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Martorell Hypertensive Ischemic Leg Ulcer and Digital Auto-amputation in an Obese Young Male with Systolic Heart Failure: Case Report

Posted by [email protected] on Mar. 17, 2021  /   0

ABSTRACT: Martorell Hypertensive Ischemic Leg Ulcers (HYTILU) are painful and rapidly enlarging lower extremity wounds associated with uncontrolled systemic hypertension. They are most commonly seen in women from the fifth to seventh decades of life. Here, we describe a unique case of HYTILU with digital autoamputation in an obese, hypertensive 27-year old male with concomitant systolic heart failure. This case serves to reinforce the importance of maintaining a high index of suspicion for this rare ulcer, as early recognition and diagnosis of HYTILU is key for the healing process that takes weeks and involves multiple modalities and disciplines.

KEYWORDS: Martorell Ulcer; Hypertensive Ischemic Leg Ulcer

INTRODUCTION: HYTILU was first described in 1945 by Spanish cardiologist Fernando Martorell in four obese females with poorly controlled systemic hypertension1. Hines and Farmer later confirmed the presence of these ulcers with their 1946 and 1947 reports and coined the term ‘hypertensive ischemic leg ulcer’2,3. Differential diagnoses include venous ulceration, calciphylaxis, and pyoderma gangrenosum. On gross examination, HYTILUs are usually found bilaterally on the dorsolateral legs or overlying the Achilles tendons. They appear as necrotic ulcers with irregular margins and surrounding erythema. Patients commonly report a livid rash over the affected area prior to ulceration4.

HYTILU is chiefly a clinical diagnosis based on patient demographics, ulcer location and characteristics, and prior medical history—94.4% of HYTILU patients have chronic hypertension and 39.7% have diabetes mellitus. The diagnosis is confirmed via deep wedge biopsy, which characteristically shows arteriolosclerosis with medial calcinosis and intimal hyperplasia5. It is believed the ulcerative lesion and intense pain of HYTILU are secondary to microvascular occlusion of the tunica media and elastic lamina6; lower extremity pulses are typically palpable, and only 50% of patients have pre-existing peripheral artery disease5. Decreased blood flow to dermal arterioles deprives the skin of nutrients and leads to impaired wound healing as well as difficulty fighting pathogens at the ulcer site. Nerve ischemia leads to unremitting pain in patients with HYTILU. This pain can be very difficult to control and unlike classic arterial disease pain, it does not improve with rest7.

CASE REPORT: A 27-year-old African American male presented with a two-year history of bilateral non-healing wounds of the lower extremities. The patient stated that the lesions began insidiously with small erythematous swollen patches that later ulcerated. Medical history was significant for obesity and uncontrolled hypertension. He was initially treated at a hospital-based wound care clinic utilizing UNNA Boots, compression stockings, and basic wound care. Weeks later, the initial wounds resolved, but unfortunately returned 3 months following his initial treatment. Repeat therapy at the wound care clinic failed to yield similar results and a more aggressive approach was then taken in the form of debridement and topical enzymatic therapy. The patient stated that this led to a worsening of the lesions, and he decided to seek a second opinion.

Physical exam revealed an obese male with significant deep, punched-out ulcerations of the dorsal aspects of the feet and toes (Figure 1). Vital signs in the office demonstrated a blood pressure of 210/104. The ulcers were classified as C6 ulcers in the CEAP classification system. The lesions were painful to the touch; neurologically, no deficits were appreciated. Non-pitting edema of the bilateral lower extremities was observed (Figure 2). Pedal pulses were intact, bilaterally. Differential diagnoses included cutaneous polyarteritis nodosa, calciphylaxis with normal renal and parathyroid function, pyoderma gangrenosum (PG), infection, arterial insufficiency, thromboangiitis obliterans (Buerger’s disease), and factitial dermatitis.

Punch biopsy demonstrated granulation and scar tissue. Incisional biopsies of lesional and perilesional skin demonstrated healing tissue, scar tissue, and changes consistent with stasis dermatitis. Tissue cultures for fungi, bacteria, and AFB were negative. Blood cultures showed no growth. CBC with differential, CMP, UA, ASO, RPR, ACE, serum immunoglobulins, hepatitis B and C, ANA, ANCA Panel, cryoglobulin, titers for Lyme disease and Rocky Mountain Spotted Fever, Rheumatoid Factor, anti-CCP, antiphospholipid antibodies, complement levels, and HIV were within normal range. Non-invasive arterial Doppler ultrasonography revealed no evidence of stenosis or occlusion. Bilateral venous Doppler ultrasonography demonstrated no evidence of DVT.

The patient reported significant weight gain over 6 months prior to presentation, and demonstrated signs of labored breathing in the office. Cardiology evaluation revealed severe uncontrolled systolic hypertension and congestive heart failure with a LVEF of 27%. A diagnosis of idiopathic cardiomyopathy was made and prompt fluid correction led to a shedding of over 120lbs of fluid weight. After stabilization of the patient’s underlying cardiac condition was complete, the lower extremity edema and ulceration demonstrated vast improvement; the patient was again managed with UNNA boots and basic wound care.

Figure 1 Top: patient’s left foot at presentation, Bottom: patient’s right foot at presentation, note gangrenous appearance of second toe.

Figure 2 Top: lateral view of patient’s left foot at presentation Bottom: patient’s feet at presentation, note significant pedal edema and tissue necrosis in both pictures.

