Scabies complicated by necrotizing lymphocytic vasculitis in an infant.

Scabies is very common among children. It is often a harmless infectious disease, responding well to antiparasitic medication. Nevertheless, severe forms can occur in immunocompromised populations like newborns and infants. We report a unique case of scabies in a three-months-old infant, complicated by generalized cutaneous lymphocytic vasculitis and unilateral acral necroses.

[Visceral Larva Migrans: A pediatric case of atypical presentation with cutaneous vasculitis in the province of Buenos Aires, Argentina]. / Toxocariosis visceral: un caso pediátrico atípico con vasculitis cutánea en la provincia de Buenos Aires, Argentina.

Toxocariosis is a parasitic disease caused by the larvae from genus Toxocara sp. There are two classic syndromes described for this entity: visceral larva migrans and ocular larva migrans, depending on larvae localization. Human being behaves as an accidental host in which Toxocara sp. does not become an adult worm. This infection is generally asymptomatic but clinical manifestations can be diverse, and they vary according to number and localization of entrenched larvae and host's immune system. In the last years it has been studied a relation between Toxocara sp. and some cutaneous manifestations. We describe the case of a 19-month infant with visceral larva migrans and cutaneous manifestations from vasculitis, explaining its form of presentation, evolution, diagnose and treatment.

La toxocariosis es una parasitosis generada por la larva del género Toxocara sp., que causa dos síndromes clásicamente definidos: larva migrans visceral o larva migrans ocular, dependiendo de la localización de la larva. Sin embargo, la mayor parte de los niños presenta una infección asintomática. El ser humano se comporta como un hospedador paraténico, en el que Toxocara sp. no llega a completar su ciclo biológico. Las manifestaciones clínicas pueden ser diversas y dependen del número y de la localización de las larvas enquistadas, así como de la respuesta inmune del huésped. En los últimos años, se ha descrito una relación entre Toxocara sp. y ciertas manifestaciones cutáneas. Se describe el caso clínico de un lactante de 19 meses con toxocariosis visceral y manifestaciones cutáneas de vasculitis. Se detalla su forma de presentación, evolución clínica, metodología diagnóstica y terapéutica empleada.

Low-Level Parasite Persistence Drives Vasculitis and Myositis in Skeletal Muscle of Mice Chronically Infected with Trypanosoma cruzi.

In chronic Trypanosoma cruzi infection, the cause of Chagas disease, life-threatening inflammatory diseases develop over time in the heart, esophagus, and colon of some patients. C57BL/6 mice infected with the myotropic Colombiana strain of T. cruzi model many of the immunological and parasitological features of human infection but succumb to chronic paralyzing myositis and skeletal muscle vasculitis, not cardiomyopathy or gastrointestinal disease. Here we show that T cell depletion in the chronic phase of this model increased tissue parasitism to acute-phase levels and induced neutrophilic skeletal muscle inflammation. Conversely, after daily treatment with the trypanocide benznidazole for 8 weeks during the chronic phase, viable parasites were no longer detectable, myositis completely resolved, vasculitis was ∼80% reduced, fibrosis was reduced, and myofiber morphology normalized. After the drug was discontinued, parasitism rebounded, and immunopathology recurred. The parasite load was statistically strongly correlated with the severity of inflammation. Thus, both T cell immunity and trypanocidal pharmacotherapy suppress to very low levels, but do not cure, T. cruzi infection, which is necessary and possibly sufficient to induce crippling chronic skeletal muscle myositis and vasculitis in the model.

Vasculitis and Thrombosis due to the Sea Lion Lungworm, Parafilaroides decorus, in a Guadalupe Fur Seal ( Arctocephalus philippii townsendi).

A free-ranging, male, yearling Guadalupe fur seal ( Arctocephalus philippii townsendi) died due to multifocal verminous vasculitis with thrombosis and several embolic infarcts in liver, kidney, and brain. Nematodes extracted from lung blood vessels were identified as Parafilaroides decorus, a parasite normally found in alveoli of California sea lions ( Zalophus californianus).

