ABSTRACT
Herpes gestationis (HG) is a rare autoimmune disease of pregnancy. We report on five patients in whom the clinical diagnosis was confirmed by histology, immunofluorescence and immunoblot studies. The time of onset ranged from the 8th week of pregnancy to the 1st day post partum. In one patient erosions and ulcerations of the oral cavity predominated while the remainder of the skin was nearly unaffected. One patient had intense pruritus without any skin eruptions during the final 8 weeks of her pregnancy, developing bullous lesions on the 1st day post partum. The other patients initially suffered from pruritic polymorphic skin lesions, followed by vesiculae and bullae. In the serum of all patients, the complement-fixing HG factor was found. Immunoblot studies with epidermal and dermal extracts demonstrated the binding of the antibodies to a 180-kD protein of the epidermis. All patients improved markedly with medium dosages of systemic corticosteroids. In four cases the newborns were healthy and mature, and in one case, the infant met all criteria for a "small-for-dates" baby. We discuss both clinical spectrum and diagnostic options of HG and review new aspects related to the pathogenesis of this disease.
Subject(s)
Pemphigoid Gestationis/diagnosis , Adult , Autoantigens/analysis , Betamethasone/administration & dosage , Blotting, Western , Complement C3/analysis , Female , Fluorescent Antibody Technique , Gestational Age , Humans , Immunoglobulin G/analysis , Infant, Newborn , Mouth Mucosa/pathology , Pemphigoid Gestationis/congenital , Pemphigoid Gestationis/drug therapy , Pemphigoid Gestationis/pathology , Prednisolone/administration & dosage , Pregnancy , Pregnancy Outcome , Skin/pathologyABSTRACT
We report on an 84-year-old female patient who presented with abundant firm skin nodules and massive lymphoedema restricted to the left leg. Metastasizing eccrine porocarcinoma was diagnosed by the unusual circumscribed pattern of the cutaneous metastases and the histological detection of intraepidermal and intradermal PAS-positive tumour cells. This diagnosis was established by the histopathological reexamination of a small skin tumour on the left ankle, which had been misinterpreted as actinic keratosis 5 years before. The initiated local radiation therapy with fast neurons and cobalt-60 resulted in partial regression of the cutaneous metastases and lymphoedema, but was not able to hamper the fatal outcome directly resulting from tumour cachexia.