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Polyurethane Tubing to Minimize Pain During Nail Injections
Practice Gap
Nail matrix and nail bed injections with triamcinolone acetonide are used to treat trachyonychia and inflammatory nail conditions, including nail psoriasis and nail lichen planus. The procedure should be quick in well-trained hands, with each nail injection taking only seconds to perform. Typically, patients have multiple nails involved, requiring at least 1 injection into the nail matrix or the nail bed (or both) in each nail at each visit. Patients often are anxious when undergoing nail injections; the nail unit is highly innervated and vascular, which can cause notable transient discomfort during the procedure1,2 as well as postoperative pain.3
Nail injections must be repeated every 4 to 6 weeks to sustain clinical benefit and maximize outcomes, which can lead to heightened anxiety and apprehension before and during the visit. Furthermore, pain and anxiety associated with the procedure may deter patients from returning for follow-up injections, which can impact treatment adherence and clinical outcomes.
Dermatologists should implement strategies to decrease periprocedural anxiety to improve the nail injection experience. In our practice, we routinely incorporate stress-reducing techniques—music, talkesthesia, a sleep mask, cool air, ethyl chloride, and squeezing a stress ball—into the clinical workflow of the procedure. The goal of these techniques is to divert attention away from painful stimuli. Most patients, however, receive injections in both hands, making it impractical to employ some of these techniques, particularly squeezing a stress ball. We employed a unique method involving polyurethane tubing to reduce stress and anxiety during nail procedures.
The Technique
A patient was receiving treatment with intralesional triamcinolone injections to the nail matrix for trachyonychia involving all of the fingernails. He worked as an equipment and facilities manager, giving him access to polyurethane tubing, which is routinely used in the manufacture of some medical devices that require gas or liquid to operate. He found the nail injections to be painful but was motivated to proceed with treatment. He brought in a piece of polyurethane tubing to a subsequent visit to bite on during the injections (Figure) and reported considerable relief of pain.
What you were not taught in United States history class was that this method—clenching an object orally—dates to the era before the Civil War, before appropriate anesthetics and analgesics were developed, when patients and soldiers bit on a bullet or leather strap during surgical procedures.4 Clenching and chewing have been shown to promote relaxation and reduce acute pain and stress.5
Practical Implications
Polyurethane tubing can be purchased in bulk, is inexpensive ($0.30/foot on Amazon), and unlikely to damage teeth due to its flexibility. It can be cut into 6-inch pieces and given to the patient at their first nail injection appointment. The patient can then bring the tubing to subsequent appointments to use as a mastication tool during nail injections.
We instruct the patient to disinfect the dedicated piece of tubing after the initial visit and each subsequent visit by soaking it for 15 minutes in either a 3% hydrogen peroxide solution, antibacterial mouthwash, a solution of baking soda (bicarbonate of soda) and water (1 cup of water to 2 teaspoons of baking soda), or white vinegar. We instruct them to thoroughly dry the disinfected polyurethane tube and store it in a clean, reusable, resealable zipper storage bag between appointments.
In addition to reducing anxiety and pain, this method also distracts the patient and therefore promotes patient and physician safety. Patients are less likely to jump or startle during the injection, thereby reducing the risk of physically interfering with the nail surgeon or making an unanticipated advance into the surgical field.
Although frustrated patients with nail disease may need to “bite the bullet” when they accept treatment with nail injections, lessons from our patient and from United States history offer a safe and cost-effective pain management strategy. Minimizing discomfort and anxiety during the first nail injection is crucial because doing so is likely to promote adherence with follow-up injections and therefore improve clinical outcomes.
Future clinical studies should validate the clinical utility of oral mastication and clenching during nail procedures compared to other perioperative stress- and anxiety-reducing techniques.
- Ricardo JW, Lipner SR. Utilization of a stress ball to diminish anxiety during nail surgery. Cutis. 2020;105:294. doi:10.12788/cutis.0013
- Ricardo JW, Lipner SR. Utilizing a sleep mask to reduce patient anxiety during nail surgery. Cutis. 2021;108:36. doi:10.12788/cutis.0285
- Ip HYV, Abrishami A, Peng PW, et al. Predictors of postoperative pain and analgesic consumption: a qualitative systematic review. Anesthesiology. 2009;111:657-677. doi:10.1097/ALN.0b013e3181aae87a
- Albin MS. The use of anesthetics during the Civil War, 1861-1865. Pharm Hist. 2000;42:99-114.
