- Conditions whose elemental lesion is one: BULLE
- The bubble is a circumscribed epidermal heave of size (0.5cm), filled with a clear liquid.
- Histologically: The bubble may result from a cleavage between the derma and epidermis (sub-epidermal bubble) inside the epidermis (intra-epidermal bubble), or from epidermal necrosis.
CONDUCT TO HOLD:
- Family history of bubble disease.
- Start age.
- How bubbles appear and evolve.
- Functional signs: pruritus, local pain.
- Circumstances of appearance: external agents, pregnancy, sun exposure, medication.
2- CLINICAL EXAMINATION:
- The topography of the eruption.
- The existence of associated skin lesions (erythematous or urticarian plaques, scars).
- The damage to the mucous membranes or its absence.
- The abnormal fragility of the skin rubbing (signedeNikolsky).
3- ADDITIONAL EXAMINATIONS:
- FNS: Blood eosinophilia
- Cytological examination of the bubble liquid.
- Skin biopsy for histological examination and direct immunofluorescence (DFI).
- Others: IME, IFI on cleavage skin, immunotransfer
4- ETIOLOGICAL DIAGNOSIS:
1- Dermatoses bubbles of exogenous origin:
- Trauma: repeated friction, pressure
- Thermal: Frostbite burns
- Solar (photonic): "sunburn"
- Drug phototoxicity
- Photophytodermatosis or "meadow dermatitis"
Contact dermatitis: caustic, allergic
2- Late skin porphyria:
- Hereditary disorder of heme synthesis by decarboxylase uroporphyrinogen (UPG)
- An accumulation of photosensitizing porphyrinic derivatives in the Skin
There are two forms:
- An acquired form that is in its fifties often under the effect of a toxic (alcohol abuse, medication such as estrogen, barbiturates, antimalarials)
- A family form affecting the youngest subjects.
The PCT combines:
- Bubble lesions mainly affect the back of the hands and sometimes the face, favored by sun exposure and trauma.
- Erosions, scars, microcysts – Abnormal skin fragility.
- Malar hypertrichosis.
- Abnormal urine colour (dark red).
- Hypersideremia, liver disturbances
- The diagnosis is confirmed by:
- The histological study of the bubble-under epidermal and above all, the elevation of urinary elimination of type III uroporphyrins.
- The prognosis is related to the severity of progressively formed liver damage.
3- Hereditary bubble dermatoses:
Rare, often expressed from childhood or the first days of life, especially bubble epidermolysis (very variable degree of severity, benign forms to lethal).
4- Polymorphic Eritrea:
- The child and the young adult.
- The diagnosis is clinical:
- The cockroach lesion: has three concentric zones
- Micro vesicular crown, o Dark Red Zone
- Bubble Central Zone
- The topography is evocative ACRALE: back of the hands, feet, elbows and knees.
- Mucous lesions in the form of bubbles quickly replaced by painful erosions on the oral, genital and eye mucous membranes.
- Fever and general discomfort often accompany the onset of the rash.
- Histology is evocative but not characteristic
- An intra-epidermal bubble by keratinocytic necrosis
- The IFD is negative.
- Its main etiology is infectious
- Recurrent Herpes,
- Mycoplasma pneumonia.
- Overall, the prognosis is good with spontaneous healing
5- Bubble toxidermies:
- Clinical tables of varying appearance and severity:
- Syndromes de STEVENS-JOHNSON
- LYELL (toxic epidermal necrolysis -NET)
- These are the most severe forms of toxidermies because the life-threatening condition is at stake.
- There is a continuum between Stevens-Johnson syndrome (SSJ) and Lyell-NET syndrome that are distinguished only by the necrotized epidermis surface, a major factor in severity
- Less than 10% of the SC in the SJS;
- More than 30% of SC in net
- 10 days after introduction of treatment
- Fever, eye burns, pharyngitis
- The rash starts in the face and upper part of the trunk.
- Erythemaous macules diffuse in 2 to 5 days
- Appearance of rapidly confluent blisters or bubbles
- Epidermal detachment in "wet linen"
- Nikolsky's sign
2- Acute epidermal necrolysis Lyell syndrome:
- Brutal start, high fever with AEG
- The eruption is first localized, quickly spread
- Painful erythema, appearance of large flattened bubbles and large epidermal detachments A scalding appearance
- SN is positive and shreds of skin come off at the slightest friction.
- The reach of the mucous membranes is constant.
- Despite advances in resuscitation, evolution is often fatal due to electrolytic disorders and overinfection.
- The drugs involved: sulfamides; INSAs, salicyles, phenylbutazone, phenobarbital, antibiotics
- A syndrome similar to Lyell can be achieved in infants, young children and adults immunosuppressed by golden staph. SSSS Staphylococcal Scalded Skin Syndrome)
6- Dermatoses autoimmune bubbles:
- Heterogeneous group of diseases, infrequent and variable prognosis.
