermis with the dermis containing a dense mixed cellular infiltrate. A late nodule shows regener. as modification of acne therapy may be required. is seen in patients with AF. secondary to hyalinized thrombotic vessels and profuse bleeding into the skin. The predomi. leucocytosis and elevated erythrocyte sedimentation rate (ESR). Propionibacterium acnes have been isolated only sporadi. iliac and knee joints. vascular hyperplasia and numerous stel. AF tends to smolder for several months. the haemorrhagic skin necrosis is surrounded by a mixed granulocytic and lymphocytic infiltrate. In contrast with acne of major importance.nant locations for bone lesions are the clavicle. Staphylococcus aureus can be discovered occasionally. Tissue typing has not shown these patients to have HLA-B27. but do not reveal any tendency for comedone formation. a wide spectrum of systemic reactions streptococcal antibody levels usually are normal.tis may make walking painful.ical disorders of young patients. Patients may demonstrate a bent-over posture because polyarthri. It is one of the most scarring acute dermatolog. direct immunofluorescence testing reveals a linear IgM and fibrin band at the dermo-epidermal junc.gue. but we do not consider this finding to be . Histologically.cally from the bone lesions of patients with AF.tion of the follicular wall and sebaceous glands. but the healing is quite slow and often leaves extensive scarring. Laboratory findings There are no consistent laboratory abnormalities in acne fulmin. fever. ankles and humerus. Bacterial cultures from blood. Systemic signs and symptoms are presented in the Cultures for bacteria from blood and bone majority of patients. Anti-staphylo.504 Zaba et al. Some follicles show distension with accumulated kerati- nized cells (microcomedone formation). osteolytic lesions may occur in the hips. The epidermis becomes necrotic. signs and symptoms are fati.ans. Aseptic osteolytic bone lesions and increased uptake on bone scans have been reported.29 This group of AF ‘sine fulminans’ patients should be delineated. Arthralgia and joint swellings may also be present and occur mainly in large joints such as iliosacral. the early pustules show an intense infiltrate of neutrophils and occasional histiocytes with invasion and destruc. as well as the sacroiliac joints.late fibroblastic cells.reactive findings. myalgias. comedones are absent. malaise. anaemia. Erythematous neovascular nodules may also be seen. However. joint fluid and skin are usually sterile. Rheumatoid factor and anti-nuclear antibody tests have resulted in negative ⁄ non.

37 in whom culture from a lytic bone lesion showed The aetiology of pyoderma gangrenosum is unclear.5–1.ever. Other labo.ated by scintigraphy (‘hot spots’). but Acne fulminans if often associated with a osteolytic lesions have also been reported in the leukemoid reaction. No some patients with both AF and erythema nodo. the primary event in posterior patients show increased uptake using technetium scleritis seems to be vasculitis initi.formed to rule out polymorphonuclear leucocytes.33 Two patients had 0. Initial lesions are osteolytic. leuke. thrombocytosis. although a patient has been described with AF association with rheumatoid arthritis described. tosis sometimes with a leukemoid reaction up to 30 lesions in AF is rare but well recognized. patients with AF have shown depressed delayed patient in whom P. promyelocytes and myel.tion include the anterior chest.39 The sites hypersensitivity to various antigens in skin of predilec. there is one sum.nancy.moid reaction.ratory findings in patients with AF may include an Imaging studies increased ESR. pathogenic bacteria have been found. and 000 ⁄ mm2. A variety of benign of liver enzymes.30. he had red fluorescence in the affected bone. elevated levels malignancy or infection. tests.504 Zaba et al. periosteal formation of new bone. normochromic histological appearances have been reported.21 In contrast with acne vulgaris. acnes.ocytes in been described.38 characteristic for P.34 Another man had a milder later on. The occurrence of bone protein. and normocytic anaemia. the peripheral blood.32 particularly the sternum and clavi. Microscopic haematuria. an normal. gangrenosum-like eruption on the lower legs. with gran. ankles and humerus. but may show decreased albumin or infiltrate of neutrophils and mononuclear cells increased a-globulin and c-globulin. characteristic leucocy. acnes. which is and haematological malig. Patients with .mented as having but it occurs with systemic diseases such as AF and osteomyelitis with negative bacterial inflammatory bowel disease.ulation tissue may mimic osteomyelitis. P. Sacroiliitis has also myeloblasts. but also developed sclerosis and thickening occurs. acnes was cultured. increased percentage of often bone biopsies are per.cles. Circulating immune complexes have been found in The cause of bone lesions is unknown.36. Analysis of biopsy specimens of the bony lesions proteinuria and other kidney abnormalities are sometimes found.31 However. rheumatoid arthritis cultures from the bone. and 70% of mechanism(s). How.cation has been noted. increased levels of C-reactive Bone involvement is common.40 Destructive lesions deposition of circulating immune complexes in resembling osteomyelitis are demonstrated on scleral or episcleral blood vessels and with an radiographs in 25% of patients.35 Approximately 50% of patients have lytic bone Posterior scleritis may be caused by autoimmune lesions demonstrated on radiographs.5% hips. No erosions or posterior scleritis of his eyes and a pyoderma ligamentous ossifi.9. Serum proteins usually are shows reactive changes only.30 A patient was docu.