Scabietic vasculitis: Report of 2 cases.

BACKGROUND: The infectious causes of cutaneous vasculitis are well known and include streptococcal infections among others. Cases resulting from parasitic infection are less frequent. Scabies, which is currently on the increase, has only been reported in a few isolated cases. Herein, we report two noteworthy cases of profuse scabies complicated by cutaneous vasculitis. PATIENTS AND METHODS: Case 1: a 90-year-old woman, residing in a nursing home, was admitted to our dermatology department complaining of pruritus, present for one month, predominantly on the inside of the thighs and on the buttocks, associated with purpuric lesions on the lower limbs. A skin biopsy revealed leukocytoclastic vasculitis. A diagnosis of scabies was based on severe pruritus and hypereosinophilia and was confirmed by microscopic examination of the parasitology sample and the skin biopsy sample. Despite thorough investigation, no other cause of vasculitis could be found. Complete regression of the skin lesions was achieved with scabies treatment only, without any specific treatment for the vasculitis. Case 2: a 74-year-old man, living in a nursing home, was hospitalized for purpuric papules on the lower limbs, present for one month. Physical examination revealed linear patterns in the interdigital spaces associated with scabies evident on dermoscopic examination. The skin biopsy revealed signs of vasculitis. As in our first case, no aetiology of vasculitis was found and a favorable outcome was achieved by means of scabies treatment alone with no specific treatment for vasculitis. DISCUSSION: Both of our patients presented scabies and vasculitis. In view of the absence of other causes of vasculitis and of the complete regression of lesions due to vasculitis without recurrence achieved with the scabies treatment alone, a diagnosis was made of scabietic vasculitis, probably as a result of cutaneous hypersensitivity reaction to humeral mediators.

Trypanosoma cruzi Causes Paralyzing Systemic Necrotizing Vasculitis Driven by Pathogen-Specific Type I Immunity in Mice.

Infectious agents are often considered potential triggers of chronic inflammatory disease, including autoimmunity; however, direct evidence is usually lacking. Here we show that following control of acute infection of mice with the myotropic Colombiana strain of Trypanosoma cruzi, parasites persisted in tissue at low levels associated with development of systemic necrotizing vasculitis. Lesions occurred in many but not all organs and tissues, with skeletal muscle arteries being the most severely affected, and were associated with myositis, atrophy, paresis/paralysis, and death. Histopathology showed fibrinoid vascular necrosis, rare amastigote nests within skeletal muscle myocytes, and massive leukocyte infiltrates composed mainly of inflammatory monocytes, F4/80(+)macrophages, and T. cruzi tetramer-specific CD8(+) T lymphocytes capable of producing gamma interferon (IFN-γ) and tumor necrosis factor alpha (TNF-α) but not interleukin-17 (IL-17). T. cruzi-specific IgG was detected in sera from infected mice, but antibody deposits and neutrophilic inflammation were not features of the lesions. Thus,T. cruzi infection of mice may be a specific infectious trigger of paralyzing systemic necrotizing vasculitis most severely affecting skeletal muscle, driven by pathogen-specific type I immune responses.

Vasculitis and infectious diseases.

