Treatment of relapsing polychondritis with dapsone

Barranco, Vincent P.

doi:10.1001/archderm.112.9.1286

Cited by 31 publications

(9 citation statements)

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“…Dapsone and colchicine may also be used in these patients. Dapsone in doses of 50 to 200 mg/day has been advocated as an effective initial therapy in patients without cardiorespiratory involvement [69][70][71] but Trentham et al [3] observed that it was not effective in most patients and resulted in a number of adverse reactions. Organ-threatening disease, including severe polychondritis, ocular or laryngotracheal involvement and systemic vasculitis require systemic corticosteroids.…”

Section: Managementmentioning

confidence: 99%

Relapsing polychondritis: a review

et al. 2013

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Relapsing polychondritis is a rare multisystem disease involving the cartilaginous and proteoglycan rich structures. The spectrum of clinical presentations may vary from intermittent episodes of painful and often disfiguring auricular and nasal chondritis, to occasional organ or even lifethreatening manifestations like airway collapse. There is lack of awareness about this disease due to its rarity. Relapsing polychondritis disease activity index has recently been validated and may help in clinical decision making and research. This article reviews the literature on this disease entity.

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Section: Managementmentioning

confidence: 99%

Relapsing polychondritis: a review

et al. 2013

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“…Zahlreiche immunsuppressive Therapien wie Azathioprin, Cyclophosphamid, Cyclosporin, Methotrexat, Plasmapherese, Dapsone sind mit unterschiedlichem Erfolg als Mono-und Kombinationstherapie eingesetzt worden, wobei aufgrund der geringen Fallzahlen keine verlässlichen Therapiestudien vorliegen [33][34][35][36][37]. In den letzten Jahren wurde das Behandlungskonzept um die "Bi-…”

Section: Relapsing Polychondritis · Vasculitisunclassified

Akuter Mesenterialinfarkt bei Polychondritis

et al. 2006

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We report about a 43-year old woman with relapsing polychondritis, admitted with progressive malaise, fatigue, anorexia and profound weight loss. Two years ago a nasal chondritis with characteristic changes of the nasal profil, scleritis, laryngitis and unspecific general symptoms (fever, fatigue, signs of a systemic inflammation) developed and relapsing polychondritis was diagnosed. The patient was treated initially with azathioprine followed by methotrexate in combination with ciclosporine and glucocorticoids. During the hospitalization her condition slowly worsened, and crampy abdominal pain developed subsequently with massive tenderness, rebound and guarding. The clinically presumed diagnosis of ileus and peritonitis was radiomorphologically confirmed with signs of enteric perforation and a laparatomy was performed. A complete occlusion of the A. mesenterica superior and stenosis of the truncus coeliacus were diagnosed followed by a revascularization with an aorto-mesenteric bypass and subsequent resection of the necrotic ischemic ileum. An association with various autoimmune disorders including vasculitic syndromes is well known for relapsing polychondritis. The case demonstrated a progressive mesenteric ischemia with the acute exacerbation caused by a vasculitic mesenterial occlusion. The unusual presentation should be considered in the differential diagnosis of uncommon abdominal symptoms during the course of relapsing polychondritis.

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“…The dramatic efficacy of dapsone, both in RP [12,13] and in DH [14] merits con sideration. Dapsone most likely acts in DH via inhibition of myeloperoxidase in poly morphonuclear leukocytes [15], which prob ably plays a role in the bullous lesion.…”

Section: Commentmentioning

confidence: 99%

Dermatitis herpetiformis Associated with Relapsing Polychondritis

Laurent

Humbert²,

Fellman³

1988

Dermatology

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A 35-year-old woman developed typical relapsing polychondritis within a year following the onset of dermatitis herpetiformis. This occurred during a reduction in the dapsone dose regimen. To our knowledge, the coexistence of these two conditions in a single patient has not yet been reported in the literature. Both the parallel course and the good response to dapsone suggest a pathogenic link between these two diseases.

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Treatment of relapsing polychondritis with dapsone

Abstract: Three patients with relapsing polychondritis were treated successfully with dapsone. Mounting evidence suggests an immune-related pathogenesis for relapsing polychondritis. Dapsone may be effective in certain immune-related diseases because of its inhibition of lysosomal enzymes.

Cited by 31 publications

References 0 publications

Relapsing polychondritis: a review

Relapsing polychondritis: a review

Akuter Mesenterialinfarkt bei Polychondritis

Dermatitis herpetiformis Associated with Relapsing Polychondritis

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