Tag Archives: pemphigus

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 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/

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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

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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