Vasculitis usually presents without a well-known underline cause (idiopathic vasculitis), nevertheless, it is sometimes possible to find out one or more causative agents (secondary vasculitis). Nowadays, thanks to the increasing amount of precise diagnostic tools, a piece of idiopathic vasculitis is reclassified as associated with probable etiology, which can be set off by several factors, such as infections. Infections are considered to be the most common cause of secondary vasculitis. Virtually, every infectious agent can trigger a vasculitis by different mechanisms which can be divided in two main categories: direct and indirect. In the former, infectious agents destroy directly the vascular wall leading, eventually, to a subsequent inflammatory response. In the latter, indirect form, they stimulate an immune response against blood vessels. Different infectious agents are able to directly damage the vascular wall. Among these, it is possible to recognize Staphylococcus spp, Streptococcus spp, Salmonella spp, Treponema spp, Rickettsia spp, Cytomegalovirus, Herpes Simplex Virus 1 and 2, and many others which have a peculiar tropism for endothelial cells. Conversely, another group of microbial agents, such as Mycobacterium tuberculosis, Mycobacterium leprae, Hepatits B Virus, Human Immunodeficiency Virus and others, trigger vasculitis in the indirect way. This is due to the fact that they can share epitopes with the host or modify self-antigens, thus leading to a cross-self reaction of the immune system. These mechanism, in turn, leads to immunological responses classified as type I-IV by Gell-Coombs. Nevertheless, it is difficult to strictly separate the direct and indirect forms, because most infectious agents can cause vasculitis in both ways (mixed forms). This paper will analyze the link between infectious agents and vasculitis, focusing on direct and indirect secondary vasculitis, and on a group of probable infection-related idiopathic vasculitis, and finally on a group of idiopathic vasculitis with microbiological triggers. Furthermore, a diagnostic and therapeutic approach to vasculitis when an underline infection has been suspected is suggested.

Superficial fibrin thrombi and other findings: a review of the histopathology of human scabietic infections.

BACKGROUND: Cutaneous infection with the mite Sarcoptes scabiei var. hominis is associated with epidermal and dermal changes. After noting superficial fibrin thrombi in two biopsies with scabies mites, we comprehensively reviewed the histopathologic findings in scabietic infections to determine the frequency of this finding. METHODS: Twenty five biopsies of scabies infection were retrieved from the archives of our institution; only cases containing scabietic mite parts or scybala were included. The microscopic features were documented. RESULTS: Nearly half (40%) of the cases showed fibrin thrombi within vessels of the superficial dermis. Other frequent findings included dermal eosinophils (88% of cases), epidermal spongiosis (76% of cases), lymphocyte atypia (64%), a superficial and deep infiltrate (52% of cases), dermal neutrophils (52%) and endothelial cell swelling (52%). Half of the cases contained polarizable mite elements. Less commonly encountered features included extravasated erythrocytes (44%), dermal edema (32%), pink 'pigtails'(28%), intraepidermal pustules (24%), plasma cells (20%) and vasculitis (4%). CONCLUSIONS: The pathologic characteristics of scabietic infection are wide-ranging. Spongiosis, superficial and deep inflammation, and dermal eosinophils and neutrophils are seen in the majority of cases. Superficial fibrin thrombi are not uncommon in scabietic infection, and may provide a helpful diagnostic clue when mites are not visible on initial sections.

Secondary choriocapillaritis in infectious chorioretinitis.

PURPOSE: To analyse the indocyanine green angiography (ICGA) patterns of hypofluorescence that are compatible with choriocapillaritis that occur secondarily to toxoplasmic retinochoroiditis (ToRC), ocular tuberculosis (including tuberculous choroiditis, TuCR and multifocal serpiginoid choroiditis, TMSC) and syphilitic chorioretinitis (SyCR). METHODS: This was a single centre, retrospective case review study. Patients with a diagnosis of ToRC, TuCR, TMSC or SyCR were identified, their charts were reviewed and fundus photographs, fluorescein angiography (FA) and ICGA pictures were assessed. RESULTS: Indocyanine green angiography was performed at the initial presentation in 63 of the 105 patients with ToRC, in 37 of the 38 patients with TuCR, in six of six patients with TMSC and in two of four patients with SyCR. The following four ICGA patterns indicated choriocapillaritis: extension of hypofluorescence beyond the hypofluorescence of the actual infectious focus as seen on fundus photography or FA (seen only in ToRC and TuCR); small dark dots around the infectious focus (seen only in ToRC); multiple 'confetti-like' hypofluorescent areas or hypofluorescent geographical confluent areas (seen only in TMSC); and widespread areas of nonperfusion visible only in ICGA (seen only in SyCR). CONCLUSIONS: Patients with secondary choriocapillaritis have distinct typical ICGA findings. ICGA is thus an important diagnostic tool that can provide an explanation for otherwise obscure visual loss and that might have diagnostic value for specific conditions like ToRC and SyCR.