- Tahara Y, Sakurai K, Ando T. Influence of chewing and clenching on salivary cortisol levels as an indicator of stress. J Prosthodont. 2007;16:129-135. doi:10.1111/j.1532-849X.2007.00178.x
Practice Gap
Nail matrix and nail bed injections with triamcinolone acetonide are used to treat trachyonychia and inflammatory nail conditions, including nail psoriasis and nail lichen planus. The procedure should be quick in well-trained hands, with each nail injection taking only seconds to perform. Typically, patients have multiple nails involved, requiring at least 1 injection into the nail matrix or the nail bed (or both) in each nail at each visit. Patients often are anxious when undergoing nail injections; the nail unit is highly innervated and vascular, which can cause notable transient discomfort during the procedure1,2 as well as postoperative pain.3
Nail injections must be repeated every 4 to 6 weeks to sustain clinical benefit and maximize outcomes, which can lead to heightened anxiety and apprehension before and during the visit. Furthermore, pain and anxiety associated with the procedure may deter patients from returning for follow-up injections, which can impact treatment adherence and clinical outcomes.
Dermatologists should implement strategies to decrease periprocedural anxiety to improve the nail injection experience. In our practice, we routinely incorporate stress-reducing techniques—music, talkesthesia, a sleep mask, cool air, ethyl chloride, and squeezing a stress ball—into the clinical workflow of the procedure. The goal of these techniques is to divert attention away from painful stimuli. Most patients, however, receive injections in both hands, making it impractical to employ some of these techniques, particularly squeezing a stress ball. We employed a unique method involving polyurethane tubing to reduce stress and anxiety during nail procedures.
The Technique
A patient was receiving treatment with intralesional triamcinolone injections to the nail matrix for trachyonychia involving all of the fingernails. He worked as an equipment and facilities manager, giving him access to polyurethane tubing, which is routinely used in the manufacture of some medical devices that require gas or liquid to operate. He found the nail injections to be painful but was motivated to proceed with treatment. He brought in a piece of polyurethane tubing to a subsequent visit to bite on during the injections (Figure) and reported considerable relief of pain.
What you were not taught in United States history class was that this method—clenching an object orally—dates to the era before the Civil War, before appropriate anesthetics and analgesics were developed, when patients and soldiers bit on a bullet or leather strap during surgical procedures.4 Clenching and chewing have been shown to promote relaxation and reduce acute pain and stress.5
Practical Implications
Polyurethane tubing can be purchased in bulk, is inexpensive ($0.30/foot on Amazon), and unlikely to damage teeth due to its flexibility. It can be cut into 6-inch pieces and given to the patient at their first nail injection appointment. The patient can then bring the tubing to subsequent appointments to use as a mastication tool during nail injections.
We instruct the patient to disinfect the dedicated piece of tubing after the initial visit and each subsequent visit by soaking it for 15 minutes in either a 3% hydrogen peroxide solution, antibacterial mouthwash, a solution of baking soda (bicarbonate of soda) and water (1 cup of water to 2 teaspoons of baking soda), or white vinegar. We instruct them to thoroughly dry the disinfected polyurethane tube and store it in a clean, reusable, resealable zipper storage bag between appointments.
In addition to reducing anxiety and pain, this method also distracts the patient and therefore promotes patient and physician safety. Patients are less likely to jump or startle during the injection, thereby reducing the risk of physically interfering with the nail surgeon or making an unanticipated advance into the surgical field.
Although frustrated patients with nail disease may need to “bite the bullet” when they accept treatment with nail injections, lessons from our patient and from United States history offer a safe and cost-effective pain management strategy. Minimizing discomfort and anxiety during the first nail injection is crucial because doing so is likely to promote adherence with follow-up injections and therefore improve clinical outcomes.
Future clinical studies should validate the clinical utility of oral mastication and clenching during nail procedures compared to other perioperative stress- and anxiety-reducing techniques.
Practice Gap
Nail matrix and nail bed injections with triamcinolone acetonide are used to treat trachyonychia and inflammatory nail conditions, including nail psoriasis and nail lichen planus. The procedure should be quick in well-trained hands, with each nail injection taking only seconds to perform. Typically, patients have multiple nails involved, requiring at least 1 injection into the nail matrix or the nail bed (or both) in each nail at each visit. Patients often are anxious when undergoing nail injections; the nail unit is highly innervated and vascular, which can cause notable transient discomfort during the procedure1,2 as well as postoperative pain.3
Nail injections must be repeated every 4 to 6 weeks to sustain clinical benefit and maximize outcomes, which can lead to heightened anxiety and apprehension before and during the visit. Furthermore, pain and anxiety associated with the procedure may deter patients from returning for follow-up injections, which can impact treatment adherence and clinical outcomes.
Dermatologists should implement strategies to decrease periprocedural anxiety to improve the nail injection experience. In our practice, we routinely incorporate stress-reducing techniques—music, talkesthesia, a sleep mask, cool air, ethyl chloride, and squeezing a stress ball—into the clinical workflow of the procedure. The goal of these techniques is to divert attention away from painful stimuli. Most patients, however, receive injections in both hands, making it impractical to employ some of these techniques, particularly squeezing a stress ball. We employed a unique method involving polyurethane tubing to reduce stress and anxiety during nail procedures.
