We present a rare case of a woman diagnosed with dermatomyositis associated with interstitial lung disease as a manifestation preceded the onset of poikilodermatosis. This presented as a generalized skin rash characterized by confluent lesions, which later was replaced by hyperpigmented and hypopigmented macules (Fig. 1), dryness and scaling, as well as alopecia. Four months later melanonychia appeared, as well as heliotrope erythema, periorbital edema, and Gottron's papules on the proximal and distal interphalangeal joints of both hands (Fig. 2), progressive muscle weakness with a symmetric, proximal distribution and weight loss of 10kg.

Fig. 1.

Image of the legs with the characteristic alternating hypo and hyperpigmented macules of the poikilodermatosis.

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Fig. 2.

Image of the hands showing Gottron's papules on the metacarpophalangeal joints superimposed on the poikilodermatosis hypopigmented lesions.

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The patient then presented dysphagia and dyspnea progressing to orthopnea, so she was admitted to the hospital. The most interesting laboratory results showed: creatinine 203U/l, lactic dehydrogenase 698U/l, aspartate transaminase 304U/l, erythrocyte sedimentation rate 20mm/h, rheumatoid factor 57IU/l, C-reactive protein 3.7mg/dl, complement fractions C3 55mg/dl and C4 11.1mg/dl.

Computed tomography revealed: interstitial lung disease, emphysematous bullae, bronchiectasis and areas of pulmonary fibrosis. During the hospital stay she developed pneumonia associated with mechanical ventilation and septic shock leading to her death.

The poikilodermatosis is a rare sign of dermatomyositis and may be the initial manifestation of the disease,1–5 as the case above described.