[Acanthamoeba meningoencephalitis: a case in an adolescent female patient with systemic lupus erythematosus]. / Acanthamöben-Meningoenzephalitis : Ein Fall bei einer jugendlichen Patientin mit systemischem Lupus erythematodes.

Meningoencephalitis caused by Acanthamoeba spp . is a rare opportunistic infection, difficult to diagnose and difficult to treat, which causes death in almost all cases. We report the neuropathologic findings of a 16-year-old girl with systemic lupus erythematosus (SLE) treated with immunosuppression who died of fulminant Acanthamoeba meningoencephalitis. Neuropathologic examination revealed multiple supratentorial and infratentorial hemorrhagic necrotizing lesions with encephalitis and vasculitis with mixed inflammatory infiltrates, fibrinoid necrosis of vessel walls, and local leptomeningitis. Acanthamoeba in the lesions may be misinterpreted as macrophages. Taking them into differential diagnostic consideration, cytological differences should be detected, and relevant additional stains for reliable differentiation of these cells can be performed. To our knowledge, this is the first published case of Acanthamoeba meningoencephalitis in a patient with SLE in Germany.

Increased serum levels of CXCL8 chemokine in acute toxoplasmic retinochoroiditis.

PURPOSE: Chemokines have been implicated in the control of leucocyte infiltration in uveitis and in modulating angiogenesis in several ocular conditions. Toxoplasmic retinochoroiditis is a common cause of posterior uveitis. This study aimed to evaluate the serum concentrations of CC and CXC chemokines in patients with acute toxoplasmic retinochoroiditis. METHODS: The levels of five chemokines (CCL2, CCL11, CXCL9, CXCL8 and CXCL10) were evaluated in the serum of patients with active toxoplasmic retinochoroiditis (n = 55) and control subjects (n = 40). In a subset of patients (n = 18), a second measure of serum levels of chemokines was performed after the completion of oral treatment with pyrimethamine (25 mg/day), sulphadiazine (1 g, four times per day), folinic acid (7.5 mg/day) and prednisone (initial dose: 1 mg/kg/day) for approximately 30 days. RESULTS: Patients with toxoplasmic retinochoroiditis, notably those presenting with vasculitis, had increased serum levels of CXCL8 (mean +/- standard error of the mean [SEM] 35.1 +/- 6.5 pg/ml) compared with control subjects (mean +/- SEM 16.0 +/- 2.3 pg/ml; p = 0.01). There were no differences between patients and controls in serum levels of the other chemokines measured. The size of ocular lesions correlated significantly with serum levels of CXCL8 and CXCL9. After treatment, there was a significant reduction in serum levels of CXCL8. Severity of vitreous opacities did not correlate with serum levels of these chemokines. CONCLUSIONS: These data suggest a role for CXCL8 in the inflammatory process of acute toxoplasmic retinochoroiditis. Furthermore, CXCL8 may be a useful marker for patient follow-up.

[Systemic vasculitis during parasitosis]. / Vascularites systémiques au cours de parasitoses.

A RARE EVENTUALITY: Although parasite infections are frequent, observations of vasculitis related to parasitosis are, however, very rare. REGARDING THE MECHANISM: The simultaneous occurrence of a parasitosis and vasculitis may be the consequence of either the direct implication of a parasite observed in the histological lesions in the onset of alteration in the vascular wall, or of immunopathological phenomena occuring during the anti-parasite immune response, or a fortuitous association. THE HUMAN PARASITOSIS IMPLIED: In most cases, vasculitis associated with parasitosis is an isolated event with varied clinical aspects. Such cases have been reported in toxoplasmosis, trichinosis, strongyliasis, ascaridiosis, sarcocystosis, amibiasis, leishmaniosis and toxocarosis.

