The Journal of Foot & Ankle Surgery 53 (2014) 62–66

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Norwegian Crusted Scabies: An Unusual Case Presentation Michael M. Maghrabi, DPM 1, Shireen Lum, DPM 2, Ameha T. Joba, DPM 2, Molly J. Meier, DPM 3, Ryan J. Holmbeck, DPM 4, Kate Kennedy, DPM 5 1

Director of Podiatric Medicine and Surgery Residency Program, Presence Health, Saints Mary and Elizabeth Medical Center, Chicago, IL Attending, Presence Health, Saints Mary and Elizabeth Medical Center, Chicago, IL 3 PGY3, Presence Health, Saints Mary and Elizabeth Medical Center, Chicago, IL 4 PGY2, Presence Health, Saints Mary and Elizabeth Medical Center, Chicago, IL 5 PGY1, Presence Health, Saints Mary and Elizabeth Medical Center, Chicago, IL 2

a r t i c l e i n f o

a b s t r a c t

Level of Clinical Evidence: 4

Scabies is a contagious condition that is transmitted through direct contact with an infected person and has been frequently associated with institutional and healthcare-facility outbreaks. The subtype Norwegian crusted scabies can masquerade as other dermatologic diseases owing to the heavy plaque formation. Successful treatment has been documented in published reports, including oral ivermectin and topical permethrin. Few case studies documenting the treatment of Norwegian crusted scabies have reported the use of surgical debridement as an aid to topical and/or oral treatment when severe plaque formation has been noted. A nursing home patient was admitted to the hospital for severe plaque formation of both feet. A superficial biopsy was negative for both fungus and scabies because of the severity of the plaque formation on both feet. The patient underwent a surgical, diagnostic biopsy of both feet, leading to the diagnosis of Norwegian crusted scabies. A second surgical debridement was then performed to remove the extensive plaque formation and aid the oral ivermectin and topical permethrin treatment. The patient subsequently made a full recovery and was discharged back to the nursing home. At 2 and 6 months after treatment, the patient remained free of scabies infestation, and the surgical wound had healed uneventfully. The present case presentation has demonstrated that surgical debridement can be complementary to the standard topical and oral medications in the treatment of those with Norwegian crusted scabies infestation. Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved.

Keywords: debridement infestation ivermectin Norwegian parasite permethrin Sarcoptes scabiei

Sarcoptes scabiei var hominis, or crusted Norwegian scabies, is a rare, atypical, and highly infectious variant of Sarcoptes scabiei (1). Clinically, this condition visually presents as a dermatitis and causes extensive hyperkeratotic lesions, nail thickening, and dystrophy (2). Patients characteristically complain of generalized pruritus, erythematous papules, and signs of scabies burrows (1,2). Newly infected patients might not experience symptoms of infection for up to 3 weeks, owing to a delayed type IV hypersensitivity reaction to the mite and its saliva, eggs, and excrement (3,4). Treatment for this condition has included both topical and systemic therapy, most commonly with oral ivermectin (5) and topical permethrin (6). Norwegian crusted scabies is an extremely contagious condition transmitted through direct contact with an infected person and has been associated with institutional and healthcare facility outbreaks (7,8). Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Michael M. Maghrabi, DPM, Director of Podiatric Medicine and Surgery Residency Program, Presence Health, Saints Mary and Elizabeth Medical Center, 2233 West Division Street, Chicago, IL 60622. E-mail address: [email protected] (M.M. Maghrabi).

In the present case description, we report a unique case of Norwegian crusted scabies with severe dystrophic and deforming plaque formation of both feet. Treatment had been delayed for 3 months, and the diagnosis was derived from inspection of a fullthickness biopsy in the operating room. Subsequent surgical debridement was performed to allow for improved penetration of topical permethrin. The treatment was successful, and the patient subsequently had a full recovery from the infestation.

Case Report A 60-year-old, nondiabetic, male nursing home patient presented to the emergency department with a 3-month history of an abnormal growth on both feet. The patient related that the abnormal growths had initially presented on the right foot and had rapidly spread to the left. The patient had increased pain in both feet on weightbearing and ambulation. He denied itching, fever, chills, nausea, vomiting, and other constitutional signs of infection. He had a medical history positive for bipolar disorder, schizoaffective disorder, hypertension, hepatitis C, and peripheral neuropathy secondary to alcoholism.

1067-2516/$ - see front matter Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2013.09.002

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Fig. 3. Radiograph of foot at initial presentation.

