46
Journal of the Royal Society of Medicine Volume 84 January 1991
Case reports
Onset of ulcerative colitis in a patient with colonic schistosomiasis
D A Gorard MB MRCP M J Hershman MS FRCS Department of Gastroenterology, Central Middlesex Hospital, Acton Lane, London NW1O 7NS Keywords: schistosomiasis; ulcerative colitis
Over 200 million people worldwide suffer from schistosomiasis. The species Schistosoma mansoni is endemic in parts of Africa, the Middle East and South America. We report a patient presenting with ulcerative colitis who was also found to have colonic schistosomiasis.
Case report A 45-year-old man presented with a 3-month history of passage of blood and mucus with his stools. He had lived in Kenya until 28-years-old, at which age he came to reside in England. Since coming to live in this country, his only foreign travel had been to India over 10 years prior to his presentation. General examination was unremarkable, but sigmoidoscopy revealed an inflamed rectal mucosa, with contact bleeding. The appearances were those of an aggressive colitis. Barium enema showed fine ulceration of the rectum, sigmoid and descending colon with loss of haustration. Rectal biopsy showed an active chronic proctitis with some goblet cell depletion consistent with ulcerative colitis. In addition numerous Schistosoma mansoni ova were present in the lamina propria (Figure 1). Blood count (including eosinophil count) and liver function tests were normal. Praziquantel (40 mg/kg) was administered, and all subsequent biopsies and stool analyses were negative
for schistosomiasis. Nevertheless following this treatment, his bowel symptoms were unchanged and further rectal biopsies continued to show features of ulcerative colitis. Colonoscopy confirmed macroscopic and microscopic features of ulcerative colitis as far as the splenic flexure. His symptoms responded to oral sulphasalazine and rectal steroids, and remain controlled on these drugs one year later.
Case presented to Clinical Section, 11 May 1990
Discussion Infection with Schistosoma mansoni occurs when the snail intermediate host sheds larvae (cerceriae) into fresh water. The cerceriae penetrate the skin or mucous membrane of man, the definitive host. The developing worms enter the general circulation and mature into adult worms in the portal venous system. They then migrate to the tributaries of the inferior mesenteric vein. Despite a mean life-span of 3-4 years, adult worms may live up to 30 years within the human host'. Eggs are laid in the submucosal veins, and may pass through the colonic wall to enter the faeces. The life-cycle is completed when human faeces containing ova contaminate water supplies infested with the snail intermediate host. Symptoms from chronic infection with Schistosoma mansoni vary and are related to the intensity of infection. Most patients are asymptomatic, others have nonspecific abdominal pains or diarrhoea. Patients with a heavy worm burden may have chronic bloody diarrhoea with pus and mucus in the stools. Colonoscopy in such patients may show a swollen granular or haemorrhagic mucosa. This schistosomal colitis is histologically distinct from ulcerative colitis, even when ova are absent from the biopsy2. In Egypt, multiple colonic polyps are associated with chronic intestinal schistosomiasis. When there is heavy or repeated infection, eggs are carried back up the portal vein, leading to portal fibrosis, hepatosplenomegaly and
portal hypertension. Our patient had asymptomatic chronic schistosomiasis, with continuing egg production 17 years after leaving an endemic area, Kenya. This only became apparent when he developed ulcerative colitis. We feel the schistosomiasis was incidental to the development of his ulcerative colitis, and the occurrence of these two common diseases in the same individual unremarkable, although previously not documented. Nevertheless, the association is of interest since there are reports of patients subsequently diagnosed as having ulcerative colitis who have initially presented with infective diarrhoea. Intestinal pathogens identified at onset of ulcerative colitis have included Salmonella spp.3'4 and Aeromonas spp.5. Such anecdotal reports suggest infectious agents are relevant in the pathogenesis of ulcerative colitis, but evidence is lacking6. As well as documenting the onset of ulcerative colitis in a patient with chronic intestinal schistosomiasis, this report reminds us of the possible prolonged longevity of Schistosoma mansoni within humans. Acknowledgments. We thank Mr Henry, Central Middlesex Hospital for permission to report this case, and Dr Domizio, St Bartholomew's Hospital, for assistance with the histology.
