Yearly Archives: 2013

Summer has always meant a lot to me. When I a kid I played Little League baseball on the southern shores of Lake Michigan. One year, our team was a solid 0-10 (no wins, 10 losses). It was July 1 and it was my birthday. I was officially 11 and we were about to officially be 0-11. We were terrible!

My good friend, Marty, was pitching that day, and things started looking up. As we entered the final inning, we were winning, but we were three outs away from victory. I will spare you the Hollywood details: We won in “Bad News Bears” fashion…a little trickery and a lot of luck. We still were the worst team in the league, but that day we were winners. I happily celebrated my 11th birthday triumphant with my friends and family.
Recently I spent a weekend with friends and family at the 16th Annual Patient Conference in San Francisco. I have to thank our hosts, Dr. Peter Marinkovich and Bay Area Support Group Leader Prem Jain for having us in their home town. Other Conference Committee members included Dr. Terry Wolinsky McDonald (co-Chair/IPPF BOD), Greg Wright (IPPF BOD), Dr. Razzaque Ahmed (2012 Annual Meeting host), Nancy Stoeckel (KabaFusion and pemphigoid caregiver), Sonia Tramel (former IPPF BOD member), and Marc Yale (Peer Health Coach). The planning started in July 2012 and ended the day before the meeting started. This year’s event was a success for many reasons, but these people made it all happen. Thank you.

If you didn’t get a chance to join us or if you did and just want a refresher, the audio has been transcribed for your reading pleasure! And PHC Jack Sherman is producing segments that will have the slides and audio combined – and be available as a DVD! You can find the 2013 Patient Conference materials online at www.pemphigus.org

This also is the first newsletter being done by someone other than me since #39 back in 2004. Thank you to Maeve Norton for her layout and graphic design skills. We look forward to this and future issues. Volunteerism is becoming more and more important in our operations as we remain lean, but still provide high-level programs and roll out new projects to benefit our community.
In celebrating our P/P family and friends, we extend our congratulations to Toby Speed who was married in May, and honor the memory of Bob Stillman who passed away in April. We are a small community – a family – and events like these affect all of us. We continue to share in the joys and sorrows of life together as one.
I hope you enjoy the Summer ahead with your family and friends. Remember, we’re here for you!

For many of us in the United States, the start of September means welcoming a new school year for our kids and grandkids and saying goodbye to summer days. For others, the start of September is actually the start of a new year. Either way, September can mean a new challenge and a fresh start. This is a good time for engaging with the IPPF and creating a positive fall experience! Volunteerism is a great way to boost your mood, and to help you feel connected and grounded.

Research on volunteerism shows volunteers enjoy better overall health, a sense of achievement, greater life satisfaction, larger social networks, and higher self-esteem (Barlow, 2005). In fact, volunteering is so successful at helping the volunteers themselves that current studies are now specifically looking at volunteering as a means of coping and success in the context of chronic disease management. Some studies show a “pronounced improvement on confidence, self-awareness, self-esteem, depression and role functioning” and reveal that participants enjoy “dramatic change in their lives in terms of how they thought of themselves and in how they related to others.” So all of this begs the question:

How might YOUR disease path change if you were more connected to the mission of helping others and improving outcomes for the IPPF community as a whole?

You probably don’t need a ton of convincing. For you, it may more be a matter of finding the time and the right opportunity to use your skill set. Trust us. . . you have skills you can share with the IPPF and put to good use. And there are few organizations out there which could use that energy more than we can! Volunteering for you might be as simple as giving literature to area dermatologists and dentists or trying to talk with them for a few minutes about the disease. It might be doing a “low stress” fundraiser.

Or, maybe you are up for a bigger challenge in response to a need you see–like the need to start a local area support group or become a patient speaker to educate young doctors in training. No matter what your personal goals or strengths, you have something to give. So, as you ease into the new year or just prepare for Fall, remember that volunteering gives you the opportunity to:

S hare Strengths
E mpower Yourself
P romote Positivity
T each Tolerance
E ngage Others
M eet Challenges
B e the Change
E xpress Yourself
R each Out

If you are interested in getting started, visit pemphig.us/volunteerIPPF for some ideas. You can also contact Will Zrnchik by emailing him at will@pemphigus.org, calling (855) 4PEMPHIGUS (855.473-6744), or talking with your Peer Health Coach about volunteering!