The physical examination revealed thick, brown plaques encompassing all the toes and most of the plantar surface of both feet (Figs. 1 and 2). The plaques were rigid, not freely moveable, and extended the entirety of the length of the toes. No purulence, no malodor, and no periwound or ascending erythema was noted. No open lesions were noted on either foot. The skin on the dorsum of the foot proximal to the Lisfranc joint was free of pathologic changes. The patient experienced pain with palpation and passive range of motion in the areas of the foot affected by plaque formation. Pedal pulses were palpable þ2/4,

bilateral. The capillary refill time was unable to be evaluated owing to the severity of the plaque formation. Protective sensation was diminished distal to the level of the malleoli, bilateral, using the 5.07/10-g Semmes-Weinstein monofilament test. The patient was admitted to the hospital and started intravenous (IV) vancomycin, with IV fluconazole (Diflucan) and topical ketoconazole 2% cream. Radiographs revealed multiple, condensed soft tissue lesions throughout both feet. This was most notable at the level of the digits and on the plantar surface of both feet. Chronic erosive changes were present at the level of the middle and distal phalanges. No radiographic evidence was found of a periosteal reaction (Figs. 3 and 4).

Fig. 2. View of dorsum surface of foot at initial presentation.

Fig. 4. Lateral radiograph at initial presentation.

Fig. 1. View of plantar surface of foot at initial presentation.

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Fig. 5. Microscopic evaluation of scabies mite.

Fig. 7. Specimen collected from surgical debridement.

A superficial biopsy specimen and samples for a culture and sensitivity test were taken at bedside. The pathology report indicated benign hyperkeratotic skin with a drying artifact and secondary infection. The special stains were negative for fungal infestation. The culture and sensitivity test revealed methicillin-susceptible Staphylococcus aureus. The infectious disease service was consulted, and the patient continued receiving IV cefazolin (Ancef). The IV vancomycin was discontinued. At that point, it was decided to take the patient to the operating room for full-thickness biopsies of both feet to acquire a conclusive diagnosis. With the patient under IV sedation with a local anesthetic block, 2 full-thickness biopsy specimens were attained, 1 from each foot. The right foot biopsy specimen was excised just proximal to the

fifth metatarsal base and measured 3 cm  2 cm. The left foot biopsy specimen was removed at the medial instep of the foot and measured 2 cm  2 cm. Both biopsy sites were then swabbed, and the samples were sent for aerobic and anaerobic culture and sensitivity testing and fungal periodic acid-Schiff staining. The biopsy specimens were sent for pathologic examination. The surgical sites were dressed with a nonadherent sterile dressing. The patient was readmitted to the floor. The biopsy specimens from both feet were positive for extensive scabies infection, Sarcoptes scabiei var hominis, also known as Norwegian crusted scabies (Fig. 5). Massive orthokeratosis and parakeratosis were noted, containing mites in all stages of development. The epidermis demonstrated psoriasis with hyperplastic and focal spongiosis. The dermis showed deep chronic inflammatory infiltrate with marked eosinophilia. The intraoperative culture and sensitivity testing revealed methicillin-resistant S. aureus and group B Streptococcus agalactiae. The anaerobic culture showed no growth of bacteria, and the fungal periodic-acid Schiff stain was negative. The patient began therapy with oral ivermectin and topical permethrin cream. He was also given oral Bactrim, in addition to the IV cefazolin (Ancef), by the infectious disease service, again, to protect against the development of bacteremia from the longstanding scabies infection. The IV and topical antifungal agents were discontinued at that point. The social worker on the case contacted the patient’s nursing home to alert them of the highly contagious nature of the patient’s condition. Owing to the severity of the plaque formations on both feet, it was decided that the patient would benefit from additional surgical debridement to facilitate quicker penetration of the topical permethrin medication. Two days after the surgical biopsy, the patient was taken to the operating room for additional surgical debridement. The plaque-like margin was delicately removed to preserve the underlying skin margins. The plaque was removed along the plantar aspect of the foot and the distal aspects of the digits. It was noted that the right third digit was encompassed within the necrotic and hyperkeratotic tissue. The distal aspect of the right third digit was disarticulated at the level of the proximal interphalangeal joint. The distal aspect of the wound was left open because of the severity of the scabies infestation. The remainder of the plaques and nails were removed from both feet, leaving a hyperemic bleeding response from all nail beds. All digits were left intact on the left foot (Fig. 6). The tissue removed from both feet was sent for pathologic examination (Fig. 7). The surgical wounds were again

Fig. 6. Intraoperative view of foot after debridement.

