Acne Fulminans

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Brigitte Dreno - One of the best experts on this subject based on the ideXlab platform.

  • Acne Fulminans and Cutibacterium Acnes phylotypes.
    Journal of the European Academy of Dermatology and Venereology : JEADV, 2020
    Co-Authors: S. Bocquet‐trémoureux, Amir Khammari, Stephane Corvec, M.-a. Dagnelie, Aurélie Boisrobert, Brigitte Dreno
    Abstract:

    Background Acne Fulminans (AF) is a rare and severe form of inflammatory Acne. It is characterized by a sudden worsening of Acne with appearance of ulceronecrotic lesions, which can be associated with systemic signs. Its pathophysiology and the best therapeutic strategy are only partially known. Objective Our main objectives were to describe the clinical and biological profile of AF patients and to determine whether there was a difference in Cutibacterium Acnes phylotype in AF compared to Acne vulgaris. The secondary objective was to assess the efficacy of different therapies. Methods A retrospective observational study was conducted in all patients followed for AF in our department between 2008 and 2018. Bacteriological samples were taken from each patient to analyse C. Acnes phylotype distribution. The therapeutic response was assessed using the ECLA and GEA scales. Results Fifteen patients with a median age of 15 years were included (12 men, 80%). A family history of Acne was found in 86.7% of patients. Nine patients (60%) had isotretinoin-induced AF. Only one patient (6.7%) showed systemic signs. The bacteriological culture was positive for C. Acnes in 80% of patients. The predominant phylotype was IA1 in 60% of patients, corresponding to the predominant phylotype in Acne vulgaris. Only 33.3% of patients were in remission after a first-line treatment with systemic corticosteroids, alone or in combination. Seven patients were treated with biotherapy, including five successfully with secukinumab. Conclusion Our results suggest that there is no specific C. Acnes phylotype associated with AF, raising the hypothesis that acute inflammation associated with AF may be more related to an abnormal cutaneous innate immunity activation. The use of preventive strategies, the impact of combined treatments and an assessment of the role of biotherapies, especially anti-IL-17, in AF treatment remain to be more investigated.

  • Acne Fulminans et phylotype de propionibacterium Acnes
    Annales De Dermatologie Et De Venereologie, 2016
    Co-Authors: Le M Moigne, S Corvec, Guillaume Ghislain Aubin, C Paugam, Saint M Jean, Amir Khammari, Brigitte Dreno
    Abstract:

    Introduction Propionibacterium Acnes ( P. Acnes ) tient un role central dans la physiopathologie de l’Acne notamment par l’activation de l’immunite innee. Les souches de P. Acnes sont classees en fonction du phylotype. Il a ete recemment demontre que le potentiel pro inflammatoire in vitro de P. Acnes differe en fonction du phylotype. L’Acne Fulminans est une forme rare et tres severe d’Acne, se manifestant par la survenue brutale de nodules inflammatoires, sur le visage et le tronc, avec parfois des signes generaux associes. L’objectif de ce travail etait de determiner le phylotype de P. Acnes chez des patients presentant une Acne Fulminans. Materiel et methodes Il s’agit d’une etude prospective, menee au CHU de Nantes, incluant tous les patients presentant une Acne Fulminans, entre septembre 2013 et mai 2016. Pour chaque patient, des donnees cliniques etaient recueillies de facon standardisee avec realisation du score echelle de cotation des lesions d’Acne (ECLA). Un prelevement bacteriologique etait realise par ecouvillonnage des lesions inflammatoires (papules ou nodules) du tronc et/ou du visage. Le phylotype, ainsi que le complexe clonal des isolats de P. Acnes etaient determines par la technique du Single-Locus Sequence-Typing (SLST) et celle de Multi-Locus Sequence-Typing (MLST). Resultats Six patients ont ete inclus, dont 5 hommes, d’âge moyen 16,8 ans (12 ; 19). Il s’agissait d’une Acne a debut pre-pubertaire pour 2/6 patients. Tous avaient des antecedents familiaux d’Acne, dont 4/6 chez la mere. L’Acne Fulminans etait induite par un traitement par isotretinoine chez 4/6 patients. Le parametre F1 (visage) du score ECLA etait en moyenne de 7,5 (1 ; 15) (avec en moyenne 22 lesions inflammatoires superficielles et 1,5 nodules par patient) et le parametre F2 (tronc) de 10 (6 ; 18) (moyenne de 7,8 nodules par patient). P. Acnes a ete identifie sur les prelevements de 5/6 patients, avec 4 isolats de phylotype IA1, dont 3 appartenaient au cluster CC18/A1 selon MLST/SLST et un au cluster CC53/D1. Un patient etait porteur d’un P. Acnes de phylotype II (cluster CC53/K2). Pour ce patient, le score F1 etait de 5 et le score F2 de 14. Staphylococcus aureus a ete identifie pour le dernier patient qui avait une atteinte predominante au niveau de la face posterieure du cou, sans atteinte du visage. Discussion Le phylotype IA1 de P. Acnes est celui que nous avons trouve chez 4/6 patients ayant une Acne Fulminans. Ce phylotype est egalement celui trouve en cas d’Acne vulgaire. Un patient avait un phylotype II, qui in vitro a une activite pro-inflammatoire plus importante que le type IA1, cependant ce patient n’avait pas un score ECLA superieur a celui des autres patients. Conclusion Notre travail est le 1 er etudiant le phylotype de P. Acnes dans l’Acne Fulminans. Il demontre que la severite de l’Acne n’est pas liee a un phylotype particulier, ce qui laisse supposer qu’elle pourrait etre liee a une anomalie de reponse de l’immunite innee cutanee.

