2. Bullous skin diseases
• The group of bullous dermatoses is very
diverse both in the clinical picture and in its
etiological and pathogenetic essence, but they
are united by a single primary morphological
element- a bubble that appears on the skin
and visible mucous membranes.
4. Classification
II. Pemphgoid:
• Bullous pemphigoid
• Scarring pemphigoid
• Benign non-acantholytic pemphigus of the
mucous membrane of the oral cavity only
Pashkov- Sheklakov.
5. Classification
III Herpetiform dermatoses
• Herpetiform dermatoses of Dühring
• Herpes of pregnant women
• Subcorneal pustulosis
IV. Benign-family familial pemphigus Guzhero-
Haley-Hailey
V. Transient acantholytic dermatosis of Grover
6. True Pemphigus
• Prmphigus is more characteristic of women.
• The disease is much more common in married
families.
• The first manifestation of dermatosis is usually
observed after the age of 50, although there
have been observations of its occurrence at a
younger age and even isolated cases in
childhood.
7. Clinical and morphological and histopathological
signs that are characteristic of all clinical forms
of disease are:
1. A long chronic undulating course of dermatoses
with the development of blisters, which tend to
generalize and merge with each other, accompanied
by a violation of the general condition of patients,
before the use of corticosteroid therapy often ends
fatally
2. The general mechanism of bladder formation by
acantholysis
8. 3 The intraepidermal arrangement of the bubbles:
suprabasal with vulgar and vegetative pemphigus and
subcornal or supra intragranular with leaf-shaped and
erythematous pemphigus.
4 Deposition of immunoglobulins of class <3 in the
intercellular space of the epidermis.
Etiology and pathogenesis a true pemphigus is still unclear.
The following theories are available: Neurogenic, Viral,
Exchange, Endocrine, Autoimmune
9. Clinical
• Characterized by appearance of flaccid bullae on
the skin and mouth cavity.
• The blisters rupture easily to leave widespread
painful erosions.
• Most patients develop the mouth lesions first.
• Oral ulcers that persists for months before skin
lesions appears on the trunk, flexures and scalp.
• Rubbing on normal skin (sliding pressure) can
cause new erosion to form(positive Nikolsky
sign).
10. Nikolsky Sign : Dislodging of epidermis by lateral finger pressure in the vicinity
of lesions, which leads to an erosion.
Shearing stresses on normal skin can cause new erosions to form
11. Diagnosis
• The presence of monomorphic rashes in the form og blisters with a
tense or sagging tire that occur on unchanged skin and or mucous
membranes.
• Detection of acantholytic cells in smears from the bottom of
erosion.
• Identification of intraepidermal blisters and cracks during
histological examination
• Determination of the presence of fixed IgG complexes in the
intercellular substance of the epidermis.
• Detection of circulating antibodies of the IgG class against antigenic
components of the intercellular gluing substance of the epidermis
in the Serum of the patient using the indirect IF method.
13. Treatment
• Corticosteroids
• Cytostatic drugs.
• Immunocorrectors.
• Plasmapheresis, hemosorption.
• Sandimmun.
• Antibiotics of a wide spectrum of action.
• Anabolic hormones, a complex of vitamins A
and E and group B.
14. Vegetative Pemphigus
• Clinical picture is characterized by the sudden
onset oof blisters, often on the mucous
membrane of the oral cavity, mainly at the
places where it passes into the skin. Flabby
blisters arise around the natural openings and
in the folds of the skin inguinal femoral,
intergluteal, axillary, mammary glands, in the
navel
16. Erythematous pemphigus
• Is typified by the appearance of erythematous
scaly plaques, thin walled bullae and denuded
areas, predominantly on the butterfly area of
the face, upper part of the back, chest and
intertriginous area.
17.
18. TREATMENT
• In acute phase, prednisolone 40-60mg daily is usually
needed to control the eruption
• Immunosuppressive agents may also be required.
• Dosage should be reduced as soon as possible to low
maintenance, taken on alternate days until treatment
is stopped.
• In very mild cases and for local recurrences, topical
glucocorticoid ointments or topical tacrolimus therapy
may be beneficial.
• Tetracycline ± nicotinamide has been reported to be
effective in some cases.
• Treatment can often be withdrawn after 2-3years.
19. Bullous pemphigoid
It is characterized by a benign course.
Bullous pemphigoid (non-acantholytic
pemphigus, parapemphigus), characterized by a
subepidermal arrangement of blisters that occur
in most cases in elderly and senile individuals.
