Driving to be held in front of a bubble dermatosis

(Last Updated On: 26 February 2020)

Introduction:

• 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:

1- INTERROGATION:

• 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:

Physical agent:

• Trauma: repeated friction, pressure
• Thermal: Frostbite burns
• Solar (photonic): "sunburn"
• Drug phototoxicity
• Photophytodermatosis or "meadow dermatitis"

Chemical agent:

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

Clinical forms:

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.

Clinical:

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).

Biology:

• 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

1– Stevens-Johnson:

• 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:

Clinical:

• 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.

Prognosis:

• 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.

Clinical

• 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

Diagnosis

• 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

Diagnosis

• 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

Etiological treatment:

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.