Norwegian Scabies Gary

F.

or crusted scabies is an unusual skin infestation the

Norwegian by Sarcoptes scabiei, distinguished

mite from the common form of scabies by the presence of remarkable hyperkeratotic lesions teeming with mites. It was first described in Norwegian lepers in 1848 by Danielssen and Boeck.' Cases have since been re¬ ported in a variety of clinical settings, usually involving chronically ill pa¬ tients.'-' Down's syndrome patients have a high incidence of Norwegian scabies,'1 although the reasons for this propensity are unclear. Immune deficient patients, such as those with lepromatous leprosy, and immune sup¬

pressed patients, usually following renal transplantation' also have de¬ veloped this peculiar eruption. Topical corticosteroid therapy can so alter the local host response that Norwegian '

scabies appears." We wish to add Bloom's syndrome to this list of immu¬ nodeficiencies in which Norweigian scabies may occur. REPORT OF A CASE A

Syndrome

Dick, MD; Walter H. C. Burgdorf, MD; William C. Gentry, Jr, MD

Norwegian scabies is an unusual Sarcoptes scabiei infestation. It has been reported often in patients who are either severely debilitated or who have congenital or iatrogenic suppression of their immune responses. We report the occurrence of Norwegian scabies in a 13-year\x=req-\ old boy with Bloom's syndrome who had impaired humoral and cell-mediated immunity. (Arch Dermatol 115:212-213, 1979)

13-year-old boy

drome

in Bloom's

was

with Bloom's syn¬ referred to the L'niversity of

Accepted

for publication Aug 28, 1978. From the Department of Dermatology, University of Minnesota Health Sciences Center,

Minneapolis. Reprints not

available.

Minnesota Dermatology Clinic for evalua¬ tion of a pruritic, hyperkeratotic, crusted eruption of three months' duration. Ini¬ tially he developed a pruritic eruption of the hands, genitalia, and trunk that was untreated. One month later, he incurred a sunburn and had persistent erythema and scaling of the upper part of the chest, shoulders, nape, and ears. These areas were treated with 0.19 triamcinolone acetonide cream with little benefit. In fact, thickened scaling lesions began to develop most prominently in the sunburned and steroidtreated areas. All family members had had pruritic skin lesions consistent with ordina¬ ry scabies. Furthermore, the patient's mother reported that there was an epidemic of scabies in the nursing home where she was employed. Examination of the skin showed an erythematous dermatitis with thick crusts and scales on the external ears, nape, shoulders, upper portion of the arms, and dorsa of the hands (Figs 1 and 2). There were excoriated, scaling papules about the umbilicus, on the foreskin, and within the fingerwebs. Erythema, telangiectasia, and dwarfism typical of Bloom's syndrome were prominent clinical features. There were no pigmentary abnormalities and the nails and hair were normal. Scrapings from both hyperkeratotic and papulovesicular lesions disclosed numerous mites. The patient was treated with gamma benzene hexachloride lotion ap¬ plied twice at an interval of one week and (ffc precipitated sulfur in petrolatum applied daily for three weeks. The patient failed to return for clinical réévaluation and immunologie investigation. However, during the course of previous hospitalizations, a number of immunologie studies had been done. These had shown normal values for serum protein electro-

phoresis, serum complement, and quantita¬ tive serum IgG and IgA. Serum IgM values remained consistently low, with values of 24, 51, and 58 mg/dL compared with normal values of 36 to 280 mg/dL. His cell-mediated immunity was examined more extensively. His lymphocytes failed to respond to phytohemagglutinin stimula¬ tion. He could not be sensitized to topical

applications

of dinitrochlorobenzene. Re-

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using histoplasmin, PPD, Candida, streptokinase/ streptodornase, and coccidioidin antigens were negative at 24 and 48 hours. A Schick test gave positive results. Attempts to culture peripheral lymphocytes for chromo¬ some analysis failed on two occasions. suits of intradermal skin tests

However, a third culture was successful and showed 9.6 breaks per cell for the 65 mitoses examined. COMMENT In Norwegian scabies, hundreds of mites infest the skin and can be recov-

Fig 1.—Crusted lesions of Norwegian scabies arising on previously sunburned skin.

scabies lesions on shoulders. Note faciès and telangiectasia of Bloom's syndrome.

