Long-lasting Localized Pemphigus Vulgaris without Detectable Serum Autoantibodies Against Desmoglein 3 and Desmoglein 1

Pemphigus vulgaris (PV) is an autoimmune blistering disease elicited by anti-desmoglein (DsG) 3 antibody. Although skin lesions tend to be distributed over the entire body, in some patients, they are confined to a restricted area. We report two patients who presented with long-lasting localized PV without detectable anti-DsG antibodies after suffering antibody-positive systemic PV. Initial treatment with prednisolone (PSL) was successful in both patients, but a local relapse occurred on the cheek or lower lip after a reduction in the PSL dose. Biopsy of the localized lesions showed suprabasal acantholysis; no serum DsG antibodies were found. Local immunosuppression therapy was effective in both patients. Based on our findings, we suggest that localized PV without detectable antibodies can develop after systemic PV.     

Multiple Familial Trichoepithelioma with Malignant Transformation

Trichoepithelioma (TE) is a benign tumor of follicular origin that presents as small, skin-colored papules predominantly on the face. When more than one family member is affected, the disease is known as multiple familial trichoepithelioma (MFT). It is a rare autosomal dominant (AD) skin disease. Malignant transformation is very rare. We present a case of MFT in a female patient and her father with malignant transformation to basal cell carcinoma (BCC) in the father. We summarized the main histological differential parameters between TE and BCC and applied immunophenotyping for both by administration of Bcl2, CD34, CD10 and androgen receptor (AR) antibodies.     

THYROID DYSFUNCTION AND THYROID ANTIBODIES IN IRANIAN PATIENTS WITH VITILIGO

Vitiligo is a common skin disorder, and the pathogenesis is unknown. An increased prevalence of autoimmune thyroid diseases has been described in these patients. The aim of this study is to assess the prevalence of thyroid dysfunction and hypoparathyroidism in patients with vitiligo.

Materials and Methods:

One hundred and nine patients (38 males and 71 females with vitiligo were enrolled. Thyroid physical examination was carried out. Thyroid function tests, thyroid antibodies, calcium and phosphorus were assessed. The collected data were analysed by SPSS version 11.

Results:

Thyromegaly was found in 30.1% of patients. Hypothyroidism was found in 16 (15.7%) out of 109 cases. Two of them had clinical and 14 had subclinical hypothyroidism. One patient had Grave''s disease. Antibody positivity was the most common disorder (anti-TPO and anti-tg were positive in 36.7 and 32.1%, respectively). No patient had hypoparathyroidism.

Conclusion:

According to our study, thyroid dysfunction, particulary hypothyroidism and thyroid antibodies increase in patients with vitiligo. We recommend thyroid antibodies assessment and thyroid function evaluation in these patients.     

Generation and characterization of chicken egg yolk antibodies against propionibacterium acnes for the prevention of acne vulgaris

Introduction:

Antigen-specific antibody has been widely used for immunological analysis in the field of diagnosis as well as in pure scientific research, where the IgY antibodies can be raised against P acnes antigen.

Material and Methods:

To produce IgY against Propionibacterium acnes, laying hens were immunized with P acnes (MTCC No: 1951) and subsequent booster injections were given. The antibodies produced were purified from the egg yolk of immunized chicken using the polyethylene glycol and ammonium sulfate precipitation method and, further, by Diethylaminoethyl (DEAE) cellulose ion-exchange column chromatography. The protein fraction of IgY was isolated from the egg yolk. The separation was rapid, and the success of each step was viewed on Sodium Dodecyl Sulphate-polyacrylamide gel electrophoresis (SDS-PAGE). The reactivity of anti–P acnes was evaluated by the Enzyme-linked immunosorbent assay (ELISA) test and the dot-immunoassay.

Results:

With ELISA, the highest titter of 1:10000 was observed on the 150^th day after vaccination. The results of dot-immunoassay suggested that anti–P acnes IgY developed a brown color as positive reaction, which showed the antigen-antibody binding even after a maximum dilution of 1/500. These results suggest that anti-acne IgY was produced and had strong specific antibody reactivity.

Conclusion:

The findings indicate that anti-acne IgY is worth utilizing as a preventive agent for acne vulgaris.     

ANTI-IDIOTYPE ANTIBODIES IN IMMUNE REGULATION OF ANCA ASSOCIATED VASCULITIS

Background:

Anti-idiotype antibodies (anti-ids) have a potential role in the immunomodulation of various autoimmune disorders. The immunoregulatory role of anti-idiotypic antibodies in ANCA-associated vasculitis needs to be studied. This study was conducted in clinically and histopathologically diagnosed ANCA-associated vasculitis (AAV) patients.