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dressed with Xeroform gauze, 4  4 gauze, Kerlix, and Coban. The patient was readmitted to the floor and continued receiving oral ivermectin and topical permethrin therapy. He was continued with IV cefazolin (Ancef) and oral Bactrim, as recommended by the infectious disease service. The pathology report from the second surgery demonstrated hyperkeratotic and orthokeratotic skin with severe, extensive scabies infection of bilateral toes and nails. No malignancy was seen. The patient was transferred from the inpatient unit to an on-campus skilled nursing facility, where he underwent physical and occupational therapy for 2 weeks. The IV cefazolin (Ancef) and oral Bactrim were continued, according to the infectious disease recommendation. The antibiotics were discontinued on the patient’s discharge back to the nursing home. The infectious disease doctor recommended an additional week of oral ivermectin treatment and instructed the patient to continue the topical permethrin cream on discharge. The patient was seen 1 week later in the office. He had residual pain from the extensive debridement of both feet, but the healing was progressing well. No signs of infection or scabies burrows or crusting were noted. The patient was told to continue with the permethrin cream on both feet until the next follow-up visit in 1 month’s time, in accordance with the recommendation of the infectious disease service. The patient did not return for follow-up until 2 months later. The infection had completely resolved at that time, and all surgical wounds had epithelialized, including the amputation site at the right third digit. The patient had discontinued the permethrin cream 1 month earlier by order of the infectious disease doctor. The patient returned for a final follow-up visit at 6 months postoperatively (Fig. 8). The patient was told to continue with regular moisturizing cream and to return to the clinic as needed. Complete resolution of the infection had been achieved, and all

Fig. 8. Complete resolution of scabies infection 6 months following surgical debridement.

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surgical wounds had healed. It is unclear what type of sanitizing precaution was used or whether other patients had been diagnosed with Norwegian crusted scabies at the patient’s nursing home.

Discussion Scabies is caused by the mite Sarcoptes Scabiei var hominis (1–9) and includes 4 variants of infection (9). The nodular form of infection is composed of pruritic nodules located in the axillae, waist, and groin (9,10). Scabies incognito typically occurs in an unusual or widespread distribution after treatment with topical or oral steroids (9,10). In infants, scabies causes widespread lesions, with the addition of pustules on the palms and soles (9,10). Crusted Norwegian scabies is the hyperkeratotic form of the disease, and the patients most at risk include the immunocompromised and institutionalized. The crusted form of this disease has been recognized as having a much greater mite count, which can be more than 1 million (4,7–10). Crusted scabies is less pruritic and will present as thick, white-gray plaques commonly found on extremities (8). The presentation can also include nonspecific-type urticarial papules and itchy excoriations and crusts (11). Norwegian crusted scabies is a rare variant of scabies infestation, and the patients most at risk include the elderly, immunocompromised, and institutionalized. A few patients with Norwegian crusted scabies have had no overt history of immunodeficiency. However, this is the most frequent parasitic infection found in patients with human immunodeficiency virus (12–14). Patients with a compromised immune system will be unable to handle the scabies mite population, which leads to the body producing an inflammatory response and hyperkeratotic reaction (11). Scabies lesions can be obscured by secondary bacterial infection, excoriation, or pre-existing dermatologic disease (9). Scabies mites are obligate human parasites that live in the upper stratum of the epidermis (15). These mites are unable to jump; however, they can crawl 2 to 5 cm/min on the skin (1). Female mites can grow to be up to 0.3 mm  0.5 mm; the male mites are smaller (1,12,15). The average human scabies infestation has up to 15 female mites, which can lay up to 3 eggs per day in the skin burrows (1). Scabies can present clinically as a cluster of pruritic lesions with tiny gray specks (16). The transmission of these mites is by direct contact and is commonly found in crowded living conditions (8,11). The pruritic condition of the skin can lead to itching and secondary bacterial infection, leading to septicemia, often requiring broadspectrum systemic antibiotics (11). The standard method of diagnosing a scabies infestation is to take a skin scraping (11,12). Other techniques such as the tape test, dermoscopy, skin biopsy, and the ink test have also been advocated in published reports. Many communities rely on the clinical diagnosis alone (12). The clinical examination should involve looking for the burrows that have been created by the mites, known as the “burrow sign” (16). This diagnosis technique is prone to error, because the burrows can be destroyed by scratching, and because scabies can mimic many infectious and noninfectious diseases of the skin (15). Scabies can often be easily misdiagnosed as eczema without the presentation of mites (15,16). Skin scrapings involve the use of a scalpel to remove a sample of the affected tissue, which is then placed on a slide with 1 drop of silicone oil (12,15). This slide is then examined under the microscope. A microscopic section of a scabies mite will show 8 legs and a biting apparatus (1). The dermoscopy test involves using a hand-held dermoscope to evaluate for the brownish-black anterior part of the embedded mite, termed the “delta wing sign” (15). The tape test involves using a transparent adhesive tape the size of a microscope slide, 25 mm  50 mm, which is applied to the

