cation, prurigo-like papules and hyperpigmentation. Other manifestations include urticaria, erythema multiforme, ichthyosis, erythroderma, bullae and.
Journal of the Royal Society of Medicine Volume 79 August 1986
483
progressive systemic sclerosis7. This appears to be due to a keloidal response to the early inflammatory component of scleroderma in predisposed individuals. In this case, however, there was no history of Raynaud's phenomenon, no soft tissue calcification or true scerodactyly. Barium studies were normal and there were no histologic changes of morphoea even on deep surgical biopsy - thus there is no evidence of scleroderma.
References
Figure 3. Biopsy of keloid nodule showing interwoven bundles of collagen with cellular proliferation inbetween
mitoses. The changes were in keeping with the early cellular, proliferative phase of keloid formation. They could, however, be possibly interpreted as being dermal manifestations of the polyfibromatosis syndrome. To date this man's disease has proved totally resistant to therapy. Several treatments including topical, intralesional and systemic steroids, paraminobenzoic acid and high-dose penicillamine have failed to halt the progression of this disabling destructive condition. Discussion The fibromatoses cover a broad spectrum of benign fibrous proliferations which may be divided into two major groups: the superficial (or fascial) fibromatoses and the deep (or musculoaponeurotic) fibromatoses1. The superficial fibromatoses comprise palmar, plantar and penile fibromatosis and knuckle pads; the deep fibromatoses consist of extra-abdominal, abdominal and intra-abdominal desmoids. Our case predominantly fulfils the criteria of a superficial fibromatosis with the added manifestation of an erosive arthropathy. The superficial group of fibromatoses are usually slow-growing and limited in extent. The clinical course may be divided into an early cellular, proliferative phase and a late collagenous, contractile phase. Our case appears to represent an unusually rapid and aggressive form of superficial fibromatosis associated with marked, spontaneous disfiguring keloid proliferations. Palmar fibromatosis (Dupuytren's contracture) is associated with plantar fibromatosis in up to 20% of cases and penile fibromatosis (Peyronie's disease) in 1-3% of cases. An increased incidence of knuckle pads has been observed2. There is also an increased incidence in epileptic3 and diabetic patients4 and patients suffering from chronic alcoholism and hepatic cirrhosis5. This case had none of these predisposing disorders. There are no previous reports of palnar and penile fibromatosis occurring in association with keloid formation, but there are reports of coexisting knuckle pads and keloid1. Although there is an increased incidence of keloid formation in Negroes, palmar fibromatosis is rarely encountered in blacks. Keloids have occurred within areas of localized scleroderma6 and keloid-like lesions have been described in thickened areas of skin in patients with
1 Enzinger FM, Weiss SW. Fibromatoses. In: Soft tissue tumours. St Louis: CV Mosby, 1983:45-70 2 Skoog T. Dupuytren's contracture. Postgrad Med 1957;21:91 3 Arieff AJ, Bell J. Epilepsy and Dupuytren's contracture. Neurology 1956;6:115 4 Davis JS, Finesilver EM. Dupuytren's contracture with a note on the incidence of contracture in diabetes. Arch Surg 1932;24:933 5 Pojer J, Radivojevic M, Williams TF. Dupuytren's disease. Its association with abnormal liver function in alcoholism and epilepsy. Arch Intern Med 1972;129:561 6 Jablonska A. Circumscribed scleroderma. In: Jablonska A, ed. Scleroderma and pseudoscleroderma. Warsaw: Polish Medical, 1975:59-60 7 James WD, Berger TG, Butler DF, et al. Nodular (keloidal) scleroderma. J Am Acad Dermatol 1984;11:1111-4
(Accepted 3 April 1986)
Endoscopic appearances in Whipple's disease
J Mayberry MD MRCP P Furness BA BM M Austin MB BCh P Toghili MD FRCP Queen's Medical Centre, University Hospital of Nottingham Keywords: Whipple's disease, endoscopy, histology
