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
48
Journal of the Royal Society of Medicine Volume 84 January 1991
large atypical nuclei. The tumour cells did not contain melanin but some were positive on S100 staining. The melanoma extended to the deep margins of the specimen. One year later, he developed increasing pain in the right eye with subsequent loss of vision. A CT scan revealed a huge mass in the right orbit and maxilla which extended deeply. In June 1989 he was started on chemotherapy using fotemustine in combination with vindesine. The tumour failed to respond to chemotherapy and he died in January 1990.
Discussion Desmoplastic melanoma is a histological variant of cutaneous melanoma that has a distinctive clinical behaviour. It was first described by Conley in 1971 with a series of seven cases'. Arising from inconspicuous superficial melanotic lesions on the head and neck, bulky subcutaneous fibrous tumours developed, which invaded deeply and recurred stubbornly, sometimes with metastases. It was argued that these lesions were a variant of malignant melanoma. The first British report of desmoplastic melanoma was in 19822. Recent publications show a peak onset in the 7th decade3'4. Desmoplastic melanomas are frequently associated with lentigo maligna or an atypical junctional melanocytic proliferation3, as occurred in our patient. Neurotropism was a feature of 27% of desmoplastic melanomas from a recent series4 and was evident on the initial and subsequent histology in our patient. Desmoplastic malignant melanoma is easily misdiagnosed as its histological appearance is often indistinct with few
Toxic shock syndrome presenting as an acute encephalopathy and diarrhoea
S R Hanafiah MB ChB S K F Chong MD MRCP Department of Paediatrics, Joyce Green Hospital, Dartford, Kent Keywords: superficial lacerations; diarrhoea; rash; convulsions
The first cases of toxic shock syndrome were reported in children'. Subsequently, toxic shock syndrome became closely associated with menstruation and the use of tampons in America2. Non-menstrual toxic shock syndrome is now increasingly recognized and is found in association with influenza, tracheitis, croup, pharyngitis, sinusitis, nasal surgery and burns3. Case report A 12-month-old child presented with acute profuse watery diarrhoea and a generalized rash with sudden deterioration of levels of consciousness on admission. She was febrile (39.80C), 10% dehydrated, shocked with hypotension (85/40 mmHg) and had peripheral vasconstriction, peripheral and central cyanosis. She had a papular erythematous rash, severe congestion of conjunctivae, inflamed and congested pharynx and buccal cavity. A superficial laceration, 1.5 cm long was noted at the base of her thumb resulting from trauma due to her habit of sucking her thumb. During the next few hours, the rash became petechial and spread all over her body. She also had tetanic convulsions and carpopedal spasms. Investigations showed haemoglobin 10.4 g/dl (normal range 11-13 g/dl), white cell count 3.0x109/1 (5-lOx 109/l)
or even no features to suggest a melanoma. Immunological staining of the S100 protein can be used to identify melanoma cells which may be few in number, amelanotic and scattered sparsely in the fibrous stroma of the tumour. It also helps to exclude epithelial and fibrohistiocytic tumours which may show similar histological features on conventional staining6. In summary, this case illustrates the typical features of desmoplastic melanoma as described in the original report by Conley et al1, and confirms the deeply invasive and recurrent nature of this tumour which results in a very poor prognosis. References 1 Conley J, Lattes R, Orr W. Desmoplastic malignant melanoma (a rare variant of spindle cell melanoma). Cancer 1971;28:914-36 2 Berry RB, Subbuswamy SG, Hackett MEJ. Desmoplastic malignant melanoma: the first British report. Br J Plast Surg 1982;
35:324-7 3 Egbert B, Kempson R, Sagebiel R. Desmoplastic malignant melanoma. A clinicohistopathologic study of 25 cases. Cancer 1988;62:2033-41 4 Jain S, Allen PW. Desmoplastic malignant melanoma and its variants. A study of 45 cases. Am J Surg Pathol 1989;13:358-73 5 Reiman HM, Goellner JR, Woods JE, Mixter RC. Desmoplastic melanoma of the head and neck. Cancer 1987;60:2269-74
(Accepted 31 July 1990. Correspondence to Dr A Anstey, Department of Dermatology, St Bartholomew's Hospital, West Smithfield, London EClA 7BE)
