Lupus erythematosus/lichen planus overlap syndrome with scarring alopecia

Lupus erythematosus (LE) and lichen planus (LP) may occur as an overlap syndrome (LE/LP). The term comprises a heterogeneous group of patients who have clinical, histological and/or immunopathological characteristics of both diseases at the same time. The disease occurs so infrequently as to limit clinical cases for study. We present a case of scarring alopecia due to LE/LP overlap syndrome. Initial biopsy of the scalp showed scarring alopecia only, with subsequent biopsies showing histological features of LE. Direct immunofluorescence was negative for the LE band, but showed features consistent with LP. We report this case as an uncommon cause of scarring alopecia illustrating the importance of multiple biopsies in the diagnosis of LE/LP overlap syndrome.     

A case of lupus erythematosus/lichen planus overlap syndrome

A case of lupus erythematosus/lichen planus overlap syndrome (LE/LP overlap syndrome) was reported. A 53-year-old woman developed violaceous erythema around the nostrils and the upper lips and atrophic scaly erythema on the cheeks and neck. Histopathological studies revealed that the patient had distinct discoid lupus erythematosus (DLE), LP, and a lesion with combined features of DLE and LP. Direct immunofluorescent (DIF) studies of the mixed lesion revealed both prominent immunoglobulin (Ig)G deposits in a granular pattern at the basement membrane zone (BMZ) and IgM deposits in the clusters of cytoid bodies; the former are more typical of LE and the latter more of LP. DIF features in combination were unique for LE/LP overlap syndrome. The patient was satisfactorily treated with topical tacrolimus. While reports support the effectiveness of tacrolimus in either LE or LP, this is the first case of LE/LP overlap syndrome treated with topical tacrolimus.     

An immunofluorescence study of primary anetoderma

Primary anetoderma (PA) has occasionally been described in association with lupus erythematosus (LE). The present study was performed to elucidate a possible causal link between PA and LE by the use of direct and indirect immunofluorescence (IF) methods. Two patients with PA were studied. Biopsy specimens were obtained from early inflammatory and atrophic anetoderma lesions and from the exposed and unexposed uninvolved skin of each patient. The pattern of immune deposits observed in one patient was indistinguishable from that which is often seen in systemic LE, and in the other patient from that which may be observed in chronic cutaneous LE. The direct IF study also showed fibrillar immune deposits in the dermis that resembled elastic fibres morphologically. The indirect IF study, however, failed to demonstrate anti-elastic fibre antibodies in the patients' sera. The results of this study and a review of the literature suggest that some cases of PA have direct IF findings similar to those of either chronic cutaneous or systemic LE. However, these findings, along with the serological findings, are insufficient to establish a diagnosis of LE in most of these PA cases.     

The presence of anti–basement membrane zone antibodies in the sera of patients with non–bullous lupus erythematosus

We performed indirect immunofluorescence (IF) studies using 1 mol/1 sodium chloride split skin to determine whether or not a positive IF is specific to patients with bullous lupus erythematosus (LE). We examined the sera from 21 patients with systemic LE (SLE), three of which were obtained from two SLE patients and one subacute cutaneous LE (SCLE) patient with bullous eruptions. As a comparison, we also studied the sera from patients with discoid LE (DLE,n= 7). SCLE (n= 1), systemic sclerosis (SSc, n= 20), bullous pemphigoid (n= 2) and normal individuals (n= 10). Sera from 16 SLE, four DLE and two SSc revealed a linear deposition of IgG isotype antibody at the epidermal side and/or the dermal side on indirect IF of split skin. The sera from three patients with bullous eruption and from 12 patients of SLE, SCLE, DLE without bullous eruption or SSc were further analysed by immunoblotting using five defined antigens, i.e, dermal extract, epidermal extract, three fusion proteins of 230 kDa bullous pemphigoid antigen (BPAG), 180 kDa BPAG, and human epidermolysis bullosa acquisita (EBA) antigen. Two SLE sera as well as one of the SCLE and the DLE serum reacted with 230 kDa BPAG in epidermal extract, and one of the SCLE and the DLE serum also reacted with the fusion protein of 180 kDa BPAG, No serum reacted with the dermal extract or the fusion protein of 230 kDa BPAG or EBA antigen. There was no consistent correlation between split–skin IF results and immunoblotting results. These results may suggest that even non–bullous LE patients often have autoantibodies to the basement membrane zone antigens, most of which are less pathogenic. Although we rarely examine the sera from non–bullous LE patients, we should keep this phenomenon in mind to avoid overestimating the results of split–skin test and immunoblotting.     

