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Human epidermis shows a non-neuronal cholinergic system including keratinocyte (kc) acetylcholine (Ach) axis which is composed by enzymes and two families of Ach receptors (muscarinic and nicotinic receptors). The activity of these two receptors can regulate the interkeratinocytes and kcs-extracellular matrix adhesion modifying the regulation of intercellular adhesion molecules like cadherins and integrins. Some authors demonstrate that acantholysis in pemphigus depends not only on anti desmogleins antibodies (abs) (mostly IgG) but even on other abs directed against kc membrane antigens (e.g. anti Ach receptors Abs). In the early phase of pemphigus pathogenesis, anti Ach receptors Abs block Ach signaling essential for cell shape and intercellular adhesion and increase the phosphorylation of adhesion molecules. Combined with the action of abs antidesmogleins, anti Ach receptors Abs cause the acantholytic phenomenon. In vitro experiments show that high doses of Ach in acantholytic kcs can rapidly reverse this pathologic event. In vivo experiments using neonatal mice model of Pemphigus have demonstrated that cholinergic agonists reduce these lesions. Therapy with pyridostigmine bromide and Nicotinamide per os or pilocarpine used topically, drugs that present cholinomimetic effects, has

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lead to encouraging results in patients affected by Pemphigus disease. Cholinergic agents could have a strategic role in the therapy of pemphigus since they could be responsible for the early stage of acantholytic diseases.

Full article available at: http://www.ingentaconnect.com/content/ben/aiaamc/2012/00000011/00000003/art00008

Although bacterial pyoderma is among the most commonly encountered dermatologic conditions in dogs, some cases present diagnostic challenges even to experienced clinicians. This article presents several unusual manifestations of pyoderma, including bullous impetigo, superficial spreading pyoderma, mucocutaneous pyoderma, and post-grooming furunculosis. Conditions mimicking pyoderma, including juvenile cellulitis, immunomodulatory-responsive lymphocytic-plasmacytic pododermatitis, and pemphigus foliaceus are also described. Diagnostic techniques used for diagnosing and characterizing pyoderma are also discussed.

Full article available at: http://www.vetsmall.theclinics.com/article/PIIS019556161200143X/abstract?rss=yes

Pemphigus vulgaris (PV) is an autoimmune mucocutaneous disease presenting clinically with blisters or erosions of the skin and mucous membrane. The main histopathologic characteristic of this disease is suprabasal vesicles due to loss of cell–cell adhesion between keratinocytes named acantholysis. Studies have shown that apoptosis is increased in PV. The purpose of this study is to investigate the role of apoptosis in blister formation in PV.

Methods

This cross-sectional study was conducted on 25 specimens of oral PV. The presence of apoptosis was evaluated using the TUNEL technique in the normal perilesional region, vesicle area, and acantholytic cells. Also, the expression of Bax pro-apoptotic marker was assessed by the biotin–streptavidin immunohistochemical method. SPSS software was used for Wilcoxon test analysis. P values <0.05 were considered significant.

Results

The percentage and intensity staining of TUNEL-positive cells were noteworthy. There were statistically significant differences between basal and parabasal) = 0.05 (, tombstone with vesicle roof (= 0.038) and basal with tombstone (= 0.038). However, the expression and staining intensity of pro-apoptotic marker Bax were weak, and no statistically significant differences were observed between the various areas.

Conclusion

The results obtained in the present study suggest that the process of apoptosis occurs early in PV because it was observed in the perilesional normal appearing tissue. Also, the process of apoptosis may cause exacerbation or speeding of the bulla formation. In other words, inhibition of apoptosis in the patients could reduce the severity of the lesions.

Full article available here: http://www.medworm.com/index.php?rid=6781830&cid=c_297_32_f&fid=28436&url=http%3A%2F%2Fonlinelibrary.wiley.com%2Fresolve%2Fdoi%3FDOI%3D10.1111%252Fjop.12022

Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rare disorder characterized by mucocutaneous involvement and associated with inflammatory bowel disease. A 42-year-old woman with ulcerative colitis who manifested verrucous and pyogenic lesions on her scalp, neck, axillae, inguinal areas, umbilicus, trunk and oral cavity for about 11 months is described. She also experienced general fatigue and swelling in her lower extremities. Histology revealed eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia, but she was negative on direct immunofluorescence for IgA, IgG and C3. She was diagnosed with PD-PSV and treated with infusions of 20% human albumin (100 mL) for 5 days, followed by methylprednisolone (40 mg/d), with remission of lesions observed after 1 month. The differential diagnosis of PD-PSV and pemphigus vegetans is discussed.

Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23138121?dopt=Abstract

Introduction: Though oral aphthosis is common, it has a significant impact on the quality of life in the patients. It is the most common oral ulcerative condition encountered in clinical practice. This study describes the characteristics and patterns of oral aphthosis seen at a tertiary dermatological centre in Singapore, with emphasis in evaluating the management gaps and in identifying underlying systemic diseases and nutritional deficiencies. Materials and Methods: This is a retrospective review of medical records over a 10-year period between June 2000 and June 2010. Two hundred and thirteen patients were identified using the search terms ‘oral ulcers’, ‘aphthous ulcers’, ‘oral aphthosis’, and ‘Behcet’s disease’. Patients with Behcet’s disease without oral ulcers and other diagnoses such as pemphigus vulgaris, lichen planus and herpes simplex were

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excluded. The remaining patients were evaluated with regard to demographic characteristics, characteristics of oral ulcers, associated connective tissue disorders and nutritional deficiencies, diagnostic tests results, treatment response as well as follow-up duration. Results: One hundred and seventy-five patients were included in this study. One hundred and one patients had recurrent oral aphthosis, with 77 having simple aphthosis and 24 having complex aphthosis. Fourteen patients (8%) fulfilled the International Study Criteria (ISG) for Behcet’s disease, of which, 85.71% had complex aphthosis. The therapeutic ladder for such patients ranged from topical steroids and colchicine through to oral corticosteroids and/or dapsone therapy. Conclusion: Recurrent oral aphthosis is a niche condition in which dermatologists are well-poised to manage. This study demonstrates that a more definitive management and therapeutic algorithm for oral aphthosis are needed for better management patients in the future. In particular, complex aphthosis needs to be monitored for progression onto Behcet’s disease.

