Pemphigus

The pemphigus is a rare dermatological disease whose one does not know the cause but which is regarded as a auto-immune Maladie. The cause would be an attack of the antibodies against the proteins désmosomiales, this would thus involve an abnormal adhérance cells between them and thus a achentolyse (separation of the skin and mucous membranes)

Clinical forms

  • the deep pemphigus (in the past pemphigus vulgar) is most frequent in France. It begin with the appearance of bubbles on the mucous membranes, especially on the level of the mouth, often taken for mouth ulcers; the fast rupture of the bubbles is followed by painful erosion which blocks the alimentation.
Then appearance of cutaneous bubbles on the level of the thorax or the scalp; the diagnosis is often carried at this period.

  • the surface pemphigus (gathering variable seborrheic, érythémateux the, foliaceous ones and herpétiformes) is of more misleading clinical presentation. There are no mucous lesions and the cutaneous lesions are surface crusts, even érythémato-squameuses plates;

  • the pemphigus paraneoplasic is exceptional

  • One finds in Brazil a disease which seems to be an endemic and particular form of pemphigus foliaceous, known as “Fogo selvagem” (wild fire)

Biology

The taking away Histologique (Biopsy) is essential and shows a intra-épidermique bubble by Acantholyse suprabasale (pemphigus deep) or under-cornea (pemphigus surface).
The direct immunofluorescence highlights deposits of IgG ± C3 surrounding in “lattice” the kératinocytes.
The positivity of the direct immunofluorescence is a criterion essential to the diagnostic.
The presence of kératinocytes necrotic and a linear deposit of IgG on the dermo-épidermique junction increase the suspicion of a paraneoplasic form.

One detects in the serum of 90% of the patients of the antibody reacting with the epitheliums malpighiens. It is of antidesmogléines 1 in the surface forms and about antidesmogléines 3 in the major forms, associated with antibodies antiplakines in the pemphigus paraneoplasic.

Evolution and Treatment

Formerly mortal, the pemphigus is currently treated by the Corticoïde S (Cortisone) or the Immunosuppresseur S.

After the treatment the evolution is variable according to the individuals.
Certain patients require prolonged treatments.

See too

External bonds

Teaching moulding of wax old presenting a case of foliaceous Pemphigus
Random links:The Band with Picsou | Theophilus Voirol | Greek orthodoxe metropolis of Panama, Central America and the Caribbean | Institutional parrot communication | Giorgio Nardone | Ligne_de_Metaxas