What Is Causing This Man’s Severe Hyperkeratotic Lower-Extremity Lesions?

Authors:
Alyssa Vivas, DO, and Amanda Rapp, MD

Citation:
Vivas A, Rapp A. What is causing this man’s severe hyperkeratotic lower-extremity lesions? Consultant. 2017;57(11):657-658.

A 69-year-old man with history of coronary artery disease, hypertension, hypothyroidism, obstructive sleep apnea, tobacco use, and medical nonadherence presented to the emergency department with dyspnea and subsequently was admitted to the intensive care unit for an exacerbation of chronic obstructive pulmonary disease. Although they were not directly related to his chief concern, severe hyperkeratotic lesions were noted on the patient’s bilateral lower extremities.

The man had not been to a health care provider in more than 10 years and had never been evaluated for this problem. These lesions had begun as minor scaling and had eventually become thick and crusted. He stated that he has difficulty ambulating and has had intermittent pain and drainage from his legs over the past several years. The lesions were not pruritic, however. He lived in a very cluttered, unclean apartment. He was not taking any medications.

Physical examination revealed diffuse nonpitting edema throughout the bilateral lower extremities, from the hips to the feet, with multiple layers of dry, scaly, gray-brown, hyperkeratotic lesions extending from the knees to the feet. There was associated malodor and diminished peripheral pulses. The lesions were limited to the lower extremities (Figure).

Answer on next page

Answer: Chronic Lymphedema

An infectious disease consultant was concerned about the possibility of scabies or other infectious skin disease. A punch biopsy specimen was taken, and the pathology report showed benign keratosis without the presence of mites or evidence of any other contagious skin process. Despite the negative biopsy results, the patient was nevertheless started on empiric therapy with permethrin and ivermectin.

A consultant podiatrist and wound care specialist determined that the hyperkeratotic lesions were a result of untreated chronic lymphedema. The patient’s legs were wrapped with wound dressings and Unna boots for compression therapy to facilitate wound healing. An occupational therapist also was consulted to administer manual lymphatic drainage therapy.

After his discharge from the hospital, the patient was instructed to follow up with the occupational therapist for continuing lymphedema treatment.

DISCUSSION

Lymphatic dysfunction is characterized by an increased amount of protein trapped in the interstitium, which leads to water retention and nonpitting edema. Lymphedema is a collection of protein-rich fluid in the interstitium caused by obstruction or dysfunction of the lymphatic system, leading to diminished lymphatic return. It occurs most commonly in the lower extremities but may also occur in the face, trunk, and genitals. Lymphedema presents as nontender, nonpitting edema with erythema and thickening of the skin. Fissuring, ulceration, and skin breakdown also may be present.

In primary lymphedema, the lymphatic system dysfunction is caused by a developmental or congenital abnormality of the lymphatic system and/or incompetence of the lymphatic valves. Examples of primary lymphedema include congenital hereditary lymphedema (Milroy disease), lymphedema praecox (Meige disease), and lymphedema tarda.¹ In secondary lymphedema, lymphatic drainage can become dysfunctional as a result of blockage of lymph nodes or disruption of lymphatic channels secondary to obesity, surgical procedures, malignancy, recurrent lymphangitis (likely from recurrent episodes of cellulitis), or infection. In our patient’s severe case, the chronic lymphedema might have resulted from a combination of obesity and a decades-long history of untreated cellulitis.

Untreated lymphedema leads to an inflammatory reaction and fibrosis in the skin.¹ The skin can thicken, scale, and develop warts, cracks, and ulcerations. Hyperkeratosis, a brawny texture, creases, and papillomatosis are common changes. Severe complications of untreated lymphedema include cellulitis, deepvein thrombosis, lymphangitis, and lymphangiosarcoma.²

The estimated prevalence of chronic lymphedema is 1.33 cases per 1000 people, which increases to 5.4 cases per 1000 in persons older than 65 years.³ Scabies, particularly crusted or Norwegian scabies, was among the primary differential diagnoses in our patient’s case. Norwegian scabies can present with similar hyperkeratosis and other examination findings; however, results of either a potassium hydroxide (KOH) preparation test of skin scrapings or a biopsy specimen will reveal the presence of mites (Sarcoptes scabiei var hominis). Norwegian scabies typically is associated with immunodeficient states and affects body parts such as the trunk, hands, feet, nails, ears, and scalp. This form of scabies is very contagious and is more difficult to eradicate than noncrusted scabies.⁴

Other differential diagnoses included chronic eczema, fungal skin infection, and ichthyosis. Chronic eczema is typically not as severe as this patient’s case, is pruritic, and usually affects the flexural surfaces, palms, and soles.⁵ Fungal skin infection can be.associated with onychomycosis, erythematous rashes, and intertrigo; patients are commonly immunodeficient or have a history of diabetes. Had the cause of this patient’s condition been a fungal infection, KOH prep test or biopsy results would have revealed hyphae and/or spores.⁶ Ichthyosis lesions are most prominent over flexural surfaces and joints, and affected skin can blister easily. Ichthyosis is an autosomal dominant disease that presents at birth with erythema, blisters, and skin erosions and gradually evolves into hyperkeratosis.⁷

Although it was not confirmed in our patient, one possible differential diagnosis was epidermodysplasia verruciformis (EV), which is a rare, autosomal recessive inherited skin disorder. The exact prevalence of EV is unknown, but approximately 600 cases have been described worldwide.⁸ It involves eruptions of wartlike lesions, hyperkeratosis, acanthosis, and plaques on the trunk, extremities, and face. EV is associated with human papillomavirus (HPV) infection and has the potential for transformation to squamous cell carcinoma, especially if exposed to sunlight or UV radiation.⁹ The diagnosis is made through HPV viral detection and typing of skin tissue. A biopsy also can be done to detect premalignant or malignant lesions. Treatment includes imiquimod, fluorouracil, systemic retinoids, interferon, and photodynamic therapy. If multiple malignant lesions are present, autotransplantation of skin from uninvolved non-sun–exposed areas can be performed. Other surgical options include cryotherapy and electrosurgical removal of diseased skin.⁹

Methods of improving lymphedema include weight loss, elevation of limbs, avoiding constrictive clothing, and local skin care to reduce the risk of infection. Physical therapy that involves external compression, exercise of the limbs, and manual lymphatic drainage are effective treatment options. Pharmacologic treatment options include topical ammonium lactate, topical urea, topical retinoid-like agents (eg, acitretin, tazarotene), benzopyrones (eg, coumarin, flavonoids), and anthelmintics (eg, albendazole). Surgery to remove excess tissue or bypass the lymphatic defect is another treatment option.

Alyssa Vivas, DO, is a resident physician in the Abrazo Central Family Medicine Residency Program in Phoenix, Arizona.

Amanda Rapp, MD, is a faculty member of the Abrazo Central Family Medicine Residency Program in Phoenix, Arizona.

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