 

Background: Pemphigus vulgaris was almost fatal before the advent of glucocorticoids. Unfortunately, the high doses and prolonged administration of glucocorticoids, which often needed to control the disease, result in numerous adverse effects many of which are serious.

Aims: To evaluate the patients with pemphigus vulgaris on treatment in respect of osteoporosis and to compare the frequency of osteoporosis in these patients with the healthy ones.

Methods: The study consisted of 40 patients with pemphigus vulgaris and 34 healthy controls. Bone mineral density measurements were obtained by dual- energy X-ray absorptiometry. Blood serum, bone parameters, and biochemical hormonal measurements were examined in both groups.

Results: When the bone mineral density values of patients with pemphigus vulgaris were compared with those of the control group, there was no significant difference between hip bone mineral density values, while lumbar region T and Z scores were found significantly low in the patient group (p = 0.034 and p = 0.006, respectively). Osteoporosis, osteopenia, and normal dual-energy X-ray absorptiometry rates in the patient group were found to be 32.5%, 32.5%, and 35%, respectively. These rates were found to be 18%, 23%, and 59% in control group, respectively. There were more fractures in the patient group and the difference was statistically significant (p = 0.004). 

Conclusion: An increase in osteoporosis frequency and secondary fracture to osteoporosis in the patients with pemphigus vulgaris was detected.

Full acticle can be viewed at: Indian Journal of Dermatology

IMPORTANCE A rare variant of mucous membrane pemphigoid (MMP) is characterized by circulating anti-laminin 332 (Lam332) autoantibodies and seems to be associated with concurrent malignant neoplasms.

OBJECTIVE To determine the prevalence and clinical significance of anti-Lam332 autoantibody detection from a large series of patients with MMP. DESIGN Multicenter retrospective study.

SETTING Four French national centers for autoimmune bullous diseases.

PARTICIPANTS One hundred fifty-four patients with MMP and 89 individuals serving as controls were included.

INTERVENTIONS Serum samples were analyzed by a new Lam332 enzyme-linked immunosorbent assay (ELISA); clinical and immunopathologic data were obtained from the patients’ medical records.

MAIN OUTCOME MEASURES The Lam332 ELISA scores were evaluated with respect to clinical characteristics, standard and salt-split indirect immunofluorescence, and bullous pemphigoid (BP) 230 and BP180-NC16A ELISAs.

RESULTS The Lam332 ELISA score was positive (≥9 U/mL) in 20.1% of serum samples from patients with MMP, 1 of 50 patients with bullous pemphigoid (BP), none of 7 with pemphigus, and 3 of 32 other controls. No relationship was evidenced between a positive ELISA Lam332 score and age; sex ratio; oral, ocular, genital, skin, or esophageal/laryngeal involvement; internal malignant neoplasm; or BP180 ELISA score. Salt-split skin indirect immunofluorescence and ELISA BP230 results were more frequently positive when Lam332 ELISA results were positive (P = .04 and .02, respectively). Patients with a positive Lam332 ELISA score frequently had more severe MMP (67.8% vs 47.2%; P = .04).

CONCLUSIONS AND RELEVANCE Results of this novel ELISA showed that serum anti-Lam332 autoantibodies are detected in 20.1% of patients with MMP. Anti-Lam332 autoantibodies are mainly detected in patients with severe MMP but not preferentially in those with a malignant neoplasm. The association between anti-Lam332 and anti-BP230 autoantibodies might arise from an epitope-spreading phenomenon.

JAMA dermatology (Chicago, Ill.)

