Biomedical subjects
Publications and source records attributed to S E Handfield-Jones.
We present a family with Buschke-Ollendorff syndrome presenting as elastic tissue naevi without evidence of osteopoikilosis. We discuss the variable expression of this syndrome in other families previously reported in the literature, the association of various connective tissue abnormalities and their correlation with the pathogenesis of this interesting condition.
Immediate hypersensitivity to latex protein is a serious and potentially life-threatening problem. This study found that eight of 867 (0.9%) medical and nursing staff in a district general hospital were allergic to latex with resultant hand eczema and/or urticaria. Wheezing occurred when latex became airborne, adsorbed to starch powder. Sensitized individuals are at risk of anaphylaxis when they themselves become patients. Measures to prevent latex sensitization include pre-employment advice regarding hand care and glove use, particularly for atopics, who are at increased risk. Gloves made from synthetic latex must be available for a sensitized individual, both to use at work and when undergoing medical examinations, surgery and dentistry. Increased awareness of the problem by health-care workers is important in order to recognize those at risk.
We report a 20-year-old female patient with telangiectasia macularis eruptiva perstans characterised by telangiectatic macular lesions and episodic flushing, two lesions of which were successfully treated by laser. Two clinically different forms of cutaneous mastocytosis have both been previously described as telangiectasia macularis eruptiva perstans, and we now propose that this term be restricted to cutaneous mastocytosis characterised by the typical telangiectatic macules.
We report the case of a 39-year-old woman with a persistent congenital vascular lesion, which unusually is continuing to enlarge. Histologically, the lesion is a thin-walled haemangioma with numerous mast cells. Currently, the precise mechanism of vessel proliferation in such lesions is unknown, but it is important in the pathogenesis of both haemangiomas and other dermatological conditions, as well as in wound healing and the formation of tumour metastases. We discuss various angiogenic factors with particular reference to the putative role of the mast cell in the pathogenesis of haemangiomas.
Malignant melanoma (MM) is rare in childhood. We report 24 cases of MM in children 16 years old or less, whose histopathological specimens were referred to our department from 1981 to 1993. In only three cases was the diagnosis of MM made clinically. In some cases histological distinction from benign Spitz naevi was difficult, but other cases were referred because of a reluctance to diagnose MM in a child. One child died of disseminated disease, two developed nodal metastases and two developed local recurrence. Of all the lesions, the one which was considered to show the least difference from a benign naevus histologically occurred in the child who died. Awareness that MM, although rare, does occur in childhood, is vital. However, diagnosis is difficult, both clinically and histopathologically.
We report our experience, and review the literature, concerning 'intensive care dermatology'. Over a period of 14 months, 27 patients who had significant cutaneous problems were seen in the intensive care unit. These included primary dermatological conditions, multisystem disorders with cutaneous signs, complications of dermatological therapy, and skin conditions developing as complications of intensive care. We discuss the diagnosis and management of dermatological problems in the intensive care unit.
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We describe a 75-year-old man demonstrating the florid clinical features of actinic granuloma of O'Brien. This rare disfiguring condition is believed to result from a granulomatous reaction of the dermis to solar-induced elastosis and is poorly responsive to topical steroids. Twelve weeks' treatment with isotretinoin prevented the development of new granulomata and produced almost complete resolution of established lesions.
Adult Still's disease (ASD) is a rare disorder of unknown aetiology, characterized by an evanescent, erythematous, maculopapular rash, fever, arthralgia, and a variety of systemic features. We report a case which illustrates the typical features of ASD, and manifests the hitherto unreported complication of diffuse cutaneous mucinosis.