扁平苔藓36例分析

目的:分析36例扁平苔藓(LP)的临床、组织病理(HP)、免疫病理(IF)、免疫印迹特点。方法:回顾分析36例LP的临床资料,HPI、F、免疫印迹检查结果。结果:36例LP中男18例,女18例,年龄12~63岁,病程1周至20年。HP诊断LP的敏感性是72.5%,一些特殊类型如色素性LP、光化性LP、肥厚性LP、毛发LP、大疱性LP、LE/LP重叠综合征等各有其特点。IF诊断LP的敏感性是75%,在IF中,Ig、C3各有11例和10例在基底膜带(BMZ)沉积,其中2例血清中检测出抗BMZ抗体。免疫印迹检查中,在表皮提取的抗原上4例类天疱疮样扁平苔藓(LPP)均出现阳性反应条带,其中2例240 kDa,2例200 kDa,3例180 kDa。结论:LP并非少见,其临床类型很多,HP和IF检查是确诊LP的主要依据。泼尼松、氨苯砜、反应停联合治疗LP有肯定效果。     

Serologic studies in patients with lupus erythematosus and psoriasis

We present four patients with coexistent lupus erythematosus (LE) and psoriasis. This is an unusual combination. All four patients had antibodies to Ro, which were absent in twenty-four control psoriatics. Antibodies to Ro occur in only 25% to 30% of unselected SLE patients, but occur in approximately 60% of "antinuclear antibody (ANA)-negative" LE patients, many of whom are highly photosensitive. The increased frequency of anti-Ro in our patients suggests that this may be a specific serologic marker for the LE/psoriasis overlap. Also, since anti-Ro correlates with photosensitivity, LE/psoriasis overlap patients may be at increased risk for photosensitivity, which occurred in two of our patients, one of whom developed severe systemic disease following ultraviolet (UVB) phototherapy. Screening for anti-Ro antibodies may be appropriate in psoriatics prior to UVB phototherapy.     

Unusual variant of lupus erythematosus or lichen planus. Clinical, histopathologic, and immunofluorescent studies.

Eleven patients with a skin disorder in which clinical, histopathologic, and immunofluorescent findings showed overlap features of both lupus erythematosus (LE) and lichen planus (LP) were observed for several years. Clinical lesions were extremely long-term and consisted primarily of livid red to violaceous atrophic patches and plaques, most common on acral aspects of the extremities. Nails were also commonly involved, often showing anonychia. Histologic changes combined cell-rich and cell-poor lichenoid patterns in the papillary dermis, suggesting both LP and LE. The major immunofluorescent finding in all patients was the presence of ovoid bodies at the dermal-epidermal (D-E) junction and in the upper dermis. Most patients showed both a linear arrangement and a clustering pattern of these bodies. Four patients also had coexistant but poorly developed linear deposits of immunoglobulins and complement at the D-E junction.     

The reliability of immunofluorescence and histopathology in the diagnosis of discoid lupus erythematosus and lichen planus

We have investigated the diagnostic reliability of the immunofluorescence (IF) technique and histopathology in discoid lupus erythematosus (DLE) and lichen planus (LP) and in diseases clinically resembling these (DLE-like and LP-like). In all cases of DLE and LP it was possible to establish the clinical diagnosis with one or both methods, when in initially negative cases the investigations were repeated on fresh biopsies. In DLE the diagnostic specificity of IF was greater than that of histopathology, and the diagnostic sensitivity of the results of both methods together was greater than that of the two methods separately. In LP the diagnostic specificity of both methods was maximal, but IF showed greater diagnostic sensitivity. These differences were not statistically significant. The most important immunohistochemical feature for diagnosis by IF was the incidence and the morphological pattern of IgG along the epidermal basement membrane. This held true for differentiation between LP and DLE and also between DLE and DLE-like diseases. Combination of the results of IF and histopathology gave the most reliable results in DLE. In LP, IF was more reliable than histopathology.     

Lichen planus pemphigoides: clinical and immunofluorescent findings in four cases

During the last 5 years we examined four black male patients who had features of both lichen planus (LP) and bullous pemphigoid (BP). The clinical disorder included classical lesions of LP along with bullae that developed subsequently and arose on both lesional and nonlesional skin. Histologic findings correlated well with the type of lesion on which biopsy was done, as did the immunofluorescent findings. All patients had features of both LP and BP by direct immunofluorescence and of BP by indirect immunofluorescence of serum and blister fluid.     