Whipple's disease is an extremely rare disorder characterized by a protracted course with multisystem involvement. In his original paper, Whipple' described the fatal illness of a young doctor who suffered from persistent diarrhoea and progressive weight loss. Later publications have emphasized the general effects of this disease which have included arthropathy, neurological impairment and pigmentation2. Up to 1947 all cases were diagnosed at post-mortem examination, but more recently the diagnosis has been established by jejunal biopsy2. Endoscopy has allowed direct visualization of the duodenum together with directed biopsy of patchy
abnormalities3~-10 Case report
0141-0768/86/ 080483-03/$02.00/0
KL was a 44-year-old, unemployed, mentally @1986 retarded man, who first presented 2 years earlier The Royal with 15kg weight loss. He had been ill for 6 months, Society of during which time he had a poor appetite and had Medicine
484
Journal of the Royal Society of Medicine Volume 79 August 1986
been tired and short of breath. He had cerebal palsy at birth and his mental age of 6 made communication difficult. However, his father described his son's continued coughing, anorexia, extreme lethargy and inability to climb the stairs of their flat. On examination he looked very ill and was wasted, with a weight * of 54.1 kg. Examination of the cardiovascular and respiratory systems revealed no abnormality and he had a normal blood pressure of 140/90 mmHg. There were areas of pigmentation on the abdomen but no palpable masses. Apart from his mental impairment, no focal neurological abnormalities were found. Investigations showed: haemoglobin 6.4g/dl with microcytic hypochromic changes; ESR (Westergren) Figure 2. Duodenal villi, distended by an infiltrate of 68 mm at one hour; true cobalamins normal at PAS-positive foamy macrophages and by spaces with the 520 mg/l (normal range 200-900) and serum folate appearance of dilated lymphatics. (H & Ex 110; reduced low at 2.3 gg/l (normal range 2.6-14); red cell folate 480/%) normal at 180 jg/l (normal range 130-600); alpha-1 acid glycoprotein raised at 3.3 g/l (normal rangeW 0.55-1.4); total serum proteins 62 g/l (normal range 63-78); albumin 33 g/l (normal range 31-51); alkaline phosphatase 704 IU/l (normal range 98-280), alanine transaminase 11 U/l (normal range up to 40). A barium swallow and meal showed coarse mucosal folds in the duodenum but at initial endoscopy no abnormality was detected and no biopsy taken. He was seen again 2 years later with an episode of _5 right-sided weakness. He was again cachetic with a weight of 54 kg. There was a rapid improvement in his right hemiparesis and in view of his previous his- j tory an endoscopy was performed to obtain biopsies from the second part of the duodenum. Endoscopy on this occasion showed pearly, yellowish, white grains in the mucosa (Figure 1) which were most prominent in the first part of the duodenum. These lesions were biopsied together with random biopsies from the remainder of the duodenum and were sent for culture, light and electron microscopy.
Histology: The duodenal villi were distended by foamy histiocytes containing PAS-positive material, and by spaces which appeared to be dilated lymphatics (Figure 2). The appearance of tissue fixed in osmium tetroxide suggested that these spaces contained fat, but an endothelium was not demonstrated at electron microscopy. Bacilliform bodies were Figure 3. Bacilliform bodies with the lamina propria (arrows). (x 20 000; reduced 57°/%)
found, confirming the diagnosis of Whipple's disease (Figure 3). Spaces within the lamina propria are well described in Whipple's disease but in this case seemed to be unusually large"1; it is suggested that these are due to selective biopsy under visual control of the pearly grains observed and described above.
Bacteriology: No organisms were grown on routine culture on various media. Treatment: The patient was commenced on a prolonged course of tetracycline at a dose of 250 mg four times daily. Following treatment a repeat endoscopy showed resolution of the abnormality seen in the duodenal mucosa. The bacilliform bodies could not be seen on the electron micrographs, but some Figure 1. Endoscopic appearance of duodenal lesions in Whipple's disease
dilated lacteals were still visible on the sections
examined by light microscopy.