From: http://www.ncbi.nlm.nih.gov/pubmed/23138144?dopt=Abstract

Pemphigus is a rare vesiculobullous autoimmune disease that exhibits blistering of the skin and oral cavity. It is caused by autoantibodies directed against antigens on the surface of keratinocytes. All forms of pemphigus are associated with the presence of circulating and skin-fixed autoantibodies. Pemphigus vegetans is a rare clinical variant of pemphigus vulgaris and comprises up to 5 percent of all pemphigus cases. In the following we present the oral presentation of pemphigus vegetans. We describe a 33-year-old man who was referred to our clinic complaining about mouth sores, tooth pain, and multiple pustules. During clinical exam we were able to recognize multiple pustules, ulcerated areas on the gingiva, and whitish mucosal plaques. Clinical, histopathological, and direct immunofluorescence findings were compatible with pemphigus vegetans.

Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23122017?dopt=Abstract

Context.-Pemphigus is a group of autoimmune vesiculobullous diseases characterized by immunoglobulin G (IgG) antibodies directed against desmosomal adhesion proteins, with IgG4 being the predominant subclass in active diseases. Direct immunofluorescence for IgG performed on fresh-frozen tissue plays a crucial role in diagnosing pemphigus. However, the diagnosis might be hindered when frozen tissue is not available. Objective.-To evaluate the usefulness of immunohistochemistry for IgG4 performed on paraffin sections as a diagnostic test for pemphigus. Design.-Eighteen immunofluorescence-proven pemphigus cases (12 pemphigus vulgaris, 6 pemphigus foliaceus) were studied. Four normal skin specimens and 32 nonpemphigus vesiculobullous disease specimens served as controls. Paraffin sections of all cases were examined immunohistochemically for IgG4 expression. Positivity was defined as distinct, condensed, continuous immunoreactivity localized to the intercellular junctions of keratinocytes. Results.-The immunostains were independently evaluated in a masked manner by 3 pathologists, with a 100% interobserver agreement. Nine of 12 pemphigus vulgaris cases (sensitivity 75.0%), and 4 of 6 pemphigus foliaceus cases (sensitivity 66.7%), were positive for IgG4 immunostain. The overall sensitivity was 72.2%. One control specimen (bullous pemphigoid) showed IgG4 positivity (specificity 97.2%). In specimens demonstrating acantholysis, 8 of 10 pemphigus vulgaris cases (sensitivity 80.0%) and 4 of 4 pemphigus foliaceus cases (sensitivity 100.0%) were positive for IgG4. The overall sensitivity for specimens with acantholytic lesions was 85.7%. Conclusion.-Immunohistochemistry for IgG4 provides a reasonably sensitive and highly specific test for diagnosing pemphigus, especially when frozen tissue is not available, and active acantholytic lesions are examined.

Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23106586?dopt=Abstract

BACKGROUND:

Rosacea is a common dermatosis that can involve the bald area of the scalp. We report the case of a man presenting clinical symptoms of rosacea of the forehead and the scalp, but with a histological picture of familial chronic benign pemphigus.

PATIENTS AND METHODS:

A 47-year-old man with a history of Hailey-Hailey disease had been presenting facial dermatosis for 5 years. The clinical features were erythema with pustules and scales located on the mid-forehead and the androgenic bald area of the frontal scalp. The histological aspect of the skin biopsy showed suprabasilar clefting and ancantholysis at all levels of the epidermis and sparse perivascular infiltrate. Direct immunofluorescence was negative. These findings were typical of Hailey-Hailey disease. Based on clinical findings, and without taking account of the skin biopsy results, treatment with doxycycline and a topical antifungal was administered for 3 months, leading to remission of symptoms.

DISCUSSION:

The site of rosacea on the bald area of the scalp in males is described in the literature, and when present, is probably enhanced by exposure to UV radiation. In this patient, the histological features were interpreted as histopathologically equivalent to Köbner phenomenon.

full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23122375?dopt=Abstract

The clinical and epidemiological features of pemphigus vulgaris (PV) are well documented but there remain few reports of oesophageal involvement of PV. Although previously considered to be rare, recent reports have suggested that up to 87% of patients with PV may have symptoms, or endoscopic features, of oesophageal disease that may be poorly responsive to conventional corticosteroid-sparing immunosuppression.

The present report details the clinical and immunological features of a 53 year old Asian female who developed symptoms and signs of oesophageal PV during therapy with azathioprine and decreasing prednisolone dosage. Oesophageal involvement occurred during stable oral disease.

Oesophageal involvement can occur without significant oro-cutaneous lesions and immunological evidence of PV. This suggests that immunological targets for oesophageal disease may differ from those of other mucocutaneous areas, and that conventional first-line systemic therapy may not be effective for oesophageal lesions.

Full article available at: http://www.ingentaconnect.com/content/ubpl/wlmj/2012/00000004/00000002/art00001

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.