  • testosterone induced Acne Fulminans in twins with kallmann s syndrome
    JAAD case reports, 2015
    Co-Authors: Melanie Saintjean, Guillaume Ghislain Aubin, Cecile Frenard, Maelle Le Bras, Stephane Corvec, Brigitte Dreno
    Abstract:

    Acne Fulminans is a rare and severe form of Acne characterized by the development of painful and inflammatory nodules on the face, chest, and back. Systemic signs are associated with the onset, including fever, chills, and musculoskeletal pain. Erythema nodosum, liver or spleen enlargement, myositis, or aseptic bone involvement have been reported more rarely. Its etiology remains unknown. It is assumed that a strong activation of the cutaneous innate immunity induced by Propionibacterium Acnes antigens could be involved. Another hypothesis is that it could be a dysregulation of some receptors expressed by sebaceous glands and keratinocytes, in particular, the androgen receptors. Moreover, these abnormalities could be genetically determined.

  • Acne Fulminans et testosterone un lien tres probable illustre par 2 cas survenus chez des jumeaux atteints d un syndrome de kallman
    Annales De Dermatologie Et De Venereologie, 2014
    Co-Authors: M Saintjean, S Corvec, Guillaume Ghislain Aubin, C Frenard, Le M Bras, Brigitte Dreno
    Abstract:

    Introduction L’Acne Fulminans est une entite rare dont la physiopathologie demeure inconnue. Nous rapportons le cas de deux jumeaux traites par testosterone injectable pour un retard pubertaire et ayant developpe dans les mois suivants une Acne Fulminans. Observations Un patient de 18 ans etait adresse en urgence pour une Acne severe du dos de debut brutal. Ce patient et son frere jumeau avaient un syndrome de Kallman (hypogonadisme central et anosmie). On notait par ailleurs un antecedent familial d’Acne chez le pere et le grand frere. Dans le cadre du retard pubertaire, un traitement par injection mensuelle de testosterone avait ete debute chez les 2 freres. Neuf mois apres, le patient developpait brutalement des lesions d’Acne nodulaires et necrotiques du dos, faisant stopper les injections. Le taux serique de testosterone etait alors normalise. Le frere jumeau developpait dans le meme temps une Acne severe nodulaire du dos traitee dans un autre centre. A l’examen clinique, on notait des lesions du dos avec de tres nombreux nodules s’etendant a la racine des bras d’evolution necrotique. Le visage presentait une Acne plus moderee sans nodule (grade 3 sur l’echelle GEA). Le prelevement local retrouvait un Propionibacterium Acnes multisensible de phylotype II. Un traitement par corticotherapie generale (0,5 mg/kg/jour) etait debute avec disparition a 1 mois des lesions necrotiques permettant d’introduire l’isotretinoine a 0,06 mg/kg/jour. Le cas a ete declare a la pharmacovigilance. Discussion Moins d’une dizaine de cas d’Acne Fulminans induite par la testosterone injectable ont ete decrits dans la litterature (1 cas d’un patient atteint d’un syndrome de Klinefelter, 4 cas de garcons traites pour petite taille et 2 cas de patients traites par anabolisants dans un contexte de musculation) et aucun chez des jumeaux. La survenue retardee par rapport au debut du traitement serait liee a la normalisation du taux de testosterone comme chez notre patient. Ces observations arguent en faveur de l’hypothese d’un lien entre Acne Fulminans et hypersensibilite androgenique peripherique des recepteurs aux androgenes cutanes (keratinocytes et sebocytes). Ce lien est evoque aussi de part l’atteinte quasi-exclusive de garcons. La survenue chez des jumeaux en meme temps dans notre cas renforce l’hypothese du role d’un facteur genetique. De maniere interessante, il a ete rapporte recemment la decouverte de 2 nouveaux loci de susceptibilite a l’Acne severe impliques dans le metabolisme des androgenes. Conclusion Notre observation d’Acne Fulminans induit par la testosterone chez des jumeaux renforce l’hypothese d’une anomalie genetique en lien avec les recepteurs androgeniques cutanes dans l’etiologie de cette maladie rare. Devant une Acne Fulminans, il convient de toujours verifier l’absence de prise dans les mois precedents de testosterone ou d’anabolisants.

