D.H. Mcgibbon

Lichen sclerosus et atrophicus and autoimmunity—a study of 350 women

A study of autoimmune related phenomena in 350 women with histologically confirmed lichen sclerosus et atrophicus revealed that 21.5% had one or more autoimmune related diseases, 21% had one or more first degree relatives with an autoimmune‐related disease, 42% had an autoantibody at a titre > 1:20, and 59.5% had one or more of these autoimmune‐relateda phenomena. No statistically significant differences in the natural history of lichen sclerosus et atrophicus were demonstrated between those patients with autoimmune‐related phenomena and those without.

The anticonvulsant hypersensitivity syndrome.

Summary Anticonvulsants can cause a characteristic hypersensitivity reaction. This multisystem reaction typically presents as fever, mucocutaneous eruptions, lymphadenopathy and hepatitis. There is cross‐reactivity between different anticonvulsants. which complicates subsequent therapy. We report three cases to illustrate both the typical features, and less common complications, of this under‐recognized and life‐threatening syndrome.

Anogenital lichen sclerosus in women.

A study of 350 women with lichen sclerosus, originally made to elucidate the relationship between lichen sclerosus and autoimmunity, led to the amassing of a considerable amount of clinical material. Our review is confined to those with anogenital lesions (342), supplemented by some new cases (15), giving a total of 357 women with biopsy proven lichen sclerosus. It demonstrates the wide age range of the condition, the association with morphoea and lichen planus and the occurrence of squamous cell carcinoma in some cases. It also shows that inappropriate surgery has continued to be carried out for benign disease.

Familial primary cutaneous amyloidosis.

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.

HTLV-1-associated cutaneous disease: a clinicopathoiogical and molecular study of patients from the U. K.

The clinieopathological features of eightpatients with cutaneous disease associated with HTLV‐1 infection are reviewed. All were U. K. residents of West Indian extraction, and two are currently alive. Disease remained confined to the skin in two patients. Five patients with a cutaneous prodromal phase developed leukaemia after a medianduration of 124 months (3months‐21years), and in one of these combination chemotherapy produced a sustained clinical remission for 20 months. Two patients developed cutaneous disease after remission of their leukaemia.

A flow cytometric study of the significance of DNA aneuploidy in cutaneous lesions

Aneuploidy has not to date been demonstrated in any entirely benign condition and thus is held to be implicit of neoplasia. DNA flow cytometry can be used to detect DNA aneuploidy rapidly. A technique has been developed to make possible flow cytometric analysis of formalin‐fixed skin. The technique was validated by study of benign and pre‐malignant dermatoses. Further studies were performed on skin conditions of questionable malignant potential. DNA aneuploidy was demonstrated in lichen sclerosus et atrophicus. Flow cytometry was unable to distinguish keratoacanthomas from squamous cell carcinomas.

Cutaneous malignant melanoma and human immunodeficiency virus (HIV) infection: a report of three cases

Cutaneous malignant melanoma was diagnosed in three patients suffering from human immunodeficiency virus (HIV) infection. Staging at presentation inversely correlated with absolute CD4 count. In addition, a notably sparse lymphocytic inflammatory response to the melanoma was observed in two cases. Established data on melanoma in non‐HIV immunosuppressed patients suggests a poor prognosis for melanoma in HIV disease.

Proximal cutaneous necrosis in association with chronic renal failure

Summary We report two patients with renal failure who developed painful symmetrical ulceration over the proximal thighs and abdominal wall. Histological examination of the ulcerated areas revealed intravascular calcification. This phenomenon has been termed calciphylaxis and is a rare complication of renal failure. The pathogenesis is poorly understood. However, abnormalities of calcium/phosphate metabolism and of coagulation are important.

Malignant angiosarcoma of the scalp: a case report with immunohistochemical studies

A case of angiosarcoma of the scalp is reported. The histogenesis of this tumour is discussed in terms of the ultrastructural and immunohistochemical findings. This type of angiosarcoma is uncommon and carries a poor prognosis; the therapeutic alternatives are discussed.

Acropustulosis of infancy

Acropustulosis of infancy is a syndrome characterized by recurrent pruritic acral vesicopustules. It occurs primarily in black male infants and hitherto has been described mainly in the North American literature. We describe four cases seen in London. This entity, therefore, should be recognized in the U.K., particularly in the differential diagnosis of infantile scabies.