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lesion and removed rapidly (17). This tape is then examined under a microscope and might demonstrate the scabies mites (18). This technique is suitable, economical, and easy to perform on unruly patients and children (17,18). The ink test will allow the burrows in the skin to become readily apparent, because they absorb the ink (1). Finally, a skin biopsy can be helpful in the diagnosis of scabies. In patients with Norwegian crusted scabies, this test can prove unreliable, because the inflammatory reaction in response to the scabies can appear as a lymphohistiocytic infiltration on analysis of the skin specimens (11). This explains why in the present case study, the initial superficial skin biopsy was negative for the scabies mite. The current treatment regimen of scabies includes both oral and topical treatment (6,8,11,12,15). The oral drug ivermectin has been approved by the Food and Drug Administration for the treatment of scabies and is given in 1 dose of 200 mg/kg of the patient’s body weight (6). Ivermectin can be administered twice with each dose approximately 1 week apart (1,12). Oral ivermectin has been shown to be as effective as topical lindane for the treatment of scabies (11). However, the published data have noted that the use of oral ivermectin increases the risk of death among elderly patients (19). Topical treatment for scabies infestation has included lindane 1% lotion, permethrin cream 5%, benzyl benzoate, and crotamiton 10% lotion or cream (1,5,6,8,11,12). The topical treatment should be left on the entire body for a set period and then rinsed with soapy water (1,12). These treatments must be repeated with multiple applications, and the entire body must be treated (1,12). The present case presentation has demonstrated that surgical debridement can be complementary to the standard topical and oral medications in the treatment of patients with Norwegian crusted scabies infestation. Few published reports have described the necessity of surgical debridement for this condition. In severe cases of plaque formation, such as in our patient, debridement will improve the ability of the topical medication to eradicate the mite.

References 1. Hengge UR, Currie BJ, Jager G, Lupi O, Schwartz RA. Scabies: a ubiquitous neglected skin disease. Lancet 6:769–779, 2006. 2. O’Donnell BF, O’Loughlin S, Powell F. Management of crusted scabies. Int J Dermatol 29:258–266, 1990. 3. Chosidow O. Scabies and pediculosis. Lancet 355:819–826, 2000. 4. Currie B, Huffam S, O’Brien D, Walton S. Ivermectin for scabies. Lancet 350:1551, 1997. 5. Dourmishev AL, Dourmishev LA, Schwartz RA. Ivermectin: pharmacology and application in dermatology. Int J Dermatol 44:981–988, 2005. 6. Walker GJA, Johnstone PW. Interventions for treating scabies. Cochrane Database Syst Rev 3:CD000320, 2006. 7. Chouela EN, Abeldano AM, Pellerano G, La Forgia M, Papale RM, Garsd A, del Carmen Balian M, Battista V, Poggio N. Equivalent therapeutic efficacy and safety of ivermectin and lindane in the treatment of human scabies. Arch Dermatol 135:651–655, 1999. 8. Khambaty M, Hsu S. Dermatology of the patient with HIV. Emerg Med Clin North Am 28:355–368, 2010. 9. Adams SP. Dermacase: crusted Norwegian scabies. Can Fam Physician 45:1462, 1999. 10. Esses SA, Eslcs J. Therapy of scabies in nursing home, hospitals, and the homeless. Semin Dermatol 12:26–33, 1993. 11. Kartono F, Lee EW, Lanum D, Pham L, Maibach HI. Crusted Norwegian scabies in an adult with Langerhans cell histiocytosis: mishaps leading to systemic chemotherapy. Arch Dermatol 143:626–628, 2007. 12. Andrews R, McCarthy J, Carapetis J, Currie B. Skin disorders including pyoderma, scabies, and tinea infections. Pediatr Clin North Am 56:6, 2009. 13. Roberts LJ, Huffam SE, Walton SF, Currie BJ. Crusted scabies: clinical and immunological findings in seventy-eight patients and a review of the literature. J Infect 50:375–381, 2005. 14. Castano-Molina C, Cockerell CJ. Diagnosis and treatment of infectious diseases in HIV-infected hosts. Dermatol Clin 15:267–283, 1997. 15. Walter B, Heukelback J, Fengler G, Worth C, Hengge U, Feldmeier H. Comparison of dermoscopy, skin scraping, and the adhesive tape test for the diagnosis of scabies in a resource-poor setting. Arch Dermatol 147:468–473, 2011. 16. McCarthy JS, Kemp DJ, Walton S, Currie BJ. Scabies: more than just an irritation. Postgrad Med J 80:382–387, 2004. 17. Albrecht J, Bigby M. Testing a test: critical appraisal of tests for diagnosing scabies. Arch Dermatol 147:494–497, 2011. 18. Katsumata K, Katsumata K. Simple method of detecting Sarcoptes Scabiei var hominis mites among bedridden elderly patients suffering from severe scabies infestation using an adhesive tape. Intern Med 45:857–859, 2006. 19. Reinties R, Hoek C. Deaths associated with ivermectin for scabies. Lancet 350:215, 1997.

Norwegian crusted scabies: an unusual case presentation.

Scabies is a contagious condition that is transmitted through direct contact with an infected person and has been frequently associated with instituti...
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