Pemphigus vulgaris (PV) is a paradigm of autoimmune disease affecting intercellular adhesion. The mechanisms that lead to cell–cell detachment (acantholysis) have crucial therapeutic implications and are currently undergoing major scrutiny. The first part of this review focuses on the classical view of the pathogenesis of PV, which is dominated by the cell adhesion molecules of the desmosome, namely desmogleins (Dsgs). Cloning of the DSG3 gene, generation DSG3 knock-out mice and isolation of monoclonal anti-Dsg3 IgG have aided to clarify the pathogenic mechanisms of PV, which are in part dependent on the fate of desmosomal molecules. These include perturbation of the desmosomal network at the transcriptional, translational, and interaction level, kinase activation, proteinase-mediated degradation, and hyper-adhesion. By the use of PV models, translational research has in turn helped shed light into the basic structure, function, and dynamics of assembly of desmosomal cadherins. The combined efforts of basic and applied research has resulted in tremendous advance into the understanding of epidermal adhesion and helped debunk old myths on the supposedly unique role of desmogleins in the mechanisms of cell–cell detachment in PV.

From: http://informahealthcare.com/doi/abs/10.3109/15419061.2013.763799

1-s2.0-S0929664612005104-gr2Lobar torsion is a rare complication after lung transplantation. Here we report a case of right middle lobe (RML) torsion after bilateral sequential lung transplantation (BLTx). This 30-year-old lady underwent BLTx for bronchiolitis obliterans due to paraneoplastic pemphigus. The right lower lobe of the donor lung was resected due to inflammatory change during procurement. The postoperative chest X-ray showed persisting RML infiltrates. Fever and leukocytosis were noted 1 week later. RML lobectomy was performed after the reconstructed chest computed tomography confirmed the diagnosis of RML torsion. Adult respiratory distress syndrome with unstable vital signs, refractory hypoxemia and respiratory acidosis occurred thereafter. After venoarterial extracorporeal membrane oxygenation support, the patient recovered slowly and was discharged 5 months after BLTx.

Full article can be purchased here: http://www.sciencedirect.com/science/article/pii/S0929664612005104

Pemphigus foliaceus (PF) is the most common autoimmune skin disease of dogs and other animal species. Although PF can spontaneously affect dogs of any breed, it appears more prevalent in Akita Inus and chow chows in the United States. The primary lesions are large pustules which rupture easily and progress rapidly to erosions and crusts. Lesion distribution most often involves the face, nasal planum, and ears. One third of affected dogs have paw pad lesions. Skin lesions of PF can remain localized or involve the entire body. The diagnosis of PF in dogs is based on historical information, clinical signs,

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and the demonstration of acantholytic keratinocytes in vesicles or pustules. (Source: Advances in Small Animal Medicine and Surgery)

Article can be purchased from : http://www.advancesinsmallanimal.com/article/PIIS1041782612000230/abstract?rss=yes

BACKGROUND: Pemphigus is an autoimmune blistering disease. According to a report, in areas of endemic pemphigus foliaceus (EPF) in Peru there are cases of pemphigus vulgaris with epidemiologic, clinical and histopathologic characteristics similar to those of “endemic pemphigus vulgaris” (EPV) in Brazil.
OBJECTIVES: To determine the clinical and epidemiologic characteristics of endemic pemphigus and the risk factors of patients for developing complications during treatment.
METHODS: A study was carried out from July 2003 to March 2008. The study population was 60 patients with EPF and 7 patients with EPV evaluated in hospitals and clinics in the Peruvian Amazon and Lima. A multivariate analysis was carried out using binary logistic regression.
RESULTS: The average age of EPF patients was 31.4 years; 55% were men; 60% presented the generalized clinical variant. Non-compliance with the treatment was seen in 57.1% of the patients. Thirty-five percent presented complications (e.g. pyodermitis and pyelonephritis) during treatment. The risk factors for developing complications during treatment were non-compliance with the treatment and having the generalized clinical form. In the EPV group, the average age was 21.7 years; 71.4% were men. All patients presented with the mucocutaneous clinical variant and the initial presentation consisted of oral mucosa lesions; 71.4% presented complications during treatment, pyodermitis being the most frequent.
CONCLUSIONS: Non-compliance with the treatment and the generalized clinical form are risk factors for the development of complications during treatment of patients with EPF. Peru indeed has EPV cases with epidemiologic characteristics similar to EPF. Living in a rural area may represent a risk factor for the development of complications during treatment of patients with EPV.

