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Background: Direct immunofluorescence (DIF) test for tissue-bound autoantibodies, has been found to be of value in the diagnosis of several dermatological disorders. The location and pattern of deposition of immunoreactants helps in classifying various immune-mediated diseases. Aims and Objectives: The aim of this study was to analyze the concordance between the clinical, histopathological and DIF diagnosis in bullous and nonbullous lesions of the skin, and thus determine the impact of immunofluorescence on diagnosis. Materials and Methods: A total of 215 skin biopsies performed in suspected immune-mediated vesiculobullous disease, vasculitis or dermatosis, were studied. Histopathological examination was done along with DIF study for deposits of immunoglobulin G(IgG), IgA, IgM, and C3. Results: Direct immunofluorescence was positive in 103/215 cases. There was very good concordance between the clinical, histological and DIF results (observed agreement = 93.4%, κ =0.90, with 95% confidence interval = 0.86-0.94). The overall sensitivity of DIF in immune-mediated skin disorders was 98.0%. DIF was positive in 52/53 cases (98.1%) in the pemphigus group and 24/25 (96.0%) bullous pemphigoid cases. None of the clinically suspected cases of dermatitis herpetiformis showed DIF positivity. A positive lupus band test was seen in 9/9 (100%) cases of lupus erythematosus. DIF was positive in 10/10 (100%) clinically suspected cases of Henoch-Schönlein purpura. In 110 cases, negative DIF results helped to rule out immune-mediated vesiculobullous disorders, lupus erythematosus and vasculitis, and the final diagnosis was made on the basis of the clinical features and/or histopathology. Conclusion: Direct immunofluorescence is a useful supplement for the accurate diagnosis of immune-mediated dermatological disorders, and helps to classify various autoimmune bullous disorders. When the clinical features/histopathology are inconclusive, the diagnosis often can be made on the basis of the DIF findings alone. A combination of the clinical features, histopathology and DIF usually gives the best results.

Keratosis lichenoides chronica also known as Nekam's disease is a rare mucocutaneous disorder, characterized clinically by asymptomatic violaceous keratotic papules arranged in linear, reticular, or plaque form usually on the trunk and extremities and histologically by interface dermatitis. The disease is considered rare with only 128 cases being reported in the literature till date and very few from India. We report a case of a 40-year-old man who presented with constellation of features of lichen planus, seborrheic dermatitis, and apthous ulcers, which upon workup was found to be Nekam's disease.

Background: Direct immunofluorescence examination is an important technique in the diagnosis of cutaneous inflammatory disorders including lichen planus, especially in clinically and histopathological doubtful cases. Objective: To study the diagnostic utility of intensity, number, and subtypes of positive immuno-reactants found in lichen planus. Materials and Methods: A detailed analysis of clinical as well as immuno-histological features of lichen planus cases was carried out. Results: The male to female ratio was 1:1.1. The largest number of patients was in 31-50 year age group. Itching was the most common presenting symptom. Papular lesions were seen in 53% cases. Remaining had hypertrophic (6), follicular (3) and mucosal (9) variants. Clinico-pathological discrepancies were observed in 3 patients. The characteristic histopathological changes including basal cell vacuolization, band-like lymphocytic infiltrate at dermo-epidermal junction were seen in all the biopsies while Civatte bodies were detected in 29% cases. The overall positive yield of direct immunofluorescence microscopy was 55%. Immune deposits at Civatte bodies and dermo-epidermal junction were detected in 47% and 8% of cases, respectively. Immunoglobulin M was the most common immunoreactant followed by immunoglobulin G. Conclusions: There was no correlation found between the number and intensity of Civatte bodies with clinical variants of disease and also between the number of positive immunoreactants and clinical severity of the disease. The frequency, number, and arrangement of Civatte bodies in clusters in the papillary dermis as well as multiple immunoglobulins deposition at the Civatte bodies on direct immunofluorescence of skin biopsies are important features distinguishing lichen planus from other interface dermatitis.