Figure 1. Rectal biopsy showing 3 ova of Scistosoma mansoni within a congested lamina propria. Distorted crypts and goblet cell depletion of ulcerative colitis are present (Haematoxylin and Eosin, 1 x105)
References 1 Manson-Bahr PEC, Bell DR. Schistosomiasis. In: Manson-Bahr PEC, Bell DR, eds. Manson's tropical diseases, 19th edn. London: Bailliere Tindall, 1987:448-85 2 Radhakrishnan S, Al Nakib B, Shaikh H, Menon NK. The value of colonoscopy in schistosomal, tuberculous, and amoebic colitis. Dis Colon Rectum 1986;29:891-5
0141-0768/91/ 010046-02/$02.00/0 © 1991 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 84 January 1991 3 Dronfield MW, Fletcher J, Langman MJS. Coincident salmonella infections and ulcerative colitis: problems of recognition and management. BMJ 1974;1:99-100 4 Taylor-Robinson S, Miles R, Whitehead A, Dickinson RJ. Salmonella infection and ulcerative colitis. Lancet 1989;i: 1145 5 Willoughby JMT, Rahman AFMS, Gregory MM. Chronic colitis after Aeromonas infection. Gut 1989;30:686-90
6 Beeken WL. Ulcerative colitis: microbial causes. In: Allan RN, Keighley MRB, Alexander-Williams J, Hawkins C, eds. Inflammatory bowel diseases. Edinburgh: Churchill Livingstone, 1983:171-6 (Accepted 12 June 1990. Correspondence to Dr D A Gorard, Department of Gastroenterology, St Bartholomew's Hospital, West Smithfield, London EC1 7BE)
Facial desmoplastic malignant melanoma
A Anstey MB MRCP J D Willdnson MB FRCP Department of Dermatology, Wycombe General Hospital, High Wycombe, Bucks HP11 2TT M M Black MD FRCP Dowling Skin Unit, St Thomas' Hospital, London SE1 7EH
47
Case presented to Section of Dermatology, 18 January 1990
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A ,
V~~~~~
J
Keywords: neurotropism, S100 protein; lentiginous hyperplasia
A patient is presented who developed a lesion on his right cheek which proved to be a desmoplastic malignant melanoma of the neurotropic type. Despite extensive surgery, the tumour recurred illustrating the deeply invasive nature ofthis type of melanoma. Desmoplastic malignant melanoma is easily missed at presentation because of its often banal clinical and histological appearance.
Case report A 64-year-old man presented in January 1986 with a 2-year history of an enlarging lesion on his right cheek. Examintion revealed a small, fibrotic lesion, 1 cm in diameter with a light brown lentigo overlying it. Histology from an excision biopsy showed a lentigo maligna overlying a dense, cellular, desmoplastic dermal tumour entrapping nerves and blood
Figure 1. H & E stain (x 106) showing lentigo maligna overlying a desmoplastic melanoma with prominent desmoplasia and sparse spindle cells
Figure 2. Prominent neural invasion by desmoplastic malignant melanoma (H & E x165)
vessels (Figures 1 and 2). S100 immunoperoxidase positively stained the spindle cell element in the dermis, which confirmed the diagnosis of desmoplastic melanoma. A wide excision of the scar was performed by plastic surgeons. There was no evidence of residual tumour on multiple sections and S100 staining. In May 1987 a subcutaneous nodule at the site ofthe scar on the right cheek was removed by wide and deep excision. This was resurfaced with an advanced rotation flap mobilized from the right cheek and neck. The histology showed a small, central, residual intradermal focus of S100 positive cells within the scar tissue. Excision appeared to be complete with generous clear margins. Six months later a right maxillary mass was excised and was confirmed histologically as recurrence of the desmoplastic melanoma. A CT scan of the head after surgery showed no evidence of persisting deep tumour. In February 1988 he underwent a right maxillectomy in an attempt to remove any occult tumour. This involved excision of the medial end of the right zygoma, the right lateral nasal bones and the anterior half ofthe boney orbital floor in addition to intervening soft tissue and nerves. Frozen sections taken from the margins of excision during surgery showed no evidence of tumour. This was covered by a left radial forearm free flap. Histology from the main soft tissue specimen again showed an infiltrative spindle cell tumour with dense fibrous stroma and scattered, elongated, pleomorphic cells with strongly eosinophilic cytoplasm and
0141-0768/91/ 010047-02/$02.00/O © 1991 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 84 January 1991
Case reports
Onset of ulcerative colitis in a patient with colonic schistosomiasis
D A Gorard MB MRCP M J Hershman MS FRCS Department of Gastroenterology, Central Middlesex Hospital, Acton Lane, London NW1O 7NS Keywords: schistosomiasis; ulcerative colitis
Over 200 million people worldwide suffer from schistosomiasis. The species Schistosoma mansoni is endemic in parts of Africa, the Middle East and South America. We report a patient presenting with ulcerative colitis who was also found to have colonic schistosomiasis.