DISCUSSION: This case is a unique presentation of Martorell HYTILU. It is typically seen in elderly women; however, this patient is a young male. Alavi et al.5 reported HYTILU patients experienced the best outcomes with immediate surgical debridement and split thickness skin-grafting. Failing that, patients also responded to conservative measures such as curettage and topical enzymatic therapy. The patient here demonstrated remarkable improvement of his ulcers after stabilization of his underlying systolic heart failure and without surgical intervention. Furthermore, he had worsening of his ulcers with conservative management prior to cardiovascular intervention. This pathergy-like response to wound manipulation is unusual for HYTILU and may suggest an alternative diagnosis such as pyoderma gangrenosum8.

            A gestalt view of the patient’s history and physical exam makes HYTILU far more likely than PG and other conditions on the differential diagnosis. However, it is crucial to accurately diagnose HYTILU versus PG and vasculitides as their respective treatments are diametrically opposed8. Calciphylaxis with normal renal and parathyroid function—also known as eutrophication—is a plausible alternative diagnosis. It is a painful, rapidly enlarging necrotic ulcer seen in the morbidly obese9. Like HYTILU, eutrophication is highly associated with hypertension and diabetes mellitus to a lesser degree. Histologically, HYTILU and eutrophication are indistinguishable and may be exist on a spectrum of the same underlying pathology. Both demonstrate intimal hyperplasia and medical calcinosis of the arterioles. HYTILU is favored here because of the patient’s severe hypertension and bilateral, mirror-like distribution of his ulcers. Eutrophication ulcers tend to be located in regions where adipose tissue is sufficiently situated. This may be seen in the inner thigh, abdomen, breasts, or upper arms8. [and are not as strongly associated with severe hypertension8.] 

It is unusual that lesional biopsies did not reveal the characteristic microangiopathic changes of HYTILU. This could be due to an inadequate biopsy, failure to comment by the pathologist, and/or bad luck such as sampling of non-diseased segments of blood vessels. However, it is important to note biopsy merely serves to confirm the diagnosis of HYTILU and rule out other etiologies; a non-diagnostic biopsy does not rule out HYTILU5.

It is also unusual the patient experienced autoamputation. As shown in figure 3, he lost the tip of his right second toe at the distal interphalangeal joint. A search of the canon revealed no cases of HYTILU with resulting autoamputation. Digital autoamputation in the context of granulomatous inflammation leads one to consider the possibility of thromboangiitis obliterans (Buerger’s disease). This is an intriguing consideration, but unlikely given the patient is a nonsmoker, had normal markers of inflammation, and no infrapopliteal occlusion or upper limb involvement. Ankle-brachial indices (ABI) and angiography would benefit the patient from a diagnostic perspective, as these modalities are usually normal in HYTILU but abnormal in diseases of larger caliber vessels such as thromboangiitis obliterans10.

In conclusion, Martorell HYTILU is a rare disease characterized by painful lower extremity ulcers. It is highly associated with systemic hypertension and most commonly seen in women aged fifty to seventy. In this case report, we demonstrated an atypical presentation and outcome of HYTILU. The patient was a young male with severe hypertension and systolic heart failure. His ulcers were significant but responded well to conservative wound care after his underlying cardiac issues were addressed. For the first time in the literature, we have described a case of digital autoamputation in a patient with HYTILU. Maintaining a high index of suspicion for this uncommon pathology is crucial in order to promptly diagnose and treat patients.

REFERENCES:

  1. Martorell F. Hypertensice ulcer of the leg. Acta Inst Policlinico Barcelona 1945;1:6–9.
  2. Hines EA, Farber EM. Ulcer of the leg due to arteriolosclerosis and ischemia, occurring in the presence of hypertensive disease (hypertensive-ischemic ulcers): a preliminary report. Proc Staff Meet Mayo Clin. 1946;21:337-346.
  3. Farber EM, Hines EA Jr, Montgomery H, Craig W. The arterioles of the skin in essential hypertension. J Invest Dermatol 1947;9:285–298.
  4. Hafner J, Nobbe S, Partsch H, Lauchli S, Mayer D, Amann-Vesti B, French LE. Martorell hypertensive ischemic leg ulcer: a model of ischemic subcutaneous arteriolosclerosis. Arch Derm, 2010;146(9): 961-68.
  5. Alavi A, Mayer D, Hafner J, Sibbald RG. Martorell hypertensive ischemic leg ulcer: an underdiagnosed entity©. Adv Skin Wound Care, 2012;25(12):563-72.
  6. Malhi HK, Didan A, Ponosh S, Kumarasinghe SP. Painful leg ulceration in a poorly controlled hypertensive patient: a case report of martorell ulcer. Case Rep Dermatol 2017;9:95-102.
  7. Pinto APFL, Silva Jr NA, Osorio CT, Rivera LM, Carneiro S, Ramos-e-Silva M, Bica BERG. Martorell’s ulcer: diagnostic and therapeutic challenge. Case Rep Dermatol, 2015;7(2):199-206.
  8. Hafner J. Calciphylaxis and Martorell hypertensive ischemic leg ulcer: same pattern-one pathophysiology. Dermatol, 2016;232(5):523-33.
  9. Munavalli G, Reisenauer A, Moses M, Kilroy S, Arbiser JL. Weight loss-induced calciphylaxis: potential role of matrix metalloproteinases. J Dermatol 2003;30:915-919.
  10. Rivera-Chavarria IJ, Brenes-Gutierrez JD. Thromboangiitis obliterans (Buerger’s Disease). Annals Med Surg, 2016;7:79-82.

FIGURE LEGENDS:

Figure 1 Top: patient’s left foot at presentation, Bottom: patient’s right foot at presentation, note gangrenous appearance of second toe.

Figure 2 Top: lateral view of patient’s left foot at presentation, Bottom: patient’s feet at presentation, note significant pedal edema and tissue necrosis in both pictures.

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