Background: Interface dermatitis (ID) refers to a pattern of skin reaction characterized by an inflammatory infiltrate that appears to obscure the dermo-epidermal junction when observed at low power examination and referred to as lichenoid tissue reaction. A wide range of inflammatory skin diseases exhibits interface change with considerable overlap of histological features. The aim of the present study was to study the clinical features and microscopic features of ID. Materials and Methods: The material for the present study consisted of skin biopsy samples collected from patients attending the outpatient Department of Dermatology. The study was conducted for a period of 3 years from 2007 to 2010. During this period, a total of 125 cases was studied. Results: In the present study, a total of 125 cases of ID was studied which presented clinically as papulosquamous disorders. Majority of the cases of ID were seen in women (57.6%). Majority of ID were lichen planus (LP) and its variants (63.2%). Clinicopathological concordance was seen in 109 cases (87.2%) and discordance in 16 cases (12.8%). Conclusion: The mere presence of an interface lichenoid inflammatory reaction should not be the sole criterion for the diagnosis of LP or one of its many variants, as now seems to be the case. A clinicopathologic correlation is absolutely essential for a conclusive diagnosis of ID.

Primary cutaneous aggressive cytotoxic epidermotropic CD8+ T-cell lymphoma is an extremely rare, rapidly progressing, cutaneous lymphoma, with frequent systemic involvement and poor prognosis, that still represents a diagnostic and therapeutic challenge, especially in the early stage. Herein, we report a case of an elderly woman with a fulminant course, who at onset presented with clinical and pathological features mimicking erythema multiforme (EM) and treated with cyclosporine that led to rapid deterioration with fatal outcome 6 months after disease onset. Histopathology showed a lichenoid, epidermotropic and nodular, angiocentric, dermal and subcutaneous infiltrate of sF1, CD8+, CD45RA+ small to medium-sized atypical lymphoid cells, which strongly expressed cytotoxic markers. Monoclonal T-cell-γ receptor was clonally rearranged and array- CGH showed numerous chromosomal imbalances. This case evidences the clinical, pathological and therapeutic challenges involved in this tumor. The first biopsy showed an interface dermatitis-like pattern, revealing the deceptive features that early cutaneous infiltrates of this aggressive lymphoma may have. A high suspicion for aggressive CTCL and a low threshold for repeat biopsies should be maintained when faced with rapidly progressing and/or ulcerative EM-like lesions, especially if immunomodulatory therapy is being considered. [ABSTRACT FROM AUTHOR]

We report a case of noninfectious vacuolar interface dermatitis associated with colonic and perianal ulceration in a patient with acquired immunodeficiency syndrome (AIDS), which responded to immunosuppressive treatment. Our findings suggest that interface dermatitis in the setting of AIDS may warrant further gastrointestinal evaluation and may respond to immunosuppression. [ABSTRACT FROM AUTHOR]

The article describes the case of an obese 31-year-old female who presented with a well-demarcated, square-shaped area of erythema and blistering over the right mid back, with a history of splenic artery aneurysm and gastroesophageal reflux disease. Topics discussed include pathologic diagnosis of subacute radiation dermatitis as a result of prior fluoroscopic procedure, and acute radiation dermatitis characterized clinically by erythema.

