Tag Archives: pemphigus

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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/

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Pemphigus and pemphigoid are uncommon dermatological entities in domestic animals and of a presumed autoimmune nature. In one form or another, they

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have been reported in the dog, cat, horse and goat. Although these diseases are considered to be bullous dermatoses, the clinical presentation may vary from ulcerative to exfoliative to proliferative depending on the individual condition. Currently, four variants of pemphigus are recognized (vulgaris, vegetans, foliaceus, erythematosus) and two of pemphigoid (bullous, cicatricial) although cicatricial pemphigoid has not yet been conclusively demonstrated in animals. Diagnosis is based on history, clinical signs, histopathology and immunopathology. Therapy must be immunosuppressive to be effective and is palliative rather than curative.

Full article available here: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1680036/

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american-quarter-horsePemphigus foliaceus (pem-fi-gus foli-a-shus) is an auto immune disease that affects humans and dogs and, to a lesser extent, cats and horses.
In horses, it is characterized by primary lesions that often begin on the head and lower extremities; secondary lesions spread to other areas, with an exudate that dries to a crust. There may be extensive edema (swelling) in the legs and abdomen (called “ventral” edema).
Equine pemphigus foliaceus (EPF) is considered rare and signs and symptoms may resemble those of other conditions such as insect bite allergies (crusty lesions), pigeon fever (ventral edema) or other skin conditions.
The primary way to diagnose EPF is by punch biopsy of the skin which is examined by a veterinary pathologist. The pathologist looks for changes consistent with this diagnosis, while also ruling out other causes.
Horses with EPF may also have systemic signs of illness – fever, depression, loss of appetite, lethargy and weight loss. The skin may be painful to touch and swelling can make it difficult to walk or lie down.