The disease can occur in all age groups, and in
rare cases in children. Approximately 10-20% of
cases are affected by mucous membranes.
20. CLINICAL
• Pemphigoid is a chronic, usually itchy, blistering
disease, mainly affecting the elderly.
• Early stages of the diseease is characterised by pruritus
• Bullae may be centered on erythematosus and
urticated base.
• Large tense bullae found anywhere on the skin
• The flexures are often affected; inner aspect of the
thigh, flexure surface of forearms, axillae, groin and
lower abdomen
• the mucous membranes usually are not.
• The Nikolsky test is negative.
21.
22.
23. Diagnostics
• Skin biopsy shows a deeper blister(than in
pemphigus) owing to a subepidermal split
through the BM
• On direct IF, perilesional skin shows linear
band of IgG and C3 along BMZ
• Indirect IF shows IgG antibodies that reacts
with the BMZ in most patients
• Hematology Eosinophilia (not always)
25. Scarring pemphigoid
• Cicatricial pemphigoid (Mucous-synechial
syndrome, synechial dermatosis) is a disease
of the mucous membranes.
• Vesico-bullous eruptions are located mainly
on the conjunctiva or in the oral cavity,
although other mucous membranes
26.
27. Differential Diagnosis
• Vulgar pemphigus,
• Herpetiform dermatitis,
a bullous variant of multiforme exudative
• Erythema,
• Pemphigoid form of red lichen planus,
• Bullous toxemia.
• Aphthous stomatitis,
• Behcet's disease,
• Stevens-Johnson syndrome,
• Recurrent herpes,
• Desquamative gingivitis,
28. TREATMENT
• Corticosteroid hormones. The initial dose of 40-
80 mg of prednisolone per day; with a scarring
pemphigoid with eye damage, higher doses may
be required.
The duration of treatment and the rate of
reduction in the daily dose are determined by the
severity of the disease.
Cytotoxic agents
Sulfon preparations
29. Dermatitis Herpetiformis
• Intensely itchy, chronic papulovesicular eruption
distributed symmetrically on extensor surfaces.
• It may start at any age, including childhood;
however, the 2nd ,3rd , and 4th decades are the
most common.
• Skin biopsy ; If a vesicle can be biopsied before it
is scratched away, the histology will be that of a
subepidermal blister, with dermal papillary
collections of neutrophils (microabscesses).
30. Dermatitis Herpetiformis
• DIF ; Granular IgA deposits in normal-appearing skin
are diagnostic for DH.
• Most, if not all, DH patients have an associated gluten-
sensitive enteropathy. Course; The condition typically
lasts for decades unless patients avoid gluten entirely.
• Differential diagnosis; scabies, an excoriated eczema,
insect bites or neurodermatitis.
• RX ; The rash responds rapidly to dapsone therapy
• gluten-free diet works very slowly. Combine the two
at the start and slowly reduce the dapsone
33. Clinical
• With the disease usually begins with
subjective sensations tingling, burning,
itching.
• Urticaroid erythmatic elements
• Tense vesicles on an edematous erythematous
base, having a pronounced tendency to group
and herptiform location.
34. Diagnosis
• Cyclic chronic course of the disease
• Eosinophilia in the bladder and often in the
blood.
• The absence of acantholytic cells.
• A negative symptom of Nikolsky
• Hypersensitivity to iodine preparations
• The presence of fixed LgA in the dermo-
epidermal zone or in the papillary dermis.
36. Treatment
• Preparations of the sulfone series:
diaminodiphenylsulfone (DDS, dapsone,
aulosulfone), diutsifon, etc. DDS is used at 200
mg per day for 5-day cycles with a break of 2
days (2-4 cycles). Diuciphone (a combination
of DDS and 6- methyluracil) is prescribed for
0.1-0.2 g per day in five-day cycles with a one-
day break.
37. Treatment
• Sulfur-containing drugs with antioxidant
properties: lipoic acid, methionine, ethamide.
• Unithiol intramuscularly in 10 ml N 10-15.
• In the bullous version of HDD, glucocorticoids
in average doses of 40-50 mg per day for 2-3
weeks with a gradual dose reduction.
• Colchicine at 0.6 mg 3 times a day for 3–4
weeks.
38. Treatment
• Gluten-free diet, i.e. the exclusion of wheat,
rice, oats, rye, barley, millet and other cereals
from food. Hypochloride diet, and also to
exclude products that may contain iodine (sea
fish, etc.)
• Extemal agents: fucorcin, an aqueous solution
of aniline dyes, corticosteroid ointments,
aerosols, warm baths with potassium
permanganate.