Cutaneous lupus erythematosus (CLE) is a common disease that may appear as a separate entity from systemic lupus erythematosus (SLE), precede SLE development, or occur as a manifestation of this systemic disease. It has a complex pathophysiology that involves genetic, environmental, and immune-mediated factors creating a self-amplification pro-inflammatory cycle. CLE is characterized by prominent type I interferons (IFNs) inflammation which are considered as the first precursors of the inflammatory cascade generated within the pathophysiology of CLE. TNF-α enhances the production of antibodies through the activation of B cells, and favors the expression of surface nuclear antigens on keratinocytes. UV light exposure favors keratinocyte apoptosis or necroptosis, which results in the release of multiple proinflammatory cytokines, including IL-6, IL-1α, IL-1β, TNF-α, IFNs, and CXCL10. Serum levels of IL-17 are elevated in patients with ACLE, SCLE, and DLE. Evidence suggests IL-22 plays a role primarily in tissue repair rather than in inflammation. High expression of BAFF and its receptors have been found in lesioned keratinocytes of patients with CLE, and patients with CLE have lower serum levels of the regulatory cytokines TGF-β and IL-10. The chemokines CXCL9 and CXCL10 (CXCR3 ligands) have an increased expression among these patients, and their expression is correlated with IFNs levels. CXCR3 ligands recruit cytotoxic type I cells through this receptor, further supporting the death of keratinocytes via necroptosis with the subsequent release of eNAs perpetuating the inflammatory cycle. Interface dermatitis is characterized by the presence of CXCR3-positive lymphocytes. This review describes the leading cytokines and chemokines present in the circulation and skin that play a fundamental role in the pathogenesis of CLE. • CLE has a complex pathophysiology that involves genetic, environmental, and immune-mediated factors. • Its pathophysiology involves a complex interaction between the innate and adaptive responses. • CLE is considered an interferonopathy, as IFNs are the most critical cytokines contributing to the disease's pathogenesis. [ABSTRACT FROM AUTHOR]

Background: The data on the histology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) is limited.Aims: To study the histopathology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) and to identify any features with diagnostic or prognostic significance.Methods: All patients admitted to the dermatology ward of government medical college, Kozhikode from January 1, 2014 to December 31, 2014 with probable or definite DRESS as per the RegiSCAR scoring system and who were willing to undergo skin biopsy were included in this prospective study.Results: The study population comprised of nine patients. The consistent histological finding documented was the predominantly lymphocytic dermal inflammatory infiltrate. Four of the five patients whose histology revealed focal interface dermatitis and keratinocyte vacuolation with or without apoptotic keratinocytes, had elevated liver transaminases. Tissue eosinophilia was associated with disease flares. The presence of atypical lymphocytes in peripheral smear and histological evidence of dense dermal inflammatory infiltrate showed an association with hepatic involvement.Limitations: The main limitations of our study were the small sample size and our inability to carry out a detailed immunohistochemistry work-up.Conclusions: In the appropriate setting, varying combinations of epidermal hyperplasia, spongiosis, parakeratosis and individually necrotic keratinocytes in the background of lymphocyte predominant dermal infiltrate (with some atypia) favor a diagnosis of drug reaction with eosinophilia and systemic symptoms. Female sex, the presence of atypical lymphocytes in peripheral smear, dense dermal inflammatory infiltrate, tissue eosinophilia and interface dermatitis with or without keratinocyte necrosis was associated with a poor prognosis. [ABSTRACT FROM AUTHOR]

Background The majority of tattoo reactions are affiliated to red pigmented areas and often suspected to be allergic in nature. A sizeable series of biopsies of such reactions has not previously been performed. The aim of this study was to type and grade epidermal and dermal changes in tattoo reactions to red/red nuances by microscopy and immunochemistry relevant for the assessment of a possible allergic pathomechanism. Methods Skin biopsies were taken from red tattoo reactions, graded by conventional microscopy and stained for T and B-lymphocytes, Langerhans cells, macrophages and tumour necrosis factor ( TNF)-α. Results The study included 19 biopsies from 19 patients. The culprit colours were red/pink ( n = 15) and purple/bordeaux ( n = 4). Interface dermatitis was clearly the lead pathology found in 78% of samples, overlapped with granulomatous (in 32%) and pseudolymphomatous reaction patterns (in 32%). Epidermal hyperkeratosis (in 89%) was common as was leakage of red pigment across the dermo-epidermal junction, with transepidermal elimination (in 28%). The dermal cellular infiltration was dominated by T-lymphocytes (in 100%), Langerhans cells (in 95%) and macrophages (in 100%). TNF-α was common. Conclusion The predominant histological pattern of chronic tattoo reactions in red/red nuances is interface dermatitis. T-lymphocytes and Langerhans cells are increased suggesting an allergic pathomechanism. TNF-α may contribute to reactions. In many cases, overlapping reactive patterns were identified. [ABSTRACT FROM AUTHOR]