initial dose: 50 mg ⁄ day. AF it is more menacing to health. severe acne (fulminans or conglobata) or hidradenitis suppurativa.5–1 mg ⁄ kg ⁄ d PO for 6 weeks. Systemic manifesta. but the absence of skin lesions does not exclude the diagnosis of SAPHO.Acne fulminans 505 Table 2 Recommended treatment for AF Drug category Comments Systemic Corticosteroids (Prednisone) Drug of choice. Although both diseases have a similar localization. Acne conglobata is found to occur more often in women than acne fulminans. Acne fulminans can be considered as a part of the spectrum of the SAPHO syndrome. Furthermore.41 frequently. decreased as condition improves Retinoids (Isotretinoin) Beneficial in combination with steroids. duration of treatment: 3–5 months Sulfones (Dapsone) Effective in case of AF with erythema nodosum. it is important not to attribute acne fulminan to severe acne conglobata.ease category. (iii) chronic recurrent multiple osteomyelitis with or without dermatosis. Skin diseases associated with this syndrome include palmoplantar pustulosis. pelvis or limb. hyperos- tosis and osteitis) and various dermatological conditions.tions such as malaise or fever have . The most frequent sites of osteoarticular involvement are the anterior chest wall. lytic bone lesions may have elevated serum also been described in SAPHO syndrome but less alkaline phosphatase levels. multiple comedones are also present in a small number of patients with AF. pelvic girdle. The cause of SAPHO is unclear. patients present usually sclerosis or osteolysis or a mixture of the two. hidradenitis suppurativa. Radiologically. The criteria of SAPHO include: (i) osteoarticular manifestations of palmoplantar pustulosis. in combined therapy with oral steroids Infliximab Therapeutic option for patients unresponsive to conventional therapies. It occurs mainly in children and young adults. vertebrae and mandible. develops in older age and has a more chronic course. effectively controlling the skin lesions. requires special therapy and is time limited.44 Differential diagnosis The disease is easily identified because of its prominent systemic features.25 mg ⁄ kg ⁄ d PO.42. Topical Compresses with urea solution Prevention of accumulation of crusts Corticosteroids Used in the active phase to diminish the intensity of inflammation AF. we believe that these diseases belong to the same auto-immune dis. gradually increased to 1 mg ⁄ kg ⁄ d. with or without dermatosis. may be increased to 100 or 150 mg ⁄ day Azathioprine Used in severe case of AF. prednisone dose: 0. although considered pathognomonic of acne conglobata. spine. It has a broad spectrum of clinical presentation. acne fulminans.43 Because the clinical similarities among them are obvious. The onset of acne conglobata is less explosive than that of AF. initial dose: 0. severe acne and various patterns of psoriasis. a clinical condition which describes an association between musculoskeletal disorders (synovitis. (ii) hyperostosis of anterior chest wall. is resistant to antibiotics.

