36º Congresso Brasileiro de Reumatologia

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Dermatomyositis is a systemic autoimmune disease characterized by symmetrical and proximal limb muscle weakness associated with typical cutaneous lesions such as the heliotrope and Gottron's papules. Rare cutaneous manifestations include vesicobolous lesions, erosive lesions and erythroderma. KUSANO (2013) was the first Brazilian author to describe a case of erythrodermic dermatomyositis, since there were no records of new cases.

Case report

L.C.D, female, black, 59 years old, reports having started periorbital edema for 4 months, with no other associated complaints and maintained even after use of oral antihistamines. It evolved with edema and erythroderma on the whole face, followed by vesicobolous formations and pruritus diffusely through the body (Figure 1), including scalp, sparing mucosal involvement. At examination, the vesicles were of varying sizes, tense and with serous content. In addition, she had alopecia, periungual erythema (Figure 2) and heliotrope (Figure 3). Initially, the patient had a histopathological diagnosis of pemphigus vulgaris (PV). Prednisone was prescribed at a dose of 1 mg / kg / day and topical clobetasol propionate, with partial improvement of the vesicobolous lesions. After 3 months with this therapy, she complained of proximal upper limb muscle weakness and pain in the shoulder girdle with limited movement. Laboratory tests: CPK 2309; CK-MB 92; PCR 34.3; FAN reagent. Magnetic resonance imaging of the shoulder revealed muscular edema in the myofascial and subcutaneous planes of diffuse form involving the deltoid, trapezius and muscles of the rotator cuff, findings suggestive of inflammatory myopathy. A new skin biopsy revealed bullous dermatitis with a subepidermal cleavage plane, distancing the diagnosis of PV. Deltoid biopsy revealed atrophy of muscle fibers, occasional foci of chronic inflammatory infiltrate with perimical fibrosa, characteristic histological alterations of DM. Pulse therapy with methylprednisolone 1g / day for 3 consecutive days, followed by maintenance with prednisone 1mg kg / mg / day. It evolved with gradual regression of dermatological lesions, as well as recovery of muscle strength. Receiving hospital discharge after clinical stabilization. Followed by outpatient follow-up, with good control of the disease.


The case report describes an extremely unusual presentation of dermatomyositis, with erythroderma and vesicoholic lesions as the predominant and initial manifestation of the disease, which made early diagnosis difficult, given the innumerable hypotheses raised before the condition. Therefore, it aims to draw attention to an adequate etiological investigation of a framework of proximal muscular weakness, as well as its therapeutic management with good results.



Rare Diseases


Andreia Coimbra Sousa, Raquel Moraes da Rocha Nogueira, Tassia Oliveira Nunes da Silva, Tâmara Aroucha Matos, Thiago Igor Aranha Gomes, Adriana Medeiros Lima, Fernando César Costa da Silva, Braulio Nunes de Souza Martins Filho, Lynara Pinheiro Moraes