The Technique
A patient was receiving treatment with intralesional triamcinolone injections to the nail matrix for trachyonychia involving all of the fingernails. He worked as an equipment and facilities manager, giving him access to polyurethane tubing, which is routinely used in the manufacture of some medical devices that require gas or liquid to operate. He found the nail injections to be painful but was motivated to proceed with treatment. He brought in a piece of polyurethane tubing to a subsequent visit to bite on during the injections (Figure) and reported considerable relief of pain.
What you were not taught in United States history class was that this method—clenching an object orally—dates to the era before the Civil War, before appropriate anesthetics and analgesics were developed, when patients and soldiers bit on a bullet or leather strap during surgical procedures.4 Clenching and chewing have been shown to promote relaxation and reduce acute pain and stress.5
Practical Implications
Polyurethane tubing can be purchased in bulk, is inexpensive ($0.30/foot on Amazon), and unlikely to damage teeth due to its flexibility. It can be cut into 6-inch pieces and given to the patient at their first nail injection appointment. The patient can then bring the tubing to subsequent appointments to use as a mastication tool during nail injections.
We instruct the patient to disinfect the dedicated piece of tubing after the initial visit and each subsequent visit by soaking it for 15 minutes in either a 3% hydrogen peroxide solution, antibacterial mouthwash, a solution of baking soda (bicarbonate of soda) and water (1 cup of water to 2 teaspoons of baking soda), or white vinegar. We instruct them to thoroughly dry the disinfected polyurethane tube and store it in a clean, reusable, resealable zipper storage bag between appointments.
In addition to reducing anxiety and pain, this method also distracts the patient and therefore promotes patient and physician safety. Patients are less likely to jump or startle during the injection, thereby reducing the risk of physically interfering with the nail surgeon or making an unanticipated advance into the surgical field.
Although frustrated patients with nail disease may need to “bite the bullet” when they accept treatment with nail injections, lessons from our patient and from United States history offer a safe and cost-effective pain management strategy. Minimizing discomfort and anxiety during the first nail injection is crucial because doing so is likely to promote adherence with follow-up injections and therefore improve clinical outcomes.
Future clinical studies should validate the clinical utility of oral mastication and clenching during nail procedures compared to other perioperative stress- and anxiety-reducing techniques.
- Ricardo JW, Lipner SR. Utilization of a stress ball to diminish anxiety during nail surgery. Cutis. 2020;105:294. doi:10.12788/cutis.0013
- Ricardo JW, Lipner SR. Utilizing a sleep mask to reduce patient anxiety during nail surgery. Cutis. 2021;108:36. doi:10.12788/cutis.0285
- Ip HYV, Abrishami A, Peng PW, et al. Predictors of postoperative pain and analgesic consumption: a qualitative systematic review. Anesthesiology. 2009;111:657-677. doi:10.1097/ALN.0b013e3181aae87a
- Albin MS. The use of anesthetics during the Civil War, 1861-1865. Pharm Hist. 2000;42:99-114.
- Tahara Y, Sakurai K, Ando T. Influence of chewing and clenching on salivary cortisol levels as an indicator of stress. J Prosthodont. 2007;16:129-135. doi:10.1111/j.1532-849X.2007.00178.x
- Ricardo JW, Lipner SR. Utilization of a stress ball to diminish anxiety during nail surgery. Cutis. 2020;105:294. doi:10.12788/cutis.0013
- Ricardo JW, Lipner SR. Utilizing a sleep mask to reduce patient anxiety during nail surgery. Cutis. 2021;108:36. doi:10.12788/cutis.0285
- Ip HYV, Abrishami A, Peng PW, et al. Predictors of postoperative pain and analgesic consumption: a qualitative systematic review. Anesthesiology. 2009;111:657-677. doi:10.1097/ALN.0b013e3181aae87a
- Albin MS. The use of anesthetics during the Civil War, 1861-1865. Pharm Hist. 2000;42:99-114.
- Tahara Y, Sakurai K, Ando T. Influence of chewing and clenching on salivary cortisol levels as an indicator of stress. J Prosthodont. 2007;16:129-135. doi:10.1111/j.1532-849X.2007.00178.x
Multiple Fingerlike Projections on the Leg
The Diagnosis: Elephantiasis Nostras Verrucosa
Histopathology revealed a benign fibroepithelial polyp demonstrating areas of hyperkeratosis, acanthosis, and focal papillomatosis (Figure, A). Increased superficial vessels with dilated lymphatics, stellate fibroblasts, edematous stroma, and plasmolymphocytosis also were noted (Figure, B). Clinical and histopathological findings led to a diagnosis of lymphedema papules in the setting of elephantiasis nostra verrucosa (ENV).
