S.E. Handfield‐Jones

The anticonvulsant hypersensitivity syndrome.

Summary 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.

Malignant melanoma in childhood

Summary 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, hut 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.

Contact sensitivity to lignocaine

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.

Adult Still's disease

Adult Stills 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.

The clinical spectrum of lipoatrophic panniculitis encompasses connective tissue panniculitis

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.

Hypopigmented mycosis fungoides

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.

Waxy keratoses of childhood

Three cases of waxy keratoses of childhood occurring in two families are described. The disorder seen in these three cases appears to be clinically and histopathologicaly distinct from previously distinct from previously described familial disorders of keratinization, which also feature multiple discrete papules.1–7

Aquagenic pruritus associated with juvenile xanthogranuloma

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.

Linear prurigo simulating dermatitis artefacta in dominant dystrophic epidermolysis bullosa

We report a family suffering from dominant dystrophic epidermolysis bullosa, in whom linear lichenified lesions and troublesome pruritus were prominent features. We consider that this clinical picture may constitute a separate subtype of this disorder.

PUVA and methotrexate therapy of psoriasis: how closely do dermatology departments follow treatment guidelines?

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 topical therapy‐PUVA (mean 81% of casenotes), methotrexate (84%); (b) repeated hospital admissions‐PUVA (16%), methotrexate (25%).