Module 8: Scaling, Crusting Disorders, and Miscellaneous Skin Conditions of Small Animals (Weeks 10 & 11) Flashcards
Introduction to autoimmune Skin Diseases
Introduction to autoimmune Skin Diseases
Definition:
Are characterized by tissue damage in anatomically diverse locations
Autoimmune disorders
Autoimmune Skin Diseases:
Inflammation is mediated by …
- Innate cells
- Migrating autoreactive lymphocytes
- Circulating pathogenic autoantibodies
- Self-reactive T cells are activated inappropriately by microbial peptides with homology to those from self
- Example: “Fogo Salvagem” (wildfire)
- Endemic form of pemphigus foliaceus (PF) in Brazil, associated with sandfly bites
- Protein of the salivary glands of the sandfly crossreact with Desmoglein -1, the main autoantibody in human PF
Molecular Mimicry
Is a diversification of the epitopes recognized by the immune system
Epitope Spreading
- an epitope is an antigenic determinant, or a site on the surface of an antigenic molecule, to which a single antibody binds
- Auto-reactive B & T cells are activated in an antigen-independent manner
- E.g. There is a high incidence of allergies in Systemin Lupus Erythematosus (SLE) patients
- Inflammatory cytokines released in response to environmental antigens like house dust mites can activate autoantibody production by B cells
Bystander activation
Autoimmune Skin Diseases:
In our patients, What are the most common autoimmune skin diseases of dogs and cats?
- Pemphigus foliaceus (PF)
- Facial Discoid Lupus erythematosus (DLE)
What condition is characterized by a specific humoral or cell-mediated immune response against the body’s tissue?
Autoimmunity
Pemphigus Complex
Pemphigus Complex
Autoimmune Dermatoses and Life-Threatening Diseases
Autoimmune Dermatoses and Life-Threatening Diseases
List the most common autoimmune skin diseases of dogs and cats:
-
Pemphigus foliaceus (PF)
- superficial
- Facial Discoid lupus eythematosus (DLE)
Term:
is a condition characterized by a specific humoral or cell-mediated immune response against the body’s own tissue
Autoimmunity
Term:
are a subfamily of desmosomal cadherins, the transmembrane constituents of desmosomes
Desmocollins
Term:
- a structure by which two adjacent cells are attached, formed from protein plaques in the cell membranes linked by filaments.
- are intercellular structures that holds cells together
desmosome
- Autoantibodies are directed against desmocollins =(form part of the desmosomes)
Pemphigus
- Systemic signs are uncommon (WNL)
- Pruritus is variable
- we don’t see secondary infections very offen
Term:
The binding of the antibodies to the desmocollins causes the release of enzymes that degrade the desmosomes
Acantholysis
Pemphigus (Term):
are immature, detached keratinocytes that are the hallmark of pemphigus diseases
Acantholytic keratinocytes
Trichophyton mentagrophytes can also cause …
Acanthosis
What are the primary lesions for Pemphigus Foliaceus?
Pustules (rarely seen)
What are the Secondary lesions for Pemphigus Foliaceus?
Crusts, scales, alopecia, erosions and erythema - Are seen more often
What is the Distribution of the lesions for Pemphigus foliaceus?
- Muzzle: dorsal aspect often affected
- Pinnae- inner and outer aspect
- Footpads: hyperkeratotic, crusty, maybe the only site affected
- CATS (maybe the only place affected) Paronychia
- General distribution
- Oral mucosa rarely affected
Term:
A skin infection that occurs around the nails
Paronychia
Differential diagnosis:
- Bacterial folliculitis
- Dermatophytosis
- Demodicosis
- P. erythematosus
- DLE
- Superficial necrolytic dermatitis (footpads)
- Zinc responsive dermatosis (footpads)
- Cutaneous lymphoma
What will you see in Skin cytology?