Elephantiasis nostras verrucosa is a complication of long-standing nonfilarial obstruction of lymphatic drainage leading to grotesque enlargement of the affected areas. Common cutaneous manifestations of ENV include nonpitting edema, dermal fibrosis, and extensive hyperkeratosis with verrucous and papillomatous lesions.1 In the beginning stages of ENV, the skin has a cobblestonelike appearance. As the disease progresses, the verrucous lesions continue to enlarge, giving the affected area a mossy appearance. Although less common, groupings of large papillomas similar to our patient’s presentation also can form.2 Ulcer formation is more likely to occur in advanced disease states, increasing the risk for bacterial and fungal colonization. Elephantiasis nostras verrucosa classically affects the legs; however, this condition can develop in any area with chronic lymphedema. Cases of ENV involving the arms, abdomen, scrotum, and ear have been documented.3-5
The pathogenesis of ENV involves the proliferation of fibroblasts and fibrosis secondary to lymphostasis and inflammation.6 When interstitial fluid builds up in the affected region, the protein-rich fluid is believed to trigger fibrogenesis and increase macrophage, keratinocyte, and adipocyte activity.7 Because of this inflammatory process, dilation and fibrosis of the lymphatic channels develop. Lymphatic obstruction can have several etiologies, most notably infection and malignancy. Staphylococcal lymphangitis and erysipelas create fibrosis of the lymphatic system and are the main infectious causes of ENV.6 Large tumors or lymphomas are insidious causes of lymphatic obstruction and should be ruled out when investigating for ENV. Other risk factors include obesity, chronic venous insufficiency, surgery, trauma, radiation, and uncontrolled congestive heart failure.1,6,8
An ENV diagnosis is clinicopathologic, involving a comprehensive metabolic panel and complete blood cell count with differential. A biopsy is needed for pathologic confirmation and to rule out malignancy. Histologically, ENV is characterized by pseudoepitheliomatous hyperplasia, dermal fibrosis, hyperkeratosis of the epidermis, and dilated lymphatic vessels.6,8 Additional studies for diagnosis include wound and lymph node culture, Wood lamp examination, and lymphoscintigraphy.
Given the chronic and progressive nature of the disease, ENV is difficult to treat. There currently is no standard of treatment, but the mainstay of management involves reducing peripheral edema. Lifestyle changes including weight loss, extremity elevation, and increased ambulation are helpful first-line therapies.3 Compression of the affected extremity using stockings or intermittent pneumatic compression devices has proven to be beneficial with long-term use.7 Patients should be followed for wound care to prevent the infection of ulcers.2 Pharmacologic treatments include systemic retinoids, which have been shown to reduce the appearance of hyperkeratosis, verrucous lesions, and papillomatous nodules.6 Prophylactic antibiotics are reserved for advanced stages of disease or in patients with recurrent infections.2,7 In severe cases of ENV that are unresponsive to medical management, surgical intervention such as lymphatic anastomosis and debulking may be considered.9,10
Other diagnoses to consider for ENV include pretibial myxedema, lymphatic filariasis, Stewart-Treves syndrome, and papillomatosis cutis carcinoides. Pretibial myxedema is an uncommon dermatologic manifestation of Graves disease. It is a local autoimmune reaction in the cutaneous tissue characterized by hyperpigmentation, nonpitting edema, and nodules on the anterior leg. Histopathology shows increased hyaluronic acid and chondroitin as well as compression of dermal lymphatics.11
Filariasis is a parasitic infection caused by Wuchereria bancrofti, Brugia malayi or Brugia timori, and Onchocerca volvulus.6 This condition presents with elephantiasis of the affected extremities but should be considered in areas endemic for filarial parasites such as tropical and subtropical countries.12 Eosinophilia and identification of microfilaria in a peripheral blood smear would indicate parasitic infection. Stewart-Treves syndrome is a rare angiosarcoma that arises in areas of chronic lymphedema. This condition classically is seen on the upper extremities following a mastectomy with lymphadenectomy, lymph node irradiation, or both.
Stewart-Treves syndrome presents with coalescing purpuric macules and nodules that eventually coalesce into cutaneous masses. Histopathology reveals proliferating vascular channels that split apart dermal collagen with hyperchromatism and pleomorphism in the tumor endothelial cells that line these channels.13
Papillomatosis cutis carcinoides is a low-grade squamous cell carcinoma that occurs secondary to human papillomavirus commonly affecting the mouth, anogenital area, and the plantar surfaces of the feet. It presents with exophytic growths and ulcerated tumors that are unilateral and asymmetrical. The presence of blunt-shaped tumor projections extending deep into the dermis to form sinuses and keratin-filled cysts is characteristic of papillomatosis cutis carcinoides.14
- Dean SM, Zirwas MJ, Horst AV. Elephantiasis nostras verrucosa: an institutional analysis of 21 cases. J Am Acad Dermatol. 2011;64: 1104-1110. doi:10.1016/j.jaad.2010.04.047
- Fife CE, Farrow W, Hebert AA, et al. Skin and wound care in lymphedema patients: a taxonomy, primer, and literature review. Adv Skin Wound Care. 2017;30:305-318. doi:10.1097/01.ASW.0000520501.23702.82
- Boyd J, Sloan S, Meffert J. Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene. J Drugs Dermatol. 2004; 3:446-448.
