types definition types etiology triggers clinical picture investigation Management
1)Medications:
-Angiotensin-converting-enzyme (ACE)
-most patients with pemphigus vulgaris first present with lesions on the mucous membranes such skin lesions appear as thin-walled faccid blisters and fragil filled with clear
inhibitors (blood pressure medications)
the mouth and genitals. fuid that easily rupture and painful erosions.
-Penicillamine (medication used in the
pemphigus vulgaris is a life-threatening disease 1)The exact etiology of the disease is unknown. *features of oral mucosal pemphigus include: -They most often arise on the upper chest, back, scalp, and face.
treatment of rheumatoid arthritis and
manifesting with blisters and erosions on both skin and 2)Autoimmune disease: Auto-antibodies igG directed against desmosome -Oral lesions in 50-70% of patients -Nikolsky's sign; characterized by the epidermal detachment caused by
Wilson's disease) the Goals of the therapy include:
mucosa in response to autoantibodies against structural components cause acantholysis and intraepiderma cleftnand(suprabasal bullae). -Superficial blistering and erosions mechanical pressure at the edge of a blister or normal skin, is usually present in
-Cephalosporin (antibiotic) -To bring the disease under control as rapidly as possible.
proteins of the desmosome). *In pemphigus vulgaris, autoantibodies bind to desmogleine 1 and 3 (the -Widespread involvement within the mouth PV.
Pemphigus vulgaris -Nonsteroidal anti-infammatory drugs (NSAIDS) -To prevent loss of serum and the development of secondary
“glue"holding the cells together)in (keratinocyte and membrane molecules). -Painful, slow-to-heal ulcers Asboe-Hansen sign:lateral extension of intact blister with gentle pressure.
-Rifampin (an antibiotic used most commonly infection.
-common type of pemphigus. average onset 40-60years. 3)Geneticfactors mayplay aroieinits development,with highestincidence among -Spread to the larynx causing hoarseness when talkir these erosions become covered by crusts with difficult tendency to heal.
for treating tuberculosis) -To promote re-epithelization.
-May affect neonates via placenta autoantibodies Jewish or Mediterranean patients. -Difficulty eating and drinking. -healing is usually without a scar, but pigmentary changes may be observed.
2)Emotional stress
transmission(neonatal pemphigus) 4) May be associated with trigger factors. -Blisters usually develop on the skin after a few weeks or months, although in some cases, -Erosions in the skin folds may develop into vegetative lesions which are granular
3)Burns
mucosal lesions may be the ony manifestation of the disease and crusted (pemphigus vegetans).
4)Exposure to sunlight (particularly the
. The skin around the nails may be painful, red, and swollen.
ultraviolet rays)
5)Infections
Diagnosis of pemphigus vulgaris;
1)skin biopsy from the skin adjacent to a lesion. Histology typically shows
Pemphigus is rare group of blistering rounded-up and separated keratinocytes (acantholytic cells) aconthylosis just
Pemphigus disease autoimmune diseases thataffect skin pemphigus foliaceus produces superficial blisters confined to the skin, without involvement of
above the basal layer of the epidermis( suprabasal bullae).
Biopsy specimen show characteristic tombstone appearance
•General measures
-wound dressings where required,
and mucous membrane. pathophysiology mucous membranes. of suprabasal acantholysis (hematoxylin and eosin stain sample) -monitor for signs of infection,
pemphigus erythematosus (Senear-Usher syndrome)
•IgG autoantibody against epidermal antigens : · Pemphigus vulgaris: IgG to -On the face,scalp and upper trunk,the lesions are Small fuid-filled blisters first, being superfcial
Pemphigus erythematosus (or SenearUsher syndrome) is -good oral care (if oral mucosa is involved).
desmoglein 1 (skin) or desmoglein 3 (mucosa) within the upper epidermis(subcoronal bullae), they rupture very easily, and only erosions may be 2) Direct Immunofuorescence testing Considered a gold standard for the
pemphigus foliaceus avariant of pemphigus foliaceus which shares clinical, •Oral therapies
• Pemphigus foliaceus: IgG to desmoglein 1 seen. diagnosis of pemphigus ,shows IgGautoantibodies (antiDsg 1 , anti Dsg3 or both)
histopathological, and serological features with - high-dose oral steroids 1-2mg/kg until improvement then
•Paraneoplastic pemphigus: IgG to desmoplakin I, desmoplakin II, plectin, -often scaly and crusty on a red and infamed base.A burning sensation or localized pain may be against the keratinocyte's cell surface suprabasal. This pattern is described as
systemiclupus erythematosus~(SLE). tapering .
periplakin, envoplakin, BP230 or A2ML1 felt. the "chicken wire" pattern or net like pattern. -immunosuppressive agents (e.g. methotrexate, azathioprine,
-The patient with pemphigus foliaceus is usually otherwise in good health.
cyclophosphamid,.mymophenolate mofetil, and othor)
3)Indirect immunofuorescence, or secondary immunofuorescence, is a
technique used in laboratories to detect circulating autoantibodies in patient
serum.