The delay in starting reduce fever and also have a favourable effect on isotretinoin therapy is necessary.5 mg ⁄ kg daily. the relapse manifests as is not the initial treatment of choice. isotretinoin (13-cis-retinoic acid) has been used The relapse usually occurs 2–8 weeks after the successfully in acne fulminans. the recommended. Isotretinoin is started after 4 weeks because they effectively control the skin lesions. 0.49 worsening of arthralgia and sometimes fever. acnes antigens. with a high isotretinoin may be bene.48.tion of steroid therapy appears The treatment of AF has been challenging.0 1. necessary.teroids dosage is may precipitate acne fulminans.47 Accordingly.0 mg ⁄ kg or even 2.2 fragility of the pilosebaceous duct.1 mg ⁄ kg ⁄ day) and gradually reduced to avoid adverse effects of a initially in patients with any evidence of prolonged course of systemic steroids. leading to Pulsed steroid treatment to control the acute massive contact with P. Thus.46.5–1.ficial. utilized as the drug of choice (Table 2). Duration of treatment depends upon individual response and usually . but Isotretinoin has been postulated to increase usually not as worsening of the cutaneous nodules. with isotretinoin started after the acute 0.g. Isotretinoin with a tend to recur when the steroid dose is minimum total dose of 120 mg ⁄ kg is lowered or discontinued too quickly. Recommended inflammatory phase of the disease may be uti. Oral steroids should recommend very be started initially to deal with systemic symptoms low doses of isotretinoin (0. In almost to be as long as 3–5 months to avoid all cases of AF.0 mg ⁄ kg daily may be mg ⁄ kg of prednisolone) are indicated in AF. systemic steroids have been relapses.45 Systemic corticosteroids (e. dosage of isotretinoin should not be less than lized. Systemic relapse rate if the dosage was reduced too quickly. acute attack when the corticos. but paradoxically. Symptoms crusting to avoid exacerbation. it reduced or stopped. Some the musculoskeletal symptoms.Acne fulminans 505 Treatment required dura. of corticosteroid treatment. but in some patients inflammatory phase of the disease has been an increase to controlled. The The combination of oral steroids and response has been generally favourable.

50 Other options can be considered. rarely 200 mg ⁄ day. dispensing or taking isotretinoin. it often hinders use of this medication. The salicy. The musculoskeletal symptoms and fever . A US Food and Drug Administration – mandated registry is now in place for all individuals prescribing. sulfasalazine and cyclosporine A). in cases of AF with erythema nodosum.taneously during isotretinoin treatment.lates have a good be used. This iPLEDGE registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy. the mean duration of hospital stay is 3–5 weeks. A remission was produced only after many months of additional therapy with azathioprine. applied on all ulcerated nodules twice daily for 7–10 days. Pyogenic inflammatory drugs. The mechanism by which it exerts an anti-inflammatory action may be related to inhibition of the myeloperoxidase-H2O2-halide system in polymorphonuclear leu- cocytes.ated with hyperplastic granulation tissue. Other topical treatments include cleansing with antibacterial agents and antiseptic lotions. oral corticosteroids. Topical.6 However. but intensive systemic antibiotic therapy is indicated to combat secondary infection when it occurs. generally respond well to non-steroidal anti- a repeat course of isotretinoin (150 mg ⁄ kg) may inflammatory drugs.2 The first step in therapy is surgical debridement. high-potency corticoster.2 mg ⁄ kg daily. including intra-articular cortico- spon. If required.oids. the inflam. Other granuloma-like vascular proliferations may occur therapies. A 16-year-old boy was described who developed within a few days AF of devastating severity. Sadly. Patients with AF usually require bed rest and possible hospi.matory effect on the acute myalgia and arthritis. but may not be effective.talization. the dose of isotretinoin should be modifying anti. More experience with it is necessary. which can be increased to 100 or 150 mg ⁄ day. The initial dose is 50 mg ⁄ day. reduced to 0. When admitted to the hospital. Pulsed dye laser is one approach said to be effective for AF and other disorders associ. The response to broad- spectrum antibiotic treatment is poor. may be beneficial in the active phase to diminish the intensity of inflammation and ulceration. If steroid. especially diaminodiphenylsulfone (dapsone) may be of limited value in AF. The healing may be rapid following pulse dye laser therapy.rheumatic drugs (methotrexate. injections. it has been shown to be effective treatment. should not be less than 3–5 months. he required both high dosage steroid and azathioprine.52 In any case dapsone may be substituted for the retinoid if it is not acceptable for whatever reason. accompanied by frequent warm compresses with 20–40% urea solution to prevent the accumulation of crusts. disease- that happens. complete healing may occur following two laser treatments. but recurrences tend to be more symptoms of SAPHO has been non-steroidal anti- frequent than in acne vulgaris. Sulfones. The most reaction can be significantly lessened or even frequently used treatment of musculoskeletal abolished.506 Zaba et al. In most cases.51 Moderate steroid therapy did not give control.