- Nakai K, Taoka R, Sugimoto M, et al. Genital elephantiasis possibly caused by chronic inguinal eczema with streptococcal infection. J Dermatol. 2019;46:E196-E198. doi:10.1111/1346-8138.14746
- Carlson JA, Mazza J, Kircher K, et al. Otophyma: a case report and review of the literature of lymphedema (elephantiasis) of the ear. Am J Dermatopathol. 2008;30:67-72. doi:10.1097/DAD.0b013e31815cd937
- Sisto K, Khachemoune A. Elephantiasis nostras verrucosa: a review. Am J Clin Dermatol. 2008;9:141-146. doi:10.2165/00128071-200809030-00001
- Yoho RM, Budny AM, Pea AS. Elephantiasis nostras verrucosa. J Am Podiatr Med Assoc. 2006;96:442-444. doi:10.7547/0960442
- Yosipovitch G, DeVore A, Dawn A. Obesity and the skin: skin physiology and skin manifestations of obesity. J Am Acad Dermatol. 2007;56:901-920. doi:10.1016/j.jaad.2006.12.004
- Iwao F, Sato-Matsumura KC, Sawamura D, et al. Elephantiasis nostras verrucosa successfully treated by surgical debridement. Dermatol Surg. 2004;30:939-941. doi:10.1111/j.1524-4725.2004.30267.x
- Tiwari A, Cheng KS, Button M, et al. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg. 2003;138:152-161. doi:10.1001/archsurg.138.2.152
- Fatourechi V. Pretibial myxedema: pathophysiology and treatment options. Am J Clin Dermatol. 2005;6:295-309. doi:10.2165 /00128071-200506050-00003
- Addiss DG, Brady MA. Morbidity management in the Global Programme to Eliminate Lymphatic Filariasis: a review of the scientific literature. Filaria J. 2007;6:2. doi:10.1186/1475-2883-6-2
- Bernia E, Rios-Viñuela E, Requena C. Stewart-Treves syndrome. JAMA Dermatol. 2021;157:721. doi:10.1001/jamadermatol.2021.0341
- Schwartz RA. Verrucous carcinoma of the skin and mucosa. J Am Acad Dermatol. 1995;32:1-24. doi:10.1016/0190-9622(95)90177-9
The Diagnosis: Elephantiasis Nostras Verrucosa
Histopathology revealed a benign fibroepithelial polyp demonstrating areas of hyperkeratosis, acanthosis, and focal papillomatosis (Figure, A). Increased superficial vessels with dilated lymphatics, stellate fibroblasts, edematous stroma, and plasmolymphocytosis also were noted (Figure, B). Clinical and histopathological findings led to a diagnosis of lymphedema papules in the setting of elephantiasis nostra verrucosa (ENV).
Elephantiasis nostras verrucosa is a complication of long-standing nonfilarial obstruction of lymphatic drainage leading to grotesque enlargement of the affected areas. Common cutaneous manifestations of ENV include nonpitting edema, dermal fibrosis, and extensive hyperkeratosis with verrucous and papillomatous lesions.1 In the beginning stages of ENV, the skin has a cobblestonelike appearance. As the disease progresses, the verrucous lesions continue to enlarge, giving the affected area a mossy appearance. Although less common, groupings of large papillomas similar to our patient’s presentation also can form.2 Ulcer formation is more likely to occur in advanced disease states, increasing the risk for bacterial and fungal colonization. Elephantiasis nostras verrucosa classically affects the legs; however, this condition can develop in any area with chronic lymphedema. Cases of ENV involving the arms, abdomen, scrotum, and ear have been documented.3-5
The pathogenesis of ENV involves the proliferation of fibroblasts and fibrosis secondary to lymphostasis and inflammation.6 When interstitial fluid builds up in the affected region, the protein-rich fluid is believed to trigger fibrogenesis and increase macrophage, keratinocyte, and adipocyte activity.7 Because of this inflammatory process, dilation and fibrosis of the lymphatic channels develop. Lymphatic obstruction can have several etiologies, most notably infection and malignancy. Staphylococcal lymphangitis and erysipelas create fibrosis of the lymphatic system and are the main infectious causes of ENV.6 Large tumors or lymphomas are insidious causes of lymphatic obstruction and should be ruled out when investigating for ENV. Other risk factors include obesity, chronic venous insufficiency, surgery, trauma, radiation, and uncontrolled congestive heart failure.1,6,8
An ENV diagnosis is clinicopathologic, involving a comprehensive metabolic panel and complete blood cell count with differential. A biopsy is needed for pathologic confirmation and to rule out malignancy. Histologically, ENV is characterized by pseudoepitheliomatous hyperplasia, dermal fibrosis, hyperkeratosis of the epidermis, and dilated lymphatic vessels.6,8 Additional studies for diagnosis include wound and lymph node culture, Wood lamp examination, and lymphoscintigraphy.
