Oleogranulomatous response in lymph nodes associated with emollient use in Netherton's syndrome.

Paraffin-based emollients are widely used in dermatological practice and are not usually absorbed through the skin. We report a case where transcutaneous transfer did occur in the context of damaged skin in Netherton's syndrome, resulting in a reversible lymphadenopathy.

Spindle and pseudoglandular squamous cell carcinoma arising in lichen sclerosus of the vulva.

An 83-year-old woman presented with a vulval mass. Radical vulvectomy was performed and histology showed a unique mixed picture of spindle, pseudoglandular and classical squamous cell carcinoma arising in vulval lichen sclerosus.

Promoter region polymorphism in the human TNF-alpha gene is not associated with lichen sclerosus.

The pathogenesis of lichen sclerosus remains unknown. However, it has been frequently associated clinically with autoimmunity. The MHC haplotype A1, B8, DR3 is associated with many autoimmune conditions and has also been associated with the uncommon allele of the tumour necrosis factor (TNF-alpha) promoter polymorphism. This allele is also associated with higher production of TNF in vivo and in vitro, and thus it has been speculated that it is the TNF-alpha gene which underlies the genetic association of many diseases with the autoimmune haplotype. There have been many reports of HLA associations with lichen scleroses, but these have not been concordant. We therefore decided to analyse the TNF-alpha polymorphism in patients with lichen scleroses to determine if TNF-alpha was likely to play a role in susceptibility or severity of lichen scleroses. No association between alleles of the TNF-alpha polymorphism and lichen scleroses was found.

Vulval involvement in lichen planus: a study of 37 women.

The overall prevalence of vulval involvement in women with lichen planus is unknown. In a series of 37 women with lichen planus, we found vulval lesions in 19 (51%). Vulval lesions are often chronic and may undergo malignant change. All patients presenting with cutaneous or oral lesions of lichen planus should be questioned about, and examined specifically for, signs of genital involvement. Long-term follow-up of these patients is necessary.

Scrotal pyoderma gangrenosum associated with dermatomyositis.

Pyoderma gangrenosum (PG) of the scrotum is extremely rare. We report a case of scrotal PG associated with dermatomyositis. Initial healing was slow despite the use of oral prednisolone and azathioprine. Healing was accelerated by the local use of a potent topical corticosteroid. PG should be considered in the differential diagnosis of ulcerating lesions of the genitalia.

Squamous cell carcinoma arising in vulval lichen planus.

We report two cases of squamous cell carcinoma arising in vulval lichen planus. Patients with lichen planus should be examined for evidence of genital involvement. If this is present, such patients should be closely monitored, and any atypical lesions biopsied.

Interleukin 1 receptor antagonist gene polymorphism association with lichen sclerosus.

Cytokines play key roles in immune responses, inflammation and fibrosis. The balance between levels of cytokines, their receptors and specific inhibitors controls inflammatory reactions in tissues. The pathogenesis of lichen sclerosus is unknown but probably involves cytokine mediators such as interleukin 1 (IL-1) and interleukin 1 receptor antagonist (IL-1ra). The IL-1ra is a competitive inhibitor of IL-1 alpha and IL-1 beta, and therefore is a powerful endogenous anti-inflammatory molecule. The gene encoding IL-1ra (designated IL-1RN) has a variable number tandem repeat polymorphism in intron 2. There are five alleles of the gene corresponding to 2, 3, 4, 5 and 6 repeats of an 86-bp sequence. We have determined allele frequencies in a control population and a group of 78 patients with lichen sclerosus. The frequency of one of the alleles is related to increasing disease severity. Thus, IL-1RN may be a candidate gene or severity factor for lichen sclerosus or may possibly be a linked marker to another, as yet undefined, gene.

Lupus pernio following facial trauma.

Cutaneous sarcoidosis may occur at sites of trauma. Two patients are reported in whom lupus pernio developed in the area of facial fractures. Injury may be a factor in the site of development of lupus pernio in some individuals and may have medico-legal implications. Cutaneous sarcoidosis is known to occur at the sites of scars and trauma but lupus pernio has not been widely recognized to show this phenomenon. We report two patients in whom lupus pernio developed at the site of facial trauma.

Giant alopecic nodule of the scalp: unusual presentation of a cellular blue naevus in an adult.

A giant cellular blue naevus of the scalp is described in a man with presentation in the third decade of life. In contrast with previous reports of children with this condition, there was no abnormality of the underlying skull bones and no evidence of malignancy.