with 40% neutrophils, left shift and toxic granulations, platelets 72x 109/1 (150-400x 109/1). Blood urea 16.8 mmol/l, (2.5-6.6 mmol/l), creatinine 76 imol/I (30-50 itmol/l) were increased. Serum calcium 1.64 mmol/l (2.1-2.6 mmolA) was low and hepatic transaminases raised: ALT 130 U/I (10-35 U/1) AST 544 U/I (20-50 U/I). Cerebrospinal fluid and blood cultures were sterile. Toxin-producing Staphylococcus aureus was grown from the laceration at the base ofher left thumb, nasopharynx and endotracheal swabs. Her chest X-ray initially showed a bullous lesion at the left hilum and subsequently showed consolidation. She was transferred to the Hospital for Sick Children (Great Ormond Street) where she was artificially ventilated for 5 days. The rash desquamated on day 10 and her subsequent recovery was uneventful. Further investigations showed no evidence of serum complement or immunoglobulin class/subclass deficiency. Aggressive resuscitation early in the illness improved the outcome. Fluid loss was replaced with dextrose saline, 0.45% saline, 0.9% saline and plasma. Dopamine (2.5-10 pg/kg/min) and prostacyclin (4.0-30 ng/kg/min) infusion were used to maintain blood pressure and to improve renal perfusion. Profound hypocalcaemia was corrected with calcium gluconate and salt-poor albumin. Convulsions were controlled with phenobarbitone and diazepam. Artificial ventilation was begun as levels of consciousness deteriorated in the presence of pneumonia. Intravenous ceftazidime, flucloxacillin and penicillin in maximal doses were given after blood cultures were taken. Oral rifampicin was given after recovery from the acute illness to eradicate toxin-producing S. aureus.
Discussion The case reported above fulfils the criteria for the diagnosis of toxic shock syndrome (see Table 1)'. Toxic shock syndrome must be considered in the differential diagnosis of any case of diarrhoea with rash, deteriorating levels of consciousness and convulsions. Other diagnoses to be considered are meningococcaemia, streptococcal scarlet fever, Kawasaki's
Case presented to Section of Paediatrics, 24 November 1989
0141-0768/91/ 010048-02/$02.00/0 © 1991 The Royal Society of Medicine
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
48
Journal of the Royal Society of Medicine Volume 84 January 1991
large atypical nuclei. The tumour cells did not contain melanin but some were positive on S100 staining. The melanoma extended to the deep margins of the specimen. One year later, he developed increasing pain in the right eye with subsequent loss of vision. A CT scan revealed a huge mass in the right orbit and maxilla which extended deeply. In June 1989 he was started on chemotherapy using fotemustine in combination with vindesine. The tumour failed to respond to chemotherapy and he died in January 1990.
Discussion Desmoplastic melanoma is a histological variant of cutaneous melanoma that has a distinctive clinical behaviour. It was first described by Conley in 1971 with a series of seven cases'. Arising from inconspicuous superficial melanotic lesions on the head and neck, bulky subcutaneous fibrous tumours developed, which invaded deeply and recurred stubbornly, sometimes with metastases. It was argued that these lesions were a variant of malignant melanoma. The first British report of desmoplastic melanoma was in 19822. Recent publications show a peak onset in the 7th decade3'4. Desmoplastic melanomas are frequently associated with lentigo maligna or an atypical junctional melanocytic proliferation3, as occurred in our patient. Neurotropism was a feature of 27% of desmoplastic melanomas from a recent series4 and was evident on the initial and subsequent histology in our patient. Desmoplastic malignant melanoma is easily misdiagnosed as its histological appearance is often indistinct with few
Toxic shock syndrome presenting as an acute encephalopathy and diarrhoea
S R Hanafiah MB ChB S K F Chong MD MRCP Department of Paediatrics, Joyce Green Hospital, Dartford, Kent Keywords: superficial lacerations; diarrhoea; rash; convulsions