Given the chronic and progressive nature of the disease, ENV is difficult to treat. There currently is no standard of treatment, but the mainstay of management involves reducing peripheral edema. Lifestyle changes including weight loss, extremity elevation, and increased ambulation are helpful first-line therapies.3 Compression of the affected extremity using stockings or intermittent pneumatic compression devices has proven to be beneficial with long-term use.7 Patients should be followed for wound care to prevent the infection of ulcers.2 Pharmacologic treatments include systemic retinoids, which have been shown to reduce the appearance of hyperkeratosis, verrucous lesions, and papillomatous nodules.6 Prophylactic antibiotics are reserved for advanced stages of disease or in patients with recurrent infections.2,7 In severe cases of ENV that are unresponsive to medical management, surgical intervention such as lymphatic anastomosis and debulking may be considered.9,10
Other diagnoses to consider for ENV include pretibial myxedema, lymphatic filariasis, Stewart-Treves syndrome, and papillomatosis cutis carcinoides. Pretibial myxedema is an uncommon dermatologic manifestation of Graves disease. It is a local autoimmune reaction in the cutaneous tissue characterized by hyperpigmentation, nonpitting edema, and nodules on the anterior leg. Histopathology shows increased hyaluronic acid and chondroitin as well as compression of dermal lymphatics.11
Filariasis is a parasitic infection caused by Wuchereria bancrofti, Brugia malayi or Brugia timori, and Onchocerca volvulus.6 This condition presents with elephantiasis of the affected extremities but should be considered in areas endemic for filarial parasites such as tropical and subtropical countries.12 Eosinophilia and identification of microfilaria in a peripheral blood smear would indicate parasitic infection. Stewart-Treves syndrome is a rare angiosarcoma that arises in areas of chronic lymphedema. This condition classically is seen on the upper extremities following a mastectomy with lymphadenectomy, lymph node irradiation, or both.
Stewart-Treves syndrome presents with coalescing purpuric macules and nodules that eventually coalesce into cutaneous masses. Histopathology reveals proliferating vascular channels that split apart dermal collagen with hyperchromatism and pleomorphism in the tumor endothelial cells that line these channels.13
Papillomatosis cutis carcinoides is a low-grade squamous cell carcinoma that occurs secondary to human papillomavirus commonly affecting the mouth, anogenital area, and the plantar surfaces of the feet. It presents with exophytic growths and ulcerated tumors that are unilateral and asymmetrical. The presence of blunt-shaped tumor projections extending deep into the dermis to form sinuses and keratin-filled cysts is characteristic of papillomatosis cutis carcinoides.14
The Diagnosis: Elephantiasis Nostras Verrucosa
Histopathology revealed a benign fibroepithelial polyp demonstrating areas of hyperkeratosis, acanthosis, and focal papillomatosis (Figure, A). Increased superficial vessels with dilated lymphatics, stellate fibroblasts, edematous stroma, and plasmolymphocytosis also were noted (Figure, B). Clinical and histopathological findings led to a diagnosis of lymphedema papules in the setting of elephantiasis nostra verrucosa (ENV).
Elephantiasis nostras verrucosa is a complication of long-standing nonfilarial obstruction of lymphatic drainage leading to grotesque enlargement of the affected areas. Common cutaneous manifestations of ENV include nonpitting edema, dermal fibrosis, and extensive hyperkeratosis with verrucous and papillomatous lesions.1 In the beginning stages of ENV, the skin has a cobblestonelike appearance. As the disease progresses, the verrucous lesions continue to enlarge, giving the affected area a mossy appearance. Although less common, groupings of large papillomas similar to our patient’s presentation also can form.2 Ulcer formation is more likely to occur in advanced disease states, increasing the risk for bacterial and fungal colonization. Elephantiasis nostras verrucosa classically affects the legs; however, this condition can develop in any area with chronic lymphedema. Cases of ENV involving the arms, abdomen, scrotum, and ear have been documented.3-5
The pathogenesis of ENV involves the proliferation of fibroblasts and fibrosis secondary to lymphostasis and inflammation.6 When interstitial fluid builds up in the affected region, the protein-rich fluid is believed to trigger fibrogenesis and increase macrophage, keratinocyte, and adipocyte activity.7 Because of this inflammatory process, dilation and fibrosis of the lymphatic channels develop. Lymphatic obstruction can have several etiologies, most notably infection and malignancy. Staphylococcal lymphangitis and erysipelas create fibrosis of the lymphatic system and are the main infectious causes of ENV.6 Large tumors or lymphomas are insidious causes of lymphatic obstruction and should be ruled out when investigating for ENV. Other risk factors include obesity, chronic venous insufficiency, surgery, trauma, radiation, and uncontrolled congestive heart failure.1,6,8
An ENV diagnosis is clinicopathologic, involving a comprehensive metabolic panel and complete blood cell count with differential. A biopsy is needed for pathologic confirmation and to rule out malignancy. Histologically, ENV is characterized by pseudoepitheliomatous hyperplasia, dermal fibrosis, hyperkeratosis of the epidermis, and dilated lymphatic vessels.6,8 Additional studies for diagnosis include wound and lymph node culture, Wood lamp examination, and lymphoscintigraphy.