Occurrence of positive immunofluorescence in the dermo-epidermal junction of sun-exposed skin of normal adults

A bright, continuous, granular deposition of immunoreactants at the dermo-epidermal junction (DEJ) of lesional skin is highly suggestive of cutaneous lupus erythematosus (LE). A recent study of the direct immunofluorescence (IF) of sun-exposed skin in normal adults has demonstrated findings similar to the bright, continuous granular pattern found in cutaneous LE. This data suggests that positive IF from sun-exposed cutaneous lupus lesions is nonspecific. Forty-one healthy adults, without a history of dermatoses or photosensitivity, presenting to the dermatology clinic for the excision of skin cancers were studied. Excess non-lesional tissue, removed from Moh's excision sites (sun-exposed face and neck) in order to obtain appropriate cosmetic closure, was examined for the deposition of immunoreactants. The specimens were incubated with iluoresceinated monovalent anti-human immunoglobulin specific for IgG, IgA, IgM, C3, Clq, and fibrinogen and examined independently by 2 immunodennatologists without prior knowledge of patient or site. None of the samples demonstrated immunoreactant deposition consistent with cutaneous LE. IF of several specimens (21/41) had a weak(1+ or 2+), interrupted pattern of fibrinogen at the DEJ, – a common, non-specific finding. Weak, interrupted, linear and granular patterns were seen with IgM (10/41), Clq (9/41), IgG (2/41), IgA (2/41), and C3 (1/41). Fibrinogen was the only immunoreactant demonstrating a bright (3+), continuous, granular pattern (4/41). This data suggests that sun-exposure alone does not induce the deposition of immunoreactants at the DEJ in a pattern similar to that found in cutaneous LE.     

Comparison of immunofluorescence microscopy, immunoblotting and enzyme-linked immunosorbent assay methods in the laboratory diagnosis of bullous pemphigoid

This prospective study investigated patients with a clinical diagnosis of bullous pemphigoid (BP) who presented to a tertiary dermatology referral centre in Singapore. All patients had blood samples and skin biopsies taken for histology, immunofluorescence (IF) and immunoblot analysis prior to initiation of treatment. We analysed 23 new cases of BP during the 1-year study period. Seventeen of 22 biopsy specimens showed subepidermal blister formation, and 12 of the 17 (71%) had a predominance of eosinophils (>50%) in the blister cavity. The dermal inflammatory infiltrate of 22 biopsy specimens was predominantly lymphocytic in nine (41%) and eosinophilic in eight (36%). The histological picture was highly suggestive of BP in 15 of 22 patients (68%), suggestive in two (9%) and poorly suggestive in five (23%). Twenty-one of 23 (91%) patients had linear deposits of IgG and C3 along the dermo-epidermal junction. Serum indirect IF was positive in 22 of 23 (96%) patients, all showing antibody binding to the roof of the induced blister on salt-split skin. All of the 23 serum samples demonstrated positive immunoblot reactivity to BP180 and/or BP230 from epidermal extracts of normal human skin. Immunoblot reactivity with BP180 and BP230 was 78% (n=18) and 52% (n=12), respectively. The BP180 NC16A antibody could be detected in 22 of 23 (96%) sera using the enzyme-linked immunosorbent assay (ELISA) technique. The sensitivity of traditional diagnostic techniques, i.e. direct IF (91%) and indirect IF (96%), was comparable with that of the newer techniques, i.e. immunoblot analysis (100%) and ELISA (96%). ELISA in combination with routine indirect IF may be a useful diagnostic tool in patients with suspected BP who refuse a skin biopsy but consent to give a serum sample.     

Lucio-Phänomen

Lucio's phenomenon (LP) occurs in patients with Lucio leprosy (LuL). Some interpret the cutaneous lesions and their histopathology as a thrombotic/occlusive condition, while others consider it leukocytoclastic vasculitis. The clinical similarities between the cutaneous manifestations of LP and antiphospholipid syndrome (APS) led us to investigate the relationship between these two pathological conditions. We studied the clinical, laboratory and histopathologic aspects of LuL and LP in one patient and compare these results to APS. The examination of antiphospholipid antibodies showed positive anticardiolipin (aCL) and lupus anticoagulant (LAC). The histopathological slides of cutaneous biopsies were stained by hematoxylin-eosin and Fite-Faraco. They showed the typical features of LuL, as well as thrombi, endothelial proliferation, vessel wall thickening and obliteration of the lumen. Leukocytoclastic vasculitis was not found. The clinical pattern of LuL in our case is identical to that described by Lucio and Latapi. The necrotic lesions of LP in our patient resembled APS. This suggests that LP could be considered APS secondary to LuL. Multidrug treatment for multibacillary patients (MDT-MP) was successful, with no need for thalidomide or systemic corticosteroids.     