Methods:

Anti-ids were tested in 100 AAV patients of which 80 had anti-MPO and 20 had anti-PR3 antibodies at various stages of disease over a period of 2-3 years. The disease activity was estimated by the Birmingham vasculitis activity score (BVAS). The affinity-purified ANCA F(ab'')2 fragments were prepared using three each of anti-MPO and anti-PR3 high titer sera and were used as idiotype coats for anti-idiotype antibody detection by ELISA. Positivity was confirmed by fluid phase inhibition ELISA.

Results:

Patients who went into remission showed 53.8% anti-ids to anti-MPO and 52.9% to anti-PR3 with low BVAS values (0-8), whereas in patients with active disease, only 12.5% had anti-ids to anti-MPO and 10% had anti-ids to anti-PR3 with comparatively high BVAS (18-32), while five cases who had relapse (BVAS 18-20) did not have anti-ids to anti-MPO or anti-PR3. An inverse correlation was noted between ANCA and anti-ids (r = −0.901).

Conclusions:

High prevalence of anti-ids in remission cases and low prevalence in active cases with absence of anti-ids in relapse cases as well as an inverse correlation of ANCA and anti-ids indicate its beneficial effect on the disease process, thus suggesting the dynamic role of anti-idiotype networks in the immunoregulation of AAV.     

Pemphigus Foliaceus Masquerading as IgA Pemphigus and Responding to Dapsone

A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology of skin lesions and direct immunofluorescence were characteristic of pemphigus foliaceus. He was treated with dexamethasone pulse therapy with inadequate response. However, relapsing skin lesions revealed a circinate arrangement with a predilection to trunk and flexures. In view of clinical features suggestive of IgA pemphigus, he was started on dapsone, to which he responded dramatically in four weeks. However, repeat biopsy continued to reveal features of pemphigus foliaceus and ELISA for anti-desmoglein 1 antibodies was positive.     

Apoptosis and Clinical Severity in Patients with Psoriasis and HCV Infection

Background:

It has been proposed that hepatitis C virus (HCV) antigens are involved in the pathogenesis of psoriasis and may contribute to severity of the disease. Increased expression of the apoptosis-regulating proteins p53 and tTG and decreased levels of bcl-2 in the keratinocytes of the skin of psoriatic patients have been reported.

Aim:

This study aims to identify the serum levels of apoptosis-regulating proteins in patients with psoriasis and without HCV infection and to study the relation between clinical severity of psoriasis and the presence of HCV infection.

Materials and Methods:

Disease severity was assessed by psoriasis area severity index score (PASI) of 90 patients with psoriasis grouped as mild (n = 30), moderate (n = 30) and severe (n = 30); 20 healthy individuals were used as controls. All groups were subjected for complete history taking, clinical examination, and tests for liver function and HCV infection. The serum levels of apoptosis related proteins: p53, tTG and bcl-2 were estimated by enzyme linked immune sorbent assay (ELISA).

Results:

There was a statistically significant (P < 0.001) correlation between clinical severity of psoriasis and presence of HCV antibodies and HCV-mRNA. In addition, significantly (P < 0.001) raised serum p53 and tTG, and reduced bcl-2 were observed among HCV-positive patients as compared to HCV-negative patients and control patients.

Conclusion:

These results conclude that clinical severity of psoriasis is affected by the presence of HCV antibodies and overexpression of apoptotic related proteins. In addition, altered serum levels of apoptosis-regulating proteins could be useful prognostic markers and therapeutic targets of psoriatic disease.     

Scle-lp overlap syndrome

A 35-year-old female presented with scaly annular and pigmented lesions all over the body of 2 years duration. Her clinical features, histopathology, immunofluorescence findings and positive ANA and anti-Ro antibodies were suggestive of subacute cutaneous lupus erythematosus-lichen planus. We report this case because of its clinical rarity.     

Role of Osteopontin in Psoriasis: An Immunohistochemical Study

Background:Osteopontin (OPN) has been postulated to have a role in several T-helper (Th) 1 and Th 17-mediated diseases including psoriasis (PS), through multiple mechanisms sharing in the onset and worsening of PS, OPN shares in induction of keratinocyte proliferation through inhibiting keratinocyte apoptosis, OPN acts as a proinflammatory agent that participates in the upregulation of Th cell lineages, among which are the Th 1 and Th 17 cells.Results:Our results showed statistically significant differences in the expression of OPN, between lesional and nonlesional skin as well as between nonlesional skin and control group (P ≤ 0.001). In addition, there was a significant difference in the expression of OPN, between control and lesional group.Conclusions:OPN involvement in PS enlarges the list of cytokines able to stimulate the inflammatory response in this disease, anti-OPN antibodies, may eventually become a useful therapeutic approach in PS.     

