Extrafacial edema is a rare manifestation of dermatomyositis (DM). We report 2 cases.

Case no. 1. The patient was an 86-year-old woman who was admitted with dysphagia, dyspnea, weakness in her 4 extremities (2/5) and edemas. Four years earlier, she had been diagnosed with cryptogenic organizing pneumonia detected by means of pulmonary function tests, imaging studies and histology. It was observed that, aside from periorbital edema, she had edema with fovea in upper and lower limbs. She had plaques with vesiculobullous eruption on her forearms and erythematous and edematous eruption on her thighs (Fig. 1). Biopsy of the thigh revealed epidermal atrophy, subepidermal bullous disease, necrotic keratinocytes, superficial perivascular infiltrate, mucin and dermal edema. Direct immunofluorescence was negative, as were tests for anti-Jo-1, anti-PL7, anti-PL12, anti-SRP-54, anti-Mi2, anti-Ku and anti-PM/Scl antibodies. Creatine kinase (CK) was 1267U/L. Electromyogram demonstrated the myopathic changes and muscle biopsy revealed perifascicular atrophy and CD4+perimysium and perivascular inflammatory infiltrate.

Fig. 1.

(a) Erythematous and edematous plaque with superficial vasiculobullae in right upper limb. (b) Edema and erythematous and edematous plaques on thighs.

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A cardiac origin of the edema was ruled out, as were renal, hepatic and thyroid origin. Albumin, after multiple hospital admissions for dyspnea, weakness and edemas, had decreased (2.5–3.2g/dL) and reached normality after treatment with corticosteroids. Having reviewed her history, we finally discovered the diagnosis of her lung disease: an elevated CK level (1370U/L). Computed tomography revealed a breast lesion that was found to be an infiltrating ductal carcinoma. She was treated with prednisone at a dose of 1mg/kg body weight (bw)/day and 5 sessions of plasmapheresis. After mastectomy, dysphagia persisted and we detected a diffuse disseminated signet ring cell adenocarcinoma. She died 4 months later due to respiratory failure.

Case no. 2. The patient was a 71-year-old man who had been treated 7 months earlier for cancer of the cavum. He presented with weakness of his upper (2/5) and lower (3/5) limbs, skin lesions and migratory swelling: it began in his neck and supraclavicular region, followed by ankles, abdomen and upper limbs. Other causes of edema were ruled out. His CK level was 728U/L and the same antibody panel was negative. Electromyogram and muscle biopsy were similar to those of case no. 1. A week with prednisone at a dose of 1mg/kg bw/day, resolved the edema; treatment was increased with methotrexate at 20mg/week, but he died 7 months later due to muscular respiratory failure.

Edematous DM1–5 could constitute up to 6% of the cases of DM.1 It is characterized by extrafacial, localized or generalized edema, with or without fovea, with no other cause of edema. It is more prevalent in women (2:1), of ages between 23 and 93 years. The diagnostic delay is more than 2 months.2 It is speculated that it is originated by ischemia of the microvasculature. To date, the migratory localization so characteristic of our case had not been reported. Vesiculobullous DM consists of subepidermal vesiculobullae with biopsy results typical of DM, with no other bullous dermatosis. Its cause has been proposed to be friction, dermal edema and mucin. The specimen for the biopsy in the first case was taken from the patient's thigh, where no clinical detachment was observed. We subsequently considered that the detachment was secondary to the edema and that the two clinical forms, edematous and vesiculobullous, could be the same manifestation or overlap. The distribution does not indicate a mechanical cause and there are similar cases of severe edema in which detachment appeared.5–7

The cause of hypoalbuminemia of the first case was due to the proinflammatory status of the interstitial lung disease and the DM that still had not been diagnosed.8,9 This hypoalbuminemia could be the cause of generalized edema, but not of the erythematous and edematous and vesiculobullous plaques. The literature demonstrates that edematous DM could be associated with a more severe myopathy and dysphagia, a finding that was not observed in our patients.1–3 Likewise, vesiculobullous DM would be closely associated with neoplasms and a worse prognosis.6,10 Extrafacial edema in DM, in any of its variants should alert physicians to a greater severity and lead to an active search for a neoplasm.