Churg-Strauss syndrome in a district hospital.

Published series on Churg-Strauss syndrome (CSS) come from tertiary referral centres. We retrospectively studied 23 patients with CSS (18 male) seen over 14 years (1982-1995) in a District General Hospital serving a population of 500,000. Mean age of onset of vasculitis was 57, 10-19 years older than in previous series. The commonest clinical features were asthma (22) and eosinophilia > 1.5 x 10(9)/l (21). Systemic vasculitis involving two or more extrapulmonary organs occurred in 22 patients, with specific organ involvement of nervous system (18), joints (13), muscles (13), lungs (11), skin (11), kidneys (11), heart (10), and bowel (7). Various classification systems were applied including the Lanham criteria, which were met in 19 patients; the American College of Rheumatology criteria, met in 14; Churg and Strauss criteria, met in four; and the Chapel Hill Consensus definition, met only in two. ANCA was detected in 10/17 patients where measured. Treatment included corticosteroids (21), cyclophosphamide (8), azathioprine (9), immunoglobulin (2), and methotrexate (1). During follow-up six patients died, two due to myocardial vasculitis (mean age 52 years), three due to infection (mean age 80 years), and one cause unknown. Significant long-term disability was due to asthma in five and neuropathy in six.

Osteosarcoma of the breast associated with fibroadenoma.

We review a rare case of osteosarcoma of the breast. Diagnostic aspects and treatment are discussed and the literature is reviewed.

Malignant 'angioendotheliomatosis'--(intravascular lymphomatosis) an unusual cutaneous lymphoma in rheumatoid arthritis.

A case of cutaneous malignant 'angioendotheliomatosis' (intravascular lymphoma) is described in a patient with RA and Sjögren's syndrome. The association of RA with this rare form of lymphoma has not been reported in the literature.

The presence of myofibroblasts in the dermis of patients with Dupuytren's contracture. A possible source for recurrence.

Samples of skin and underlying cord obtained at dermofasciectomy for Dupuytren's contracture have been examined for the presence of smooth muscle alpha-actin (SM alpha-actin), a marker for myofibroblasts. 15 of the 20 samples stained positively for SM alpha-actin corresponding with areas of hypercellular Dupuytren's tissue. In 12 of these 15 samples SM alpha-actin-positive hypercellular Dupuytren's tissue extended into the dermis, in three cases reaching the epidermis. In eight samples, diffusely distributed cells positive for SM alpha-actin and resembling fibroblasts were seen in the dermis. These cells appeared to be separate from the Dupuytren's foci. The presence of hypercellular foci and isolated fibroblasts positive for SM alpha-actin within the dermis may explain the high recurrence rate of Dupuytren's disease after fasciectomy.

Fatal neutropenic enterocolitis associated with sulphasalazine therapy for rheumatoid arthritis.

Neutropenia is a recognized complication of sulphasalazine therapy in patients with rheumatoid arthritis. It is usually mild, transient and rarely associated with serious sequelae. We describe a patient with rheumatoid arthritis who developed fatal neutropenic enterocolitis complicated by tracheo-oesophageal fistula following treatment with sulphasalazine.

A new protocol for the treatment of stage I cutaneous malignant melanoma; interim results of the first 806 patients treated.

A protocol is described which has been used in a prospective study of 806 patients with stage I malignant melanoma. Excisional biopsies are performed as soon as possible and Breslow thickness and Clark's levels determined. Patients are allocated to low, medium and high risk groups and definitive excision is carried out with a clearance of 2, 20 and 50 mm respectively. Preliminary results are reported on the 806 patients treated over 7 years in three plastic surgery units. No local recurrences have occurred so far in the low and medium risk groups, which suggests that the protocol is safe.

Lymphocytic interstitial pneumonitis in association with Sjogren's syndrome.

