Malignant melanoma arising from a congenital pigmented naevus in a patient with sarcoidosis: cause or coincidence?

A case is described of a 40-year-old man who developed sarcoidosis and at the same time malignant change in a congenital pigmented naevus of his foot. It is proposed that the sarcoidosis may have acted as a predisposing cause of the melanoma in this case. The difficulties of diagnosis and management of patients with coexistent malignant melanoma and sarcoidosis are discussed.

Scleromyxoedema and thrombotic thrombocytopaenic purpura: two rare conditions both responding to plasma exchange.

We report the case of a 66-year-old female who over an 18-month period developed severe, disabling scleromyxoedema with pulmonary fibrosis. Treatment with oral prednisolone and melphalan had failed to prevent disease progression. Treatment with a 5-day course of plasma exchange, intravenous cyclophosphamide (500 mg) and methyl-prednisolone (1 g on 3 consecutive days) was unfortunately followed by the development of thrombotic thrombocytopaenic purpura (TTP). After 17 extra plasma exchanges, she recovered and there has been a dramatic improvement in her skin signs. We postulate that the extra plasma exchanges which she received as a consequence of developing TTP have contributed to this result. To our knowledge, TTP has never been associated with scleromyxoedema, but it is likely to be a coincidence in this case.

Familial lichen planus. A report of nine cases from four families with a brief review of the literature.

Nine members of four families, each of whom suffered from lichen planus, are described. The disease appears to differ from the nonfamilial form in its earlier age of onset, tendency to chronicity, increased severity, and the frequent presence of "atypical" disease.

Periodic exophthalmos associated with the blue rubber bleb naevus syndrome: a case report.

A 71-year-old woman with the blue rubber bleb naevus syndrome experienced 4 episodes of self-limiting unilateral exophthalmos. Is is suggested that a vascular malformation in the orbit is responsible for this phenomenon. A biopsy sample was taken of one of the cutaneous lesions and studied by light and transmission electron microscopy. The histological appearances are discussed in relation to possible mechanisms causing this periodic exophthalmos.

Forty years of diarrhoea in a patient with urticaria pigmentosa.

A patient with urticaria pigmentosa who gave a 40-year history of diarrhoea was found to have systemic mastocytosis with gut involvement. The radiological appearance of the gut in this disease, although not widely recognized, are specific and should be looked for carefully in patients with urticaria pigmentosa who complain of gastro-intestinal symptoms. Gastro-intestinal symptoms, due mainly to alterations in bowel motility or peptic ulceration, are said to occur in some 25-50% of cases of systemic mastocytosis (3, 6). These symptoms have usually been ascribed to generalized histamine release acting on the gut, although cases where mast cell infiltration of the bowel has occurred have also been reported (4, 5). In a review of the radiological features (2), increased gastric rugosity with or without evidence of peptic ulceration and nodular space-filling defects of the bowel mucosa were the most commonly found. Occasionally, diffuse thickening of the bowel wall was seen. It was concluded that these appearance were probably due to local release of vasoactive substances causing submucosal oedema following mast cell accumulation in the gut. Another result of such infiltration may be malabsorption (1).

Portuguese-type amyloid neuropathy in an English family.

A 31-year-old woman thought to be suffering from a psychiatric illness was found to have peripheral and autonomic neuropathy, keratoconjunctivitis sicca and vitreous opacities. Her mother had died 10 years previously, aged 42 years from an undiagnosed illness with similar features. Histological proof of amyloidosis was obtained in both cases. This is the second report of familial amyloid neuropathy in an English family.

Pyoderma gangrenosum complicating non-Hodgkin's lymphoma.

A case of pyoderma gangrenosum which occurred in a patient with non-Hodgkin's lymphoma is described. The lesion started in the foot where dye was introduced for a lymphangiogram.