Given the chronic and progressive nature of the disease, ENV is difficult to treat. There currently is no standard of treatment, but the mainstay of management involves reducing peripheral edema. Lifestyle changes including weight loss, extremity elevation, and increased ambulation are helpful first-line therapies.3 Compression of the affected extremity using stockings or intermittent pneumatic compression devices has proven to be beneficial with long-term use.7 Patients should be followed for wound care to prevent the infection of ulcers.2 Pharmacologic treatments include systemic retinoids, which have been shown to reduce the appearance of hyperkeratosis, verrucous lesions, and papillomatous nodules.6 Prophylactic antibiotics are reserved for advanced stages of disease or in patients with recurrent infections.2,7 In severe cases of ENV that are unresponsive to medical management, surgical intervention such as lymphatic anastomosis and debulking may be considered.9,10
Other diagnoses to consider for ENV include pretibial myxedema, lymphatic filariasis, Stewart-Treves syndrome, and papillomatosis cutis carcinoides. Pretibial myxedema is an uncommon dermatologic manifestation of Graves disease. It is a local autoimmune reaction in the cutaneous tissue characterized by hyperpigmentation, nonpitting edema, and nodules on the anterior leg. Histopathology shows increased hyaluronic acid and chondroitin as well as compression of dermal lymphatics.11
Filariasis is a parasitic infection caused by Wuchereria bancrofti, Brugia malayi or Brugia timori, and Onchocerca volvulus.6 This condition presents with elephantiasis of the affected extremities but should be considered in areas endemic for filarial parasites such as tropical and subtropical countries.12 Eosinophilia and identification of microfilaria in a peripheral blood smear would indicate parasitic infection. Stewart-Treves syndrome is a rare angiosarcoma that arises in areas of chronic lymphedema. This condition classically is seen on the upper extremities following a mastectomy with lymphadenectomy, lymph node irradiation, or both.
Stewart-Treves syndrome presents with coalescing purpuric macules and nodules that eventually coalesce into cutaneous masses. Histopathology reveals proliferating vascular channels that split apart dermal collagen with hyperchromatism and pleomorphism in the tumor endothelial cells that line these channels.13
Papillomatosis cutis carcinoides is a low-grade squamous cell carcinoma that occurs secondary to human papillomavirus commonly affecting the mouth, anogenital area, and the plantar surfaces of the feet. It presents with exophytic growths and ulcerated tumors that are unilateral and asymmetrical. The presence of blunt-shaped tumor projections extending deep into the dermis to form sinuses and keratin-filled cysts is characteristic of papillomatosis cutis carcinoides.14
- Dean SM, Zirwas MJ, Horst AV. Elephantiasis nostras verrucosa: an institutional analysis of 21 cases. J Am Acad Dermatol. 2011;64: 1104-1110. doi:10.1016/j.jaad.2010.04.047
- Fife CE, Farrow W, Hebert AA, et al. Skin and wound care in lymphedema patients: a taxonomy, primer, and literature review. Adv Skin Wound Care. 2017;30:305-318. doi:10.1097/01.ASW.0000520501.23702.82
- Boyd J, Sloan S, Meffert J. Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene. J Drugs Dermatol. 2004; 3:446-448.