Fig 2—Norwegian

easily. This is in contrast to ordi¬ nary scabies where a small number of mites colonize the skin surface and are hard to find. It is believed that a large number of mites initially colonize a normal host, but that by the time clinical disease occurs some four weeks after the infestation, the host response has decreased the number of mites present. Both humoral and cellular immune responses to the scabies mite have been demon¬ strated.7 Orkin and Maibach8 suggest that the clinical lesions and symp¬ toms of ordinary scabies develop as a result of these host immunological responses. Supporting this is the shorter incubation period and paucity of mites in scabetic reinfestation. Both the host's immune response and the character of his skin may be important in modifying his response to the scabies mite. While in many cases of Norwegian scabies the pa¬ tient's condition has been described as debilitated without a description of the patient's immunologie status, it may well be that either humoral or cell-mediated immunity or local cuta¬ neous factors were responsible for the unusual clinical course. The appear¬ ance of Norwegian scabies in patients with iatrogenic immune suppression, such as renal transplant patients, and with natural deficiencies, such as lepromatous leprosy, supports this concept. Localized immunosuppresered

sion,

as

tions of

produced by topical applica¬ glucocorticosteroids, has been

associated with the appearance of Norwegian scabies.6 9 The nature of the host's skin may be important in permitting infestation with a multitude of organisms. In our patient, clinical lesions developed at sites that had been damaged by previous ultraviolet light exposure. Patients with Down's syndrome, whose skin tends to be xerotic and

hyperkeratotic, are prone to develop hypertrophie crusted lesions with scabetic infestation. Regardless of immunological abnormalities, is the clinical appearance of Norwegian scabies in¬ fluenced by abnormalities of cuta¬

keratinization? Fain10 has recently suggested a third factor in producing Norwegian scabies. While it has generally been believed that the same parasite causes ordinary and Norwegian scabies, he demonstrated a morphologic differ¬ ence in the mites recovered from patients with Norwegian scabies. These organisms had fewer cuticular scales on the dorsal and lateral surfaces of the venter of the female mite. We did not have an opportunity to search for these changes in our neous

case.

The epidemiology of our case is unclear. Often a patient with Norwe¬ gian scabies serves as the source for an epidemic of ordinary scabies.3-4 However, we do not know if our patient's mother carried scabies into the nursing home where she worked or if she brought the mite into her family

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from work. The clinical appearance of Norwe¬ gian scabies results from an infesta¬ tion that is influenced by many factors, including host immunological status, host epidermal factors, and intrinsic features of the parasite. To understand the role of these variables better in the production of clinical disease, each of these factors should be evaluated in patients with Norwe¬ gian scabies. The cost of color reproduction was assumed by Westwood Pharmaceuticals Inc, Buffalo, NY, This investigation was supported in part by Public Health Service research training program in Academic Dermatology training grant T 32 AM 07165-03.

References WD, Allen BR, Beveridge GW: Norwegian scabies during immunosuppressive therapy. Br Med J 4:211-212, 1973. 2. Schiff BL, Ronchese F: Norwegian scabies. Arch Dermatol 89:236-239, 1964. 3. Haydon JR Jr, Caplan RM: Epidemic scabies. Arch Dermatol 103:168-173, 1971. 4. Hubler WR Jr, Clabaugh W: Epidemic Norwegian scabies. Arch Dermatol 112:179-181, 1. Paterson

1976. 5. Espy

PD, Jolly HW Jr: Norwegian scabies. Arch Dermatol 112:193-196, 1976. 6. Clayton R, Farrow S: Norwegian scabies following topical corticosteroid therapy. Postgrad Med J 51:657-659, 1975. 7. Mellanby K: Scabies. Middlesex, England, EW Classey Ltd, 1972. 8. Orkin M, Maibach HI: Current concepts in parasitology: This scabies pandemic. N Engl J Med 298:496-498, 1978. 9. Millard LG: Norwegian scabies developing during treatment with fluorinated steroid therapy. Acta Derm Venereol 57:86-90, 1977. 10. Fain A: Epidemiological problems of scabies. Int J Dermatol 17:20-30, 1978.

Norwegian scabies in Bloom's syndrome.

Norwegian Scabies Gary F. or crusted scabies is an unusual skin infestation the Norwegian by Sarcoptes scabiei, distinguished mite from the common...
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