Full article can be viewed here: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962012000600003&lng=en&nrm=iso&tlng=en

Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blistering skin eruptions, and histologically acantholysis, keratinocyte necrosis and interface dermatitis are its hallmark features. A 58-year-old female presented with recurrent, severe, recalcitrant stomatitis and widespread erosions/blistering lesions of one-year duration. Treatment with repeated courses of systemic corticosteroids at a peripheral center would provide temporary relief. She also had fever, productive cough, odynophagia and poor oral intake, herpes zoster ophthalmicus, pain in the abdomen, and watery diarrhea. An array of investigations revealed chronic lymphocytic leukemia (CLL), mediastinal and para-aortic lymphadenopathy, bronchiolitis obliterans, and vertebral osteoporosis/fractures. With the diagnosis of CLL-associated PNP she was managed with dexamethasone-cyclophosphamide pulse (DCP) therapy for 3 cycles initially, followed by COP regimen (cyclophosphamide, vincristine, and prednisolone) for 5 cycles. Remission is being maintained with chlorambucil and prednisolone pulse therapy once in 3 weeks with complete resolution of skin lesions and adequate control of CLL.

Full article can be viewed here: http://www.hindawi.com/crim/dm/2012/207126/

A year before I went to the IPPF’s fabulous 2012 Annual Meeting in Boston, I had no interest in gathering for a weekend with doctors, researchers, Peer Health Coaches and other pem-pals. What good could it do?

Since being diagnosed with PV in December 2010, I’d been under the care of a terrific dermatologist. While the initial diagnosis had scared me, I’d read enough on the Internet to feel sufficiently informed. I’m a writer, not a scientist, and, frankly, all that stuff about proteins and B cells scrambled my brain. Did I really need to understand what was happening in my body? My doctor did, and that was good enough.

All I wanted was to go about my life without those nasty sores. So I took my medicine, went for labs, and waited for my condition to clear up.
Only it didn’t clear up.
I flared – badly. Clearly there was more going on than I realized. It was time to accept the fact that I had a chronic disease, one that was still in the early stages of being unraveled by scientists. If I was going to learn how to live with pemphigus over the long haul, I needed the standard tools:

• basic knowledge about the immune system to communicate with my doctor better
• a connection to the medical community to begin to grasp what researchers already know and where current research is headed
• shared experiences with others to better understand how diet, sleep, and stress affect me
• a community for ongoing support
The announcement about the meeting in Boston promised all that so I signed myself up. My daughter, Zoe, accompanied me. With a background of science, Zoe not only was great company and support but translated the molecular biology stuff. Handy!

As we checked in for the weekend, we were warmly welcomed by IPPF staff. Friday’s evening reception was a cozy mingling of patients, family members, and doctors in a setting suited to making new friends and getting to know some of the weekend presenters close up in a casual environment.
Saturday’s workshops covered the gamut of topics related to pemphigus and pemphigoid, their diagnoses and the choices doctors make when prescribing treatment regimes. My favorite talk was the one led by pioneer Dr. Sam Moschella on “Pemphigus Before Prednisone.” He described corn starch baths and treatment with an arsenic derivative called carbosone. Listening to him, the gravity and complexity of the disease hit home.

There was a lot of talk about rituximab, a B-cell targeting drug that has signified a real change in pemphigus treatment. This was particularly relevant to me, as I had just undergone Rituxan™ therapy. In one of the smaller breakout groups, I learned about Dr. Animesh Sinha’s work in genetics. In another, Dr. Vikki Noonan, a dentist, gave us great tips on oral hygiene.

One of the best parts of the weekend was making new friends. Zoe and I sat with a woman from Canada and her daughter at the Saturday night gala dinner and had a wonderful time sharing our lives and many laughs.
Oh, and the delicious food and the generosity of the event sponsors was beyond compare!
What good can a weekend with the IPPF do? It can educate, entertain, and inspire. It can bring to life the truth of the IPPF motto, “A common hope, an uncommon bond”. In short, a weekend can do lots of good!
I will be back again this year in San Francisco. I hope to see you there.