- Multiple acantholytic keratinocytes
- Nondegenerate neutrophils
- +/- Eosinophils
Localized Discoid Lupus Erythematosus
Localized Discoid Lupus Erythematosus
- Is the second most common autoimmune skin disease after PF
- Only skin is affected, no systemic signs
- Lesions are exacerbated by UV light
Discoid Lupus Erythematosus (DLE)
List the Clinical Signs for DLE:
- Initial Lesions:
- Depigmentation and erythema of the planum nasale
- Can progress to erosions, ulcers, scaling and crusts
- Depigmentation and erythema of the planum nasale
- Lesions usually heal with a scar – loss of cobblestone architecture of the nose
Describe the distribution of the lesions for DLE:
- Often limited to the planum nasale and dorsal aspect of the muzzle
- Other areas can be affected
- Mucocutaneous junctions, scrotum, pinnae
Differential diagnosis:
- PF
- P. erythematosus
- Dermatomyositis (skin and muscle, scars)
- Zinc responsive dermatosis
Pemphigus erythematosus
Pemphigus erythematosus
- Is uncommon
- It is a benign form of Pemphigus
- It does have some similarities to Discoid Lupus erythematous (DLE) - a crossover between PF and DLE?
Pemphigus erythematosus
List the clinical signs of Pemphigus erythematosus:
- Lesions are similar to PF, localized to the face and planum nasale
- Primary Lesions are rare (Pustules)
- Secondary lesions are common (Crusts, Erosion, Alopecia, erythema)
Describe the diagnosis of histopathology for Pemphigus erythematosus:
- Subcorneal or intraepidermal pustule
- Basal keratinocyte vacuolization and presence of apoptotic cells in the basal layer
(T/F) Most cases of the autoimmune disease need lifelong treatment
True
(T/F) Approximately 50% of the cases respond to oral glucocorticoids as a sole therapy
True
(T/F) An early lesion of facial discoid lupus erythematosus is nasal depigmentation
True
Generalized DLE
Generalized DLE
- Is a newly recognized canine variant of cutaneous lupus erythematosus
- Ten cases were recently described, criteria for diagnosis included:
- > 3 months history of generalized skin lesions - Skin lesions resembling those of human - annular (discoid) to polycyclic plaques with depigmentation, adherent scaling, follicular plugging and central alopecia below the neck
- Presence of microscopic lesions typical of DLE interface dermatitis with basal cell damage
Generalized discoid lupus erythematosus (GDLE)
Clinical Signs:
- 40% had mucocutaneous lesions most commonly around the genitalia
- Pruritus in 40% of cases
- Pain in 30% of cases
(NO systemic clinical signs)
Histopathology:
- interface dermatitis with basal cell vacuolization and apoptosis
Vesicular cutaneous lupus erythematosus
Vesicular cutaneous lupus erythematosus
- Ulceration dermatitis affecting predominantly the ventrum of shelties and collies also reported in border colli
- Mucocutaneous junctions and the concave aspect of the pinnae can also be affected
- Age of onset: 3-8 years old
- Onset usually during summer
- Females may be over-represented
Vesicular cutaneous lupus erythematosus
- no systemic signs
- may be painful
- it affects the quality of life
Lupus disease responds very nicely to …
Cyclosporine
Mucocutaneous lupus erythematosus
Mucocutaneous lupus erythematosus
- Age 3-13
- Presence of mucocutaneous erosive lesions and lupus-specific histopathology
- Most dogs start showing between 4-8 years of age
- German Shepard Dogs
- Dyschezia/dysuria
Mucocutaneous lupus erythematosus
What are the main differential diagnoses?
- Mucocutaneous pyoderma
- Mucous membrane pemphigoid (autoimmune)
Exfoliative Cutaneous Lupus erythematous
Exfoliative Cutaneous Lupus erythematous
- German Short-haired Pointers
- Lesions:
- Scaling - most common
- Alopecia
- Erythema
- Erosions
- Ulcers
Exfoliative Cutaneous Lupus erythematous
- Distribution: Muzzle, pinna, Dorsum
- Progressive, poorly responsive to treatment!
Life-threatening Skin Diseases
Life-threatening Skin Diseases
- Atopic dermatitis
- Canine generalized demodicosis
- Otitis externa/media
- Deep pyoderma
common diseases Difficult to control
- Thymoma-associated exfoliative dermatosis
- Severe scaling and erythema
- Alopecia
- Start on the head, neck and pinae
- Pruritus absent or minimal; may be intense with secondary bacterial or Malassezia infections
- Systemic signs
- Respiratory signs
- Cats
- Paraneoplastic alopecia - pancreatic adenocarcinoma
- Rare
- cats (> 10 years)
- Associated with Pancreatic adenocarcinoma
- Glistering skin
Paraneoplastic syndromes