- The conditions whose primary lesion is a : BUBBLE
- The bubble is an epidermal lifting circumscribed dimension (>0,5cm), filled with a clear liquid.
- Histologiquement : The bubble may result from cleavage between the dermis and epidermis (bubble subepidermal) within the epidermis (bubble intra- epidermal), or necrosis epidermal.
TO BEHAVE :
1- EXAMINATION :
- family history of bullous disease.
- early age.
- Mode of occurrence and evolution of bubbles.
- functional signs : pruritus, local pain.
- Circumstances of appearance : external agents, pregnancy, sun exposure, drug intake.
2- PHYSICAL EXAMINATION :
- The topography the eruption.
- The existence of skin lesions related (erythematous plaques or hives, scars).
- Achieving mucous or lack.
- Abnormal fragility of the skin friction (signedeNikolsky).
3- ADDITIONAL TESTS :
- FNS : eosinophilia
- cytology bubble liquid.
- Skin biopsy for histological examination and direct immunofluorescence (IFD).
- Other : NAME, IFI skin cleaved, immunotransfert
4- ETIOLOGICAL DIAGNOSIS :
1- Dermatoses bullous original exogenously :
Agent physique :
- trauma : friction rehearsals, pression
- Thermal : burns frostbite
- Solar (photonics) :" sunburn "
- drug phototoxicity
- Photophytodermatose or "phytophotodermatitis"
Contact Dermatitis : caustic, allergic
2- Porphyria cutanea tarda :
- hereditary synthesis of heme deficiency disorder uroporphyrinogen decarboxylase (UPG)
- An accumulation derivatives porphyrin photosensitizers in the skin
shapes clinics :
There are two forms :
- An acquired form is revealing middle age often under the influence of a toxic (Alcohol abuse, estrogen drug such, barbiturates, antipaludéens)
- A familial affecting younger patients.
PCT associates :
- bullous lesions affecting mainly the back of the hands and sometimes the face, favored by sun exposure and trauma.
- erosions, scars, of microkystes Abnormal skin fragility.
- Hypertrichosis malar.
- Discolored urine (dark red).
- a hypersidérémie, liver disturbances
- The diagnosis is confirmed by :
- Histological study of the epidermal -under bubble and above, the rise of the urinary excretion of type III uroporphyrin.
- The prognosis is related to the severity of the progressively constituted liver damage.
3– Dermatoses bullous hereditary :
Rares, often expressed in childhood or the early days of life, including epidermolysis bullosa (degree of severity varies, benign forms to lethal).
4– Erythema polymorphous :
- Children and young adults.
- The diagnosis is clinical :
- The lesion badges : comprises three concentric zones
- micro vesicular Crown, o Red Zone dark
- Bullous central area
- The topography is suggestive ACRALE : back of the hands, feet, elbows and knees.
- of the injury mucous as quickly replaced by erosions bubbles painful on the oral mucosa, genital and eye.
- Fever and general malaise accompany, often, the onset of rash.
- Histology is suggestive but not characteristic
- A bubble intra epidermal necrosis keratinocyte
- The IFD is negative.
- Its main cause is infectious
- recurrent herpes,
- Mycoplasma pneumonia.
- overall, the prognosis is good with spontaneous recovery
5- bullous Eruptions :
- Tables clinical appearance and varying severity :
- Syndromes de STEVENS-JOHNSON
- LYELL (toxic epidermal necrolysis -NET)
- These are the most severe forms of drug eruptions because the prognosis is at stake.
- There is a continuum between Stevens-Johnson syndrome (SSJ) and Lyell-NET syndrome that are distinguished only by the surface of necrotic epidermis, major factor in gravity
- Lower than 10 % SC in SJS ;
- Better than 30 % SC in the NET
1– Stevens-Johnson :
- 10 days after initiation of treatment
- Fever, eye burns, pharyngite
- The rash begins on the face and upper trunk.
- Macules érythèmeuses broadcasting in 2 at 5 j
- Blisters or bubbles quickly confluent
- epidermal detachment in "wet towel"
- Nikolsky's sign +
2- necrolysis epidermal acute syndrome from Lyell :
- sudden onset, high fever with AEG
- The rash is first located, is spreading rapidly
- painful erythema, flattened appearance of large bubbles and large epidermal detachment A scalded appearance
- SN is positive and shreds of skin are detached at the lower friction.
- Mucous membrane is constant.
- Despite advances in resuscitation, evolution is often fatal by electrolyte disorders and superinfection.
- The drugs in question : sulfamides ++ ; AINS, salicylés, phenylbutazone, phenobarbital, antibiotics
- An identical Lyell syndrome can be achieved in infants, young children and adults immunocompromised by Staphylococcus aureus. SSSS Staphylococcal Scalded Skin Syndrome)
6- Bullous disorders Autoimmune :
- A heterogeneous group of diseases, infrequent and variable prognosis.