A Clinicopathological Study of Pemphigus in Eastern India with Special Reference to Direct Immunofluorescence

Background:Pemphigus is a group of chronic autoimmune vesico-bullous disorders in which the epidermis and the basement membrane zone are the focus of attack resulting in cutaneous and mucosal blister formation. Direct immunofluorescence (DIF) test is a very sensitive test for the diagnosisAim:To study the clinico histopathological patterns of pemphigus in eastern India. The study also aims to correlate DIF with clinical and histologic findings as well as severity of skin involvement [scoring systems].Results:In our study Pemphigus vulgaris (PV) was the predominant type with 32 cases followed by 8 cases of pemphigus foliaceus (PF) and a single case of IgA pemphigus. Mean age at presentation was late middle age. Majority of the patients, 26 (63.41%) initially had cutaneous involvement followed by mucosal involvement. In this study group 36 (87.80%) patients showed acantholytic cells on histopathological examination. Most patients of PV showed suprabasal blister 20 (62.50%) followed by intraspinous 5 (15.62%) and subcorneal 5 (15.62%) blister. In majority 28 (87.50%) of the PV patients IgG and C3 antibodies were deposited throughout the epidermis. The strength of antibody positivity was strong in most of the patients (71.87%). In cases of PF mostly IgG 6 (75%) antibodies were deposited in the upper epidermis. DIF intensity had poor correlation with disease activity/severity except in PF.Conclusion:Almost 85.36% cases of pemphigus were diagnosed clinicopathologically. But 6 cases couldn’t be diagnosed accurately on clinicopathological basis and in them DIF was confirmatory. Two cases of pure mucosal PV and 1 case of IgA pemphigus was confirmed by DIF. Two cases of bullous pemphigoid clinico-histologically mimicking PV were also excluded by DIF. So it appears from our study that DIF is confirmatory for diagnosis of pemphigus in all cases.     

Disseminated Cutaneous Histoplasmosis,an Initial Manifestation of HIV,Diagnosed with Fine Needle Aspiration Cytology

Acute progressive disseminated histoplasmosis (PDH) may be the initial manifestation of human immunodeficiency virus (HIV). However, cutaneous involvement is very rare. We present an unusual case of acute PDH with multiple diffuse cutaneous papulonodular lesions predominantly on the face, trunk, and upper extremities, diagnosed initially with fine needle aspiration cytology (FNAC). Subsequent serological tests revealed positivity for antibodies for HIV 1 and 2. The cytomorphological features were further confirmed by biopsy and histochemical stains. FNAC is a rapid, cost-effective tool that may be utilized in the diagnosis of papulonodular forms of PDH and for initiating prompt therapy. We discuss the clinical features, clinical differentials, and treatment of the condition.     

Childhood Epidermolysis Bullosa Acquisita: Confirmation of Diagnosis by Skin Deficient in Type VII Collagen,Enzyme-linked Immunosorbent Assay,and Immunoblotting

Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disorder characterized by autoantibodies against Type VII collagen. It usually affects adults; childhood EBA is rare. We describe a 10-year-old girl presenting with recurrent tense blisters predominantly on legs, dorsa of hands and feet accompanied by oral erosions since the age of 5 years. Direct immunofluorescence (IF) microscopy showed linear deposition of IgG and C3 along the basement membrane zone (BMZ); indirect IF microscopy on salt-split skin revealed staining of IgG to the dermal side of the split. The patient''s serum did not show BMZ staining in recessive dystrophic epidermolysis bullosa skin deficient for Type VII collagen, thus confirming autoantibody reactivity against Type VII collagen. Circulating antibodies against the immunodominant noncollagenous 1 domain of Type VII collagen were detected by ELISA and immunoblotting studies. The patient was treated with oral corticosteroids and dapsone with good improvement.     

DETECTION OF R576 INTERLEUKIN-4 RECEPTOR AN ALLELE GENE, SERUM INTERLEUKIN-4, AND EOSINOPHILIC CATIONIC PROTEIN IN ATOPIC DERMATITIS PATIENTS

Atopic dermatitis (AD) is a chronic pruritic skin disease. It results from a complex interplay between strong genetic and environmental factors. The aim of this work was to study some biochemical markers of the dermatosis. This included detection of R576 interleukin-4 receptor alpha allele gene. Twenty five patients with AD and 25 controls participated in this study.     