- Their diversity requires the combination of Clinical, Immunopathological Immunopathological Anatomopathological Examinations.
- Characterized by the presence of auto-antibodies directed against:
- Desmosomes of the keratinocytes of the epidermis
- Structures of the dermo-epidermal junction.
a) Intra–epidermal DBAIs: pemphigus
- Touch the skin and mucous membranes
- AutoCAs are directed against desmosome proteins and are responsible for acantholysis and intraepidermal cleavage.
- There are three main types of pemphigus:
- Vulgar Pemphigus where the cleavage is suprabasal,
- Superficial pemphigus where the cleavage is under-horned,
- Parneoplasic Pemphigus.
- 50 years old but can be at any age
- Characterized by:
– A very often onset on the oral mucosa: painful and recurrent erosions
– An eruption of bubble lesions isolated without pruritus, without underlying rash, sign of Nikolski positive.
- The diagnosis is based on:
- Cytodiagnostic (mucous membranes) of the bubble floor reveals acantholytic cells
- Histology, an intra-epidermal bubble with acantholysis
- The DFI of skin biopsy or peribullous mucous membrane: IgG and C3 deposits on the keratinocyte membrane, taking on a mesh aspect.
- In the serum, the presence of anti-substance anti-substance epidermal.
- Target antigen is desmoglein, a major component of desmosome.
b) The superficial pemphigus:
- Erythemato-scaly lesions on seborrheic regions
- No mucous lesions.
- Histology is close to the previous form
- It can be induced by drugs containing radical sulfydryl (D-penicillamine; captopril)
c) DBAI sub-epidermal pemphigoides:
- Sub-epidermal DBAI are linked to the production of auto-antibodies directed against different JDE proteins.
- We can see:
- The bubble pemphigoid
- Scarring Pemphigoid
- The Gravidic Pemphigoid
- Herpetiform dermatitis
- Linear IgA dermatitis
- epidermolysis bubbles aquises
1/ Pemphigoid bubble:
- This is the most common of the DBAIs
- After 70 years
- Intense pruritus before eruption
- A polymorphic eruption with tense bubbles of varying size on urticarian erythemato-papuleous closets
- The rarity of mucous membrane damage
- The absence of Nikolski's sign
- Inconsistent eosinophilia
- Biopsy with histology a sub-epidermal bubble
- IFD of IgG and complement linear deposits in the basal membrane area.
- IFI: presence of circulating anti-membrane basal antibodies
- Two targeted antigens were identified corresponding to two hemidesmosome proteins.
2/ Scarred Pemphigoid:
- It is characterized by elective impairment of the mucous membranes in the elderly:
- Gingivitis, bully stomatitis
- Synechiating conjunctivitis with risk of blindness.
- The diagnosis is similar to that of the bubble pemphigoid.
3/ Herpetiform dermatitis:
- Teenager or young adult
- Diffuse prurit, long isolated.
- An eruption of annular-clustered blisters on erythemato-papulent closets, willingly symmetrical
- Associated with gluten enteropathy, most often asymptomatic.
- Evolution by thrusts
- Histology shows an sub-epidermal cleavage associated with micro-abscesses of the papillary derma to polynuclear neutrophils and eosinophils.
- IFD the presence of granular IgA deposits at the top of dermal taste buds
- The search for serum autoantibodies is negative.
- Circulating antibodies antireticulin, antiendomysium, antigliadine and antitransglutaminase
- Hail biopsy shows vositaire atrophy
4/ Gravidic Pemphigoid:
- 2nd and 3rd trimester often in the peri-umbilical and
- May recur in later pregnancies.
- Fetal prognosis is dominated by the risk of prematurity and low fetal weight. 5/ Linear IgA Dermatosis
- Happens at any age, especially in children
- Characterized by an IgA deposit at the IFD.
CONDUCT TO HOLD:
- The severity of the condition should be assessed on the extent of the bubble rash and the general condition.
- All extensive bubble dermatoses warrant hospitalization;
- Epidermal or pemphigus necrolysis very extensive.
- An electrolytic hydro rebalancing.
- Adequate and adequate nutritional intake
- Symptomatic treatment involves evacuating the liquid contents and disinfecting
The treatment of major autoimmune bubble diseases relies mainly on
Local or general corticosteroid therapy: The life-threatening prognosis remained reserved primarily because of the complications of this long-term treatment in an elderly person.
Scarring pemphigoid is a difficult treatment. The new immunomodulators (CELLEPT) greatly improved his prognosis.
Herpetiform dermatitis: gluten-free diet and Disulone.
Pemphigus is treated with general corticosteroid therapy associated with immunosuppressants or not.