The first cases of toxic shock syndrome were reported in children'. Subsequently, toxic shock syndrome became closely associated with menstruation and the use of tampons in America2. Non-menstrual toxic shock syndrome is now increasingly recognized and is found in association with influenza, tracheitis, croup, pharyngitis, sinusitis, nasal surgery and burns3. Case report A 12-month-old child presented with acute profuse watery diarrhoea and a generalized rash with sudden deterioration of levels of consciousness on admission. She was febrile (39.80C), 10% dehydrated, shocked with hypotension (85/40 mmHg) and had peripheral vasconstriction, peripheral and central cyanosis. She had a papular erythematous rash, severe congestion of conjunctivae, inflamed and congested pharynx and buccal cavity. A superficial laceration, 1.5 cm long was noted at the base of her thumb resulting from trauma due to her habit of sucking her thumb. During the next few hours, the rash became petechial and spread all over her body. She also had tetanic convulsions and carpopedal spasms. Investigations showed haemoglobin 10.4 g/dl (normal range 11-13 g/dl), white cell count 3.0x109/1 (5-lOx 109/l)
or even no features to suggest a melanoma. Immunological staining of the S100 protein can be used to identify melanoma cells which may be few in number, amelanotic and scattered sparsely in the fibrous stroma of the tumour. It also helps to exclude epithelial and fibrohistiocytic tumours which may show similar histological features on conventional staining6. In summary, this case illustrates the typical features of desmoplastic melanoma as described in the original report by Conley et al1, and confirms the deeply invasive and recurrent nature of this tumour which results in a very poor prognosis. References 1 Conley J, Lattes R, Orr W. Desmoplastic malignant melanoma (a rare variant of spindle cell melanoma). Cancer 1971;28:914-36 2 Berry RB, Subbuswamy SG, Hackett MEJ. Desmoplastic malignant melanoma: the first British report. Br J Plast Surg 1982;
35:324-7 3 Egbert B, Kempson R, Sagebiel R. Desmoplastic malignant melanoma. A clinicohistopathologic study of 25 cases. Cancer 1988;62:2033-41 4 Jain S, Allen PW. Desmoplastic malignant melanoma and its variants. A study of 45 cases. Am J Surg Pathol 1989;13:358-73 5 Reiman HM, Goellner JR, Woods JE, Mixter RC. Desmoplastic melanoma of the head and neck. Cancer 1987;60:2269-74
(Accepted 31 July 1990. Correspondence to Dr A Anstey, Department of Dermatology, St Bartholomew's Hospital, West Smithfield, London EClA 7BE)
with 40% neutrophils, left shift and toxic granulations, platelets 72x 109/1 (150-400x 109/1). Blood urea 16.8 mmol/l, (2.5-6.6 mmol/l), creatinine 76 imol/I (30-50 itmol/l) were increased. Serum calcium 1.64 mmol/l (2.1-2.6 mmolA) was low and hepatic transaminases raised: ALT 130 U/I (10-35 U/1) AST 544 U/I (20-50 U/I). Cerebrospinal fluid and blood cultures were sterile. Toxin-producing Staphylococcus aureus was grown from the laceration at the base ofher left thumb, nasopharynx and endotracheal swabs. Her chest X-ray initially showed a bullous lesion at the left hilum and subsequently showed consolidation. She was transferred to the Hospital for Sick Children (Great Ormond Street) where she was artificially ventilated for 5 days. The rash desquamated on day 10 and her subsequent recovery was uneventful. Further investigations showed no evidence of serum complement or immunoglobulin class/subclass deficiency. Aggressive resuscitation early in the illness improved the outcome. Fluid loss was replaced with dextrose saline, 0.45% saline, 0.9% saline and plasma. Dopamine (2.5-10 pg/kg/min) and prostacyclin (4.0-30 ng/kg/min) infusion were used to maintain blood pressure and to improve renal perfusion. Profound hypocalcaemia was corrected with calcium gluconate and salt-poor albumin. Convulsions were controlled with phenobarbitone and diazepam. Artificial ventilation was begun as levels of consciousness deteriorated in the presence of pneumonia. Intravenous ceftazidime, flucloxacillin and penicillin in maximal doses were given after blood cultures were taken. Oral rifampicin was given after recovery from the acute illness to eradicate toxin-producing S. aureus.
Discussion The case reported above fulfils the criteria for the diagnosis of toxic shock syndrome (see Table 1)'. Toxic shock syndrome must be considered in the differential diagnosis of any case of diarrhoea with rash, deteriorating levels of consciousness and convulsions. Other diagnoses to be considered are meningococcaemia, streptococcal scarlet fever, Kawasaki's
Case presented to Section of Paediatrics, 24 November 1989
0141-0768/91/ 010048-02/$02.00/0 © 1991 The Royal Society of Medicine