- Their diversity requires the combination of exams Clinic, Anatomopathologique Immunopathologique.
- Characterized by the presence of autoantibodies directed against :
- Desmosomes epidermal keratinocyte
- Structures of the dermo-epidermal.
a) The Dbai intraepidermal : the pemphigus
- Affecting the skin and mucous membranes
- The autoantibodies are directed against proteins of desmosomes and are responsible for the acantholyse a can intraepidermal cleavage.
- There are three main types of pemphigus :
- pemphigus vulgaris where cleavage is suprabasal,
- Pemphigus where cleavage is superficial sub-horny,
- Pemphigus paraneoplastic.
- 50 years but can be at any age
- Characterized by :
– An early very often on the buccal mucosa : painful and recurrent erosions
– Bullous rash of isolated lesions without pruritus, without erythema under- underlying, signe de Nikolski positive.
- Diagnosis is based on :
- Cytodiagnostic (mucous ++) bubble the floor reveals acantholytic cells
- histology, a bubble with intraepidermal acantholysis
- The IFD biopsy of the skin or mucosa péribulleuse: deposits of IgG and C3 on the membrane of keratinocytes, taking an aspect in mesh net.
- In serum, the presence of anti-epidermal intercellular substance antibody.
- Target antigen is desmoglein, a major component of the desmosome.
b) The pemphigus superficial :
- erythematous scaly lesions on the seborrheic areas
- Lack of mucosal lesions.
- Histology is close to the previous form
- It can be induced by medicines comprising the radical sulfydryl (D-penicillamine ; captopril)
c) DBAI Subepidermal pemphigoid :
- The DBAI subepidermal are related to the production of autoantibodies directed against different proteins of the DEJ.
- On distingue :
- The bullous pemphigoid
- The scarring pemphigoid
- The gestational pemphigoid
- Dermatitis herpetiformis
- Linear IgA dermatitis
- epidermolyses bulleuses aquises
1/ Pemphigoïde bulleuse :
- This is the most common DBAI
- After 70 years
- Intense itching before the eruption
- An polymorphous rash strained bubbles of varying size on cupboards erythematous papular urticarial
- The scarcity of mucosal
- The absence of signs of Nikolski
- Une fickle éosinophilie
- Biopsy with histological sub-epidermal bubble
- IFD linear deposits of IgG and complement in basement membrane zone.
- IFI : presence of anti-basement membrane antibodies circulating
- Two target antigens were identified corresponding to two proteins hemidesmosomes.
2/ cicatricial pemphigoid :
- It is characterized by an elective mucosal in the elderly :
- Gingivitis, stomatitis bulleuse
- Conjunctivitis synéchiante with risk of blindness.
- The diagnosis is similar to that of bullous pemphigoid.
3/ Dermatitis herpetiformis :
- The teenager or young adult
- diffuse itching, long isolated.
- An eruption of vesico-bubble annular array on closets erythematous papular, symmetrical willingly
- Associated with gluten enteropathy, usually asymptomatic.
- Changes in spurts
- Histology shows cleavage subepidermal associated with micro-abscess the papillary dermis with neutrophils and eosinophils.
- DFI's presence deposits granular d'IgA atop the dermal papillae
- The search for serum autoantibodies is negative.
- Circulating antibodies antireticulin, antiendomysium,antigliadine et antitransglutaminase
- The biopsy of the small intestine shows atrophy villositaire
4/ gestational pemphigoid :
- 2nd and 3rd quarter often in the periumbilical region
- May recur in subsequent pregnancies.
- The fetal prognosis is dominated by the risk of prematurity and low birthweight Fetal. 5/ Linear IgA dermatosis
- Occurs at any age especially in children
- Characterized by a deposition of IgA to the IFD.
TO BEHAVE :
- The severity of the condition must be assessed on the extent of the blistering rash and general condition.
- All bullous dermatosis extended warrant hospitalization ;
- epidermal necrolysis and pemphigus extensive.
- A hydro electrolytic rebalancing.
- nutritional intake suitable and sufficient
- Symptomatic treatment consists in evacuating the liquid contents and disinfect
etiological treatment :
The treatment of major diseases autoimmune bullous based mainly on
The corticosteroids locale or General : The prognosis remains reserved mainly due to complications of this long-term treatment in the elderly.
The pemphigoïde scarring is a difficult treatment. The novel immunomodulatory (CELLEPT) have greatly improved prognosis.
The dermatitis herpetiformis : gluten-free diet and dapsone.
The pemphigus is treated with corticosteroids with or without immunosuppressive.