Herpetiform pemphigus

Orpha code: 208524OMIM code:

Definition

A rare superficial pemphigus disease characterized by severe intractable pruritus with erythematous or urticarial plaques and sometimes vesicles organized in a herpetiform pattern. Mucosae are generally spared. Eosinophilia in peripheral blood and low titers of circulating autoantibodies are observed in many cases. Histology can show an aspect of either pemphigus (superficial or deep), or an intraepidermal infiltrate rich in eosinophils (eosinophilic spongiosis).

Disease data
Classification

Disease

ORPHA code
208524
OMIM code
-
ICD10 code
L10.8
ICD11 code
-

No additional description.

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