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Atypical Scabies in Systemic Lupus Erythematosus

1998; King Faisal Specialist Hospital and Research Centre; Volume: 18; Issue: 6 Linguagem: Inglês

10.5144/0256-4947.1998.534

0975-4466

J. Fernando Val‐Bernal, M. Carmen Gonzaléz‐Vela, Sonsoles Yáñez, Concepción Mira, Víctor M. Martínez‐Taboada, Vicente Rodríguez‐Valverde,

Herpesvirus Infections and Treatments

Case ReportsAtypical Scabies in Systemic Lupus Erythematosus J. Fernando Val-Bernal, MD, PhD M. Carmen González-Vela, MD Sonsoles Yáñez, MD, PhD Concepción Mira, MD V. Martínez-Taboada, and MD, PhD V. Rodriguez-ValverdeMD, PhD J. Fernando Val-Bernal Address reprint requests and correspondence to Dr. Val-Bernal: Departmento de Anatomía Patológica, Hospital Universitario Marqués de Valdecilla, Avda. Valdecilla 1, 39008, Santander, Spain. From the Department of Anatomical Pathology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Search for more papers by this author , M. Carmen González-Vela From the Department of Anatomical Pathology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Search for more papers by this author , Sonsoles Yáñez From the Department of Services of Dermatology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Search for more papers by this author , Concepción Mira From the Department of Anatomical Pathology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Search for more papers by this author , V. Martínez-Taboada From the Department of Rheumatology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Search for more papers by this author , and V. Rodriguez-Valverde From the Department of Rheumatology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Search for more papers by this author Published Online::1 Nov 1998https://doi.org/10.5144/0256-4947.1998.534SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionRecent reports have increasingly linked scabies with congenital or acquired immunosuppression. It is important to be aware of this form of infestation, because its unusual clinical signs may be easily overlooked. Scabies can mimic several diseases, including cutaneous or systemic lupus erythematosus (SLE).1 However, the infestation has rarely been reported in patients with SLE. The objective of this report is to describe a rare case of atypical (exaggerated) scabies in SLE that illustrates a pitfall in the recognition of the infestation, since such recognition took place belatedly. We hope that this case will draw attention to the possibility of scabies whenever a widespread erythematosquamous dermatosis develops in a case of SLE.CASE REPORTA 39-year-old woman had SLE for about 17 years, which was manifested as malar eruption, oral ulcers, leukopenia and arthritis. She also had a history of several episodes of pleural effusion. The patient was being treated with prednisone (30 mg/day), enalapril maleate (10 mg/day), and chloroquine (250 mg/day).In September 1995, she presented with nonpruritic erythematosquamous plaques, without alopecia or scars in the scalp. She was treated with topical application of betamethasone valerate. Two months later, she developed a generalized papulosquamous eruption (Figure 1). Physical examination revealed erythematous and erythematosquamous papules without itching, particularly affecting abdomen, arms, legs and buttocks. Routine laboratory findings, including creatinine level, were normal. Serum protein electrophoresis demonstrated a polyclonal increase in gammaglobulin, and IgA was 663 mg/dL (normal, 90-450). Cryoglobulins were trace-positive (IgG, IgA). ANA was positive at 1:1280, with a homogenous pattern. The serum levels of C3 and C4 were within the normal range. The rest of the immunologic studies were normal or negative. Although there was no clinical or serological activity of the disease, the lesions were presumed to be secondary cutaneous lupus erythematosus. Two punch biopsies were performed and scabies was diagnosed. Treatment with 1% lindane lotion in two courses resolved the cutaneous manifestations of the patient. She remained asymptomatic 24 months without relapse of her skin lesions.Figure 1. Clinical appearance of scabies manifesting as a disseminated erythematosquamous papular dermatosis. The lesions are evident in the trunk and in the right upper arm.Download FigureMATERIALS AND METHODSThe patient fulfilled the revised 1982 criteria for SLE of the American Rheumatism Association. The surgical specimens were fixed in 10% buffered formaldehyde solution, embedded in paraffin, and routinely processed. Consecutive 4 μm sections were stained with hematoxylin and eosin. Immunohistochemistry stains were