Viriato Campelo - One of the best experts on this subject based on the ideXlab platform.

  • Acne Fulminans successfully treated with prednisone and dapsone
    Anais Brasileiros De Dermatologia, 2012
    Co-Authors: Rafael Bandeira Lages, Sebastião Honório Bona, Flávia Veríssimo Melo E Silva, Andressa Karoline Lima Gomes, Viriato Campelo
    Abstract:

    Acne Fulminans is a rare condition and the most severe form of Acne. It involves the sudden onset of febrile and multisystemic symptoms, with poor response to ordinary therapy in patients who previously had mild to moderate Acne. It is characterized by hemorrhagic ulcerative crusting lesions on the face, chest and upper back. The authors report a case of Acne Fulminans that was successfully treated with oral prednisone and dapsone.

  • Acne Fulminans successfully treated with prednisone and dapsone Acne Fulminans tratada com prednisona associada à dapsona
    Sociedade Brasileira de Dermatologia, 2012
    Co-Authors: Rafael Bandeira Lages, Sebastião Honório Bona, Flávia Veríssimo Melo E Silva, Andressa Karoline Lima Gomes, Viriato Campelo
    Abstract:

    Acne Fulminans is a rare condition and the most severe form of Acne. It involves the sudden onset of febrile and multisystemic symptoms, with poor response to ordinary therapy in patients who previously had mild to moderate Acne. It is characterized by hemorrhagic ulcerative crusting lesions on the face, chest and upper back. The authors report a case of Acne Fulminans that was successfully treated with oral prednisone and dapsone.A Acne Fulminans é uma condição rara e a mais severa forma de Acne. Manifesta-se com um quadro agudo, febril e multissistêmico, resistente à terapêutica convencional em doentes com antecedente de Acne leve ou moderada. As lesões são caracteristicamente úlcero-hemorrágicas e acometem preferencialmente tórax e face. Os autores relatam um caso de Acne Fulminans com excelente resposta terapêutica ao tratamento empregado

S L Karvonen - One of the best experts on this subject based on the ideXlab platform.

  • increased chemiluminescence of whole blood and normal t lymphocyte subsets in severe nodular Acne and Acne Fulminans
    Acta Dermato-venereologica, 1995
    Co-Authors: S L Karvonen, Liisa Rasanen, E Soppi, Heikki Hyoty, Matti Lehtinen, Timo Reunala
    Abstract:

    To investigate the inflammatory and immunological aspects of severe Acne, we examined the luminol-enhanced chemiluminescence of whole blood, T-cell subsets and natural killer cell functions in 11 patients with severe nodular Acne and 4 patients with Acne Fulminans. In patients with severe nodular Acne, the active phase of the disease, compared to the values in remission (means 47 mV, SD 24.8 and 32 mV, SD 8.3, p < 0.05). The patients with Acne Fulminans also showed high values in the active phase of the disease (mean mV 68.3, SD3.5) compared to remission (mean 30.5 mV, SD 15.3). No marked alterations were seen in the percentages of T-helper cells, T-suppressor cells or DR-positive lymphocytes or in the levels of soluble interleukin 2 receptor. The percentages and activities of natural killer cells did not show any significant changes either. Five patients (4 with severe nodular Acne and one with Acne Fulminans, accounting for 33% of all patients) carried HLA Cw6 antigen, which is a significantly increased frequency compared to health controls (pc = 0.015). The present chemiluminescence results suggest that peripheral blood neutrophils are activated in patients with severe Acne.