Following publication of treatment guidelines for patients with psoriasis, a six-centre audit was undertaken to assess current therapeutic practice for two second-line treatments, PUVA and methotrexate. The audit consisted of random sampling of casenotes by external auditors from a paired dermatology department, and assessment by questionnaire. One hundred and eight PUVA and 118 methotrexate casenotes were audited. The commonest indications for treatment were: (a) failure of tropical therapy--PUVA (mean 81% of casenotes), methotrexate (84%); (b) repeated hospital admissions--PUVA (16%), methotrexate (25%). For both PUVA and methotrexate, some aspects of treatment were well documented: PUVA--psoralen dosage (91%), response to PUVA (89%), cumulative lifetime UVA dosage (81%); methotrexate--pretreatment assessment of full blood count (91%), urea and electrolytes (85%), liver function tests (84%). For other aspects documentation was less complete: PUVA--no documentation of presence/absence of skin cancer history (66%), note of photoactive drugs (32%); methotrexate--concurrent medication (69%), history of presence/absence of liver disease (36%). Another aspect which was poorly documented in both PUVA and methotrexate notes was whether advice on contraception/fertility had been given. There was no indication in 29 of 32 casenotes of females of child-bearing age receiving PUVA, and 52 of 63 case notes of relevant patients on methotrexate. This project has demonstrated that formal, multicentre audit based on published guidelines is a practical proposition.
Two patients with widespread, chronic, relapsing panniculitis resulting in disfiguring lipoatrophy are reported. Histology in both cases showed a mixed septal and lobular panniculitis, with lipophagia. The clinical appearance and histology suggested a diagnosis of lipoatrophic panniculitis. Both cases had features of connective tissue panniculitis, which is likely to be a subtype of this condition. Treatment of lipoatrophic panniculitis can be difficult. Our first patient initially responded well to antimalarial therapy, allowing plastic surgical repair of the defects to be carried out.
Anticonvulsants can cause a characteristic hypersensitivity reaction. This multisystem reaction typically presents as fever, mucocutaneous eruptions, lymphadenopathy and hepatitis. There is cross-reactivity between different anticonvulsants, which complicates subsequent therapy. We report three cases to illustrate both the typical features, and less common complications, of this under-recognized and life-threatening syndrome.
While the relationship of aquagenic pruritus (AP) to haematoproliferative conditions is well-recognized, an association with histiocytic disorders has not previously been described. The case of a child with both AP and juvenile xanthogranulomata (JXG) is reported. The cutaneous lesions were typical of JXG, both clinically and histologically, and in addition there were radiological changes consistent with JXG in the lungs. AP is rare in childhood and the coincidental occurrence with unusually extensive JXG seems unlikely.
Lignocaine is a rare contact allergen, in contrast to other local anaesthetics such as benzocaine, which commonly sensitize. The case of a patient sensitized to lignocaine through the use of a topical medication for the treatment of haemorrhoids is reported. Previous reports show that application of a lignocaine-containing preparation for pruritus ani is the most frequent cause of sensitization to lignocaine. The significance of sensitization to lignocaine is discussed in the light of its widespread use both in local anaesthesia and as an anti-arrhythmic.
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We report three cases of Wegener's granulomatosis presenting with cutaneous ulceration resembling pyoderma gangrenosum. Wegener's granulomatosis classically affects the upper and lower respiratory tracts and the kidneys. Skin involvement occurs in up to 50% of patients. Increased awareness that cutaneous involvement can take the form of pyoderma gangrenosum and that it can be a presenting sign may lead to more rapid diagnosis of Wegener's granulomatosis.
We report the case of a 25-year-old Jamaican woman with hypopigmented mycosis fungoides. She first developed a hypopigmented patch on her arm at the age of 11 years. Further lesions developed on the trunk and limbs over a period of 10 years. The lesions were completely impalpable. Skin biopsy showed an infiltrate of atypical lymphocytes, some with cerebriform nuclei, suggesting a diagnosis of mycosis fungoides. The lesions cleared with PUVA therapy.
A 46-year-old caucasian female presented in January 1991 with a 17-year history of severe pemphigoid gestationis. She had been on systemic steroids for 10 years since her last pregnancy in 1981 and required doses of 20-80 mg prednisolone daily to control her disease. A chemical oophorectomy was induced using a new luteinizing hormone releasing hormone (LHRH) analogue known as goserelin. A complete remission occurred within 6 months of initiating this treatment and the systemic steroids were discontinued.