performed by using the streptavidin-biotin-peroxidase method and a Techmate 500-220 automated immunostainer (Biotek, Santa Barbara, CA, USA). With the use of a pressure cooker, sections were boiled for a short time in sodium citrate buffer prior to the staining. Diaminobenzidine was used as chromogen. The following antibodies were utilized: CD3 (monoclonal, Dako, Glostrup, Denmark, 1:100) for T-lymphocytes; CD20 (monoclonal, Dako, 1:50) for B-lymphocytes; CD68 (KP1, monoclonal, Dako, 1:25) for macrophages; and carcinoembryonic antigen (CEA, polyclonal, Dako, 1:500) for neutrophils.PATHOLOGIC FINDINGSTwo punch biopsy specimens of the erythemato-squamous papules were obtained from the abdomen and left leg, respectively. Histologic examination revealed a slightly acanthotic epidermis with focal parakeratosis, intracorneal pustules, and a mite of scabies in a burrow within the epidermal cornified layer (Figure 2) (Figure 2. Papular lesion of scabies showing a scale-crust composed of parakeratotic cells and eosinophilic material, a scabie egg residing in the stratum corneum (on the left), and an adult mite within a subcorneal burrow (on the right). A mixed-cell perivascular infiltrate is present in the dermis (H&E, 25x).). In the superficial dermis, there was a perivascular inflammatory infiltrate of mixed cell type with mononuclear cells, mainly macrophages, a few lymphocytes, neutrophils, and sparse eosinophils (Figure 3). Serial sections were required to demonstrate eggs and mites. Immunohistochemical stains showed that macrophages were the predominant cells in the infiltrate. Fewer T-lymphocytes and very few B-lymphocytes and neutrophils were detected.Figure 2. Papular lesion of scabies showing a scale-crust composed of parakeratotic cells and eosinophilic material, a scabie egg residing in the stratum corneum (on the left), and an adult mite within a subcorneal burrow (on the right). A mixed-cell perivascular infiltrate is present in the dermis (H&E, 25x).Download FigureFigure 3A. The inflammatory-cell infiltrate consists of histiocytes, lymphocytes, and neutrophils arranged around blood vessels of both superficial and deep plexuses (H&E, 80x).Download FigureFigure 3B. Monoclonal antibody CD68 (KP1) decorating the cytoplasm of most of the cells of dermal infiltrate (160x).Download FigureDISCUSSIONIn classic scabies, the lesions are few and roughly symmetrical. These are frequently eczematous and occur in the hands, flexor surface of the wrists, elbows, and the anterior axillary folds. Other typical locations are female breasts, around the umbilicus, penis, and lower portion of the buttocks. However, there are special forms of scabies resulting in unusual clinical presentations, atypical locations and unusual extension of the disease closely simulating other entities. Some unusual forms of scabies are the crusted (Norwegian) scabies and the atypical (exaggerated) scabies.2 In many cases of these unusual forms, the pruritus of classic scabies lessens or disappears, the sites of involvement are different, i.e., the scalp, face, back, and nails, and burrows are less apparent. It has been said that scabies is a great imitator. Thus, it can masquerade as psoriasiform dermatosis, contact dermatitis, Darier disease, dermatitis herpetiformis, bullous pemphigoid, malignant lymphoma, and systemic or cutaneous lupus erythematosus,1 among other entities.Crusted scabies has been associated with a wide group of disorders, including mental retardation, diabetes mellitus, and immunosuppressed status. New features associated with severe Sarcoptes scabiei infestations exist. Severe forms of infestation develop preferentially in cases of imbalance in host-parasite relationships. Immune deficiency is one etiologic factor that is being increasingly recognized. Forms of scabies associated with congenital, acquired or iatrogenic suppression of the immune response include the following: 1) spontaneous essential immunodeficiency with Pneumocystiscarinii infection in patients who do not have acquired immune deficiency syndrome (AIDS);3 2) HTLV-I-associated T-cell leukemia;4 3) HTLV-III infection (AIDS);2 4) lepromatous leprosy;5 5) dialysis with or without immunosuppression;6 6) corticosteroid therapy, including topical treatment;7 7) renal transplantation;8 8) bone marrow transplantation;9 9) Bloom’s syndrome;10; and 10) SLE.11-16Scabies has rarely been described in patients with SLE. A review of the literature revealed eleven reported cases.11-16 Scabies in these eleven cases appeared to be more severe than usual, and seven of the patients developed crusted scabies. Atypical or exaggerated type of scabies was observed in only four