Definitions- general :
- group of many diseases and heterogeneous. secondary to achieving the various constituents of the skin : dermis, the epidermis or the dermal-epidermal junction.
- Their causes are multiple, Hereditary or acquired, foremost among which drug eruptions and autoimmune diseases.
- The diagnosis is usually based on clinical examination and histology, often supplemented by immunological tests.
- Lesions in relief, translucent, from 1 at 2 mm, containing a clear serous fluid, located in healthy skin (example : varicella) or erythematous skin (example : eczema).
- raised lesions whose size is larger than the vesicles (5 mm to several cm) containing a liquid that may be clear, yellowish, or hemorrhagic, elapsing after fracture
sign de Nikolsky :
- sign from Nikolsky (bullous detachment caused by gentle rubbing of healthy skin : in Lyell's syndrome).
- erosion (or exulcération) : superficial loss of substance flat bottom, healing without scarring.
- visible lesions, secondary coagulation of serous exudate, bleeding or purulent, that correspond to a different evolutionary stage of primitive elementary lesions : bubbles, vesicles, pustules.
REMINDER FROM THE STRUCTURE FROM THE SKIN :
- The skin coating comprises the skin and its annexes.
- The skin is made, s & rsquo; outside to the & rsquo; interior by 3 distinct zones: l & rsquo; epidermis, the dermis and the & rsquo; hypoderme.
1) The epidermis The epidermis East a epithelium malpighien pluristratifié compound from 5 couches cell:
2) The dermis consists connective member :
- d & rsquo; a substance basic,
- from connective fibers
- d & rsquo; elements cell (fibroblasts, fibrocytes).
3) L’hypoderme : It is a tissue adipose Split in lobules through of the bays conjunctive.
- In the dermis and l’hypoderme cheminent the formations vascular and nerve
4) The annexes : The skin contains various formations say annexes:
– the follicle Pilosebaceous form of hair and from the gland sebaceous appendue at canal pilaris.(1,2,3)
– the glands antiperspirants eccrines and apocrines.(4,5)
systems from junction interkératinocytaire and JDE.
- The cohesion from the skin and to solidity are insured through of the systems d & rsquo; membership located, d & rsquo; a part between the keratinocyte, else part between l & rsquo; epidermis and the dermis. The main structure ensuring membership interkératinocytaire East the desmosome.
the dermo epidermal junction :
Interface between epidermis and dermis The JDE is a zone d & rsquo; membership between l & rsquo; epidermis and the dermis She East developed through the keratinocytes and fibroblasts ensures epidermal adhesion to the dermis
mechanisms from formation of the bubbles :
- It is the alteration from these different systems d & rsquo; membership through of the process divers who East at originally from the formation of the thisthem.
– The loss from membership interkératinocytaire, or acantholyse, results the formation from bubbles intraepidermal,
– while that achieving the DEJ causes the formation of a bubble subepidermal.
– Bubbles through necrosis epidermal: The necrosis keratinocyte can result d & rsquo; a allergy drug (syndrome de Lyell, The Stevens-Johnson) or physical aggression of the epidermis (gelures, second degree burns)
– Bubbles original auto-immune: The different protein component the systems from junction can to be the target d’autoanticorps. Antibodies directed against desmosomal components are the origin of pemphigus. Antibodies against components of the DEJ are responsible for diseases autoimmune bullous subepidermal.
– Bubbles of toxin or metabolic: secondary to the action of a bacterial toxin (exfoliatine staphylococcique). In porphyria cutanea tarda, this is an anomaly of heme metabolism causing accumulation of phototoxic products.
– Bubbles through anomaly hereditary. of the anomalies genetic from the synthesis from some constituents of the structures membership are causing bullous genodermatoses. This is the case of bullous congenital epidermolysis (dermal-epidermal bubbles)
– Bubbles spongiotic: All the dermatoses accompanied a edema interkératinocytaire (spongiose) can give intraepidermal bubbles related to hypertension and keratinocyte suffering. This is the case of acute eczema and certain viral dermatoses
DIAGNOSTIC ETIOLOGICAL :
- all toxiderma bullous need a hospitalization for surveillance, even and the forms are minor Frequently what the syndromes from Lyell and from Stevens-Johnson.