Case report A 45-year-old man presented with a 3-month history of passage of blood and mucus with his stools. He had lived in Kenya until 28-years-old, at which age he came to reside in England. Since coming to live in this country, his only foreign travel had been to India over 10 years prior to his presentation. General examination was unremarkable, but sigmoidoscopy revealed an inflamed rectal mucosa, with contact bleeding. The appearances were those of an aggressive colitis. Barium enema showed fine ulceration of the rectum, sigmoid and descending colon with loss of haustration. Rectal biopsy showed an active chronic proctitis with some goblet cell depletion consistent with ulcerative colitis. In addition numerous Schistosoma mansoni ova were present in the lamina propria (Figure 1). Blood count (including eosinophil count) and liver function tests were normal. Praziquantel (40 mg/kg) was administered, and all subsequent biopsies and stool analyses were negative
for schistosomiasis. Nevertheless following this treatment, his bowel symptoms were unchanged and further rectal biopsies continued to show features of ulcerative colitis. Colonoscopy confirmed macroscopic and microscopic features of ulcerative colitis as far as the splenic flexure. His symptoms responded to oral sulphasalazine and rectal steroids, and remain controlled on these drugs one year later.
Case presented to Clinical Section, 11 May 1990
Discussion Infection with Schistosoma mansoni occurs when the snail intermediate host sheds larvae (cerceriae) into fresh water. The cerceriae penetrate the skin or mucous membrane of man, the definitive host. The developing worms enter the general circulation and mature into adult worms in the portal venous system. They then migrate to the tributaries of the inferior mesenteric vein. Despite a mean life-span of 3-4 years, adult worms may live up to 30 years within the human host'. Eggs are laid in the submucosal veins, and may pass through the colonic wall to enter the faeces. The life-cycle is completed when human faeces containing ova contaminate water supplies infested with the snail intermediate host. Symptoms from chronic infection with Schistosoma mansoni vary and are related to the intensity of infection. Most patients are asymptomatic, others have nonspecific abdominal pains or diarrhoea. Patients with a heavy worm burden may have chronic bloody diarrhoea with pus and mucus in the stools. Colonoscopy in such patients may show a swollen granular or haemorrhagic mucosa. This schistosomal colitis is histologically distinct from ulcerative colitis, even when ova are absent from the biopsy2. In Egypt, multiple colonic polyps are associated with chronic intestinal schistosomiasis. When there is heavy or repeated infection, eggs are carried back up the portal vein, leading to portal fibrosis, hepatosplenomegaly and
portal hypertension. Our patient had asymptomatic chronic schistosomiasis, with continuing egg production 17 years after leaving an endemic area, Kenya. This only became apparent when he developed ulcerative colitis. We feel the schistosomiasis was incidental to the development of his ulcerative colitis, and the occurrence of these two common diseases in the same individual unremarkable, although previously not documented. Nevertheless, the association is of interest since there are reports of patients subsequently diagnosed as having ulcerative colitis who have initially presented with infective diarrhoea. Intestinal pathogens identified at onset of ulcerative colitis have included Salmonella spp.3'4 and Aeromonas spp.5. Such anecdotal reports suggest infectious agents are relevant in the pathogenesis of ulcerative colitis, but evidence is lacking6. As well as documenting the onset of ulcerative colitis in a patient with chronic intestinal schistosomiasis, this report reminds us of the possible prolonged longevity of Schistosoma mansoni within humans. Acknowledgments. We thank Mr Henry, Central Middlesex Hospital for permission to report this case, and Dr Domizio, St Bartholomew's Hospital, for assistance with the histology.
