Bulleuse Dermatosis

The bulleuses dermatoses are the whole of the dermatological affections which can present bulleuses lesions in their clinical signs.

Private clinic

The elementary lesion is the bubble, one also finds other lesions elementary. Certain bulleuses dermatoses are accompanied by eczema, urticaria, disorder of the pigmentation, ulceration, a prurit and scars.

The clinical context is important for the orientation diagnosis. The age, the pregnancy, the catch of drug give invaluable information to the clinician.

The cytodiagnostic of Tzanck, and the examinations in direct and indirect immunofluorescence help with the diagnosis etiologic.

The general state of the patients, the associated tares must be known to choose the treatment.

Etiology

The causes of bulleuse dermatosis are distributed in a following way:

the Toxidermie S (pigmented erythema fixes, polymorphic erythema, Syndrome of Lyell)
physical Causes (Brûlure, Frottement S) or chemical (Dermite of the meadows)
infectious Causes (bulleux Impétigo, épidermolyse staphylococcic acute, polymorphic erythema infectious)
the late Porphyrie cutaneous
hereditary causes (épidermolyse bulleuse congenital: dystrophic Épidermolyse bulleuse, Épidermolyse bulleuse epidermolytic, Incontinentia pigmenti, Mastocytose bulleuse, Acrodermatitis enteropathica, Porphyrie congenital etc)
autoimmune forms (Pemphigoïde bulleuse, Pemphigus, Pemphigoïde gestationis, Dermatite herpétiforme and épidermolyse bulleuse acquired)

Congenital bulleuse dermatosis

In this group of dermatoses, one gathers those diagnosed in the infant and the child. They are due to genetic anomalies person in charge of a bad cohesion of the various structures of the skin.

One speaks about épidermolyse bulleuse hereditary, that one classifies according to the cleaved cutaneous zones: the simple, jonctionnelle or dystrophic bulleuse épidermolyse.

Other genetic diseases the such enteropathic acrodermatite are due to a deficiency in Zinc.

There exist Génodermatose S like the Incontinentia Pigmenti being accompanied by disorder of the cutaneous pigmentation.

Auto-immune bulleuse dermatosis

Generally acquired, this type of dermatosis is due to the effect of antibody on the components of the skin, like the Desmosome S and hémidesmosome S, proteins of cohesion between cells and extracellular matrix.

Pemphigus

The pemphigus are acquired bulleuses dermatoses. They are expressed in the typical form of the vulgar pemphigus, but there exist rare forms of pemphigus, the such pemphigus seborrheic , the foliaceous pemphigue and the vegetating pemphigus .

Pemphigoïde

The bulleuse pemphigoïde is a definite subepidermic auto-immune dermatosis bulleuse, on the immunological level, by a autoimmunisation against two proteins of structure of the dermo-épidermique junction (basal Membrane), of a molecular mass of 230 kD (called BPAG1 or AgPB230) and of 180 kD (called BPAG2, AgPB180 or collagen XVII), located in the hémidesmosomes.

Other forms

There exist particular forms of autoimmune bulleuses dermatoses.

In the expectant mother after the first quarter, one finds the pemphigoïde gestationis characterized by the bulleuse eruption of the perish-umbilical area, repeating with each pregnancy, and the catch of œstroprogestatives hormones (contraceptive pill)

Comparison

Clinical elements allowing to differentiate the various autoimmune dermatoses bulleuse most characteristic:

Infectious bulleuses dermatoses

Many infectious germs are responsible for bulleuses lesions.

Bulleux Impétigo

Running in the child, the Impétigo streptoccocic or staphyloccocic can be characterized by a surface bulleuse eruption, made small fragile bubbles on a érythémateuse skin. One notes the appearance of crust yellowish on the lesions surrounding the nostrils or the mouth.

Polymorphic erythema

The polymorphic erythema makes party of the bulleuses dermatoses whose certain pathogenic agents are viruses or bacteria (Mycoplasme, herpes virus, chlamydia). The lesions take the aspect of rosettes centered by a bubble and being distributed on the hands, the elbows, the knees and the back. Inconstant, the deterioration of the general state of the patient makes all the gravity of the affection.

Medicamentous bulleuses dermatoses

The catch of drugs and by extension the exposure to toxic substances or irritating even of the aggressions by physical agents (sun) can lead to the development of a bulleuse dermatosis.

Syndrome of Lyell

The syndrome of Lyell is a serious affection started quasi exclusively by the catch of a drug. Among the treatment most often incriminéw, one finds the Sulfamidé S, the Barbiturique S, the anti-inflammatory drugs not stéroïdiens etc

Late cutaneous Porphyrie

Affection with share, this disease can let appear lesions bulleuse of the photo-exposed cutaneous zones the such hands and the face. Drugs can start bulleuses dermatoses by using the sun like Co-factor. These bulleuses lesions are generally cicatricial since they leashes of the cyst while disappearing.