patients.11,13An exaggerated form of scabies with generalized papulosquamous lesions occurred in our patient upon treatment for SLE. This special form can be designated scabies incognito, as the lesions were ascribed to SLE. In our case, systemic and topical corticosteroid therapy aggravated a primary cutaneous involvement, resulting in a severe form of infestation. The role of immunologic mechanisms in limiting the number of scabies mites is not well defined. However, the cellular immunity of the host appears to play a significant role.17 Histology suggests that a cell-mediated reaction causes the lesions of classic scabies. Lymphocytes constitute the majority of cells in the inflammatory infiltrate and T-cells dominate.17 The ratio of T-cells to B-cells in the lesions is greater than in the peripheral blood.18 This suggests a selection of T-lymphocytes in the inflammatory infiltrate. SLE is characterized by an alteration of humoral and cellular immunity,19 with loss of suppressor T-cell function.20 Corticosteroid therapy decreases T-cell response. It is not surprising, therefore, that in our case macrophages were predominant in the infiltrate. The exaggerated form of scabies in the present case may be the result of underlying SLE and oral and topical usage of corticosteroids. Secondary bacterial infection may complicate scabies. The mite scybala and the fissures probably contribute to bacterial colonization.It is necessary to emphasize the importance of a rapid diagnosis of scabies and that of the secondary bacterial infection, because four patients reported with this complication in SLE died of septicemia.12,13,15,16 These patients had developed crusted scabies.ARTICLE REFERENCES:1. Bastian HM, Lindgren AM, Alarcón GS. "Scabies mimicking systemic lupus erythematosus" . Am J Med. 1997: 102;305-6. Google Scholar2. Orkin M. "Scabies in AIDS" . Semin Dermatol. 1993; 12:9-14. Google Scholar3. Kobayashi M, Miyoshi I, Sonobe H, Taguchi H, Kubonishi I. "Association of Pneumocystis carinii pneumonia and scabies" . J Am Acad Dermatol. 1982; 248:1973. Google Scholar4. Suzumiya J, Sumiyoshi A, Kuroki Y, Inoue S. "Crusted (Norwegian) scabies with adult T-cell leukemia" . Arch Dermatol. 1985; 121:903-4. Google Scholar5. De Almeida Barbosa A, Correia Silva TM, Ribeiro Santos MI, Gomez Garrido MF, Niljay Patel B, Cavalcanti M, Oliveira Riccio MC. "Coexistence of an unusual form of scabies and lepromatous leprosy: a case report" . Path Res Pract. 1996; 192:88-90. Google Scholar6. Lempert KD, Baltz PS, Welton WA, Whittier FC. "Pseudouremic pruritus: a scabies epidemic in a dialysis unit" . Am J Kidney Dis. 1985; 5:117-9. Google Scholar7. Clayton R, Farrow S. "Norwegian scabies following topical corticosteroid therapy" . Postgrad Med J. 1975; 51:657-9. Google Scholar8. You shock E, Glazer SD. "Norwegian scabies in a renal transplant patient" . JAMA. 1981; 246:2608-9. Google Scholar9. Barnes L, McCallister RE, Lucky AW. "Crusted (Norwegian) scabies. Occurrence in a child undergoing a bone marrow transplant" . Arch Dermatol. 1987; 123:95-7. Google Scholar10. Dick GF, Burgdorf WHC, Gentry WC. "Norwegian scabies in Bloom’s syndrome" . Arch Dermatol. 1979; 115:212-3. Google Scholar11. Bernstein B, Mihan R. "Hospital epidemic of scabies" . J Pediatr. 1973; 83:1086-7. Google Scholar12. Timpatanapong P, Tasanapradit P, Indulak S, Sundaravej P. "Norwegian scabies in acute SLE patient treated with high-dose corticosteroid" . J Med Assoc Thai. 1974; 57:514-6. Google Scholar13. Ting HC, Wang F. "Scabies and systemic lupus erythematosus" . Int J Dermatol. 1983; 22:473-6. Google Scholar14. Mikheev GN. "Development of hyperkeratotic or Norwegian scabies in two patients with lupus erythematosus" . Vestnik Dermatol Venerol. 1984; 4:57-61. Google Scholar15. Wanke NC, Melo C, Balassiano V. "Crusted scabies in a child with systemic lupus erythematosus" . Rev Soc Bras Med Trop. 1992; 25:73-5. Google Scholar16. Chen DY, Lan JL. "Crusted scabies in systematic lupus erythematosus: a case report" . Chinese J Microbiol Immunol. 1993; 26:44-50. Google Scholar17. Cabrera R, Agar A, Dahl MV. "The immunology of scabies" . Semin Dermatol. 1993; 12:15-21. Google Scholar18. Falk ES, Matre R. "In situ characterization of cell infiltrates in the dermis in human scabies" . Am J Dermatopathol. 1982; 4:9-15. Google Scholar19. Schoenfeld Y, Schwartz RS. "Immunologic and genetic factors in autoimmune diseases" . N Engl J Med. 1984; 311:1019-29. Google Scholar20. Morimoto C. "Loss of suppressor T-lymphocyte function in patients with systemic lupus erythematosus (SLE)" . Clin Exp Immunol. 1978; 32:125-33. 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