Category Archives: Around the Globe

To evaluate the significance of the association of malignancy with autoimmune blistering diseases, we studied the incidence of internal malignancies in pemphigus and bullous pemphigoid based upon 496 cases of pemphigus and 1113 cases of bullous pemphigoid in Japan. Results showed that (1) an association between internal malignancies and pemphigus was observed in 25 out of 496 cases (5.0%), while that with bullous pemphigoid was seen in 64 out of 1113 cases (5.8%). Such association ratios were significantly higher than that of the controls aged over 70 years old (0.61%); (2) The average ages of pemphigus/bullous pemphigoid with malignancy were 64.7 and 69.2 years, respectively. The association ratio of malignancy with pemphigus increased by age, while that with pemphigoid was not correlated with aging;

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(3) Lung cancer was most common in pemphigus and gastric cancer in bullous pemphigoid; (4) There were no significant differences in the titers of circulating antibody, the presence or extent of mucous involvement or annular erythema between bullous pemphigoid patients with malignancy and without malignancy. Our results indicated that detailed examination for internal malignancy is essential for those patients with pemphigus or bullous pemphigoid.

Abstract from: http://www.ncbi.nlm.nih.gov/pubmed/7772576

In BP lesional skin, immunohistochemistry and confocal microscopy were performed for CD4+, CD25+, forkhead/winged helix transcription factor (FOXP3)+, transforming growth factor (TGF)-β+ and interleukin (IL)-10+ cells. In addition, the number of CD4+CD25++FOXP3+ Tregs in peripheral blood was assessed by flow cytometry, and the levels of TGF-β and IL-10 were determined in serum samples by enzyme-linked immunosorbent assay before and after steroid therapy. Controls included patients with psoriasis, atopic dermatitis (AD) and healthy donors.

The frequency of FOXP3+ cells was significantly reduced in skin lesions from patients with BP (P < 0.001) compared with psoriasis and AD. Moreover, the number of IL-10+ cells was lower in BP than in psoriasis (P < 0.001) and AD (P = 0.002), while no differences were observed in the number of TGF-β+ cells. CD4+CD25++FOXP3+

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Treg in the peripheral blood of patients with BP was significantly reduced compared with healthy controls (P < 0.001), and augmented significantly after steroid therapy (P = 0.001). Finally, TGF-β and IL-10 serum levels were similar in patients with BP compared with healthy controls. However, after therapy, BP patients showed significantly higher IL-10 serum levels than before therapy (P = 0.01).

Full article available at: http://onlinelibrary.wiley.com/doi/10.1111/jdv.12091/abstract;jsessionid=C37D521517222D9766F5D0D339765626.d04t01?deniedAccessCustomisedMessage=&userIsAuthenticated=false

Antiga, E., Quaglino, P., Volpi, W., Pierini, I., Del Bianco, E., Bianchi, B., Novelli, M., Savoia, P., Bernengo, M.G., Fabbri, P. and Caproni, M. (2013), Regulatory T cells in skin lesions and blood of patients with bullous pemphigoid. Journal of the European Academy of Dermatology and Venereology. doi: 10.1111/jdv.12091

“I have been in remission now for over 1 year (YES!!!). That is complete remission with no symtpoms and no meds. I was diagnosed in 2004 and rode the roller coaster of varying doses of prednisone coupled with Cellcept at first and then Imuran. After many ups and downs, 4 years of meds, and a very slow taper of prednisone, I was able to go off of all meds and have been doing great for the past year. My treatment was with Dr. Anhalt, who I am extremely grateful to for his knowledge and compassion.

I have never done IVIG or Rituxan although these therapies were discussed as options had I not finally started doing well on the meds I was on. I do have to say, however, that I have never taken zinc or niacinamide, so I cannot owe my remission to that.

I think that it is wonderful that we have alternative therapies such as Rituxan and IVIG. My understanding is that most of the time, these treatments are used for those patients who do not respond well to traditional therapies. I know there are people in {the discussion)} group who have had tough battles and have not responded well to prednisone and immunosuppressive therapies. I think that is when these other treatments come into play.

I think using supplements can be very wonderful and if it works for some, then that is great. I don’t think, however, it’s always enough for everyone. And, I think with this disease, the treatment and journey is different for us all.”

More stories available at: http://www.pemphinremission.com/

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

 A small, but perfectly formed group of PEM Friends braved the floods  in the UK on Monday 26th November 2012 to have a sociable lunch together and share their experiences of Pemphigus and Pemphigoid. Even though it was a low turnout of  just seven patients, there was a wide diversity of the diseases represented and a great deal of empathy and common interest.

People came from as far away as Leicestershire and Suffolk.   We met in the self-service restaurant of Peter Jones in Sloane Square, London.

We were joined by Dr. Jane Setterfield, a Consultant Dermatologist with a great wealth of knowledge in the field of blistering diseases, particularly when there is oral involvement. Dr. Setterfield was able to answer our many questions and suggest possible actions for those attending.

Some of the particularly helpful sources of further information that Dr. Setterfield guided the group to were:-

http://www.dermnetnz.org/

http://emedicine.medscape.com/dermatology

http://www.bad.org.uk/site/1/default.aspx

It was a very enjoyable and useful event.

 Pemphigus can be a tough disease to live with but many patients now in remission have found inspiration, this is Sally’s story:

I have been in remission now for over 1 year (YES!!!). That is complete remission with no symptoms and no meds. I was diagnosed in 2004 and rode the roller coaster of varying doses of prednisone coupled with Cellcept at first and then Imuran. After many ups and downs, 4 years of meds, and a very slow taper of prednisone, I was able to go off of all meds and have been doing great for the past year. My treatment was with Dr. Anhalt, who I am extremely grateful to for his knowledge and compassion.

I have never done IVIG or Rituxan although these therapies were discussed as options had I not finally started doing well on the meds I was on. I do have to say, however, that I have never taken zinc or niacinamide, so I cannot owe my remission to that.

I think that it is wonderful that we have alternative therapies such as Rituxan and IVIG. My understanding is that most of the time, these treatments are used for those patients who do not respond well to traditional therapies. I know there are people in {the discussion)} group who have had tough battles and have not responded well to prednisone and immunosuppressive therapies. I think that is when these other treatments come into play.

I think using supplements can be very wonderful and if it works for some, then that is great. I don’t think, however, it’s always enough for everyone. And, I think with this disease, the treatment and journey is different for us all.

Story found at: http://pemphinremission.com/

 

We report on a patient with longstanding multicentric Castleman’s disease, hyaline-vascular type, who developed a follicular dendritic cell sarcoma, and finally presented a fatal paraneoplastic pemphigus. We review all four cases of such a triple association described in the literature so far.

Full article available here: http://link.springer.com/article/10.2478%2Fs11536-012-0097-6

Experience in the Texas Department of Criminal Justice Dermatology Clinic

In this article, the authors review the skin conditions seen in a dermatology referral clinic for inmates in the Texas Department of Criminal Justice system at the University of Texas Medical Branch. A database search of dermatology visits over a 34-month period yielded 3,326 adult outpatient encounters for analysis. Psoriasis, actinic keratoses, and hair diseases were the most commonly encountered diagnoses. Dermatophytes were the most common infection, keloids the most common benign tumor, and pemphigus the most common autoimmune disease.

Full article available at: http://jcx.sagepub.com/content/18/4/302.abstract?rss=1