Acne conglobata with septicemia. the bones and general. When the treatment is given too control.53 the areas affected by the acute disease may After starting infliximab. unnecessary pharmacological organism appears to begin to destroy his own treatments and resid. Scarring and fibrosis may result from nodules may begin to noticeably decrease and this acute inflammatory process. inflix. but residual radiographic changes.tively treated with corticosteroids monoclonal antibody targeted against TNF-a. a chimeric IgG1-kappa Recurrent AF is extremely rare. For the patients with disfiguring disease.ious results. Relapses can occur. Promptly recognizing this disorder will aid of acne. The early institution of therapy count provide an objective means of monitoring can diminish the degree of scarring and other the therapeutic response. Serial and mani. Acne fulminans: report of clinical Discontinuation of therapy findings and treatment of twenty-four patients. The relapse usually 3 Burns RE. Appropriate diagnosis of AF make the disease sometimes difficult to AF allows the patients to avoid multiple recognize. AF unresponsive to conventional therapies.imab might be considered as a treatment Prognosis option. osteoarticular symp. 59: 387–9. which may correlate changes associated with this potentially with the clinical course. 54 2 Karvonen SL. . when Arch Dermatol 1959. South kg occurring as quickly as 1. back and clavicular pain completely than scarring are rare. Sequelae other synovitis. patients rapidly develop destructive changes. occurs 2–8 weeks after the acute attack. They suffer the pain and disability from the skin lesions. high as 41 °C has been reported. Colville JM. late. but resolve.5 kg ⁄ week and Dakota Med dramatic fever as 2006. the marrow. In tissues – the skin. ulcerative cutaneous persist. resolve with treatment.tory cytokines such as IL-1. The prognosis for patients effec. 79: 361–363. The multiple clinical manifestations of in appropriate management.toms. may inflama.ual scarring of the skin. In acne fulminans. IL-6 and remain. such as sclerosis and hyperostosis. Bone lesions typically neutralizes TNF-a.fests as worsening of arthralgia and measurements of the ESR and white blood cell sometimes fever. after 1-year treatment the risk of relapse is AF is the most aggressive and destructive form small.506 Zaba et al.sional mild musculoskeletal pain in decreasing cytokines of acute phase proteins. Occa.22 Infliximab. Acne fulminans: a case report. Devastating weight losses of 12 References 1 Neely GM. 28: 572–579. the patient’s consultations. and isotretinoin is good. but usually not as worsening of the skin lesions. bisphosphonates and infliximab have been the corticosteroid dosage is reduced or stopped administrated with var. Hein MS. J Am may be followed by an exacerbation of the Acad Dermatol 1993. especially when steroid treatment is reduced. AF does not recur when brought under the kidneys. leading to a reduction in pro.

