Martorell Hypertensive Ischemic Ulcer Successfully Treated With Punch Skin Grafting

Most lower extremity ulcers may be classified into 1 or more of these etiological groups: venous insufficiency, arterial insufficiency, or neuropathy. However, other less common causes do exist and should be considered for differential diagnosis when atypical features arise or when the wound does not respond to an accurate standard treatment.¹

In 1945, Martorell² described 4 cases of supramalleolar ulcers with underlying arteriolitis in obese women with uncontrolled hypertension. From then on, different authors have published various clinical and histological features that characterize these lesions.^2,3 The typical clinical presentation is a painful ulcer over the Achilles tendon or dorsolateral leg. Multiple bilateral lesions are frequently present. Skin infarction begins as a livid, painful area that rapidly turns into a necrotic lesion with purple-reddish edges. The development of satellite lesions is common. Most patients are more than 60 years old and present with usually well-controlled arterial hypertension for many years as well as type 2 diabetes mellitus. Some patients describe a minor trauma as the trigger, but others only report a spontaneous onset with an initial livid macule.³

Considering the typical histopathology of the underlying arterioles, which show arteriolosclerotic changes with a distinctive wall thickening that results in a narrow lumen, the term hypertensive ischemic leg ulcer (HYTILU) has been coined.⁴ However, the eponymous Martorell ulcer continues to be used. Martorell HYTILUs have a higher vascular resistance due to a decrease in the compensatory dilatation of the arterioles supplying the dermal capillary bed.⁴ This vascular resistance may exist in hypertensive patients with thorough blood pressure control for years. Consequently, it seems that an adequate therapeutic control of hypertension may not be sufficient to either prevent the occurrence or stop the progression of Martorell HYTILUs.³ The authors present the case of a woman with well-controlled hypertension and diabetes who developed extremely painful bilateral Martorell HYTILUs and had a successful response to treatment with punch skin grafting.

A 68-year-old Caucasian woman presented to the Department of Dermatology, Hospital Universitario Infanta Leonor in Madrid, Spain, with an extremely painful 10-cm x 5-cm superficial ulcer on the distal medial aspect of her left leg (Figure 1). The ulcer had developed 6 months prior after minimal trauma, and despite antibiotic therapy and conventional local wound care, there was unstoppable progression during the previous month prior to presentation at the hospital. The wound edges were irregular with a peripheral purpuric halo, necrotic tissue on the upper margin of the wound, and both red granulation tissue and slough in the wound bed. She also presented with a recent 1-cm ulcer, with livid halo and slough in the wound bed, on the anterolateral supramalleolar region of the contralateral leg (Figure 2). The patient had a history of well-controlled hypertension (under antihypertensive therapy for more than 20 years), well-controlled type 2 diabetes mellitus, chronic cardiac insufficiency, atrial fibrillation, and chronic venous insufficiency. Her current treatment included acenocumarol, amiodarone, insulin, an angiotensin-converting enzyme inhibitor, a beta blocker, and a diuretic. She had never smoked. Physical examination revealed signs of chronic venous stasis. Distal pulses were palpable, and the ankle-brachial index was normal. Arterial and venous duplex ultrasound found a slight insufficiency of the left great saphenous vein. A biopsy of the wound edge showed hyalinization of the dermal blood vessels with minimal perivascular inflammatory infiltrate. Hematologic and rheumatologic work-up did not show relevant findings. Considering the above findings, the diagnosis of Martorell HYTILU was made. 

The anticoagulant treatment was replaced by heparin. Pain control was poor despite opioids, with a score of 8 out of 10 on the Visual Analog Score for pain. Neither rest nor lower limb elevation relieved the pain, and dressing changes were unbearable due to pain. Consequently, despite the presence of slough in the wound bed and necrotic tissue on the superior aspect of the wound, early punch grafting in an outpatient setting was decided.

The donor site was the anterolateral aspect of the left thigh. After tumescent lidocaine injection to the donor site, the thin split-thickness skin grafts were obtained with either a 4-mm or 6-mm punch and were placed on both wound beds (Figure 3). The grafted ulcers were covered with an interface dressing, and an alginate was used as a secondary dressing. For the outermost layer, a 2-layer compression bandage system was used. The donor site (Figure 4) was covered with an alginate sheet and gauze as a secondary dressing. The first dressing change took place 6 days after the surgical procedure. Frequency of subsequent dressing changes ranged from 5 to 7 days, depending on schedule availability. Blood pressure and glucose levels remained in normal ranges during the whole treatment period. 

Reduction in both permanent and peak pain started a few hours after punch grafting. At week 4, complete reepithelialization could be observed, except for the upper margin of the wound on the right leg (Figure 5), which was the area that could not be covered with grafts due to necrotic tissue. After autolytic debridement during those 4 weeks, which was well tolerated, this area finally presented with adequate granulation tissue and was covered with punch grafts. Figure 6 shows the successful result 3 weeks after the second surgical procedure.