Background: Annular lichenoid dermatitis of youth (ALDY) is an uncommon disease clinically reminiscent of morphea, annular erythema or mycosis fungoides.Objective: To describe the histological and clinical features of a small series of patients with ALDY and to review the literature.Patients: We describe the clinical and histological features of six patients (age range 7-79 years) with asymptomatic erythematous macules and patches with a red-brownish border and central hypopigmentation, mostly distributed on the groin and flanks. Histologically, all cases showed lichenoid dermatitis limited to the tips of rete ridges, with many intraepidermal CD8+ and some CD4+ T cells. T cell receptor rearrangement was absent in all cases. A total of 44 patients with a consistent clinical and histological picture have been described. The disease is sensitive to topical and/or systemic corticosteroids.Conclusions: ALDY is a unique lichenoid dermatitis for whose diagnosis a clinical-pathological correlation is essential. The disease typically affects young patients, more rarely adults and elderly. [ABSTRACT FROM AUTHOR]

Background: Direct immunofluorescence (DIF) test for tissue-bound autoantibodies, has been found to be of value in the diagnosis of several dermatological disorders. The location and pattern of deposition of immunoreactants helps in classifying various immune-mediated diseases. Aims and Objectives: The aim of this study was to analyze the concordance between the clinical, histopathological and DIF diagnosis in bullous and nonbullous lesions of the skin, and thus determine the impact of immunofluorescence on diagnosis. Materials and Methods: A total of 215 skin biopsies performed in suspected immune-mediated vesiculobullous disease, vasculitis or dermatosis, were studied. Histopathological examination was done along with DIF study for deposits of immunoglobulin G(IgG), IgA, IgM, and C3. Results: Direct immunofluorescence was positive in 103/215 cases. There was very good concordance between the clinical, histological and DIF results (observed agreement = 93.4%, κ =0.90, with 95% confidence interval = 0.86--0.94). The overall sensitivity of DIF in immune-mediated skin disorders was 98.0%. DIF was positive in 52/53 cases (98.1%) in the pemphigus group and 24/25 (96.0%) bullous pemphigoid cases. None of the clinically suspected cases of dermatitis herpetiformis showed DIF positivity. A positive lupus band test was seen in 9/9 (100%) cases of lupus erythematosus. DIF was positive in 10/10 (100%) clinically suspected cases of Henoch-Schönlein purpura. In 110 cases, negative DIF results helped to rule out immune-mediated vesiculobullous disorders, lupus erythematosus and vasculitis, and the final diagnosis was made on the basis of the clinical features and/or histopathology. Conclusion: Direct immunofluorescence is a useful supplement for the accurate diagnosis of immune-mediated dermatological disorders, and helps to classify various autoimmune bullous disorders. When the clinical features/histopathology are inconclusive, the diagnosis often can be made on the basis of the DIF findings alone. A combination of the clinical features, histopathology and DIF usually gives the best results. [ABSTRACT FROM AUTHOR]

Background: Direct immunofluorescence examination is an important technique in the diagnosis of cutaneous inflammatory disorders including lichen planus, especially in clinically and histopathological doubtful cases. Objective: To study the diagnostic utility of intensity, number, and subtypes of positive immuno-reactants found in lichen planus. Materials and Methods: A detailed analysis of clinical as well as immuno-histological features of lichen planus cases was carried out. Results: The male to female ratio was 1:1.1. The largest number of patients was in 31-50 year age group. Itching was the most common presenting symptom. Papular lesions were seen in 53% cases. Remaining had hypertrophic (6), follicular (3) and mucosal (9) variants. Clinico-pathological discrepancies were observed in 3 patients. The characteristic histopathological changes including basal cell vacuolization, band-like lymphocytic infiltrate at dermo-epidermal junction were seen in all the biopsies while Civatte bodies were detected in 29% cases. The overall positive yield of direct immunofluorescence microscopy was 55%. Immune deposits at Civatte bodies and dermo-epidermal junction were detected in 47% and 8% of cases, respectively. Immunoglobulin M was the most common immunoreactant followed by immunoglobulin G. Conclusions: There was no correlation found between the number and intensity of Civatte bodies with clinical variants of disease and also between the number of positive immunoreactants and clinical severity of the disease. The frequency, number, and arrangement of Civatte bodies in clusters in the papillary dermis as well as multiple immunoglobulins deposition at the Civatte bodies on direct immunofluorescence of skin biopsies are important features distinguishing lichen planus from other interface dermatitis. [ABSTRACT FROM AUTHOR]

