Présentation clinique

Clinical Setting

F 62 ans Lésions bulleuses, sur fond plus ou moins érythémateux, à contenu clair ou sanguinolent, généralisées prédominant sur les extrémités. Pas d'antécédents ou de prise médicamenteuse. Pas de lésion muqueuse. Prurit modéré.

(Peau badigeonnée d'éosine)

F 62 yo Tense large bullous lesions, generalised, mainly the extremities, on a more or less erythematous background. Moderate pruritus. No medical treatment. No specific clinical history.

(Skin covered by eosin)

Lésions erythémateuses infiltrées au niveau du tronc.

Biopsie Chirurgicale (Siège)

Surgical biopsy (Site)

Lésion du tronc

Lesion of the abdomen

IgG

C3

Diagnostic Proposé:

Pemphigoide Bulleuse

Proposed Diagnosis:

Bullous Pemphigoid (Cell-Poor Variant)

 

Arguments

• Tense and often intact blisters arising on normal or erythematous skin. They may measure up to several centimeters in diameter and are typically dome-shaped . Often they contain clear or bloodstained fluid. Any area of the body may be affected, but the blisters are most commonly located about the lower abdomen, the inner aspect of the thighs and on the flexural surfaces of the forearms, the axillae and groins.
• The lesions are often pruritic
• biopsy is taken from an established blister, the changes are most often those of an inflammatory (cell-rich) variant
• A typical finding in bullous pemphigoid is retention of the dermal papillary outline (festooning) which project like sentries into the vesicle cavity
• infiltrate of eosinophils and mononuclears surrounds the blood vessels
• The adjacent papillary dermis is often edematous and, very occasionally, eosinophil microabscesses are a feature
• Cell-poor (non-inflammatory) features are occasionally seen if biopsies are taken from lesions arising on non-inflamed skin
• Bullous pemphigoid is characterized by a linear anti-basement membrane zone antibody using the indirect immunofluorescent technique. Although IgG is invariably present (and most commonly of the IgG4 subclass), other immunoglobulins, including IgE, may be represented
• Complement (C3) is also usually present and is sometimes the sole immunoreactants

For more details go to:

Bullous pemphigoïd

(Ref. Pathology of the skin, PH McKee, 3rd Ed. Elsevier-Mosby)

Pour plus de détails voir:

Pemphigoide bulleuse

(in Dermatologie, H Saurat et al, 3ème Ed. Masson)