Biomedical subjects
Publications and source records attributed to S H Wakelin.
Brachioradial pruritus (BRP) is an uncommon localized pruritic dermatosis only previously described from the tropics and subtropics. We report two cases in British residents who had a history of prolonged ultraviolet radiation exposure, and who responded to strict photoprotection of the affected sites.
Bullous pemphigoid (BP) is an acquired bullous disorder which predominantly affects the elderly. It is rare in children, and may be clinically indistinguishable from other immunobullous disorders. As routine histology may be non-specific, a definitive diagnosis of childhood BP usually depends on the results of direct and indirect immunofluorescence investigations. We report a 5-year-old girl who developed bullous pemphigoid, associated with atypical immunofluorescence findings. Indirect immunofluorescence on split-skin showed a pure dermal pattern of IgG binding. This is usually suggestive of epidermolysis bullosa acquisita, but Western immunoblotting was positive with epidermal extracts, confirming a diagnosis of BP. Dermal binding on split-skin occurs in about 5% of adult cases of BP, and has not been reported previously in childhood BP.
Astophoderma of Pasini and Pierini is generally regarded as an atrophic variant of morphoea. It arises most commonly on the trunk and abdomen. We describe a 53-year-old man who had a 12-year history of unilateral atrophoderma of Pasini and Pierini affecting the left side of his trunk in a zosteriform distribution. Within these clinically atrophic lesions, there were localized areas of induration which were typical of morphoea. The patient also had a preceding history of balanitis xerotica obliterans. The occurrence of atrophoderma of Pasini and Pierini, morphoea and lichen sclerosus within the same individual supports the view that these conditions are closely related, and suggests that common factors may be involved in their aetiologies.
Pustulation is a major feature in several different dermatoses, and it may also occur as a manifestation of drug hypersensitivity. Acute generalized exanthematous pustulosis (AGEP) is an uncommon eruption characterized by acute, extensive formation of sterile pustules, fever and peripheral blood leucocytosis. It shares several clinical and histological features in common with pustular psoriasis. Most reported cases have been triggered by ingestion of broad spectrum antibiotics, particularly betalactams and macrolides. There is usually rapid resolution of the eruption on drug withdrawal. We report the case of a 58 year-old woman who developed AGEP shortly after commencing treatment with the calcium channel blocker diltiazem hydrochloride. The eruption followed a biphasic course, and improved following treatment with systemic corticosteroids and methotrexate. AGEP appears to be a rare adverse cutaneous reaction to diltiazem, whereas a wide range of other skin eruptions have been reported more commonly with this drug.
Sarcoidosis is a chronic granulomatous disease of unknown origin that affects multiple organs and may present with a variety of skin lesions. Involvement of the nails is rare and almost invariably associated with underlying bone disease. We describe a patient with sarcoid nail dystrophy in whom this diagnosis was confirmed by a proximal nail fold biopsy. Radiologic investigation did not show evidence of an associated bone dystrophy in this case.
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A 55-year-old woman is described with a short history of generalized lichen sclerosus et atrophicus in whom extensive blistering and ulceration occurred. The disease was confined to extragenital sites at presentation, with no clinical evidence of mucosal involvement. The degree of blistering, rapid evolution and widespread involvement of non-genital skin in this case are uncommon features. There was evidence of improvement following treatment with hydroxychloroquine. We suggest that this drug may be of therapeutic benefit in cutaneous lichen sclerosus.
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The immunobullous diseases bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) are very rare in childhood. Although case studies have been detailed, there are no reports of a large series of patients documenting the effectiveness of treatment and long-term prognosis. We report the clinical presentation, immunopathologic features, disease course, and long-term prognosis of BP and EBA in a series of 11 children. The initial diagnoses based on clinical features were BP (5), EBA (3), and chronic bullous disease of childhood (CBDC) (3). These were subsequently revised from BP to EBA (2), CBDC to BP (2), and CBDC to BP or EBA (1) following the results of direct and indirect immunofluorescence and immunoblotting. Analysis of IgG subclasses in eight cases showed that the predominant subclasses were IgG1 (8) and IgG4 (6). The clinical features appeared to be highly variable, and in patients presenting with inflammatory blistering, laboratory studies were required in order to differentiate between BP and EBA. All patients improved on treatment with corticosteroids and/or sulfones, although treatment regimens showed wide variation. Their diseases tended to remit within 2 years, and their long-term prognosis was good.
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