Positive Direct Immunofluorescence Findings Research Articles

A Cross-Sectional Study to Correlate Serum Complement C3 and C4 Levels With Clinical and Pathological Severity in Cutaneous Small-Vessel Vasculitis.

IntroductionThe role of serum C3 and C4 levels as a marker of disease activity in cutaneous small-vessel vasculitis (CSVV) has been sparsely studied, especially in India. The primary objective was to determine the correlation between clinico-histopathological severity and serum C3 and C4 levels in CSVV. The secondary objective was to determine the association between direct immunofluorescence (DIF) findings and serum C3 and C4 levels and clinico-histopathological findings.MethodThis prospective cross-sectional study included all the clinically diagnosed cases of CSVV that satisfied the pathological criteria for CSVV. A clinical disease activity grade and a histopathological severity grade were calculated in all patients (N=50).ResultsSerum C3 and C4 levels (n=44) were diminished in 4.5% of cases. There was no significant correlation between the serum C3 and C4 levels and the clinical and histopathological severity. DIF was positive in 60.0% of cases (n=45), and IgA was the predominant immune deposit (46.7%). No significant association was detected between the DIF findings and the serum C3 and C4 levels, histopathological severity, and clinical disease activity grade. Positive DIF findings were significantly associated with palpable purpura and cutaneous necrosis. A significant association was detected between gastrointestinal involvement and IgA positivity.ConclusionIn CSVV, serum C3 and C4 may not be used as markers of disease severity, and a positive DIF finding may indicate an underlying gastrointestinal involvement.

Direct immunofluorescence findings in livedoid vasculopathy: a 10‐year study and literature review

Direct immunofluorescence (DIF) findings in patients with livedoid vasculopathy (LV) may have benefits for disease differentiation when clinical presentations and/or histopathological findings are inconclusive. To investigate DIF findings in patients with a clinical and histopathological diagnosis of LV. DIF findings of 62 patients with LV were analysed, and the published literature in the PubMed database was also reviewed and summarized. This study demonstrated deposition of immunoreactants in blood vessels (BVs) in 59 of the 62 patients (95.2%), and almost all cases were positive for a combination of multiple immunoreactants. Complement C3 and IgM formed the most common combination. The most common pattern was deposition in BVs and at the dermoepidermal junction (DEJ) (59.3%), followed by deposition in BVs alone (40.7%). Immunoreactant deposition in BVs involved superficial BVs with or without deep BVs. The median age of patients with positive DIF findings was significantly higher than that of patients with negative DIF findings for LV (P<0.03). More recent lesions (present for <6months) had a significantly higher percentage of positive results than older lesions (present for ≥6months) (85.2% vs. 14.8%, respectively; P<0.001). In both the present study and in the published literature, DIF study in patients with LV showed positive immunoreactants ranging from 42.9% to 100%. C3 and IgM were the most common immunoreactants deposited in BVs, while the most common pattern was immunoreactant deposition in BVs and at the DEJ. Older patients and those with more recent lesions (<6months) had a significantly higher percentage of positive DIF results for LV than did younger patients and those with older lesions (≥6months).

Lupus Erythematosus Profundus: A Case Series and Review of Literature

Objective: Lupus Erythematosus Profundus (LEP) is a rare subset of Lupus erythematosus and clinically presents as indurated subcutaneous painful nodules and plaques. The objective of the study was to evaluate clinical, histopathological and supplementary laboratory parameters to diagnose Lupus erythematous profundus.Methods and materials: This was a retrospective study, a clinical series of histo-pathologically proven cases of 11 patients. Data were collected from clinic records from 1996 to 2010. A detailed history including onset of disease, age, sex, residence, hospital ID number and clinical examination, association with Discoid lupus erythematosus (DLE) or Systemic lupus erythematosus (SLE), laboratory workup, histopathological evaluation and Direct Immunofluorescence data was taken from the records.Result: There were total of 11 patients, with mean years of presentation of 26 years. Male to female ratio was 3:8. Face was the commonest site of distribution. Exclusively LEP presentation was seen in 83% and in 18% associated with DLE and 9% with SLE. Clinically lesions varied from nodules, indurated plaques, atrophy and ulcer. In laboratory work up ANA was positive in 9% of the cases, others baseline investigations were within normal limits. On histopathological evaluation, lobular panniculitis and hyaline fat necrosis was seen in all patients. The infiltrates were predominantly lymphohistiocytic. Direct immunofluorescence (DIF) could be done in 3 out of 11 cases. Lupus band at basement membrane zone was seen in all the 3 cases. Deposition of predominantly IgG followed by IgM, IgA and C3 were seen. Two of the three cases with positive DIF findings had no interface pathology.Conclusion: Diagnosis of LEP is based on clinico-pathological correlation. An early diagnosis and prompt treatment may help prevent disfigurement.

