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Martorell hypertensive ischemic leg ulcer must not be confounded with pyoderma gangrenosum: Management is totally different


Hafner, Jürg; Nobbe, Stephan; Läuchli, Severin; Kerl, Katrin; French, Lars E; Pelivani, Nedzimin; Böhler, Kornelia; Mayer, Dieter; Senet, Patricia (2013). Martorell hypertensive ischemic leg ulcer must not be confounded with pyoderma gangrenosum: Management is totally different. Reviews in Vascular Medicine, 1(1):5-8.

Abstract

Introduction: Martorell hypertensive ischemic leg ulcer (HYTILU) and pyoderma gangrenosum share a violaceous, rapidly progressive necrotic wound border and excruciating pain as essential clinical features. Clinicians unaware of Martorell HYTILU readily misdiagnose pyoderma gangrenosum or necrotic vasculitis. This can be detrimental since disease management is totally different.
Material and methods: This review includes two patient series with Martorell hypertensive ischemic leg ulcer (HYTILU), 31 patients from the University Hospital of Zurich and 64 patients from a randomized controlled trial in France. The analysis of confounding Martorell HYTILU and histological study is based on the Zurich data and the analysis of therapeutic issues is based on the French and Zurich data.
Results: Fifty-two percent (16/31 patients) were referred with an erroneous diagnosis of pyoderma gangrenosum, and another 19% (6/31 patients) with an erroneous diagnosis of necrotizing vasculitis. The hallmark of Martorell HYTILU are the location at the laterodorsal leg or Achilles tendon (in the present series 100%) in a patient with usually long-standing and controlled hypertension (95–100%), often accompanied by diabetes (40–60%). Histology of a several centimeter long, 4–6 mm narrow, and deep (to fascia) performed spindle-shape skin and ulcer biopsy shows a skin infarction with sclerotic subcutaneous arterioles. Arteriolosclerosis is defined by thick vessel walls at the cost of a narrow lumen, and in approximately 70% of histologic sections part of the arterioles show a striking medial calcification. The narrow lumen may be occluded by acute or organized thrombosis. In the French multicenter serie, at 8 weeks only 10% (3/31 patients) healed by conservative means, and this could not be improved with the use of topical application of platelet-derived growth factor-BB (becaplermin) (18% healing, 5/28 patients, no significant difference). In the Zurich series, 84% of patients (26/31) had wound surgery (necrosectomy, split skin graft) to heal the ulcer and 10% (3/31) died from wound infection and sepsis, amongst two under immunosuppression received for misdiagnosed pyoderma gangrenosum.
Conclusions: Martorell HYTILU can easily be confounded with pyoderma gangrenosum or necrotizing vasculitis. Physicians involved in wound treatment should be sensitized to the typical clinical setting: a progressive and extremely painful skin infarction at the laterodorsal leg in a hypertensive and often diabetic subject. Diagnosis is confirmed histologically on a long-enough, narrow, but deep spindle-shape skin and ulcer biopsy, showing a highly characteristic form of stenotic and occlusive subcutaneous arteriolosclerosis. Management is essentially based on wound surgery. Conservative means are almost uneffective. Immunosuppression must be avoided, and antibiotic treatment introduced as required.

Abstract

Introduction: Martorell hypertensive ischemic leg ulcer (HYTILU) and pyoderma gangrenosum share a violaceous, rapidly progressive necrotic wound border and excruciating pain as essential clinical features. Clinicians unaware of Martorell HYTILU readily misdiagnose pyoderma gangrenosum or necrotic vasculitis. This can be detrimental since disease management is totally different.
Material and methods: This review includes two patient series with Martorell hypertensive ischemic leg ulcer (HYTILU), 31 patients from the University Hospital of Zurich and 64 patients from a randomized controlled trial in France. The analysis of confounding Martorell HYTILU and histological study is based on the Zurich data and the analysis of therapeutic issues is based on the French and Zurich data.
Results: Fifty-two percent (16/31 patients) were referred with an erroneous diagnosis of pyoderma gangrenosum, and another 19% (6/31 patients) with an erroneous diagnosis of necrotizing vasculitis. The hallmark of Martorell HYTILU are the location at the laterodorsal leg or Achilles tendon (in the present series 100%) in a patient with usually long-standing and controlled hypertension (95–100%), often accompanied by diabetes (40–60%). Histology of a several centimeter long, 4–6 mm narrow, and deep (to fascia) performed spindle-shape skin and ulcer biopsy shows a skin infarction with sclerotic subcutaneous arterioles. Arteriolosclerosis is defined by thick vessel walls at the cost of a narrow lumen, and in approximately 70% of histologic sections part of the arterioles show a striking medial calcification. The narrow lumen may be occluded by acute or organized thrombosis. In the French multicenter serie, at 8 weeks only 10% (3/31 patients) healed by conservative means, and this could not be improved with the use of topical application of platelet-derived growth factor-BB (becaplermin) (18% healing, 5/28 patients, no significant difference). In the Zurich series, 84% of patients (26/31) had wound surgery (necrosectomy, split skin graft) to heal the ulcer and 10% (3/31) died from wound infection and sepsis, amongst two under immunosuppression received for misdiagnosed pyoderma gangrenosum.
Conclusions: Martorell HYTILU can easily be confounded with pyoderma gangrenosum or necrotizing vasculitis. Physicians involved in wound treatment should be sensitized to the typical clinical setting: a progressive and extremely painful skin infarction at the laterodorsal leg in a hypertensive and often diabetic subject. Diagnosis is confirmed histologically on a long-enough, narrow, but deep spindle-shape skin and ulcer biopsy, showing a highly characteristic form of stenotic and occlusive subcutaneous arteriolosclerosis. Management is essentially based on wound surgery. Conservative means are almost uneffective. Immunosuppression must be avoided, and antibiotic treatment introduced as required.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiovascular Surgery
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2013
Deposited On:31 Jan 2013 14:21
Last Modified:07 Dec 2017 19:19
Publisher:Elsevier
ISSN:2212-0211
Publisher DOI:https://doi.org/10.1016/j.rvm.2012.08.001

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