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==Introduction==
==Introduction==
The pemphigus complex is a group of uncommon to rare '''autoimmune diseases''' which can occur in different species, but mainly dogs and cats.
The pemphigus complex is a group of uncommon to rare [[Autoimmune Diseases|'''autoimmune diseases''']] which can occur in different species, but mainly dogs and cats.
Different forms of pemphigus include:
Different forms of pemphigus include:
==Clinical Features==
==Clinical Features==
===[[Pemphigus Vulgaris|Pemphigus vulgaris]]===
===[[Pemphigus Vulgaris|Pemphigus vulgaris]]===
It is more common in the German Shepherd Dog and the Collie but remains a very rare disease.
It is more common in the German Shepherd Dog and the Collie but remains a very rare disease. Males are predisposed, even though females tend to be prone to autoimmune diseases in general, and the age of onset is usually past 6 years old.
The '''oral cavity is affected in 90% of cases'''. Disease affects the mucocutaneous junctions, ears, claws, axilla and groin. The paw pads and nails may slough. There are vesicles or bullae, erosions and ulcers.
It is one of the more '''severe''' forms of pemphigus and the patients are often ill with anorexia, depression and fever. Secondary bacterial infection is common.
It is one of the more '''severe''' forms of pemphigus and the patients are often ill with anorexia, depression and fever.
Secondary bacterial infection is common.
===[[Pemphigus Foliaceus|Pemphigus foliaceus]]===
===[[Pemphigus Foliaceus|Pemphigus foliaceus]]===
===Pemphigus erythematosus===
===Pemphigus erythematosus===
It is similar to pemphigus foliaceus but lesions are usually '''confined to the face''', especially the nose.
It is similar to pemphigus foliaceus but lesions are usually '''confined to the face''', especially the nose. There are pustules, crusts, erosions and depigmentation of the nasal planum and the dorsum of the muzzle. The paws and genitals are sometimes affected.
There are pustules, crusts, erosions and depigmentation of the nasal planum and the dorsum of the muzzle.
The paws and genitals are sometimes affected.
No oral or mucosal lesions are present.
No oral or mucosal lesions are present.
===Pemphigus vegetans===
===Pemphigus vegetans===
This is very rare but has been reported in the dog.
This is very rare but has been reported in the dog. It is thought to be a '''benign version of pemphigus vulgaris'''. The disease manifests as a vesiculopustular disorder that evolves into verrucous vegetations and papillomatous proliferations, which ooze and are studded with pustules.
Animals are usually otherwise healthy.
Animals are usually otherwise healthy.
This is very rare, and is a blistering disease affecting the '''mucosa and mucocutaneous junctions'''.
This is very rare, and is a blistering disease affecting the '''mucosa and mucocutaneous junctions'''.
It is seen in conjunction with neoplasias such as the thymoma, thymic lymphoma and splenic sarcoma.
It is seen in conjunction with neoplasias such as the [[Thymus Neoplasia|thymoma, thymic lymphoma]] and splenic sarcoma.
Systemic signs will be present due to the neoplastic process and the skin condition.
Systemic signs will be present due to the neoplastic process and the skin condition.
<u>Pemphigus vulgaris</u>: there is suprabasilar acantholysis with resultant cleft and vesicle formation. Basal epidermal cells remain attached to the basement membrane zone like a row of 'tombstones'.
<u>Pemphigus vulgaris</u>: there is suprabasilar acantholysis with resultant cleft and vesicle formation. Basal epidermal cells remain attached to the basement membrane zone like a row of 'tombstones'.
<u>Pemphigus foliaceus</u>: there is intragranular or subcorneal acantholysis with cleft and vesicle or pustule formation. Within the vesicle or pustule, acanthocytes may be individual, associated together into rafts, or adheret to the overlying epidermis. Neutrophils or eosinophils may predominate in the vesicles or pustules.
<u>Pemphigus foliaceus</u>: there is intragranular or subcorneal acantholysis with cleft and vesicle or pustule formation. Within the vesicle or pustule, acanthocytes may be individual, associated together into rafts, or adherent to the overlying epidermis. [[Neutrophils]] or [[eosinophils]] may predominate in the vesicles or pustules.
<u>Pemphigus erythematosus</u>: histology is usually identical to pemphigus foliaceus, except that there is often a lichenoid cellular infiltrate of mononuclear cells, plasma cells, and neutrophils or eosinophils or both.
<u>Pemphigus erythematosus</u>: histology is usually identical to pemphigus foliaceus, except that there is often a lichenoid cellular infiltrate of mononuclear cells, plasma cells, and neutrophils or eosinophils or both.
==Treatment==
==Treatment==
Severely affected patients may require '''antibiotics''' to control any secondary bacterial infections.
Severely affected patients may require '''antibiotics''' to control any secondary bacterial infections.