We present the case of a 10-year-old boy with an initial clinical and histopathologic diagnosis of actinic lichen nitidus of the face and neck, occurring in spring and summer and resolving in winter. He was treated with 1% hydrocortisone cream 1 time daily for 14 days and consistent sun protection was recommended. [ABSTRACT FROM AUTHOR]

Common inflammatory skin diseases can be correctly diagnosed with clinical examination. However, unusual presentations do exist and may cause difficulties in the differentiation among different entities, and histopathology is required for an accurate diagnosis. In this regard, dermoscopy and reflectance confocal microscopy (RCM) can play an important role in increasing the specificity of the diagnosis, offering the possibility of improved management of patients. In this review, the authors have analyzed dermoscopic patterns of lichen planus, discoid lupus erythematosus, psoriasis and alopecias as prototypes of inflammatory skin diseases, and correlated them with RCM microscopical features. RCM, thanks to its correspondence to histology and to the possibility of an immediate correlation to dermoscopy, provides additional information useful for increasing the sensibility and specificity of dermoscopy. [ABSTRACT FROM AUTHOR]

Interface dermatitis includes diseases in which the primary pathology involves the dermo-epidermal junction. The salient histological findings include basal cell vacuolization, apoptotic keratinocytes (colloid or Civatte bodies), and obscuring of the dermo-epidermal junction by inflammatory cells. Secondary changes of the epidermis and papillary dermis along with type, distribution and density of inflammatory cells are used for the differential diagnoses of the various diseases that exhibit interface changes. Lupus erythematosus, dermatomyositis, lichen planus, graft versus host disease, erythema multiforme, fixed drug eruptions, lichen striatus, and pityriasis lichenoides are considered major interface diseases. Several other diseases (inflammatory, infective, and neoplastic) may show interface changes. [ABSTRACT FROM AUTHOR]

The article focuses on amyloidosis disease, which is caused by the accumulation of abnormal host-derived β-pleated sheets in tissue. It says that amyliodosis manifests in biopsy material with amyloid deposition in blood vessels and affected tissues. It mentions that keratinocyte-derived amyloidosis represents a spectrum which includes lichen amyloidosus and macular amyloidosis.

Background: Dermatomyositis (DM) belongs to a group of diseases known as idiopathic inflammatory myopathies. It manifests by a proximal inflammatory myopathy of extensor muscles and pathognomonic skin lesions like Gottron's papules (GP). Nowadays, one of the most important aspects for the diagnosis continues to be muscular biopsy, although there are publications were it has been observed that histological alterations of the GP are more relevant to the diagnosis of DM. Objective: To determine the histological alterations of Gottron's papules. Material and methods: Retrospective, transversal, observational and descriptive study of 30 biopsies performed Center (Centro Dermatólogico Dr. Ladislao de la Pascua). Results: The histological characteristics found in GP allow the diagnosis of collagen vascular disease highlighting the importance of hydropic degeneration of the basal layer (HDBL; 60%) and thickening of the basal membrane (TBM; 56.7%). The most affected gender was the female (80%) and the highest incidence was found in the age groups 11-20 and 41-50 (bimodal distribution). Conclusions: It is necessary to perform a methodological design of diagnostic test with the objective of demonstrating that the sensibility and specificity is greater in the histopathological study of the GP than in the muscle biopsy, as it is observed in this pilot study. [ABSTRACT FROM AUTHOR]

