Download Norwegian Crusted Scabies: An Unusual Case Presentation

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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63
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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M.M. Maghrabi et al. / The Journal of Foot & Ankle Surgery 53 (2014) 62–66
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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M.M. Maghrabi et al. / The Journal of Foot & Ankle Surgery 53 (2014) 62–66
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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M.M. Maghrabi et al. / The Journal of Foot & Ankle Surgery 53 (2014) 62–66
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.
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