- items d’orientation:
- l & rsquo; age of patient (pemphigoïde bullous of topic age, epidermolysis bullous hereditary, epidermolysis staphylococcique and syndrome from Kaposi-Juliusberg from l & rsquo; child)
- The antecedents personal and family (existence from case family for the dermatoses bullous congenital), the mode scalable (the character quickly extensive being rather in favor d & rsquo; a toxiderma or d & rsquo; a origin infectious)
- l’existence from signs functional Related (pruritus frequent in the pemphigoïde bullous),
- the existence of triggers (pregnancy pemphigoid gestationis, sun exposure for porphyria cutaneous late, taken drug in the toxidermies bullous)
EXAM CLINIQUE :
- specify the characteristics of the injury : cut of the bubbles, aspect from the skin Underlying, presence from cocardes in favor erythema multiforme, looking for Nikolsky's sign, topography of lesions, mucosal lesions and cutaneous signs and associated extracutaneous.
- resounding bullous disease : dehydration, malnutrition, surinfection.
- assess evolution grace at a count daily of Name from bubbles.
REVIEWS ADDITIONAL :
- The diagnostic etiological:
- A eosinophilia East sought sure the cell count sanguine (NFS) and is a Well sign in favor d & rsquo; a pemphigoïde bulleuse.
- The cytodiagnostic from Tzanck research of the cell acantholytiques in favor a pemphigus, or of the cell ballonnisées in favor d & rsquo; a infection through a virus from herpes.
- The biopsy d & rsquo; a bubble recent precise the level of cleavage intraepidermal or subepidermal.
The data immunopathologiques allow the diagnostic specific of the diseases bullous Autoimmune ;
- review in immunofluorescence direct (IFD) d & rsquo; a biopsy from skin péribulleuse
- review of serum in immunofluorescence indirect (IFI) looking the presence d’autoanticorps antiépiderme or anti junction dermoepidermique
DIAGNOSTIC FROM GRAVITY :
- The peeling of the skin leads to a loss of physiological functions of the skin.
- The risk of superinfection, dehydration and malnutrition are correlated with the extent of the detachment and terrain (existence of defects).
1/ Erythema polymorphous bullous :
- erythema polymorphous East a syndrome mucocutaneous.
signs clinics :
- The board clinique typical: occurrence from injury in « badges » formed from many zones concentric dont the central East bullous
- reaching mucosa is intensity and frequency variables: postbulleuses erosions of the oral mucosa. of the violations genital and conjunctival are possible.
- Topography: predominance acrale of the injury (palms, plants).
- The alteration from the state general East moderate
- erythema polymorphous: syndrome answering at from very many etiologies making intervene a mechanism d’hypersenbility.
- erythema postherpetic polymorph is the most common
- Outraged the care local, the repose sure the treatment from the cause when East found
2/ SYNDROME FROM STEVENS-JOHNSON AND SYNDROME FROM LYELL (OR necrolysis EPIDERMAL TOXIC) :
Syndrome The Stevens-Johnson :
- This syndrome East currently close of syndrome from Lyell.
- of the bullous and haemorrhagic lesions in the oral cavity, associated with crusted lesions of the external lips side. Genital lesions are frequently associated, as well as conjunctival lesions that are the severity of the syndrome.
- The state general East often thirsty
- Topography central with reached of trunk.
- Etiology: pharmaceuticals ++++
Syndrome from Lyell :
- The is a syndrome mucocutaneous grave, secondary at a necrosis acute from l & rsquo; epidermis, the plus often original médicamenteuse.
signs clinics :
- prodromes type like illness.
- The beginning East the plus often brutal, the injury mucous before habitually the injury skin.
- reaching mucous, often intense, includes from manifestations eye (conjunctivitis, keratitis) and buccopharyngées, sometimes related at a reached tracheobronchial grave, oesophagienne and genital.
- reaching cutaneous East incorporated initially through of the injury erythematous diffuses or in macules confluent evolving in 1 at 3 days towards a peeling off cutaneous. The skin take so a aspect from « laundry wet » with sign from Nikolsky .