  • bone disease in adolescents with Acne Fulminans and severe cystic Acne radiologic and scintigraphic findings
    American Journal of Roentgenology, 1994
    Co-Authors: L S Laasonen, S L Karvonen, T L Reunala
    Abstract:

    Acne Fulminans is an uncommon form of ulcerative Acne with acute onset. It usually affects adolescent boys who have associated musculoskeletal pain and septic fever. Osteolytic bone lesions have been reported in these patients. Severe cystic Acne occurs almost equally in both sexes, but it has a less dramatic clinical course than Acne Fulminans and rarely causes ulcerative skin lesions and systemic symptoms. In this study we investigated the imaging features of bone lesions associated with Acne Fulminans and determined if patients with severe cystic Acne have similar bone lesions.From 1970 through 1991, 24 patients with Acne Fulminans were treated in the dermatologic departments of Finnish hospitals. Radiologic (plain radiographs or conventional tomograms) or scintigraphic data were available for 21 patients and analyzed retrospectively. For comparison, 20 consecutive patients with severe cystic Acne were examined prospectively with scintigraphy.Ten patients (48%) with Acne Fulminans had lytic bone lesion...

  • delayed hypersensitivity to propionibacterium Acnes in patients with severe nodular Acne and Acne Fulminans
    Dermatology, 1994
    Co-Authors: S L Karvonen, W.j. Cunliffe, L. Räsänen, K T Holland, Jaakko Karvonen, Timo Reunala
    Abstract:

    Background: Increased hypersensitivity reactions to Propionibacterium Acnes may be involved in the pathogenesis of severe Acne. Objective: To study delayed and immediate hypersensitivity reactions to P. Acnes in patients with severe nodular Acne (SNA) and Acne Fulminans (AF). Methods: We performed lymphocyte stimulation and skin tests for P. Acnes antigens on 11 patients with SNA and 7 patients with AF. Results: The patients with SNA had similar mean lymphocyte stimulation indices (mean 13.96, SD 8.6) to P. Acnes during active disease as had healthy controls (12.63, SD 6.46). After the treatment the mean stimulation index was significantly elevated (23.47, SD 13.84, p = 0.006). A similar increase occurred in the patients with AF (mean 17.04, SD 5.74, and 33.42, SD 27.17, respectively). Two of 7 patients with SNA and 3 of the 7 patients with AF but none of the 10 control subjects showed positive 48-hour intradermal tests to P. Acnes. Conclusion: Specific cell-mediated immunity to P. Acnes increases during the course of severe inflammatory Acne.

Timo Reunala - One of the best experts on this subject based on the ideXlab platform.

  • increased chemiluminescence of whole blood and normal t lymphocyte subsets in severe nodular Acne and Acne Fulminans
    Acta Dermato-venereologica, 1995
    Co-Authors: S L Karvonen, Liisa Rasanen, E Soppi, Heikki Hyoty, Matti Lehtinen, Timo Reunala
    Abstract:

    To investigate the inflammatory and immunological aspects of severe Acne, we examined the luminol-enhanced chemiluminescence of whole blood, T-cell subsets and natural killer cell functions in 11 patients with severe nodular Acne and 4 patients with Acne Fulminans. In patients with severe nodular Acne, the active phase of the disease, compared to the values in remission (means 47 mV, SD 24.8 and 32 mV, SD 8.3, p < 0.05). The patients with Acne Fulminans also showed high values in the active phase of the disease (mean mV 68.3, SD3.5) compared to remission (mean 30.5 mV, SD 15.3). No marked alterations were seen in the percentages of T-helper cells, T-suppressor cells or DR-positive lymphocytes or in the levels of soluble interleukin 2 receptor. The percentages and activities of natural killer cells did not show any significant changes either. Five patients (4 with severe nodular Acne and one with Acne Fulminans, accounting for 33% of all patients) carried HLA Cw6 antigen, which is a significantly increased frequency compared to health controls (pc = 0.015). The present chemiluminescence results suggest that peripheral blood neutrophils are activated in patients with severe Acne.