===Pemphigus vulgaris===
===Pemphigus vulgaris===
This is a severe disease that is '''often fatal''', and animals may fail to respond to treatment.
This is a severe disease that is '''often fatal''', and animals may fail to respond to treatment. '''Aggressive''' treatment with '''predisolone and azathioprine''' should be instituted as soon as a diagnosis is made. Prednisolone with '''chlorambucil''' is preferred in cats.
'''Aggressive''' treatment with '''predisolone and azathioprine''' should be instituted as soon as a diagnosis is made.
Prednisolone with '''chlorambucil''' is preferred in cats.
===Pemphigus foliaceus===
===Pemphigus foliaceus===
This disease is less severe, but if left untreated, can be fatal.
This disease is less severe, but if left untreated, can be fatal. Mild localised lesions can be treated with '''topical steroids''', but more extensive disease usually requires '''oral prednisolone'''. More than half the patients require an additional cytotoxic treatment that is synergistic with corticosteroids. This is usually azathioprine or cyclophosphamide in dogs, and chlorambucil in cats. Cyclosporin can also be considered.
Mild localised lesions can be treated with '''topical steroids''', but more extensive disease usually requires '''oral prednisolone'''.
More than half the patients require an additional cytotoxic treatment that is synergistic with corticosteroids.
This is usually azathioprine or cyclophosphamide in dogs, and chlorambucil in cats.
Cyclosporin can also be considered.
===Pemphigus erythematosus===
===Pemphigus erythematosus===
This can initially be treated by '''sun avoidance and topical steroids'''.
This can initially be treated by '''sun avoidance and topical steroids'''. In dogs, a combination of tetracycline and niacinamide is effective in up to 25% of cases. Vitamin E or oral glucocorticoids may also be added to the regimen. Topical cyclosporin can also be helpful.
In dogs, a combination of tetracycline and niacinamide is effective in up to 25% of cases. Vitamin E or oral glucocorticoids may also be added to the regimen.
Topical cyclosporin can also be helpful.
===Pemphigus vegetans===
===Pemphigus vegetans===
===Paraneoplastic pemphigus===
===Paraneoplastic pemphigus===
The '''underlying neoplasia''' needs to be identified and treated or controlled.
The '''underlying neoplasia''' needs to be identified and treated or controlled. Additional treatment may involve systemic glucocorticoids with added cytotoxic agents if necessary.
Additional treatment may involve systemic glucocorticoids with added cytotoxic agents if necessary.
==Monitoring==
==Monitoring==
Patients should be monitored at 2-4 week intervals at first, and medications should be reduced to the minimal dose necessary to control clinical signs.
Patients should be monitored at 2-4 week intervals at first, and medications should be reduced to the minimal dose necessary to control clinical signs. Routine haematology and biochemistry should be performed to identify any organ damage by drug therapy.
Routine haematology and biochemistry should be performed to identify any organ damage by drug therapy.
The medical therapy has many '''side effects''', which can include: bone marrow suppression, hepatotoxicity, nephrotoxicity, haemorrhagic cystitis or immunosuppression and the development of opportunistic infections.
The medical therapy has many '''side effects''', which can include: bone marrow suppression, hepatotoxicity, nephrotoxicity, haemorrhagic cystitis or immunosuppression and the development of opportunistic infections.
==Prognosis==
==Prognosis==
Remission is rare for pemphigus vulgaris and foliaceus, and '''lifelong therapy''' is usually necessary.
Prognosis varies with the form of the disease and the severity. Remission is rare for pemphigus vulgaris and foliaceus, and '''lifelong therapy''' is usually necessary. Pemphigus erythematosus and vegetans are usually mild diseases which are '''self-limiting''' and respond well to therapy.
Pemphigus erythematosus and vegetans are usually mild diseases which are '''self-limiting''', which respond well to therapy.
'''Secondary bacterial infections''' contribute to patient morbidity.
'''Secondary bacterial infections''' contribute to patient morbidity.
Helton Rhodes, K. (2011) '''Blackwell's five minute veterinary consult: dermatology''' ''Wiley-Blackwell''
Helton Rhodes, K. (2011) '''Blackwell's five minute veterinary consult: dermatology''' ''Wiley-Blackwell''
{{review}}
[[Category:Expert Review - Small Animal]]
[[Category:Type II Hypersensitivity Diseases]]
[[Category:Type II Hypersensitivity Diseases]]
[[Category:Integumentary System - Autoimmune Reactions]]
[[Category:Integumentary System - Autoimmune Reactions]]
[[Category:Oral Cavity - Immune Mediated Pathology]]
[[Category:Oral Cavity - Immune Mediated Pathology]]
[[Category:Antibody Mediated Autoimmune Diseases]]
[[Category:Antibody Mediated Autoimmune Diseases]]