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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.81 no.2 Rio de Janeiro Mar./Apr. 2006
Efficacy of dapsone in two cases of amyopathic dermatomyositis*
Liliana GalrãoI; Jean RealeII; Isabella LimaIII; Mittermayer SantiagoIV
IRheumatologist at the Center for Rheumatology of the Hospital Santa
Izabel HSI Salvador (BA), Brazil
IIMedical student at Universidade Federal da Bahia UFBA - Salvador (BA), Brazil
IIIRheumatologist at the Center for Rheumatology of the Hospital Santa Izabel HSI Salvador (BA), Brazil
IVAssociate Professor at Bahia School of Medicine and Public Health EBMSP and Coordinator of Rheumatology at the Center for Rheumatology of the Hospital Santa Izabel HSI Salvador (BA), Brazil
Dapsone is a drug primarily used for the treatment of Hansens disease, and it has also been employed in cases of bullous lupus erythematosus and some types of cutaneous vasculitis. Recently, its efficacy in the treatment of cutaneous lesions in dermatomyositis has been observed. We present two cases of dermatomyositis, amyopathic form, which were refractory to habitual treatment, but had an excellent response to dapsone therapy.
Keywords: Autoimmune diseases; Dapsone; Dermatomyositis
Cutaneous manifestations of dermatomyositis (DM) often persist after therapy with steroids, immunosuppressing agents, antimalarian drugs or their combination.1,2 For half a century, dapsone has been used for its anti-inflammatory properties, particularly aimed at leukocytes, for the treatment of Hansens Disease and autoimmune diseases, such as bullous lupus erythematosus and relapsing polychondritis. Literature has shown some reports of DM with good response of its cutaneous manifestations to the use of dapsone. We hereby present two patients with refractory amyopathic dermatomyositis, who responded to treatment with dapsone.
Fifty five-year-old white male patient, who presented, four years ago, erythematous pruritic cutaneous eruptions, with the finding of photosensitivity in the face and upper trunk, shawl Sign and V-Sign on physical examination, besides heliotrope and Gottrons Sign. Muscle strength was preserved (degree: 5+/5+). Histopathological study of the skin revealed an epidermis with discrete acanthosis, edematous dermis with mononuclear infiltrate surrounding vessels and annexes, besides decrease of pillous follicles. Antinuclear factor (ANF) was negative, and Creatinophosphokinase (CPK) values were within normal range. No muscle biopsies or electroneuromyography (ENMG) were carried out. The case was diagnosed as amyopathic dermatomyositis, and treatment with chloroquine diphosphate at 250 mg/day was begun, without satisfactory response. Dapsone at 50 mg/day was then introduced and after two months the dose was built up to 100 mg/day, with a significant improvement. Later on, patient had the onset of an insidious ventilatory-dependent thoracic pain associated with weight loss, with the finding, on tomographic investigation, of a pulmonary nodule, the histopathological examination of which revealed an adenocarcinoma.
Forty three-year-old white female patient, with the onset, 7 years previously, of a dermatological picture characterized by an erythematous eruption, with findings on clinical examination of photosensistivity in the face, arms and cervical regions (Shawl Sign). Face eruption was diffuse, with heliotrope. Patient also presented Gottrons Sign in metacarpal-phalangeal and proximal interphalangeal joints, and slight allopecia. There was not, in any moment, any complaint of muscle weakness, with the observation of preserved muscle strength on physical examination (degree: 5+/5+). ANF was positive at 1:160 dillution, with a fine speckled pattern. Anti-SSA and anti-SSB antibodies were negative. Histopathological examination revealed normal muscles, and overlying skin likewise. Several CPK and aldolase assays were performed during follow-up, with all values within normal ranges. No histopathological examinations of the affected skin or ENMGs were carried out. The case was diagnosed as amyopathic dermatomyositis, and treatment was initiated with prednisone at 5 mg/day and hydroxichloroquine at 400 mg/day, which had no efficacy for the improvement of the cutaneous picture. Dapsone was then begun at 50 mg/day, and dose increased to 100 mg/day after two months, as no satisfactory results had been obtained with lower dosages. After the increase to 100mg, patient obtained a progressive improvement in the course of the following 12 months, and the medication was then withdrawn with no relapses.
Amyopathic dermatomyositis can make up to 10% of DM cases, and it is defined as an autoimmune disease with typical manifestations of DM, with no clinical evidences for proximal muscle weakness and with normal muscle enzymes.3 Treatment of DM cutaneous manifestations with antimalarian and immunosuppressing drugs is not always efficable. Dapsone (4,4-diaminodiphenylsulphone) has an anti-inflammatory action, for inhibiting polimorphonuclear leukocytes and the activation of the alternative pathway of the complement cascade.4 Having been first used in the treatment of Hansens Disease, it has also been used as a second-line agent in the treatment of cutaneous manifestations of autoimmune diseases, such as cutaneous lupus,5 cutaneous vasculites, bullous dermatites and herpes-like dermatitis.1 In 1994, Konohana e Kawashima reported, in a patient with a classical refractory dermatomyositis, an improvement of cutaneous eruption and myositis, with decrease in CPK serum levels after two weeks of daily use of 75 mg of dapsone.6 Later on, Cohen described a satisfactory response to dapsone in two patients who had been diagnosed with oligomyopathic and amyopathic dermatomyosites, the tratement of wich has been inefficable with corticosteroids and immunosupressing agents.2 In both cases presented here of chloroquine-refractory amyopathic dermatomyositis, after excluding the possibility of G-6-PD deficiency, we were able to prove the efficacy of such drug for this condition, even in a patient who had DM probably secondarily to a neoplasia. These data allow us to conclude that dapsone can be used for the treatment of DM cutaneous manifestations which do not respond to therapy with chloroquine.
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2. Cohen JB. Cutaneous involvement of dermatomyositis can respond to dapsone therapy. Int J Dermatol. 2002;41:182-4. [ Links ]
3. Sontheimer R. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol. 1999;11:475-82. [ Links ]
4. Coleman MD. Dapsone: models of action, toxicity, and possible strategies for increasing patient tolerance. Br J Dermatol. 1993;129:507-13. [ Links ]
5. Tsutsui K, Imai T, Hatta N, Sakai H, Takata M, Takehara K. Widespread pruritic plaques in a patient with subacute lupus erythematosus and hypocomplementemia: response to dapsone therapy. J Am Acad Dermatol. 1996;35:133-5. [ Links ]
6. Konohana A, Kawashima J. Successful treatment of dermatomyositis with dapsone. Clin Exp Dermatol. 1994;69:367. [ Links ]
Liliana DAlmeida Galrão
Núcleo de Reumatologia do Hospital Santa Izabel
Praça Almeida Couto, 500 - Nazaré
40000-000 - Salvador - BA
Tel./Fax: (71) 3326-5276
on January 05, 2005.
Approved by the Consultive Council and accepted for publication on September 27, 2005.
Conflict of interest: None
* Work done at Center for Rheumatology at Hospital Santa Izabel - HSI - and Bahia School of Medicine and Public Health EBMSP - Salvador (BA), Brazil.