A Cross-sectional Study of Direct Immunofluorescence in the Diagnosis of Immunobullous Dermatoses

Background:

Autoimmune blistering diseases are a group of bullous disorders characterized by pathogenic antibodies directed at the target antigens, which are components of the desmosomes or adhesion complex at the dermoepidermal junction. Direct immunofluorescence (DIF) is invaluable in the diagnosis of these lesions.

Aim:

The aim of this study was to evaluate the sensitivity of DIF in immunobullous dermatoses and to study the pattern of DIF. The study also aims to correlate DIF with clinical and histologic findings and to analyze discrepancies.

Materials and Methods:

Total 100 biopsies received over a period of 2 years in the Department of Pathology were analyzed. DIF, histopathology and clinical data were reviewed.

Results:

Out of 100, 89 cases showed DIF patterns concordant with clinical/histologic diagnosis. The sensitivity of DIF was 94.44% (51/58) in the pemphigus and 84% (21/25) in the bullous pemphigoid (BP) group, 100% each in dermatitis herpetiformis (2/2) and linear IgA disease (1/1). A total of 11 histologically proven cases of immunobullous lesions were DIF negative-four (three of pemphigus vulgaris and one of BP) due to having no epidermis, three (cases of BP) owing to sampling/technical errors and the remaining four (cases of pemphigus vulgaris) due to being on treatment.

Conclusion:

Immunofluorescence helps confirm the diagnosis of bullous lesions in which there is clinical and the histopathologic overlap. Sampling errors contributed to false negative (FN) results.     

Epidermolysis bullosa acquisita with moderately severe Dysphagia due to esophageal strictures

Epidermolysis bullosa acquisita (EBA) is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient's disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig) will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.     

Radiation Port Cutaneous Metastases: Reports of Two Patients Whose Recurrent Visceral Cancers Presented as Skin Lesions at the Site of Previous Radiation and Literature Review

Radiation therapy is associated with a variety of complications, including the development of primary skin cancers in the radiated region. However, it is rare for patients with visceral cancers who are treated with radiation therapy to subsequently develop cutaneous metastasis within the radiation port. We describe two patients with internal malignancies who developed cutaneous metastases within their radiation ports following radiotherapy. In addition, we used PubMed to perform an extensive literature review and identify additional reports of cutaneous metastasis within a radiation port. We excluded patients who developed melanoma or primary skin cancers in the radiation port. We also excluded patients with non-solid organ malignancies. Herein, we summarize the characteristics of 23 additional patients who experienced radiation port cutaneous metastases and explore possible mechanisms for the occurrence of radiation port cutaneous metastases.     

A Report of 10 Individuals with Weathering Nodules and Review of the Literature

Weathering nodules are a benign skin condition that usually present as papules on the helices of patients with significant prior sun exposure. They are easily recognized clinically and blanch upon application of pressure to the adjacent helical rim: a positive blanch sign. We describe the clinical presentation of weathering nodules in 10 patients, nine men and one woman, aging from 38 to 70 (median 59), and their associated risk factors. Eight patients had a history of actinic keratosis, three had a history of nonmelanoma skin cancer, and all patients had increased Sun exposure through outdoor activities. Weathering nodules are rarely mentioned in the literature and may be confused with other cutaneous disorders. Therefore, it is paramount for clinicians to become familiar with weathering nodules and include them in the differential diagnosis of ear nodules. Appropriate diagnosis will help avoid unnecessary biopsies while reassuring the patient that the lesions are benign.     

Simulation-Based Cutaneous Surgical-Skill Training on a Chicken-Skin Bench Model in a Medical Undergraduate Program

Background:

Because of ethical and medico-legal aspects involved in the training of cutaneous surgical skills on living patients, human cadavers and living animals, it is necessary the search for alternative and effective forms of training simulation.

Aims:

To propose and describe an alternative methodology for teaching and learning the principles of cutaneous surgery in a medical undergraduate program by using a chicken-skin bench model.

Materials and Methods:

One instructor for every four students, teaching materials on cutaneous surgical skills, chicken trunks, wings, or thighs, a rigid platform support, needled threads, needle holders, surgical blades with scalpel handles, rat-tooth tweezers, scissors, and marking pens were necessary for training simulation.

Results:

A proposal for simulation-based training on incision, suture, biopsy, and on reconstruction techniques using a chicken-skin bench model distributed in several sessions and with increasing levels of difficultywas structured. Both feedback and objective evaluations always directed to individual students were also outlined.

Conclusion:

The teaching of a methodology for the principles of cutaneous surgery using a chicken-skin bench model versatile, portable, easy to assemble, and inexpensive is an alternative and complementary option to the armamentarium of methods based on other bench models described.