Vasodilation is not the only approach to the management of cutaneous ulceration in systemic sclerosis

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Abstract

Digital ulceration (DU) is a major cause of pain, decreased work capacity and increased reliance on other in systemic sclerosis (SSc) [1]. Phosphodiesterase inhibitors and endothelin receptor antagonist therapy have an important role in SSc-DU management [2]. A 49-year-old lady with a 9-year history of diffuse cutaneous SSc presented with recurrent painful DU despite treatment with diltiazem, ramipril and candesartan. The addition of sildenafil, and subsequently bosentan, did not accelerate DU healing or prevent the emergence of new painful DUs (Fig. 1A). The identification of significant calcinosis cutis (Fig. 1B) led to commencement of minocycline (100 mg bd) which achieved rapid (within 4 months), complete and sustained healing of all active ulceration with no further new cutaneous ulceration (Fig. 1C). Treatment was complicated by minocycline-induced hyperpigmentation (Fig. 1D), but the patient did not wish to discontinue treatment. The case highlights the need to consider aetiological drivers of SSc-DU when considering management. Vasodilator therapy forms the mainstay of treatment of ischaemic SSc-DU, but evidence-based strategies for the prevention and management of SSc-DU in which mechanical factors and/or calcinosis cutis contribute to aetiopathogenesis are lacking. Minocycline is a treatment option for calcinosis-related SSc-DU that is often overlooked in recommendations on SSc-DU management.
Original languageEnglish
Pages (from-to)1559
Number of pages1
JournalRheumatology
Volume56
Issue number9
Early online date9 May 2017
DOIs
Publication statusPublished - 1 Sep 2017

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Systemic Scleroderma
Vasodilation
Skin
Calcinosis
Minocycline
Therapeutics
Ramipril
Diffuse Scleroderma
Hyperpigmentation
Phosphodiesterase Inhibitors
Diltiazem
Vasodilator Agents
Pain

Keywords

  • Journal Article

Cite this

Vasodilation is not the only approach to the management of cutaneous ulceration in systemic sclerosis. / Pauling, John D.

In: Rheumatology, Vol. 56, No. 9, 01.09.2017, p. 1559.

Research output: Contribution to journalArticle

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abstract = "Digital ulceration (DU) is a major cause of pain, decreased work capacity and increased reliance on other in systemic sclerosis (SSc) [1]. Phosphodiesterase inhibitors and endothelin receptor antagonist therapy have an important role in SSc-DU management [2]. A 49-year-old lady with a 9-year history of diffuse cutaneous SSc presented with recurrent painful DU despite treatment with diltiazem, ramipril and candesartan. The addition of sildenafil, and subsequently bosentan, did not accelerate DU healing or prevent the emergence of new painful DUs (Fig. 1A). The identification of significant calcinosis cutis (Fig. 1B) led to commencement of minocycline (100 mg bd) which achieved rapid (within 4 months), complete and sustained healing of all active ulceration with no further new cutaneous ulceration (Fig. 1C). Treatment was complicated by minocycline-induced hyperpigmentation (Fig. 1D), but the patient did not wish to discontinue treatment. The case highlights the need to consider aetiological drivers of SSc-DU when considering management. Vasodilator therapy forms the mainstay of treatment of ischaemic SSc-DU, but evidence-based strategies for the prevention and management of SSc-DU in which mechanical factors and/or calcinosis cutis contribute to aetiopathogenesis are lacking. Minocycline is a treatment option for calcinosis-related SSc-DU that is often overlooked in recommendations on SSc-DU management.",
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AB - Digital ulceration (DU) is a major cause of pain, decreased work capacity and increased reliance on other in systemic sclerosis (SSc) [1]. Phosphodiesterase inhibitors and endothelin receptor antagonist therapy have an important role in SSc-DU management [2]. A 49-year-old lady with a 9-year history of diffuse cutaneous SSc presented with recurrent painful DU despite treatment with diltiazem, ramipril and candesartan. The addition of sildenafil, and subsequently bosentan, did not accelerate DU healing or prevent the emergence of new painful DUs (Fig. 1A). The identification of significant calcinosis cutis (Fig. 1B) led to commencement of minocycline (100 mg bd) which achieved rapid (within 4 months), complete and sustained healing of all active ulceration with no further new cutaneous ulceration (Fig. 1C). Treatment was complicated by minocycline-induced hyperpigmentation (Fig. 1D), but the patient did not wish to discontinue treatment. The case highlights the need to consider aetiological drivers of SSc-DU when considering management. Vasodilator therapy forms the mainstay of treatment of ischaemic SSc-DU, but evidence-based strategies for the prevention and management of SSc-DU in which mechanical factors and/or calcinosis cutis contribute to aetiopathogenesis are lacking. Minocycline is a treatment option for calcinosis-related SSc-DU that is often overlooked in recommendations on SSc-DU management.

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