- Nakai K, Taoka R, Sugimoto M, et al. Genital elephantiasis possibly caused by chronic inguinal eczema with streptococcal infection. J Dermatol. 2019;46:E196-E198. doi:10.1111/1346-8138.14746
- Carlson JA, Mazza J, Kircher K, et al. Otophyma: a case report and review of the literature of lymphedema (elephantiasis) of the ear. Am J Dermatopathol. 2008;30:67-72. doi:10.1097/DAD.0b013e31815cd937
- Sisto K, Khachemoune A. Elephantiasis nostras verrucosa: a review. Am J Clin Dermatol. 2008;9:141-146. doi:10.2165/00128071-200809030-00001
- Yoho RM, Budny AM, Pea AS. Elephantiasis nostras verrucosa. J Am Podiatr Med Assoc. 2006;96:442-444. doi:10.7547/0960442
- Yosipovitch G, DeVore A, Dawn A. Obesity and the skin: skin physiology and skin manifestations of obesity. J Am Acad Dermatol. 2007;56:901-920. doi:10.1016/j.jaad.2006.12.004
- Iwao F, Sato-Matsumura KC, Sawamura D, et al. Elephantiasis nostras verrucosa successfully treated by surgical debridement. Dermatol Surg. 2004;30:939-941. doi:10.1111/j.1524-4725.2004.30267.x
- Tiwari A, Cheng KS, Button M, et al. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg. 2003;138:152-161. doi:10.1001/archsurg.138.2.152
- Fatourechi V. Pretibial myxedema: pathophysiology and treatment options. Am J Clin Dermatol. 2005;6:295-309. doi:10.2165 /00128071-200506050-00003
- Addiss DG, Brady MA. Morbidity management in the Global Programme to Eliminate Lymphatic Filariasis: a review of the scientific literature. Filaria J. 2007;6:2. doi:10.1186/1475-2883-6-2
- Bernia E, Rios-Viñuela E, Requena C. Stewart-Treves syndrome. JAMA Dermatol. 2021;157:721. doi:10.1001/jamadermatol.2021.0341
- Schwartz RA. Verrucous carcinoma of the skin and mucosa. J Am Acad Dermatol. 1995;32:1-24. doi:10.1016/0190-9622(95)90177-9
- Dean SM, Zirwas MJ, Horst AV. Elephantiasis nostras verrucosa: an institutional analysis of 21 cases. J Am Acad Dermatol. 2011;64: 1104-1110. doi:10.1016/j.jaad.2010.04.047
- Fife CE, Farrow W, Hebert AA, et al. Skin and wound care in lymphedema patients: a taxonomy, primer, and literature review. Adv Skin Wound Care. 2017;30:305-318. doi:10.1097/01.ASW.0000520501.23702.82
- Boyd J, Sloan S, Meffert J. Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene. J Drugs Dermatol. 2004; 3:446-448.
- Nakai K, Taoka R, Sugimoto M, et al. Genital elephantiasis possibly caused by chronic inguinal eczema with streptococcal infection. J Dermatol. 2019;46:E196-E198. doi:10.1111/1346-8138.14746
- Carlson JA, Mazza J, Kircher K, et al. Otophyma: a case report and review of the literature of lymphedema (elephantiasis) of the ear. Am J Dermatopathol. 2008;30:67-72. doi:10.1097/DAD.0b013e31815cd937
- Sisto K, Khachemoune A. Elephantiasis nostras verrucosa: a review. Am J Clin Dermatol. 2008;9:141-146. doi:10.2165/00128071-200809030-00001
- Yoho RM, Budny AM, Pea AS. Elephantiasis nostras verrucosa. J Am Podiatr Med Assoc. 2006;96:442-444. doi:10.7547/0960442
- Yosipovitch G, DeVore A, Dawn A. Obesity and the skin: skin physiology and skin manifestations of obesity. J Am Acad Dermatol. 2007;56:901-920. doi:10.1016/j.jaad.2006.12.004
- Iwao F, Sato-Matsumura KC, Sawamura D, et al. Elephantiasis nostras verrucosa successfully treated by surgical debridement. Dermatol Surg. 2004;30:939-941. doi:10.1111/j.1524-4725.2004.30267.x
- Tiwari A, Cheng KS, Button M, et al. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg. 2003;138:152-161. doi:10.1001/archsurg.138.2.152
- Fatourechi V. Pretibial myxedema: pathophysiology and treatment options. Am J Clin Dermatol. 2005;6:295-309. doi:10.2165 /00128071-200506050-00003
- Addiss DG, Brady MA. Morbidity management in the Global Programme to Eliminate Lymphatic Filariasis: a review of the scientific literature. Filaria J. 2007;6:2. doi:10.1186/1475-2883-6-2
- Bernia E, Rios-Viñuela E, Requena C. Stewart-Treves syndrome. JAMA Dermatol. 2021;157:721. doi:10.1001/jamadermatol.2021.0341
- Schwartz RA. Verrucous carcinoma of the skin and mucosa. J Am Acad Dermatol. 1995;32:1-24. doi:10.1016/0190-9622(95)90177-9
A 61-year-old man presented with painful skin growths on the right pretibial region of several months’ duration. The patient reported pain due to friction between the lesions and underlying skin, leading to erosions. His medical history was remarkable for morbid obesity (body mass index of 62), chronic venous stasis, and chronic lymphedema. The patient was followed for wound care of venous stasis ulcers. Dermatologic examination revealed multiple 5- to 30-mm, flesh-colored, fingerlike projections on the right tibial region. A biopsy was obtained and submitted for histopathologic analysis.