4)Tzank smear.
Bullous diseases Almost all cases of PNP are associated with an underlying cancerous (malignant)
process:
-Lymphoproliferative neoplasms are the most common underlying disease (~84% •Extensive refractory mucostomatitis (often the only presenting feature ofPNP), with painful
Paraneoplastic PNP is a rare autoimmune bullous disease variant;it is the of PNP cases): specifically, non-Hodgkin lymphoma is the most mucous me'mbrane erosions, blisters, haemorrhagic crusting, and/or ulceration involving the
pemphigus (PNP) least common but most serious form of pemphigus, common type of underlying cancer in adults. oropharynx, nasopharynx, conjunctiva, lips, tongue,palate, and/or genitals
-followed by chroniclymphocytic leukaemia (CLL). •Diffuse polymorphic skin lesion.
-thyomoma
Symptoms
·severe pruritus
·may have history of eczematous or urticarial lesions before bullae formation
·Associated conditions ·Physical exam
1)Skin biopsy
•bullous pemphigoid (BP)is an -drug use ·tense bullae with clear exudate
·Pathogenesis ·may be hemorrhagic ·shows subepidermal blisters which are often with eosinophil-rich infiltrates
autoimmune blistering 1.diuretics
•auto-antibodies (lgG) against hemidesmosomes in the epidermal-dermal junction ·does not easily rupture 2)Immunofuorescence DIF and IDIF
2.metformin
disorder(subepidermal bullae), ·more common in those >70 years of age •antibodies are below the epidermis ·shows a linear band of immunoglobulins targeting hemidesmosomes and
Bullous pemphigoid characterized by bullae (> 1 cm
3.neuroleptics
-neurologic conditions ·rarely in infants •main autoantigens are BP180 and BP230
•in adults:
typically symmetrically distributed on trunks and extremities complement at
•this activates complement and infammatory reaction which cause epidermal- epidermal-dermal junction
large, fuid-containing tens blister) 1.multiple sclerosis
dermal splitting 3)Complete blood count (CBC)
2.dementia •ininfants:
and severe pruritus 3.Parkinson disease ·palms and soles more commonly affected ·may show eosinophilia
·spares mucous membranes
·negative Nikolsky sign
·may have vesicles
Dermatitis herpetiformis may cause skin, oral, and gastrointestinal symptoms.
Causes Skin symptoms:
1. Gluten-free diet.
dermatitis herpetiformis is a -Gluten triggers production of IgA antibodies and an autoimmune process •Groups of extremely itchy blisters; a burning sensation may develop
2.Dapsone
thattargetsthe skin and gut. •Commonly affected areas are the elbows, forearms, knees, buttocks, back, and/or scalp,
chronicskin condition with an -In coeliac disease, gluten causes intestinal infammation resulting in diarrhoea, although the face and groin may sometimes be affected 1)Skin biopsy is usually necessary to confirm DH. is the treatment of choice for DH, as itusually reducesitch
autoimmune origin associated with •The skin rash tends to develop equally on the left and right sides of the body Lesional skin may show: within 3 days. Dose ranges from 25-300 mg daily.
-DH can appear at any age but is most commonly tiredness, weight loss, abdominal discomfort, and metabolic consequences of
-Subepidermal blisters -The effects of dapsone are seen rapidly. Symptoms of itch
celiac disease(gluten sensitive diagnosed in those between the age of 15 and 40 . malabsorption. Oral symptoms:
Dermatitis herpetiformis enteropathy)that is characterized by
Subtopic 1
- It's more common in men than women and is rare in -The majority (>90%) of patients with DH also have gluten-sensitive •Blisters in the mouth -Neutrophil and eosinophil infammatory cells in the dermal papillae resolve within several hours and new blister formation ceases
in 24-36 hours. It is an effective option in
children enteropathy.Gastrointestinal symptoms are usually timild; some patients remain •Tooth enamel defects (discoloration, pitting, or banding)
development of symmetrical, on the symptom-free. Gastrointestinal symptoms: 2) DIFOF is the standard gold test for DH is charcteerised by granular deposit of the early stages of gluten-free diet initiation.
IgA at the dermoepidermal junction papillae. -There are reports that combination therapy with dapsone
extensor surfaces, bilateral pruritic · There is a genetic predisposion DH may be exacerbated by some medications ·Stomach pain
and sulfasalazine may be effective in patients who do not
papulovesicular lesions. (eg, potassium iodide, non- steroidal anti-infammatory medications,progesterone), ·Diarrhea
·Constipation tolerate increasing doses of dapsone monotherapy.
and premenstrually
·Bloating
by fatema okoff
1)Medications:
-Angiotensin-converting-enzyme (ACE)
-most patients with pemphigus vulgaris first present with lesions on the mucous membranes such skin lesions appear as thin-walled faccid blisters and fragil filled with clear
inhibitors (blood pressure medications)
the mouth and genitals. fuid that easily rupture and painful erosions.
