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

Journal of the Royal Society of Medicine Volume 84 January 1991 Table 1. Criteria for diagnosis (1) Temperature: >38.90C (2) Rash: erythematous rash (3) Desquamation: palms and soles about 2 weeks later (4) Hypotension and poor peripheral perfusion. (5) Clinical or laboratory abnormalities of at least 3 systems: (i) Gastrointestinal; diarrhoea and/or vomiting at the onset of illness (ii) Hepatic; raised liver enzymes (alanine and aspartate transaminases, triaditis and centrilobular necrosis (iii) Muscular; myalgia and/or raised creatine phosphokinase, arthralgias and synovitis (iv) Renal; raised urea and/or creatinine or pyuria without renal infection, acute renal failure (renal and pre-renal). (v) Cardiovascular; hypotension, shock and cardiomyopathy (vi) Neurological; deteriorating levels of consciousness and seizures (vii) Mucous membranes; ulceration of buccal mucosa, oesophagus, vagina, bladder, conxjunctival injection, subconjunctival haemorrhages and strawberry tongue (6) Negative results on blood and CSF cultures is suggestive in the presence of toxin-producing S. aureus grown from wound/ laceration/nasopharynx. Toxin-producing S. aureus grown from wound/laceration/nasopharynx. (7) No laboratory test is available for confirming the diagnosis of toxic shock syndrome. A significant rise in TSST-I serum antibody in association with clinical manifestation is strong retrospective support for diagnosis

disease, erythema multiformae, leptospirosis and typhus5. The pathogenetic mechanism of toxic shock syndrome is due to production of staphylococcal toxic shock syndrome toxin I(TSST-I). This toxin has been isolated in America and reproduces the disease in animal models6. TSST-I acts as an initiator for the in vivo release of endogenous shock-inducing mediators particularly Interleukin I (IL-I) and tumour

Fournier's gangrene of the scrotum following day case vasectomy

A Patel FRCS J W A Ramsay MS FRCS H N Whitfield MChir FRCS Department of Urology, St Bartholomew's Hospital, West Smithfield, London EC1 7BE Keywords: vasectomy; Fournier's gangrene

In 1883, Alfred Fournier, a French venereologist described five patients with unexplained gangrene of the male genitalia, a syndrome that has since borne his name'.

Case report A 31-year-old man underwent day case vasectomy under local anaesthesia (2% plain lignocaine) after preparation of the skin with aqueous chlorhexidine solution, through a midline incision, at his local hospital. The scrotum appeared red and swollen 3 hours later. The patient had suffered a mild diarrhoeal illness prior to operation. Delayed medical consultation despite the immediate appearances of the wound, allowed a systemic septic illness to become established over the next 48 h. On admission to this hospital there was established Gram-negative septicaemic shock, anuria and uraemia secondary to acute tubular necrosis, and Fournier's gangrene of the scrotum and penis. The patient

49

necrosis factor (TNF)7. Anti-TSST-I antibody may be measured and levels greater than 1: 100 are thought to be protective. Antibody levels increase with age and more than 80% of adults 20 years and older have protective levels of antibody. Absence or inability to produce adequate levels of antibody may be an important risk factor in the development of the disease8. Children with endogenous strains of toxin-producing S. aureus and who produce adequate levels of anti-TSST-I antibody may be protected from the disease. References 1 Todd J, Fishaut M, Kapral F, Welch F. Toxic shock syndrome associated with phage group I staphylococcus. Lancet 1978; ii:1116-18 2 Davis JP, Chesney PJ, Ward PJ, et al. Toxic shock syndrome: epidemiologic features, risk factors and prevention. NEngi J Med 1980;303:1429-35 3 MacDonald K, Osterholm M, Hedberg C, et al. Toxic shock syndrome: a newly recognised complication of influenza and influenza-like illness. JAMA 1987;257:1053-58 4 Buchdahl R, Levin M, Wilkins B, et al. Toxic shock syndrome. Arch Dis Child 1985;60:563-7 5 Scully R, Mark E, McNeely B. Case records ofthe Massachusetts General Hospital. N Engl J Med 1986;314 1:302-9 6 de Azavedo J. Animal models for toxic shock syndrome: overview. Rev Infect Dis 1989;II,Supplement I:S205-S209 7 Parsonett J. Mediators in the pathogenesis of toxic shock syndrome: overview. Rev Infect Dis 1989;II,Supplement I: S263-S269 8 Jacobson J, Kasworm E, Daly J. Risk of developing toxic shock syndrome associated with toxic shock syndrome toxin I following non-genital staphylococcal infection. Rev Infect Dis 1989;II,

Supplement I:S8-S13 (Accepted 3 July 1990. Correspondence to Dr S R Hanafiah, Priority Services Unit, South Western Hospital, Landor Road, London SW9)

was admitted to intensive care, where inotropic support was provided, together with continuous arteriovenous haemofiltration. Parenteral antibiotic treatment with metronidazole, benzylpenicillin and Ceftazidime, was commenced prior to emergency radical debridement of the gangrenous tissues (Figure 1). Cardiorespiratory arrest occurred postoperatively, and after successful resuscitation there was pulmonary oedema, adult respiratory distress syndrome and disseminated intravascular coagulation. Histology of the penoscrotal tissues confirmed necrosis of the dermis and subcutaneous tissues, extensive vascular thrombosis and abundant Gram-positive cocci. Bacteriological specimens revealed the presence of (l-haemolytic streptococci. Gentamicin and vancomycin were added to the antibiotic regimen. The patient remained unconscious and required ventilation on the intensive care unit for 2 weeks. During this time, further surgical debridement was performed on four occasions with platelet cover. An EEG carried out for prognostic purposes was grossly abnormal and there was no response to external stimuli. A week later, the patient regained consciousness and the capacity for both speech and purposeful movement. In order to facilitate further dressings and aid skin coverage, a left orchidectomy was carried out, and the right testis was secured in the superficial inguinal pouch while the scrotum and penis were left to granulate. Complete re-epithelialization had occurred by 3 months.

Discussion Many common surgical procedures have been complicated by Fournier's gangrene including herniorrhaphy,

Case presented to Section of Urology, 28 April 1988

0141-0768/91/ 010049-02/$02.00/0 © 1991 The Royal Society of Medicine