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Many times when seeing a physician for pemphigus or pemphigoid they are quick to prescribe a systemic treatment that will hopefully help you reach remission. This can be a good thing. However, sometimes the obvious may be overlooked.  For example, if you are in pain,  having trouble eating or swallowing, your clothes are sticking to your lesions, the blisters on your scalp make bathing and showering difficult, or perhaps you are having chronic nosebleeds. These symptoms can be managed with topical treatments, but they are often forgotten. There are different options available for different body locations in many different strengths. Be candid with your doctor and let them know where you are having disease activity and how severe it is. Although, ultimately, the systemic treatment is going to make the difference in the long run.  Topical treatment can help relieve many of your symptoms along the way!

If you’re not sure which medications to ask for or their strengths, just “Ask a Coach”!

Remember, when you need us we are in your corner!

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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

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Background  Pemphigus foliaceus (PF) is a chronic cutaneous autoimmune blistering

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disease that is characterized by superficial blistering of the skin, and according to the current perspective is caused by autoantibodies directed against desmoglein (Dsg) 1.

Objectives  To examine early acantholysis in the skin of patients with PF at an ultrastructural level.

Methods  Two Nikolsky-negative (N−), five Nikolsky-positive (N+) and two lesional skin biopsies from immunoserologically defined patients with PF were studied by light and electron microscopy.

Results  We found no abnormalities in N− PF skin, whereas all the N+ skin biopsies displayed intercellular widening between desmosomes, a decreased number of desmosomes and hypoplastic desmosomes in the lower epidermal layers. Acantholysis was present in two of five N+ biopsies, but only in the upper epidermal layers. The lesional skin biopsies displayed acantholysis in the higher epidermal layers. Hypoplastic desmosomes were partially (pseudo-half-desmosomes) or completely torn off from the opposing cell.

Conclusion  We propose the following mechanism for acantholysis in PF: initially PF IgG causes a depletion of nonjunctional Dsg1, leading to intercellular widening between desmosomes starting in the lower layers and spreading upwards. Depletion of nonjunctional Dsg1 impairs the assembly of desmosomes, resulting in hypoplastic desmosomes and a decreased number of desmosomes. In addition, antibodies might promote disassembly of desmosomes. In the upper layers of the epidermis, where Dsg3 is not expressed and cannot compensate for Dsg1 loss, ongoing depletion of Dsg1 will finally result in a total disappearance of desmosomes and subsequent acantholysis.

Full article available at: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2133.2012.11173.x/abstract;jsessionid=624E75DA95767387AA80E95C275F4100.d02t01