com/article/1072815-overview 7 Seukeran DC. 1998. Acne fulminans with arthritis in iden- tical twins treated with isotretinoin. Bra¨mswig J et al. Arch WebMD. Kist JM. Gantes M. . Degitz K. 77: 328–330. Trygstad O. Hodgkins MB et al. Weenig RH et al. Dermatologica 1988. 72: 148– 149. Semin Arthritis Rheum 1999.megaly and erythema nodosum migrans. Cunliffe WJ et al. Acne fulminans: 27Zaba R. 13Karvonen SL. Lancet 1999. 177: 16–18. Beck MH. 5 Plewig G. 196. 1985. 17: 351–353. 10Reizis Z. Acne fulminans following measles infection. Acta Derm Venereol (Stockh) 1992. Acne: Morphogenesis and Treatment. Erythema nodosum and circulating immune complexes in acne fulminans after treatment with isotretinoin. Iinuma S et al. 4: 501–505. eMedicine from investigation of acute febrile ulcerative acne. Arch Dermatol 1985. Kolodney MS. 113: 444–449. 9 Williamson DM. Holt PJA.medscape. Clin Exp Derma. Acne fulminans in late-onset congenital adrenal hyperplasia. Br J Dermatol 1999. Arch Dermatol 1988. 354: 739–740. Acute febrile ulcerative 25Gonzales T. Burns RE. Baker H. 17Traupe H. Crocket KV. 290: 820. 189: 344–349. 124: 429–432. Rasanen L. Arch Dermatol 1989. Stein KH. 104: 182–187. Currey HL. J Am Acad Dermatol 1991. Int J Dermatol 2005. The treatment of acne (last accessed: 15 January 2010). Leyden JJ. 52: 118–120. J Am Acad Dermatol 2005. Hansel G. 141: 28Mehrany K. 19Wollina U. Koch A et al. fulminans: a review of 25 cases. Bustabad S et al. 36: 471– 473. Miller RA. 124: 414–417. Gatecliff M et al. Eruptive pyogenic granulomas and acne fulminans in two siblings treated with isotretinoin: a possible common pathogenesis. Kligman AM.ans type following testosterone therapy in three excessively tall boys. Cunliffe WJ. J Rheumatol 1971. Delayed hypersensitivity to Propionibacterium acnes in patients with severe nodular acne and acne fulminans. 1975. Acne fulminans: review. Acne fulminans associated with inflammatory bowel disease: report of a case. 23Honma M. 18Heydenreich G. Familial acne fulminans. J R Soc Med 1984. Acne fulminans with synovitis- acne-pustulosis. 12: 389–390.tol 1977. Available at: Dermatol 1977. Dermatology 1994. Berlin. Acne in pubertal boys undergoing treatment with androgens. Zultak M. Fiskadaal HJ. 12McAuley D. Br Med J 1985. 29: 159–171. von Muhlendahl KE. Acute ulcerate acne conglobata (acne fulminans) with erythema nodosum. Case reports: acne fulminans in Marfan syndrome. Dermatol 8 Jansen T. Testosterone and anabolic steroids and acne fulminans. Arch Dermatol associated with arthritis in monozygotic twins. Bouchaud-Chabot A. Acne of the fulmin. Wendling D et al. J Drugs Dermatol 2005. Schwartz RA: Acne fulminans. Springer. SAPHO syndrome: a long-term follow-up study of 120 cases. Br J Dermatol 1991. Int J 307–309. 37: 254–257. J Dermatol 2009. 14Perkins W. Acne fulminans with hepatospleno. 44: 132–133. Acne fulminans conglobate acne with polyarthralgia. 2: 351–354.hyperostosis-osteitis (SAPHO) syndrome treated with infliximab. 16Fyrand O. 21Kellett J. 20Placzek M. Cunliffe WJ. 22Iqbal M. Pritchard MH. 121: 91–93. Schmidt H et al. 24Darley CR. Plewig G. 6 Goldschmidt H. Clin Exp Dermatol 1992.Acne fulminans 507 4 Kelly PA. Acne fulminans. http://emedicine. 125: 571–572. Trattner A. 26Wong SS. Benali K et al. The effect of treatment with 13-cis-retinoid acid on the metabolic burst of peripheral blood neutrophils from patients with acne. Chalmers RJG. H. 15Blanc D. Hodak E et al. 11Hayem G. Murakami M. 24: 886–888.