Positive Direct Immunofluorescence Is of Better Value than ELISA-BP180 and ELISA-BP230 Values for the Prediction of Relapse after Treatment Cessation in Bullous Pemphigoid: A Retrospective Study of 97 Patients

Background: ELISA-BP180 values and direct immunofluorescence (DIF) are prognostic factors for relapse after treatment cessation in bullous pemphigoid (BP). Objective: To determine the relevance of ELISA-BP230 antibodies for predicting relapse 6 months after treatment cessation. Methods: We retrospectively selected patients with BP and available data from ELISA-BP180 and -BP230 and DIF performed at treatment cessation. The rate of relapse was calculated at 6 months. We compared ELISA-BP180 and -BP230 values and DIF in patients with relapse and remission. Results: We included 97 patients. At 6 months, 25.6% of patients showed relapse. The proportion of patients with an ELISA-BP230 value ≥27 UA/ml was higher, but not significantly, for those with relapse than for those with remission (p = 0.11). The frequency of positive DIF findings was significantly higher for patients with relapse (p = 0.005). Conclusion: DIF is of better value than ELISA-BP180 and -230 tests to predict relapse after treatment cessation in BP.

'Clues' for the histological diagnosis of tinea: how reliable are they?

'Clues' for the histological diagnosis of tinea: how reliable are they?

Bullous Tinea Pedis With Direct Immunofluorescence Positivity

Skin biopsy for direct immunofluorescence (DIF) testing is an essential tool in the diagnosis of blistering diseases. In the majority of cases, positive epidermal immunofluorescent staining is indicative of an autoimmune bullous disease (AIBD). We identified 2 patients with bullous dermatophyte infection diagnosed on hematoxylin- and eosin-stained sections who had positive DIF findings on biopsy of perilesional skin. We subsequently reviewed the literature regarding positive DIF findings in conditions other than AIBD. Other infections, including herpesviridae, scabies, and orf, have rarely been reported to yield positive DIF findings, with positive staining at the dermoepidermal junction. Some genodermatoses and many inflammatory skin diseases, including lichen planus, psoriasis, graft-versus-host disease, among others, may also have DIF findings mimicking those of both intra- and subepidermal AIBD. Although rare, positive DIF results occur in conditions other than AIBD. In many instances, the pathophysiological mechanisms behind immunoreactant deposition in these conditions are poorly understood. Misleading DIF results may lead to delay in correct diagnosis and treatment. Clinicians should be aware of potential alternate sources of positivity when there is lack of clinical correlation with immunofluorescence findings.

Immunofluorescence of the outer root sheath: an aid to diagnosis in pemphigus

The direct immunofluorescence (DIF) pattern of the outer root sheath (ORS) of the hair follicle in patients with pemphigus resembles that of perilesional skin. We performed DIF on anagen and telogen hair ORS from patients with pemphigus and compared the findings with those of perilesional skin. To assess the sensitivity of DIF in the ORS of anagen and telogen hair in patients with pemphigus with positive DIF results in perilesional skin. In total, 50 patients with active pemphigus and positive DIF findings in perilesional skin were selected for the study. Anagen hair was obtained from the scalp by the same method used for a trichogram, and telogen hair was selected by combing the hair and collecting the loose strands of hair from the comb. The hair type was further confirmed by microscopy. Hair samples were transported in Michel medium and prepared for DIF by mounting on a slide without sectioning. Anagen and telogen hair samples were also collected from 50 controls. Immunological deposits characteristic of pemphigus were found in the ORS of anagen and telogen hairs in all 50 patients, but in none of the 50 controls. Positive DIF findings were seen in scalp hair irrespective of the presence of scalp lesions. Of the 50 patients with pemphigus, 46 were diagnosed with pemphigus vulgaris, 2 with pemphigus vegetans, and 1 each with pemphigus foliaceus and pemphigus erythematosus, based on the clinical, histopathological and DIF findings. All the pemphigus variants had positive DIF findings in the ORS. Using DIF on telogen hair ORS promises to be a useful tool for diagnosis and follow-up in cases of pemphigus.