- alteration important general condition
- of the violations visceral can to be encountered : leucopénie, thrombocytopenia, anemia, liver and pancreatic, and mostly reached bronchopulmonary from prognosis very pejorative.
histology: necrosis from the all the height from the epidermis with peeling off bullous junctional ++++
- A hospitalization in a specialized center is needed as soon as the diagnosis is made.
- The treatment repose sure a resuscitation electrolyte suitable, of the contributions protidiques and caloric important, of the measures from Warming and sure of the care antiseptic particularly rigorous.
3/ BUBBLES ORIGINAL INFECTIOUS :
- impetigo bullous streptococcique (streptococcus b-hemolytic of group A) or staphylococcique (staph golden) is common among the & rsquo; child, favored by the lack of hygiene and sometimes rampant as small school or family epidemics due to its highly contagious.
- bubbles intraepidermal very superficial, sub-corneas,
- topography gladly periorificial.
- The bubbles evolve towards the formation from crusts mélicériques yellowish characteristics.
- epidermolysis staphylococcique
- epidermolysis staphylococcique, rare in l & rsquo; adult, East secondary at the secretion d & rsquo; a toxins through some staphylococci gold.
4/ DISEASES BULLOUS AUTOIMMUNE :
- group from diseases rares dont the diagnostic specific need the recourse at dermatologist and the prescription exam spécialisésA / Diseases from the JDE
- The diseases bullous from the JDE are multiples and are linked at the production d’autoanticorps directed against different protein component the JDE.
- Type from description: The Pemphigoïde bullous
- It is the Dermatose bullous auto-immune from l & rsquo; adult the plus common. She occurs mostly in of the topics elderly (age way : 75 years). The beginning: signs non specific : pruritus isolated, placards eczematiform or urticarial.
- The bubbles based on an erythematous base, urticaria or healthy skin.
- strained, solid, at liquid clair, from big cut and prevail from way symmetrical sure the trunk and the racine of the membres.
- Nikolsky : –
exams Additional :
- A eosinophilia sanguine.
- histology watch a bubble subepidermal.
- L’IFD: fixation linear d’IgG and from C3 the long from the JDE.
- L'IFI: antibody circulating remarkable the JDE ;
- bains antiseptic, hydration and nutrition compensating the losses Electrolyte and protein.
- The treatment classical repose sure the corticosteroids General or sure the dermocorticoides very fort
4/ mPHIGUS :
- The pemphigus are of the diseases Autoimmune rares who affecting the skin and the mucous.
- The autoanticorps present in the serums of the ill are directed against of the constituents of desmosomes.
- On two main types of pemphigus :
– the pemphigus vulgar, or the cleavage East suprabasal ;
– the pemphigus superficial, or the cleavage East sub-horny ;
Type from description: The pemphigus vulgar
- topics young (40 years)
- beginning often mucous mouth with of the erosions post bullous extended and painful
- Reaching genital and nasal also common
- Reaching cutaneous non CASTROTHEODORICIENS: bubbles flanges sure skin healthy predominant in periungueal, gutter vertebral, face and scalp
- Nikolsky +++
exams Additional :
- The diagnostic East confirmed through review histologic d & rsquo; a bubble recent (cleavage intra epidermal deep.
- IFD: presence d’IgG and from complement at the surface of the keratinocyte: a aspect in « mesh from filet »)
- IFI: setting in evidence antibody circulating IgG directed against the surface of the keratinocyte.
- Care local +++
- corticotherpie +/- immunosuppressive
5/ DISEASES BULLOUS ORIGINAL METABOLIC :
- porphyrie cutanea tarda
- The porphyries skin are of the diseases the plus often hereditary, officials from l’accumulation from porphyrine photosensibilisantes through deficits enzyme portant sure the metabolism from heme
6/ BUBBLES FROM CAUSE EXTERNAL :
- Chemical: application from products caustics,
- bites insect (prurigo strophulus)
- agents physical thermal (burns or exposition at cold) or mechanical(bubbles dues to the friction)
Courses of Dr. Laroum. C – Faculty of Constantine