Biologic therapies targeting tumor necrosis factor (TNF)-alpha have become a mainstay in the management of a number of autoimmune diseases. We report a series of adverse skin eruptions in six patients (four females, two males, age: 21-58 years, mean: 39) receiving 4 months to 10 years (mean 3.1 years) of anti-TNF-alpha therapies (infliximab, n = 4; adalimumab, n = 1 or etanercept, n = 1). The following drug-associated diagnoses were made in eight skin biopsies performed at Massachusetts General Hospital between 3/2007 and 10/2010: pustular folliculitis, psoriasis, interface dermatitis, neutrophilic eccrine hidradenitis, Sweet's syndrome, lupus, vasculitis and palmoplantar pustulosis. The descriptions of neutrophilic eccrine hidradenitis-like and Sweet's-like hypersensitivity eruptions induced by anti-TNF-alpha therapies are the first such cases described in the literature. Each cutaneous eruption improved or resolved with switching to a different TNF-alpha inhibitor, discontinuation of the anti-TNF-alpha agent, and/or topical or systemic steroids. There was a clear chronologic relationship with, and clinical remission upon withdrawal or steroid suppression of the anti-TNF-alpha agents. The mechanism for such diverse cutaneous eruptions among this class of medications remains poorly understood. The cutaneous adverse reaction profile of TNF-alpha inhibitors is broad and should be considered in the histopathologic differential in this clinical setting. Hawryluk EB, Linskey KR, Duncan LM, Nazarian RM. Broad range of adverse cutaneous eruptions in patients on TNF-alpha antagonists. [ABSTRACT FROM AUTHOR]

Introduction: To establish the clinical profile of Herpes Zoster Ophthalmicus (HZO) in individuals less than 30 years of age and to correlate clinical manifestation with their immune status. Materials and methods: A retrospective chart review was performed of patients younger than 30 years of age who presented with HZO from June 2010 to June 2011. Data was collected on their demographics, medical history, clinical presentation, results of serological investigations, and visual outcome. A detailed evaluation of clinical profile with ocular implications and a sequel was done in each case. Results: The mean age of the patients was 23.25 years. Ophthalmic features presented included lid edema, ptosis, keratitis, and superficial punctate keratitis with dry eye, optic neuritis. None of them was found to be HIV positive. Final visual acuity was 20/40 or better in 90% of the subjects. Conclusion: Immunocompetent young adults do present with features of HZO. However, the disease spectrum in HIV-negative patients is localized, less severe, and more amenable to therapy. [ABSTRACT FROM AUTHOR]

Lichenoid tissue reaction or interface dermatitis embrace several clinical conditions, the prototype of which is lichen planus and its variants, drug induced lichenoid dermatitis, special forms of lichenoid dermatitis, lichenoid dermatitis in lupus erythematosus, and miscellaneous disorders showing lichenoid dermatitis, the salient clinical and histological features of which are described to facilitate their diagnosis. Background of lichenoid reaction pattern has been briefly outlined to enlighten those interested in this entity. [ABSTRACT FROM AUTHOR]

Dermatomyositis is a systemic autoimmune disease that primarily affects skeletal muscle, skin, and the lungs. Dermatomyositis is characterized by autoantibodies, tissue inflammation, parenchymal cell damage and death, and vasculopathy. This review focuses on recent advances regarding the role of cytokines and interferon in the pathogenesis of the disease. Evidence for the role of a particular cytokine is based on data showing dysregulated levels in tissue and/or blood; correlation with histopathologic or clinical markers of disease activity; and, rarely, clinical efficacy of targeted cytokine inhibitors. Many of the recent advances pertain to elucidation of the role of interferons in both muscle and skin disease in dermatomyositis. Although a great deal of progress has been made regarding the role of interferon in the disease, many critical questions remain unanswered. [ABSTRACT FROM AUTHOR]