We describe a patient with Sjogren's syndrome who developed what appeared to be fibrosing alveolitis. Transbronchial lung biopsy indicated lymphocytic interstitial pneumonitis (LIP), rather than fibrosing alveolitis, which was treated successfully with steroids. Both conditions are well recognized in association with Sjogren's syndrome and the distinction is important in view of the good response to therapy of LIP.

Eosinophilic angiocentric fibrosis of the upper respiratory tract: a mucosal variant of granuloma faciale? A report of three cases.

Three patients with an unusual stenosing lesion affecting the upper respiratory tract are described. The lesion involved the mucosa of the nasal septum and lateral nasal wall in two cases and the mucosa of the sub-glottic region of the larynx in one. The lesion leads to thickening of the sub-mucosal connective tissues by an usual pathological process. It begins as a focal peri-vascular sub-epithelial exudate of eosinophils accompanied by collections of plasma cells and lymphocytes without fibrinoid necrosis. These foci progress to form extensive areas of peri-vascular fibrosis showing a characteristic angiocentric whorling with an 'onion-skin' pattern. This process leads to thickening and matting of the mucosa which becomes adherent to the underlying structures. As fibrosis progresses, the lympho-plasmacytic component regresses but the eosinophils remain. The fibrosis does not resolve and the consequent stenosis requires surgical relief. No other site appears to be affected by this process, but one case was associated with granuloma faciale. The cause is unknown. We have given the term 'eosinophilic angiocentric fibrosis of the upper respiratory tract' to this condition to encompass its cardinal features.

Peutz-Jeghers syndrome and metastasising colonic adenocarcinoma.

A case of metastasising colonic carcinoma associated with Peutz-Jeghers syndrome in a 39 year old man is described. The caecal adenocarcinoma had metastasised widely to regional lymph nodes and was associated with several other colonic Peutz-Jeghers polyps, showing no evidence of dysplasia or malignancy. It was not possible to determine whether the carcinoma had arisen from a Peutz-Jeghers polyp. The patient also had gastric and small intestinal polyps. The serum carcinoembryonic antigen (CEA) was normal at presentation in this patient and in one other reported case. The use of this determination as a screening test for so-called high risk groups is, therefore, not supported by this report. The risk of malignancy is not known. It will only be determined by careful follow up of a defined Peutz-Jeghers syndrome population and comparison of carcinoma incidence with a matched sample of the general population. A national registry of Peutz-Jeghers syndrome patients in the United Kingdom would help to resolve this question.

Percutaneous Trucut lung biopsy in the diagnosis of localised pulmonary lesions.

In a prospective evaluation of percutaneous Trucut needle biopsy for localised intrathoracic lesions in 89 patients histological specimens were obtained in 81. Malignancy was diagnosed in 66 cases. Subsequently definitive histological reports were available in 18 of these patients with complete concordance of malignant cell type. Sixteen patients had non-malignant histological appearances, which were later confirmed objectively in six. In three patients there was no follow up information, but in the remainder the clinical course was entirely consistent with the histological appearances of the biopsy specimens. Adequate specimens were obtained from only two of the five lesions less than 2 cm in diameter. Lesions deeper than 8 cm from the site of biopsy were associated with significantly more haemorrhagic complications than more superficial lesions. Comparison with other series indicates that Trucut needle biopsy which produces histological specimens has greater diagnostic accuracy than cytological techniques for both malignant and non-malignant localised lesions. It is concluded that this technique has a definite place in the investigation of this common problem in carefully selected patients provided that strict attention is paid to the details of the technique.

Metastatic Crohn's disease of thigh and forearm.

Cutaneous involvement with Crohn's disease at a site separated from the gastrointestinal tract by normal skin is termed metastatic Crohn's disease. Such a complication is extremely rare and previous reports have emphasised the flexural distribution of the ulcerated lesions. We report a patient who developed Crohn's disease involving the skin of the thigh and forearm several months after a panproctocolectomy for colorectal Crohn's disease.