-Penicillamine (medication used in the
pemphigus vulgaris is a life-threatening disease 1)The exact etiology of the disease is unknown. *features of oral mucosal pemphigus include: -They most often arise on the upper chest, back, scalp, and face.
treatment of rheumatoid arthritis and
manifesting with blisters and erosions on both skin and 2)Autoimmune disease: Auto-antibodies igG directed against desmosome -Oral lesions in 50-70% of patients -Nikolsky's sign; characterized by the epidermal detachment caused by
Wilson's disease) the Goals of the therapy include:
mucosa in response to autoantibodies against structural components cause acantholysis and intraepiderma cleftnand(suprabasal bullae). -Superficial blistering and erosions mechanical pressure at the edge of a blister or normal skin, is usually present in
-Cephalosporin (antibiotic) -To bring the disease under control as rapidly as possible.
proteins of the desmosome). *In pemphigus vulgaris, autoantibodies bind to desmogleine 1 and 3 (the -Widespread involvement within the mouth PV.
Pemphigus vulgaris -Nonsteroidal anti-infammatory drugs (NSAIDS) -To prevent loss of serum and the development of secondary
“glue"holding the cells together)in (keratinocyte and membrane molecules). -Painful, slow-to-heal ulcers Asboe-Hansen sign:lateral extension of intact blister with gentle pressure.
-Rifampin (an antibiotic used most commonly infection.
-common type of pemphigus. average onset 40-60years. 3)Geneticfactors mayplay aroieinits development,with highestincidence among -Spread to the larynx causing hoarseness when talkir these erosions become covered by crusts with difficult tendency to heal.
for treating tuberculosis) -To promote re-epithelization.
-May affect neonates via placenta autoantibodies Jewish or Mediterranean patients. -Difficulty eating and drinking. -healing is usually without a scar, but pigmentary changes may be observed.
2)Emotional stress
transmission(neonatal pemphigus) 4) May be associated with trigger factors. -Blisters usually develop on the skin after a few weeks or months, although in some cases, -Erosions in the skin folds may develop into vegetative lesions which are granular
3)Burns
mucosal lesions may be the ony manifestation of the disease and crusted (pemphigus vegetans).
4)Exposure to sunlight (particularly the
. The skin around the nails may be painful, red, and swollen.
ultraviolet rays)
5)Infections
Diagnosis of pemphigus vulgaris;
1)skin biopsy from the skin adjacent to a lesion. Histology typically shows
Pemphigus is rare group of blistering rounded-up and separated keratinocytes (acantholytic cells) aconthylosis just
Pemphigus disease autoimmune diseases thataffect skin pemphigus foliaceus produces superficial blisters confined to the skin, without involvement of
above the basal layer of the epidermis( suprabasal bullae).
Biopsy specimen show characteristic tombstone appearance
•General measures
-wound dressings where required,
and mucous membrane. pathophysiology mucous membranes. of suprabasal acantholysis (hematoxylin and eosin stain sample) -monitor for signs of infection,
pemphigus erythematosus (Senear-Usher syndrome)
•IgG autoantibody against epidermal antigens : · Pemphigus vulgaris: IgG to -On the face,scalp and upper trunk,the lesions are Small fuid-filled blisters first, being superfcial
Pemphigus erythematosus (or SenearUsher syndrome) is -good oral care (if oral mucosa is involved).
desmoglein 1 (skin) or desmoglein 3 (mucosa) within the upper epidermis(subcoronal bullae), they rupture very easily, and only erosions may be 2) Direct Immunofuorescence testing Considered a gold standard for the
pemphigus foliaceus avariant of pemphigus foliaceus which shares clinical, •Oral therapies
• Pemphigus foliaceus: IgG to desmoglein 1 seen. diagnosis of pemphigus ,shows IgGautoantibodies (antiDsg 1 , anti Dsg3 or both)
histopathological, and serological features with - high-dose oral steroids 1-2mg/kg until improvement then
•Paraneoplastic pemphigus: IgG to desmoplakin I, desmoplakin II, plectin, -often scaly and crusty on a red and infamed base.A burning sensation or localized pain may be against the keratinocyte's cell surface suprabasal. This pattern is described as
systemiclupus erythematosus~(SLE). tapering .
periplakin, envoplakin, BP230 or A2ML1 felt. the "chicken wire" pattern or net like pattern. -immunosuppressive agents (e.g. methotrexate, azathioprine,
-The patient with pemphigus foliaceus is usually otherwise in good health.
cyclophosphamid,.mymophenolate mofetil, and othor)
3)Indirect immunofuorescence, or secondary immunofuorescence, is a
technique used in laboratories to detect circulating autoantibodies in patient
serum.