  • delayed hypersensitivity to propionibacterium Acnes in patients with severe nodular Acne and Acne Fulminans
    Dermatology, 1994
    Co-Authors: S L Karvonen, W.j. Cunliffe, L. Räsänen, K T Holland, Jaakko Karvonen, Timo Reunala
    Abstract:

    Background: Increased hypersensitivity reactions to Propionibacterium Acnes may be involved in the pathogenesis of severe Acne. Objective: To study delayed and immediate hypersensitivity reactions to P. Acnes in patients with severe nodular Acne (SNA) and Acne Fulminans (AF). Methods: We performed lymphocyte stimulation and skin tests for P. Acnes antigens on 11 patients with SNA and 7 patients with AF. Results: The patients with SNA had similar mean lymphocyte stimulation indices (mean 13.96, SD 8.6) to P. Acnes during active disease as had healthy controls (12.63, SD 6.46). After the treatment the mean stimulation index was significantly elevated (23.47, SD 13.84, p = 0.006). A similar increase occurred in the patients with AF (mean 17.04, SD 5.74, and 33.42, SD 27.17, respectively). Two of 7 patients with SNA and 3 of the 7 patients with AF but none of the 10 control subjects showed positive 48-hour intradermal tests to P. Acnes. Conclusion: Specific cell-mediated immunity to P. Acnes increases during the course of severe inflammatory Acne.

Om Schofield - One of the best experts on this subject based on the ideXlab platform.

  • G49 Acne Fulminans induced by oral isotretinoin
    Archives of Disease in Childhood, 2014
    Co-Authors: Ai Mackenzie, Cg Sinclair, Om Schofield
    Abstract:

    A 13 year-old male presented with a one year history of Acne vulgaris, unresponsive to six months of lymecycline, with comedones, pustules and a few small scarring cysts of his face, chest and upper back. He had a history of motor dyspraxia for which he was under neurology review. He was commenced on oral isotretinoin at 20 mg (0.33 mg/kg/day) and reviewed at one month and found to have significantly deteriorated with tender confluent abscesses, ulceration and necrosis of the trunk associated with cervical lymphadenopathy and general malaise. A diagnosis was made of Acne conglobata, oral isotretinoin was discontinued and he was treated with 20 mg prednisolone, clobetasol propionate cream and antiseptics. His condition continued to deteriorate with pyrexia, arthralgia, elevated inflammatory markers and a marked neutrophilia. A diagnosis of Acne Fulminans was made at this stage; and prednisolone was increased to 60 mg (approximately 1 mg/kg/day), combined with erythromycin 2 g/day, which significantly improved both the cutaneous lesions and systemic symptoms. After one month, oral isotretinoin was cautiously re-introduced at 10 mg daily and was gradually increased to a maximum of 70 mg daily (1 mg/kg). Oral steroids were slowly reduced and stopped after 11 months. Non-classical congenital adrenal hyperplasia was excluded with a normal 17-OH level after completing the course of oral corticosteroids. Oral isotretinoin was discontinued after a total cumulative dose of 150 mg/kg. He now has no active Acne vulgaris but has been left with significant scarring. Acne Fulminans is an unusual condition of sudden onset with nodulocystic lesions evolving into haemorrhagic nodules and plaques that rapidly undergo suppuration and necrotic degeneration with extensive scarring. Systemic involvement includes pyrexia, cachexia, weakness, arthralgia/arthritis, myalgia, erythema nodosum and hepatosplenomegaly. 1 The aetiology is unclear in many cases, with bacterial infection, abnormal immunological reaction, drug-induced mechanisms and underlying endocrinopathies having been hypothesised. 1,2 We report this case to highlight a very rare but devastating complication of oral isotretinoin. References Jansen T, Plewig G. Acne Fulminans. Int J Dermatol. 1998; 37:254–25 Melnik B, Jansen T, Grabbe S. Abuse of anabolic-androgenic steroids and bodybuilding ache: An underdestimated health problem. J Dtsch Dermatol Ges. 2007; 5:110-117