Yamagata T et al. Bone lesions of acne fulminans: polymorphonuclear leucocyte cytotoxicity by dapson.tinoin and ‘pulse’ corticosteroids. gangreno. osteitis (SAPHO) syndrome and acne fulminans: are they part of the same disease 30Nault P. 2: 327–328. Miyatake J. Plewig G. Pediatr acne conglobata. Acne fulminans ‘sine 43Chua SL. Ranza R. 34: 241– 243. 93: 1632–1633.sum: a review.plexes and leukemoid reaction treated with 39Hunter LY. Rasmussen I. Rheum Dis 1980. Pediatr Dermatol 1998. Eur J 37Benson WE. 20: 353–357. Hensinger RN. Lassonde M. Acne fulminans and 13-cis-retinoic acid. 1992. Destructive arthritis steroids and azathioprine. 39: 403–405. Surv Ophthalmol. Acne fulminans with circulating 559–568. Acne fulminans: part of 138–142. Wiese F et al. as the main manifestation of the SAPHO syndrome: 31Knuchel M. 46von den Driesch P.tol 1996. Person DA. Reis G. lytic bone lesions responsive to dapsone. Cunliffe WJ. Japanese. 51–53. acne. 62: 214–220. Herna´ ndez MV. Acne fulminans Klin Monalsbl Augenhailk 1980. Ann Bras Acta Derm Venereol (Stockh) 1973. literature. Bang D. immune com. Strosberg JM. 297–316. 15: 396– 398. J Derma. rativa . Haneke E. 49Cavicchini S. Molin L. fulminans’. Thyresson N. Tokura Y. Effective treatment of acne fulminans- associated granu. Can˜ete JD et al. Arch Dermatol 1985.lation tissue with the pulsed dye 38Schwaegerle SM. . Dahlgren C. Semin Arthritis Rheum 2008. Pyoderma laser. Fuksiewicz W. Merle A et al. Nippon Hautkr 1986. 14: 39–42. hyperostosis. Clin Exp Dermatol. St-Antoine P. J Am Acad Dermatol 1988. the spectrum of SAPHO. coexisting with pyoderma gangrenosum-like eruptions and J Dermatol posterior scleritis.Acne fulminans 507 29Thomsen KF. possibilities . 61: 1145–1151. 58: 3–10. Schell H. 12: associated with acne fulminans: a case report. 41Knitzer RH. Naika Gakkai Zasshi 2004. 52Stendahl O. Acne fulminans: tratamento de 34Strom S. Acute febrile ulcerative conglobate acne with leukemoid reaction. 1987. Bostrom H. Acne fulminans: 33Tamaki C. J Rheumatol 1982. 9: 344–346. 36Rochels R. 25: 299–301. 57: Dermatol 1997. Delayed hypersensitivity and febrile with isotre. Ravenscroft J et al. 61: 1092–1098.case report. Clin Exp Dermatol 2000. 23: 37–41. Semin Arthritis Rheum 1991. Acne fulminans 48Choi EH. Knochennekrose bei acne response to infliximab therapy and review of the conglobata. Nham NX et al. Acta Derm Venereol (Stockh) 1977. Senitzer D et al. 20: Hidradenitis suppu. Luderschmidt CR. 1988. A case report and review of the literature. Post Derm Alerg 2008. Bergfeld WF. Echography in posterior scleritis. 11 pacientes com o a¢cido 13-cis-retino¢ ico. 53: 306–312. Br J Dermatol 2007. 45Allison MA. 156: 54Siegel D. 32: Dermatol 1992. 47Hartmann RR. 121: 44Moll C. the therapeutic 247–255. Case of acne Therapie mit 13-cis-Retinsa¨ure und Indometazin. Ann 463–466. herpetiformis. 18: 51Woolfson H. pustulosis. 50Friedlander SF. Ravenscroft J. Z fulminans associ. J Clin Invest 1978. Needleman BW. Ilium osteitis 662–664. Z Hautkr 1986. The inhibition of 40Jemec GBE. Dunn CL. J Am Acad possible mechanism in the treat. Acne fulminans treated 32Palatsi R. with sacroiliitis during isotretinoin therapy. with osteolytic lesions. Acne fulminans with a 1408. 177: 611–613. 35Kurokawa S.ment of dermatitis Dermatol 1989. Synovitis. Acne fulminans spectrum? Clin Exp Dermatol 2009. 19: 378–383. Road H. Posterior scleritis. 25: 42Chua SL. associated with acne. Dermatol 1983. 37: 299–306.ated with hemophagocytosis. Brezzi A et al. Angus JE. Musculoskeletal syndromes 53Szulczyn´ ska-Gabor J.

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