T cell subset heterogeneity in a series of patients with mycosis fungoides and Sézary syndrome

The diagnostic value of the identification of T cell subsets in skin biopsies from a series of eight patients (clinical stages I-IV) with cutaneous T cell lymphoma (6 with mycosis fungoides and 2 with Sézary syndrome) was determined with an indirect immunoperoxidase technic using commercially available monoclonal anti-T lymphocyte antibodies. Each patient had a mixture of helper and suppressor T cells within both the dermis and the epidermis, although helper T cells predominated approximately 2:1 over suppressor cells in all except two patients. This pattern is not appreciably different from that reported in benign inflammatory dermatoses. One patient with Sézary syndrome who had had an unusually protracted course was found to have a greater number of suppressor cells than helper cells within the epidermis. We conclude that the in situ identification of T cell subsets adds little, if any, diagnostic sensitivity to standard histopathologic methods in cutaneous T cell lymphoma.     

Value of direct immunofluorescence for differential diagnosis of cicatricial alopecia

BACKGROUND: There are diverse causes of cicatricial alopecia characterized by lack of follicular ostia and irreversible loss of hair. While clinical differentiation between the causes may be difficult, particularly with regard to lichen planus (LP), lupus erythematosus (LE) and pseudopelade of Brocq (PB), it has been suggested that both histopathologic examination and direct immunofluorescence studies (DIF) are necessary for an accurate diagnosis. OBJECTIVE: The aim of this study was to evaluate the diagnostic value of DIF studies in addition to histopathology in patients with cicatricial alopecia as a clinical feature. METHODS: 136 scalp biopsy specimens received for histopathology and DIF during a 5-year period were reviewed. RESULTS: Definitive diagnosis was achieved by careful evaluation of scalp biopsies. The most prevalent diagnoses in order of frequency were LP (26%), LE (21%) and folliculitis decalvans (20%). PB was diagnosed in 10%. In most cases, the diagnosis could be made on the basis of histopathology and independently of DIF. Characteristic DIF patterns showed high specificity, but low sensitivity for LP, and high specificity and sensitivity for LE. The DIF pattern in PB showed no difference to LP. CONCLUSIONS: Histopathology permits diagnosis in the majority of cicatricial alopecias. DIF is of value in histopathologically inconclusive cases, particularly when LE is in question.     

Lichen planus and lupus erythematosus overlap syndrome

A 45-year-old woman with livid plaques showing central atrophy and erythematous vesicular borders over both dorsa of feet and buttocks, and follicular and papular lesions over buttocks and lumbar area, was difficult to diagnose as either lichen planus (LP) or lupus erythematosus (LE). The histological studies from two places showed features of both LE and LP. Laboratory findings were within normal limits first, but follow up studies for two years showed persistent albuminurea, leucopenia, arthritis and erythema over the exposed areas with same histology suggesting that eruption may be an unusual variant of LE.     

Lichenoid tissue reaction

Lichenoid tissue reaction (LTR) is characterised by epidermal basal cell damage which takes the form of liquefaction degeneration or cell death either apoptosis or necrosis with an associated cascade of histologic events in epidermis and dermis. LTR is found in clinical conditions with lichenoid poikilodermatous and pigmentary dermatoses. A selected group of fifty lichenoid and pigmentary dermatoses such as Lichen planus (LP) Discoid lupus erythematosus (DLE) Lichenoid melanodermatitis (LM) and Lichen nitidus (LN) were studied. In LP basal cell liquefaction degeneration was extensive in comparison to other disease with large number of Civatte bodies and colloid bodies. There were significant vasodilatation in upper dermis inside the massive band like infiltrate. PAS positive basement membrane was disrupted in reaction area. Hypergranulosis was conspicuous. Chronic DLE showed spotty lichenoid reaction in the form of basal cell liquefaction degeneration. Civatte bodies and colloid bodies were infrequent. Infiltrate was more focal but could be band like. Epidermal atrophy and thickening of PAS positive basement membrane were important differentiating features, LM or Melanodermatitis toxica revealed focal mild to moderate liquefaction degeneration of basal cells with atrophy of the epidermis. The infiltrate although band like was less dense with marked pigmentary incontinence in clumps and giant melanophages. Civatte bodies, colloid bodies were not found and vascular changes were less prominent. LN showed localised basal cell damage with claw like rete ridges clutching a dense infiltrate. The dermal infiltrate often showed multinucleated giant cell. Civatte bodies and colloid bodies were not present. In some cases of the overlap syndrome LP/LE a careful study of lichenoid tissue reaction could distinguish these two diseases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Calcium enhances the sensitivity of immunofluorescence for pemphigus antibodies

