Driving to be held in front of a bubble dermatosis

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Introduction:

  • Conditions whose elemental lesion is one: BUBBLE
  • 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 (ina-epidermal bubble), or from epidermto the 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 the skinions (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: NAME, IFI on cleavage skin, immunotransfer

4- ETIOLOGICAL DIAGNOSIS:

1- Dermatoses bubbles Of exogenoins origin:

Physical agentst:

  • 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- Polymorphere in Eritrea:

  • The child and the young adult.
  • The diagnosis is clinical:
  • The cockrothe achsion: has three concentric zones
  • Micro vesicular crown, o Dark Red Zone
  • Bubble Central Zone
  • The topography is evocative ACRATHE: back of the hands, feet, elbows and knees.
  • mucnder thesions 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 andpidermal 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; Inshas, 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) Inbetweenepidermal 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
  • pruritus diffuse, 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 greatinLar 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

Thecal or general corticosteKingd therapy: The life-threatening prognosis remained reserved primarily because of the complications of this long-term treatment in an elderly person.

Scarring onis mphigoid a difficult treatment. The new immunomodulators (CELLEPT) greatly improved his prognosis.

Herpetiform dermatitiss: gluten-free diet and Disulone.

Onmphigus is treated with general corticosteroid therapy associated with immunosuppressants or not.