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Background: Vulval carcinoma is a relatively rare disorder that may have various aetiologies. Objectives To document the features and outcome in a series of patients with this disorder., Methods: Retrospective analysis of patients presenting to a vulval clinic over a 5-year period., Results: Twenty-one women presented with a squamous cell carcinoma (SCC) and two with a verrucous carcinoma (VC). The age range was 43-83 years. Twenty-one had well-established (1-30 years) vulval symptoms prior to developing their tumour. Specific tumour-related symptoms ranged from 3 weeks to 11 months. Eight had had a prior diagnosis of lichen sclerosus (LS) or lichen planus (LP), only two of whom were on regular treatment and follow-up. At presentation, 12 patients had clinical signs of LS, three of LP, and five had some changes of both LS and LP. Two patients had multifocal vulval intraepithelial neoplasia (VIN3). Only one had no evidence of any background vulval skin disease. The commonest histological changes noted in the epithelium either adjacent to or distant from the SCC were those of atrophic LS (n = 8), LS with squamous cell hyperplasia (n = 3), LS with hyperplastic foci and lichenoid infiltrate (n = 4), and LS with differentiated VIN3 (n = 1). Four cases demonstrated the changes of LP, and three showed VIN3. All patients were treated surgically and, in those who had lymphadenectomy, four had positive nodes. There have been two deaths due to metastatic disease, and one further patient has developed a second primary SCC at a different site., Conclusions: An underlying skin disorder prior to the development of their carcinoma was found in 22 of 23 patients with vulval SCC and is therefore an important risk factor.

A 67-year-old man presented with discrete mucinous papules on the scalp and fingers, and bilateral carpal tunnel syndrome. Both features remitted spontaneously within several months. The acral distribution, and histopathological features, were in keeping with the recently described acral persistent papular mucinosis. Involvement of the scalp, however, has not previously been described, and transient mucin deposition is unusual. The history of bilateral carpal tunnel compression raises the possibility of extracutaneous involvement, not previously reported in this group of patients.

The clinical details of four fair-skinned women with recurrent blistering and skin fragility are presented. All had considerable sun-exposure and had used sunbeds for years. Specific causes of bullous dermatoses including drug ingestion, porphyria, cutaneous amyloidosis and epidermolysis bullosa acquisita were excluded. Long-term recurrent exposure to high doses of sunbed radiation in chronically sun-damaged skin appears to be a further cause of pseudoporphyria.

Extra-mammary Paget's disease is an uncommon entity. It has been associated with a underlying sweat gland carcinoma. This paper presents a patient with metastatic endocervical carcinoma and vulval Paget's disease. We believe that the Paget's disease developed as a direct consequence of the cervical tumour. In patients with extra-mammary Paget's disease with no sweat gland carcinoma a careful search to exclude other primary malignant tumours is advised.

The ultrastructural localization of the IgA deposits in the skin of 15 patients with linear IgA bullous dermatosis of adults (LAD), 13 with chronic bullous dermatosis of childhood (CBDC) and three with childhood cicatricial pemphigoid (CCP) were studied. The site of the antigen was determined using sera from two LAD, 13 CBDC and two CCP patients. In all 31 patients the IgA was located predominantly below the lamina lucida (sublamina densa). Similarly, the indirect immunoelectron microscopic studies demonstrated the antigen to be present at the same site, below the lamina densa. This suggests that in linear IgA bullous dermatosis the antibody reacts with the antigen in the sublamina densa region of the basement membrane zone.

Five dark-skinned individuals presented with widespread well-demarcated hypopigmented lesions, biopsy of which revealed the histopathological features of mycosis fungoides. Ultrastructural studies showed focal invasion of the epidermis by mycosis cells with degenerative changes in adjacent melanocytes and keratinocytes. The majority of melanocytes exhibited swelling of cytoplasmic organelles and disordered melanogenesis with production of spherical incompletely melanized melanosomes. In addition disintegrating melanocytes were occasionally seen. Perifollicular repigmentation within hypopigmented areas occurred in two patients following clearing of the epidermal infiltrate with PUVA therapy. Mycosis fungoides may present with areas of cutaneous hypopigmentation.

Two cases of toxic epidermal necrolysis are described in patients suffering from pustular psoriasis. In one of these cases there were no recognized predisposing causes. In the other, although there were alternative possibilities, we consider that the most likely cause of the toxic epidermal necrolysis was pustular psoriasis.

Primary cutaneous amyloidosis is uncommon in Europeans. A British family is described in which an extremely rare variant was inherited as an autosomal dominant. The subtlety of physical signs in this family contrasted with the severity of the associated pruritus. Transepidermal elimination of amyloid was a characteristic histological feature.

In a study of 46 patients with nodular prurigo (NP), potential metabolic causes of pruritus, such as anaemia, hepatic dysfunction, uraemia and myxoedema, were present in 50%. Focal causes of pruritus were important in 37% and included insect bites, venous stasis, folliculitis and nummular eczema. Psycho-social disorders were recorded in over 50% of patients and were considered relevant in 33%. Clinical and histological appearances ranged from classical NP to chronic lichenified eczema. Neural hyperplasia was not a prominent feature.

Using an indirect immunoperoxidase technique, both epithelial membrane antigen and carcinoembryonic antigen were identified within the ducts and secretory coils of the eccrine sweat gland. Antibodies to epithelial membrane antigen stained the intercellular canaliculi of the secretory coils, as did those antisera to CEA which showed activity against normal cross-reacting antigen (CEX, NCA). Those without such activity showed minimal or no staining of intercellular canaliculi. There is a difference in antigenic expression between the acinar cells and their intercellular canaliculi, and the cells of eccrine ducts.

Occasional reports have appeared in the literature describing a relationship between pemphigus vulgaris and malignancy (Krain & Bierman, 1974; Tagami et al., 1976). Carcinoma of the ovary, stomach, breast, endometrium and bronchus have all been associated with pemphigus (Krain, 1974). The association between pemphigus and thymoma is well known (Stillmand & Baer, 1972). Pemphigus has also been described in connection with various lymphoproliferative malignancies (Naysmith & Hancock, 1976). The purpose of this paper is to describe a patient who suffered from a rare retroperitoneal tumour in association with pemphigus vulgaris. His serum contained a high titre of anti-skeletal muscle antibody, although he showed no clinical manifestations of myasthenia gravis. He also had immunological manifestations suggestive of lupus erythematosus.