Indirect immunofluorescence (IF) to detect pemphigus and pemphigoid autoantibodies is commonly performed with monkey esophagus (ME) as substrate and phosphate-buffered saline (PBS) as a diluent. The purpose of this study was to evaluate comparative IF titers using human skin (HS) as substrate with variations in the buffers employed. Substrates (ME or HS) were incubated in PBS, Tris-acetate-buffered saline (TAS), TAS with 5 mM CaCl+2 (TAS-Ca+2), and PBS or TAS with 1 mM EDTA, prior to incubation with pemphigus or pemphigoid sera for indirect IF. We examined sera from 11 patients with pemphigus vulgaris (PV), 10 patients with Brazilian pemphigus foliaceus (BPF), and 4 patients with bullous pemphigoid. In 20 of 21 pemphigus sera, endpoint indirect IF titers were highest on normal skin with TAS-Ca+2. Six sera (2 PV and 4 BPF) had endpoints that were 5 double dilutions higher than the endpoints obtained with ME and PBS. Six sera (3 PV and 3 BPF) were 4 double dilutions higher, 7 sera (3 PV and 4 BPF) were 2-3 double dilutions higher, and 2 PV sera were equivalent with both substrate/buffers. Preincubation of either tissue with EDTA prior to indirect IF abolished PV and BPF antibody binding completely. Exposure to EDTA after the tissue was incubated with PV or BPF sera did not affect indirect IF titers. In the presence of Ca+2, the antigen was resistant to trypsin in concentrations of 0.001%; however, in the absence of added Ca+2 it was destroyed by 0.0001% trypsin. These differences were not observed with bullous pemphigoid sera; all 4 sera had similar endpoint indirect IF titers. This study shows a significant increase in the sensitivity of indirect IF assays for pemphigus autoantibodies by the use of Ca+2-supplemented buffers on human skin. This finding may also have implications for procedures designed to purify and/or detect pemphigus antigens.     

A Study of lupus erythematosus with particular reference to generalized discoid lupus

The classification of lupus erythematosus (LE) is difficult because of variable and multisystem involvement, occurrence of transitional forms, and overlapping with connective tissue disorders. One of the earliest attempts at classification was made by O'Leary (1934), who classified LE clinically into four main types: (1) chronic discoid lupus erythematosus (DLE), or fixed type with the typical erythematous, scaly, well-demarcated eruption, usually showing follicular plugging and atrophy, confined to the head region; (2) generalized DLE, or chronic disseminated type differing from the localized discoid type in that the erythematous plaques are found not only on the head area but also below the neck region (O'Leary considered that constitutional systems were generally lacking in these two types); (3) subacute disseminated LE; and (4) acute disseminated LE. The last two categories reflect what we now call systemic lupus erythematosus (SLE) of varying severity. Many authors have used O'Leary's clinical classification, but progress in laboratory medicine—in particular the discovery of the LE cell—has led to other schemes, the most widely accepted at present being that related to the American Rheumatism Association's (ARA) preliminary criteria for the diagnosis of SLE (Cohen et al., 1971). These criteria have been criticized by many authorities because they do not include important data, such as those relative to the antinuclear antibody test, serum anti-native-DNA, serum complement levels, many neurological abnormalities, renal biopsy changes, and cutaneous immunofluorescent (IF) findings. However, Dubois (1974b) pointed out that all categorizations of LE are arbitrary, and he accepted the ARA criteria as a successful method for differentiating LE. This report involves the clinical and laboratory findings in 123 patients with LE seen at the Mayo Clinic during the 3-year period 1971–1974 and an assessment of the validity and usefulness of O'Leary's classification.     

Lupus erythematosus associated with erythema multiforme: report of two cases and review of the literature

Rowell's syndrome (RS) is a rare presentation of lupus erythematosus (LE) with erythema multiforme‐like lesions associated with antinuclear, anti‐La (SS‐B)/anti‐Ro (SS‐A) antibodies and rheumatoid factor (RF) positivity. This syndrome is suggested to be a different variant of cutaneous lupus erythematosus by some authors in literature. Here we present a 64‐year‐old woman with LE and a 51‐year‐old woman with LE and Sjögren syndrome (SS) who had erythema multiforme‐like eruptions and discuss the coexistence of lupus erythematosus and erythema multiforme.