Ignorance of the Martorell HYTILU hinders its diagnosis and, consequently, this condition is considered to be a rare cause of leg ulcers.^5,6 Martorell HYTILU, or angiodermite nécrotique in French, recently has been suggested to share the same pathophysiology as calciphylaxis.^3,7 These 2 entities present with skin infarction as a result of subcutaneous stenotic arteriolosclerosis with medial calcinosis. Moreover, both diseases have been associated with similar risk factors: arterial hypertension, diabetes mellitus (types 1 and 2), secondary or tertiary hyperparathyroidism (in end-stage kidney disease), and oral anticoagulation with vitamin K antagonists.⁷ Regarding treatment, calciphylaxis and Martorell HYTILU benefit from the same management strategy in the acute phase, which is enormously painful and presents with progressive necrosis. The control of cardiovascular risk factors must be optimized; oral anticoagulation with vitamin K antagonists should be replaced by heparine, and a proactive wound approach should be established. Oral anticoagulation with vitamin K antagonists may exacerbate the condition, because the activation of a protein that inhibits pathological calcification, α 2- Heremans-Schmid glycoprotein, depends on vitamin K.^3,7

Early debridement and skin grafting is recommended to promote wound healing and reduce pain.^3,7-9 A sequential treatment combining necrosectomy and negative pressure wound therapy may improve the wound bed preparation before skin grafting.^3,7 However, some have described clinical worsening after necrosectomy and suggested that the condition may be exacerbated by tissue trauma, thus wound care should be conservative.¹⁰

The present authors agree with Hafner et al⁷ and other experts,^3,8,9 who suggest that early surgical management is more effective than conservative treatment for both pain control and wound healing. Hafner et al³ presented a case series of Martorell HYTILUs and showed the effectiveness of a treatment protocol consisting of surgical debridement of necrotic tissue, vacuum-assisted negative pressure when necessary, and autologous split-thickness skin grafting (0.2-mm thick meshed graft). That study³ observed a striking significant pain reduction after skin grafting. Some patients required several skin grafting procedures to achieve final complete healing.³

Punch grafting is a traditional method, which can be performed in an outpatient basis, to obtain thin split-thickness skin grafts containing epidermis with minimal papillary dermis. Regarding time-efficiency, the choice of punch size varies when considering the wound surface area (the broader the area means the wider the punch). In this case, the authors used both 4-mm and 6-mm punches, but a unique size also could have been chosen. Skin grafts were obtained under a local anesthetic, removed from the patients’ thigh, and directly placed on the ulcer. The donor site healed by secondary intention.

As the authors observed in this patient, even if the wound bed does not present with the perfect tissue for receiving skin grafts (covered with slough and necrotic tissue in peripheral areas), the lesion will take advantage of early skin grafting. Punch grafting is especially interesting due to the fact that even if some grafts do not succeed to adhere to the wound bed, they do release growth factors and cells that enhance wound epithelialization.^9,11 The angiogenic effect of skin grafts counteract the ischemic and proinflammatory environment in these wound beds, which is an essential promoter of wound healing and a constraining factor for skin necrosis. The success in the treatment of this patient relied on the nonaggressive and appropriately spaced dressing changes after skin grafting, so that the created microenvironment, by both adhered and nonadhered grafts, could promote wound healing.

Punch grafting is a simple and cost- effective traditional technique to enhance wound healing, which may be performed in an outpatient setting and has been associated with significant and quick pain reduction in ulcers with different underlying causes.^9,11 Regarding Martorell HYTILU, Nordström and Hansson⁹ showed a stronger pain relief effect on permanent pain than on peak pains, so the reduction of analgesics, especially opioids, should be gradual. Since it is a well- accepted treatment by the patient, some ulcers can benefit from multiple grafts if a complete epithelialization is not achieved after the first procedure,¹¹ as in the case reported herein.

As in this case, simultaneous occurrence of venous insufficiency is not rare. Consequently, compression therapy must be prescribed during ulcer treatment and should be maintained with compression stockings as a preventive measure after wound healing.⁶

Clinical presentation of Martorell HYTILUs, with severe pain and inflamed livid margins, may lead to misdiagnosis of pyoderma gangrenosum (PG) or necrotizing vasculitis.^1,3,7 In contrast to PG, Martorell HYTILUs do not present with pustules and are not related to the typical underlying neutrophilic disorders such as inflammatory bowel disorders, rheumatoid arthritis, or hematoproliferative diseases. Treatment for PG consists of suppression of inflammatory disease activity with systemic glucocorticosteroids or other immunosuppressive therapies. Considering that surgical debridement is contraindicated in order to not worsen PG lesions, an accurate differential diagnosis with this entity is essential. As the typical subcutaneous arteriolosclerosis may not be found in superficial biopsies and the inflammatory response may be striking, misdiagnosis with PG is not rare. Moreover, considering that PG is a diagnosis of exclusion with no pathognomonic, clinical, or histological findings, differential diagnosis may be a challenge and misdiagnosis very detrimental, due to the different treatment strategies for both entities. Diagnosis of necrotizing vasculitis is supported by histological findings, and its initial treatment consists of systemic immunosuppression.⁷

Martorell HYTILUs may represent a diagnostic and therapeutic challenge in clinical practice. In many cases, misdiagnosis is due to lack of knowledge. The reported case emphasizes the fact that uncontrolled high arterial hypertension is not a required diagnostic criterion. The authors support the opinion of other experts,^3,7,9,10 who suggest an early surgical treatment focused on coverage with skin grafts should be the core strategy to alleviate pain and stop the uncontrolled necrotic progression of this lesion. 

Affiliations: Department of Dermatology, Hospital Universitario Infanta Leonor, Madrid, Spain; Centro de Especialidades Vicente Soldevilla, Hospital Virgen de la Torre, Madrid, Spain; Hospital Universitario de Fuenlabrada, Madrid, Spain; and Hospital General Universitario Gregorio Marañón, Madrid, Spain

Correspondence: Elena Conde Montero, PhD, Hospital Infanta Leonor, Department of Dermatology, Alcalá 124, 4b, Madrid, Madrid 28009, Spain; elenacondemontero@gmail.com

Disclosure: The authors disclose no financial or other conflicts of interest.