4)Tzank smear.
Bullous diseases Almost all cases of PNP are associated with an underlying cancerous (malignant)
process:
-Lymphoproliferative neoplasms are the most common underlying disease (~84% •Extensive refractory mucostomatitis (often the only presenting feature ofPNP), with painful
Paraneoplastic PNP is a rare autoimmune bullous disease variant;it is the of PNP cases): specifically, non-Hodgkin lymphoma is the most mucous me'mbrane erosions, blisters, haemorrhagic crusting, and/or ulceration involving the
pemphigus (PNP) least common but most serious form of pemphigus, common type of underlying cancer in adults. oropharynx, nasopharynx, conjunctiva, lips, tongue,palate, and/or genitals
-followed by chroniclymphocytic leukaemia (CLL). •Diffuse polymorphic skin lesion.
-thyomoma
Symptoms
·severe pruritus
·may have history of eczematous or urticarial lesions before bullae formation
·Associated conditions ·Physical exam
1)Skin biopsy
•bullous pemphigoid (BP)is an -drug use ·tense bullae with clear exudate
·Pathogenesis ·may be hemorrhagic ·shows subepidermal blisters which are often with eosinophil-rich infiltrates
autoimmune blistering 1.diuretics
•auto-antibodies (lgG) against hemidesmosomes in the epidermal-dermal junction ·does not easily rupture 2)Immunofuorescence DIF and IDIF
2.metformin
disorder(subepidermal bullae), ·more common in those >70 years of age •antibodies are below the epidermis ·shows a linear band of immunoglobulins targeting hemidesmosomes and
Bullous pemphigoid characterized by bullae (> 1 cm
3.neuroleptics
-neurologic conditions ·rarely in infants •main autoantigens are BP180 and BP230
•in adults:
typically symmetrically distributed on trunks and extremities complement at
•this activates complement and infammatory reaction which cause epidermal- epidermal-dermal junction
large, fuid-containing tens blister) 1.multiple sclerosis
dermal splitting 3)Complete blood count (CBC)
2.dementia •ininfants:
and severe pruritus 3.Parkinson disease ·palms and soles more commonly affected ·may show eosinophilia
·spares mucous membranes
·negative Nikolsky sign
·may have vesicles
Dermatitis herpetiformis may cause skin, oral, and gastrointestinal symptoms.
Causes Skin symptoms:
1. Gluten-free diet.
dermatitis herpetiformis is a -Gluten triggers production of IgA antibodies and an autoimmune process •Groups of extremely itchy blisters; a burning sensation may develop
2.Dapsone
thattargetsthe skin and gut. •Commonly affected areas are the elbows, forearms, knees, buttocks, back, and/or scalp,
chronicskin condition with an -In coeliac disease, gluten causes intestinal infammation resulting in diarrhoea, although the face and groin may sometimes be affected 1)Skin biopsy is usually necessary to confirm DH. is the treatment of choice for DH, as itusually reducesitch
autoimmune origin associated with •The skin rash tends to develop equally on the left and right sides of the body Lesional skin may show: within 3 days. Dose ranges from 25-300 mg daily.
-DH can appear at any age but is most commonly tiredness, weight loss, abdominal discomfort, and metabolic consequences of
-Subepidermal blisters -The effects of dapsone are seen rapidly. Symptoms of itch
celiac disease(gluten sensitive diagnosed in those between the age of 15 and 40 . malabsorption. Oral symptoms:
Dermatitis herpetiformis enteropathy)that is characterized by
Subtopic 1
- It's more common in men than women and is rare in -The majority (>90%) of patients with DH also have gluten-sensitive •Blisters in the mouth -Neutrophil and eosinophil infammatory cells in the dermal papillae resolve within several hours and new blister formation ceases
in 24-36 hours. It is an effective option in
children enteropathy.Gastrointestinal symptoms are usually timild; some patients remain •Tooth enamel defects (discoloration, pitting, or banding)
development of symmetrical, on the symptom-free. Gastrointestinal symptoms: 2) DIFOF is the standard gold test for DH is charcteerised by granular deposit of the early stages of gluten-free diet initiation.
IgA at the dermoepidermal junction papillae. -There are reports that combination therapy with dapsone
extensor surfaces, bilateral pruritic · There is a genetic predisposion DH may be exacerbated by some medications ·Stomach pain
and sulfasalazine may be effective in patients who do not
papulovesicular lesions. (eg, potassium iodide, non- steroidal anti-infammatory medications,progesterone), ·Diarrhea
·Constipation tolerate increasing doses of dapsone monotherapy.
and premenstrually
·Bloating
by fatema okoff