Journal of the Royal Society of Medicine Volume 79 August 1986 Discussion
Whipple's disease is a chronic multisystem disease2, which traditionally has been diagnosed by histological examination of small-bowel biopsies in which PAS-positive macrophages fill the lamina propria. Electron microscopy has shown the macrophages to contain rod-shaped bacteria. The purpose of this report is to draw attention to the endoscopic changes that may be observed. Less than 10 cases of endoscopic diagnosis of the disease have been reported and there are some descriptive differences (Table 1). In general, the Table 1. Endoscopic findings in Whipple's disease White punctate lesions diffusely distributed throughout the duodenum7 -10 Blunting of the valvulae conniventes Friable erythematous mucosa4 Whitish-yellow confluent nodules and plaques5 6
abnormalities are in the first or second part of the duodenum and usually consist of whitish-yellow punctate lesions, although on occasions they take the form of nodules. Endoscopic biopsy of such lesions in the duodenum may well produce more useful material for examination in possible cases of Whipple's disease. It can also be used to follow progress after treatment, and biopsies within a week of therapy may show an improvement. References 1 Whipple GH. A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissue. Bull Johns Hopkins Hosp 1907;18:382-91 2 Comer FM, Brandt LJ, Abissi CJ. Whipple's disease: a review. Am J Gastroenterol 1983;78:107-44 3 Borsch G, Schneider J, Schejbal V. Intestinaler Morbus Whipple eine meist dicodenoshopisch stellbare Diagnose. Medizinische Welt (Stuttgart) 1983;34:639-41 4 Crane S, Schlippert W. Duodenoscopic findings in Whipple's disease. Gastrointest Endosc 1978;24:248-9 5 Ewers HD, Gasch J, Hamburger B, et al. Endoscopic documentation of Whipple's disease. Endoscopy 1976;8:169-71 6 Gaertner U, Petzold D, Heisig N, Vogel P, Otto HF. Endoskopische befunde bei Morbus Whipple. Deutsche Medizinische Wochenschrift 1978;103:1740-2 7 Lita RG, Royo VA, Brotons BB, Reinosu JG, Grimactav JL. Contribucion endoscopica al diagnostio de la enfermedical de Whipple. Rev Esp Enferm Apar Dig 1978;54:607-18 8 Massarrat 5, Janzen R, Schmitz-Moorman P. Endoskopische befunde bei M Whipple. Leber Magen Darm 1977;7:52-3 9 Puppala AR, Singh S, Munaswamy M. Whipple's disease, endoscopic documentation, light and electron microscopic features in an eighty-year-old man. Am J Gastroenterol 1978;70:407-11 10 Vopicelli NA, Salyer WR, Milligan F1), Bayless TM, Yardley JH. The endoscopic appearances of the duodenum in Whipple's Disease. Johns Hopkins Med J
1976;188:19-23 11 Whitehead R. Mucosal biopsy of the gastrointestinal tract. 2nd ed. Philadelphia: WB Saunders, 1979:122-5 (Accepted 10February 1986)
485
Cutaneous vasculitis: a presenting feature in Hodgkin's disease
M E Kesseler MRCP D N Slater MRCPath Rotherham District General Hospital, Rotherham Keywords: cutaneous vasculitis, Hodgkin's disease
Cutaneous involvement by Hodgkin's disease is uncommon and is rarely the presenting feature. However, nonspecific lesions are common and occur in 15-50% of cases'. Many lesions result from responses to itching and include excoriations, lichenification, prurigo-like papules and hyperpigmentation. Other manifestations include urticaria, erythema multiforme, ichthyosis, erythroderma, bullae and herpes zoster1'2. We describe two patients in whom cutaneous leukocytoclastic vasculitis was the presenting feature of Hodgkin's disease and mimicked pruritic excoriations. Case reports Case 1: A 72-year-old man presented with a threemonth history of generalized pruritis and weight loss of 6 kg. He suffered from chronic obstructive airways disease and his medication included salbutamol, acepifylline and methylcysteine hydrochloride. Examination revealed numerous 1-5 mm diameter necrotic lesions on the trunk and limbs. The lesions were considered to represent excoriations from scratching. The liver was palpable 10 cm below the costal margin but there was no splenomegaly nor lymphadenopathy. Abnormal investigations included low haemoglobin (10 g/dl), serum B12 (163jug/l), serum folate (5 p/l) and plasma albumin (30 g/l); elevated platelet count (728 x 109/l), ESR (117 mm/hour Westergren), serum ferritin (500 ng/ml), alpha-2 globulin, alkaline phosphatase (592 U/l) and ALT (50 U/l). Normal investigations included white cell count, red cell indices, blood urea, electrolytes, bilirubin, glucose, acid phosphatase and T4. Blood and urine immunoelectrophoresis was normal. Antibodies to nuclei, mitochondria and smooth muscle were absent. Chest X-ray, ultrasound of the liver and spleen and isotope liver scan were normal. Culture and serology for fungi and viruses were negative. However, Staphylococcus aureus was isolated from the skin and nose and treatment was started with flucloxacillin. The skin lesions were also occluded but no improvement was observed. The pruritis became marginally better on an antihistamine. Four weeks after presentation, the patient developed axillary lymphadenopathy and node biopsy revealed lymphocyte-depleted Hodgkin's disease. Skin biopsy demonstrated a small vessel necrotizing leukocytoclastic vasculitis with epidermal necrosis and ulceration. However, before further treatment was instituted, the patient developed pneumonia and died. Autopsy demonstrated Hodgkin's disease restricted to the para-aortic and axillary lymph nodes. Hepatic enlargement was secondary to cor pulmonale. Further skin lesions biopsied also demonstrated necrotizing vasculitis and inmmunofluorescence identified perivascular IgM and C3.
0141-0768/86/ 080485-02/$02.00/0 C 1986 The Royal Society of Medicine
