R.C. Holmes

The specific dermatoses of pregnancy.

The terminology of the specific dermatoses of pregnancy has become increasingly confusing, with several names in use for identical clinical disorders. On the basis of our own study of sixty-four patients and a review of the literature, we propose a simplified classification: (1) herpes gestationis (pemphigoid gestationis); (2) polymorphic eruption of pregnancy; (3) prurigo of pregnancy; and (4) pruritic folliculitis of pregnancy.

A comparative study of toxic erythema of pregnancy and herpes gestationis.

We compared the clinical features, histopathology, immunopathology and immunogenetics of 30 patients with toxic erythema of pregnancy and 24 patients with herpes gestationis. Although we found some clinical and histopathological overlap we highlighted several important differences. In toxic erythema of pregnancy prominent striae were frequently present. Herpes gestationis was suggested by the occurrence of periumbilical lesions, acute exacerbations immediately after delivery, and persistence of the eruption for more than 3 weeks post‐partum. In herpes gestationis, immunofluorescence studies were consistently positive, there was a high frequency of HLA‐B8 and an association with autoimmune thyrotoxicosis. Toxic erythema of pregnancy did not share these immunological features. Therefore we feel that toxic erythema of pregnancy and herpes gestationis should continue to be classified as separate disorders.

Clues to the aetiology and pathogenesis of herpes gestationis

In a Study of twenty‐five patients with herpes gestationis we found that 80% possessed the HLA antigen DR3, which confers increased immune responsiveness and a predisposition to ‘auto‐immune disease’. In five patients the development of herpes gestationis coincided with a change in sexual partner, suggesting that the development of herpes gestationis may depend on exposure to an antigen derived from the father. This might share determinants with a component of the basement membrane zone of skin. Although anti‐basement membrane zone antibodies are present in HG it is not clear whether they play a pathogenic role. The infrequency of neonatal involvement and the lack of correlation between immunofluorescence findings and clinical activity in our patients suggested that the antibodies might be a result of tissue damage rather than its cause. Two patients In our study were exceptional in that episodes of herpes gestationis were followed by normal pregnancies. In these patients the relationship of their DR antigens to those of the fetus may have been important in determining whether or not the pregnancy would be affected by herpes gestationis.

The specific dermatoses of pregnancy: a reappraisal with special emphasis on a proposed simplified clinical classification

There is little insight into the pathogeneses of most of the specific dermatoses of pregnancy, and therefore they have remained poorly classified. The terminology has become increasingly confusing with several names in use for similar clinical conditions. In an attempt to rationalise the nomenclature we have critically reviewed the clinical descriptions of the dermatoses specifically related to pregnancy and we have proposed a simplified clinical classification into three broad categories: (1) pemphigoid gestationis, (2) polymorphic eruption of pregnancy and (3) pregnancy prurigo.

Angiokeratoma corporis diffusum in a patient with normal enzyme activities

A 34-year-old man is described with angiokeratoma corporis diffusum. This eruption was once thought to be diagnostic of Anderson-Fabry disease; however, recent studies have shown that it may also occur in the enzyme disorders fucosidosis and sialidosis. In our patient with widespread angiokeratomas, the results of enzyme studies were normal, and there were no systemic problems or significant family history. Our case demonstrates that angiokeratoma corporis diffusum can occur in a benign form without systemic features.

Steatocystoma multiplex with unusually prominent cysts on the face

A patient is described who had unusually large facial cysts which histologically were typical of steatocystoma multiplex. An excellent cosmetic result was obtained after plastic surgery.

An immunoelectron microscopy study of the relationship between herpes gestationis and polymorphic eruption of pregnancy

Five patients with typical bullous herpes gestationis and five patients with polymorphic eruption of pregnancy were studied. Shave biopsies were taken from both involved and uninvolved skin and examined by routine immunofluorescence. They were also examined by immunoelectron microscopy employing a multistep peroxidase‐antiperoxidase technique which has been shown to be more sensitive than immunofluorescence. Despite the increased sensitivity of immunoelectron microscopy all cases of polymorphic eruption of pregnancy were completely negative. Although there is a considerable clinical and histopathological overlap between herpes gestationis and polymorphic eruption of pregnancy our findings indicate that these conditions are pathogenetically distinct and should be classified as separate disorders.

Contact sensitization to p-tertiary butylphenol (PTBP) resin in plastic nail adhesive.

Nail dystrophy and dermatitis of the nail folds and finger tips are reported in three women using the same brand of adhesive (Eylure® Nailfix®) to affix plastic nails (Eylure®). Contact sensitization to p‐tertiary butylphenol (PTBP) resin was demonstrated in ail patients. The subcontractor responsible for supplying the adhesive had changed just prior to the outbreak of symptoms. The new subcontractor subsequently confirmed that there was PTBP resin in their formulation. The subcontract has since reverted to the original company.

Mycosis fungoides: progression towards Sézary syndrome reversed with chlorambucil.

A 74‐year‐old man with mycosis fungoides and strikingly prominent follicular lesions is described. One year after the onset of his eruption he developed features suggestive of progression towards Sézary syndrome with generalized pruritus and increasing erythema and oedema of the face and neck. He also had a significant number of circulating Sézary cells (92% of a total white cell count of 26,700 cells/mm3). Following the administration of chlorambucil, extension of his erythema abated. With continued treatment there was a marked reduction in the number of circulating Sézary cells and his clinical condition improved considerably.

Malignancy associated dermatomyositis with fibrosing alveolitis. Report of a case with a discussion of the management.

A fatal case of dermatomyositis is described in which fibrosing alveolitis (mural type) resulted in a rapid deterioration of respiratory function and death within 6 weeks of the onset of respiratory symptoms.