[Cerebral vasculitis secondary to Toxocara canis and Fasciola hepatica co-infestation]. / Vasculite cérébrale secondaire à une co-infestation par Toxocara canis et Fasciola hepatica.

Neurological complications of cosmopolitan parasitosis are rare. We report a case of a cerebral and systemic vasculitis secondary to a visceral larva migrans syndrome associated with a Toxocara canis and Fasciola hepatica co-infestation.

Systemic vasculitis with lymphocytic temporal arteritis and Toxocara canis infection.

Vasculitis associated with helminthic infection is rare. We report a case of systemic negative antineutrophil cytoplasmic antibodies vasculitis associated with Toxocara canis infection documented by enzyme-linked immunosorbent assay and Western blot analysis. This case report is unusual because of lymphocytic temporal arteritis and renal involvement. The spontaneous remission of systemic vasculitis without use of steroids or immunosuppressive agents favors a close relationship between vasculitis and the parasite that is more than a coincidental association. To our knowledge, this is the first observation of temporal vasculitis associated with helminthic infection, extending the clinical spectrum of visceral larva migrans.

Disseminated acanthamoebiasis presenting as lobular panniculitis with necrotizing vasculitis in a patient with AIDS.

BACKGROUND: Disseminated acanthamoebiasis is a rare entity, almost exclusively occurring in the immunocompromised host. METHODS: We report an unusual case of a 35-year-old female with recurrent sinusitis and multiple skin nodules demonstrating a necrotizing panniculitis, shown to be due to disseminated acanthamoebiasis. RESULTS: Histologic sections showed a neutrophilic lobular panniculitis with 20- to 30-microm trophozoites consistent with Acanthamoeba species. CONCLUSIONS: A review the literature shows that the histopathological presentation of acanthamoebiasis often eludes initial diagnostic attempts and that central nervous system (CNS) involvement is frequent and ultimately fatal. When amoebiasis is suspected, knowledge of the trophozoite and cyst forms may be helpful in distinguishing Acanthamoeba species from Entamoeba histolytica.

Intraocular inflammatory reactions without focal necrotizing retinochoroiditis in patients with acquired systemic toxoplasmosis.

PURPOSE: To describe the occurrence of intraocular inflammatory reactions as the sole ophthalmic manifestation of acquired systemic toxoplasmosis. METHODS: Review of medical records for 10 patients with uveitis and evidence of recent Toxoplasma gondii infection. RESULTS: Patient ages ranged from 3 to 51 years. Ocular symptoms were present in each of eight adult patients. Inflammation was unilateral in nine patients; it manifested as vitreous humor cells and haze (10 patients), anterior chamber cells (seven patients), and retinal vasculitis (seven patients). No patient had necrotizing retinochoroiditis upon initial examination. Inflammation resolved in each of nine patients who had follow-up examinations. Foci of retinitis or inactive retinochoroidal scars were seen in four of these nine patients during follow-up examinations, at intervals of 2.0 weeks to 2.5 years after initial examination. CONCLUSIONS: Retinal vasculitis and associated inflammatory reactions may be the only ophthalmic disorder during the early stages of a newly acquired T. gondii infection. Later development of retinitis or scars consistent with toxoplasmic retinochoroiditis in the same eyes suggests that the initial, isolated inflammation may be caused by the presence of parasites in retinal tissue. These cases may have implications for understanding the original source of retinal infection in patients who have recurrent toxoplasmic retinochoroiditis and for treatment of newly acquired T gondii infection.

Vasculitis owing to infection.

Infections are a recognized cause of secondary vasculitis. A variety of pathogens have a propensity to involve blood vessels. Vasculitis, non-vasculitic vasculopathy, and mycotic aneurysms lead to infarction and hemorrhage of nervous system tissue. Treatment of infection-related vasculitis should include appropriate antimicrobial therapy directed against the offending pathogen, and appropriate management of cerebrovascular complications.