2018/Arthritis Rheumatol. 2018 Oct;70(10):1698.

Mertz P, Herber M, Jeannel J, Korganow AS, Guffroy A.

The patient, a 69‐year‐old woman with asthenia, anorexia, and weight loss of several weeks’ duration, was referred for a subacute onset of painful permanent acrocyanosis of the arms and legs without Raynaud’s phenomenon. The patient had concomitantly developed grade I/IV dyspnea. Physical examination revealed livedoid acrocyanosis of distal phalanges of the hands (A), associated with flame‐shaped hemorrhages (B) (arrowheads) and dilated capillaries at the proximal nailfolds (B) (arrow), Gottron’s sign over the metacarpophalangeal and proximal interphalangeal joints, and a discrete heliotrope rash with a palpable V‐shaped rash on the upper chest and forehead. Fine bibasilar crackles were present, and computed tomography showed interstitial lung disease (ILD) (C). Immunodot assay revealed anti–PL‐12 antibodies. Anti–PL‐12 syndrome is usually described as an amyopathic cluster in anti–aminoacyl–transfer RNA synthetase syndrome (ARS). Pulmonary involvement is the most common leading manifestation, with most cases presenting as isolated ILD with dyspnea 1, 2. Acute ischemic phenomena are rare in ARS and are generally associated with a history of Raynaud’s phenomenon. In our case, the discovery of vascular abnormalities of the arms and legs is what led us to the diagnosis of ARS. Little is known about the pathogenesis of subacute vascular manifestations in ARS. It might be due to several causes of immune‐mediated vasculopathy 3. The patient’s clinical manifestations initially responded well to high‐dose steroid therapy and prostaglandin infusion (D), but she experienced a recurrence with cold weather on day 28 (E and F). The treatment regimen was replaced with intravenous pulse cyclophosphamide and bosentan with a satisfactory outcome by day 63 (G–I). In addition to classic dermatologic manifestations, such as Gottron’s papules, mechanic hands, or cuticular overgrowth, physicians should note the existence of uncommon acral manifestations, such as ischemic symptoms, that can also indicate ARS and an increased risk of developing necrosis.


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