Figure 1. Rectal biopsy showing 3 ova of Scistosoma mansoni within a congested lamina propria. Distorted crypts and goblet cell depletion of ulcerative colitis are present (Haematoxylin and Eosin, 1 x105)
References 1 Manson-Bahr PEC, Bell DR. Schistosomiasis. In: Manson-Bahr PEC, Bell DR, eds. Manson's tropical diseases, 19th edn. London: Bailliere Tindall, 1987:448-85 2 Radhakrishnan S, Al Nakib B, Shaikh H, Menon NK. The value of colonoscopy in schistosomal, tuberculous, and amoebic colitis. Dis Colon Rectum 1986;29:891-5
0141-0768/91/ 010046-02/$02.00/0 © 1991 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 84 January 1991 3 Dronfield MW, Fletcher J, Langman MJS. Coincident salmonella infections and ulcerative colitis: problems of recognition and management. BMJ 1974;1:99-100 4 Taylor-Robinson S, Miles R, Whitehead A, Dickinson RJ. Salmonella infection and ulcerative colitis. Lancet 1989;i: 1145 5 Willoughby JMT, Rahman AFMS, Gregory MM. Chronic colitis after Aeromonas infection. Gut 1989;30:686-90
6 Beeken WL. Ulcerative colitis: microbial causes. In: Allan RN, Keighley MRB, Alexander-Williams J, Hawkins C, eds. Inflammatory bowel diseases. Edinburgh: Churchill Livingstone, 1983:171-6 (Accepted 12 June 1990. Correspondence to Dr D A Gorard, Department of Gastroenterology, St Bartholomew's Hospital, West Smithfield, London EC1 7BE)
Facial desmoplastic malignant melanoma
A Anstey MB MRCP J D Willdnson MB FRCP Department of Dermatology, Wycombe General Hospital, High Wycombe, Bucks HP11 2TT M M Black MD FRCP Dowling Skin Unit, St Thomas' Hospital, London SE1 7EH
47
Case presented to Section of Dermatology, 18 January 1990
s.~~
A ,
V~~~~~
J
Keywords: neurotropism, S100 protein; lentiginous hyperplasia
A patient is presented who developed a lesion on his right cheek which proved to be a desmoplastic malignant melanoma of the neurotropic type. Despite extensive surgery, the tumour recurred illustrating the deeply invasive nature ofthis type of melanoma. Desmoplastic malignant melanoma is easily missed at presentation because of its often banal clinical and histological appearance.
Case report A 64-year-old man presented in January 1986 with a 2-year history of an enlarging lesion on his right cheek. Examintion revealed a small, fibrotic lesion, 1 cm in diameter with a light brown lentigo overlying it. Histology from an excision biopsy showed a lentigo maligna overlying a dense, cellular, desmoplastic dermal tumour entrapping nerves and blood
Figure 1. H & E stain (x 106) showing lentigo maligna overlying a desmoplastic melanoma with prominent desmoplasia and sparse spindle cells
Figure 2. Prominent neural invasion by desmoplastic malignant melanoma (H & E x165)
vessels (Figures 1 and 2). S100 immunoperoxidase positively stained the spindle cell element in the dermis, which confirmed the diagnosis of desmoplastic melanoma. A wide excision of the scar was performed by plastic surgeons. There was no evidence of residual tumour on multiple sections and S100 staining. In May 1987 a subcutaneous nodule at the site ofthe scar on the right cheek was removed by wide and deep excision. This was resurfaced with an advanced rotation flap mobilized from the right cheek and neck. The histology showed a small, central, residual intradermal focus of S100 positive cells within the scar tissue. Excision appeared to be complete with generous clear margins. Six months later a right maxillary mass was excised and was confirmed histologically as recurrence of the desmoplastic melanoma. A CT scan of the head after surgery showed no evidence of persisting deep tumour. In February 1988 he underwent a right maxillectomy in an attempt to remove any occult tumour. This involved excision of the medial end of the right zygoma, the right lateral nasal bones and the anterior half ofthe boney orbital floor in addition to intervening soft tissue and nerves. Frozen sections taken from the margins of excision during surgery showed no evidence of tumour. This was covered by a left radial forearm free flap. Histology from the main soft tissue specimen again showed an infiltrative spindle cell tumour with dense fibrous stroma and scattered, elongated, pleomorphic cells with strongly eosinophilic cytoplasm and
0141-0768/91/ 010047-02/$02.00/O © 1991 The Royal Society of Medicine