A Determination of the Range of Oral Conditions Submitted for Microscopic and Direct Immunofluorescence Analysis

Direct immunofluorescence (DIF) testing is a useful adjunct for the diagnosis of immune-mediated oral vesiculobullous diseases, helping to identify separate, histologically similar, but prognostically different, conditions. It is unknown how often biopsy of these lesions yields positive DIF results. A total of 270 consecutive archival cases submitted to a reference laboratory in Buffalo, New York, over a 2-year span were examined. These specimens were submitted to establish or rule out a diagnosis of a DIF-positive oral vesiculobullous disease. Demographic, clinical, and diagnostic information, based on conventional microscopic and DIF analysis, was tabulated. To assess the contribution of DIF to successful diagnosis, three pathologists examined the hematoxylin and eosin-stained slides of the known DIF-positive specimens without knowledge of the DIF results. Approximately 48% of the specimens demonstrated positive DIF findings and consisted of pemphigus vulgaris, mucous membrane pemphigoid, lichen planus, linear immunoglobulin A disease, and chronic ulcerative stomatitis. The remaining specimens had negative DIF findings and consisted of numerous non-specific inflammatory conditions. Of particular interest were several cases of epithelial dysplasia and squamous cell carcinoma. Of the DIF-positive cases, only pemphigus vulgaris could be diagnosed reliably by conventional microscopy alone. Approximately half of biopsies of oral conditions that clinically resembled typically DIF-positive vesiculobullous diseases did not yield positive findings on DIF testing. Instead, a wide range of oral diseases can mimic these lesions clinically. With the exception of pemphigus vulgaris, DIF is essential for establishing a definitive diagnosis for known DIF-positive diseases.

Papular acantholytic dyskeratosis of the anogenital area with positive direct immunofluorescence results

Acantholytic dyskeratosis is a distinct histological pattern characterized by hyperkeratotic and parakeratotic epidermis with intraepidermal clefts harbouring acantholytic and dyskeratotic keratinocytes. This histopathological pattern is uncommon in dermatoses of the anogenital region. We report a 30-year-old woman who had numerous smooth whitish papules on the labia majora, perineum and perianal region, which coalesced into plaques in some areas. Microscopically, the lesions showed prominent suprabasal and intraspinous acantholysis with dyskeratotic keratinocytes. Direct immunofluorescence examination revealed intercellular Ig G and C(3) within the epidermis. We were unable to find a similar case of papular acantholytic dyskeratosis of the anogenital area with positive direct immunofluorescence findings reported in the literature, thus in this report, the clinicopathological features of a unique case are presented.

Immunologic Abnormalities Associated With Primary Anetoderma

Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence. Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor. These findings indicate that there is an immunologic involvement in primary anetoderma.

Immunologic abnormalities associated with primary anetoderma

<h3>• Background and Design.—</h3> Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence. <h3>Results.—</h3> Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor. <h3>Conclusions.—</h3> These findings indicate that there is an immunologic involvement in primary anetoderma. (<i>Arch Dermatol.</i>1992;128:799-803)

Direct immunofluorescence for the study of cutaneous drug eruptions

Forty-one patients with drug eruptions were retrospectively evaluated to correlate clinical, direct immunofluorescence, and dermatopathologic findings. Forty-six biopsy specimens were obtained for immunofluorescence in addition to specimens for dermatopathologic study. Eighteen patients had blood vessel fluorescence. Fourteen patients had basement membrane zone fluorescence. Eleven patients had dermatopathologic evidence of lichenoid changes; 8 of these had basement membrane zone fluorescence. Triamterene-hydrochlorothiazide caused the most eruptions (six patients). Positive direct immunofluorescence findings may aid the diagnosis of drug eruptions, especially in the presence of a nonspecific clinical picture or histopathologic findings.

Immunofluorescence findings in human renal amyloidosis.

Positive direct immunofluorescence findings have been occasionally reported for human renal amyloidosis. However, no substantial information describing the frequency of immunomicroscopic pattern has been reported. Twenty-seven renal biopsy specimens with proven amyloidosis were studied by direct immunofluorescence using monospecific antisera against IgG, IgA, IgM, IgD, IgE, kappa and lambda light chains, C3, C4 properdin, alpha-2 macroglobulin, albumin, transferrin, and fibrinogen. Ten biopsy specimens were negative. The remaining specimens showed principally a diffuse immunofluorescent staining of the mesangial areas with various of the above fluorescein-labeled antisera except IgE, albumin, and transferrin antisera, which gave a consistently negative reaction. IgD, properdin, or fibrinogen were weakly identified in only one case. Immunoglobulins and C3 were observed in 14 cases. Kappa and/or lambda light chains were found in nine of ten biopsy specimens so tested. Positive immunofluorescent staining of other elements of the nephron was rarely observed. These results suggest that various immunoreactants, in addition to light chains, can be frequently detected in renal amyloidosis and that passive nonselective absorption of plasma proteins does not explain these immunomicroscopic findings.