Biomedical subjects
Publications and source records attributed to J I Harper.
This is a case report of a 25-year-old man, with severe lipoid proteinosis of the oropharynx and larynx, who underwent microlaryngeal surgery. The condition is discussed with particular reference to the surgical management of the laryngeal lesions.
A 61-year-old woman is presented with essential cryoglobulinaemia and a 25-year history of recurrent ulceration of the lower legs. She developed multiple benign angiomatous nodules on the face, earlobes and neck. Histologically, they simulated an angiosarcoma and are therefore of considerable diagnostic importance.
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Topical methoxsalen photochemotherapy has been assessed in 22 patients suffering from recalcitrant palmoplantar pustulosis or psoriasis predominantly involving the hands and feet. Although 20 out of 22 patients improved and good results were obtained in exactly half of those treated, only 4 patients were classified as being "clear" or "minimally involved" at the end of 12 weeks. Two patients have shown no improvement at all. Minor local side effects were relatively common and included symptomatic erythema, blistering and local pigmentation. The histological findings in post PUVA treated skin are discussed and the pros and cons of topical photochemotherapy in this group of diseases is reviewed.
The clinical differential diagnosis of erythroderma plus immunodeficiency and failure to thrive in neonates includes graft-versus-host-disease (GVHD), Omenn's syndrome (OS), and Netherton's syndrome (NS). In addition to immunological investigations, skin biopsy is an important part of the diagnostic work-up. We reviewed biopsies from 25 patients that were retrieved from the archives of the Department of Histopathology at Great Ormond Street, of which 9 were OS, 11 were GVHD, and 5 were NS. Five patients had two biopsy specimens. Both OS and GVHD show dyskeratosis and basal vacuolation. OS always shows acanthosis and almost always parakeratosis. GVHD shows a flat epidermis and rarely parakeratosis. OS and GVHD can be distinguished after immunohistochemistry for LCA and CD68 by the relative proportions of lymphocytes and macrophages in the dermal infiltrate (predominantly lymphocytes in OS, relatively more macrophages in GVHD). Skin biopsy diagnosis of OS is difficult before 6 weeks of age because the features are poorly developed. NS can be distinguished by psoriasiform acanthosis, thickening of the basement membrane, prominent dermal blood vessels, absence of dyskeratosis, and basal layer vacuolation, and a dermal infiltrate in which lymphocytes and macrophages are equally represented. Thus, the main difference between GVHD and OS is in the proportion of lymphocytes and macrophages in the infiltrate on immunohistochemical staining for LCA and CD68, while OS and NS may be distinguished on H&E morphology alone.
The histology of skin biopsies from 46 cases of different forms of congenital ichthyosis was reviewed. Sections were examined for hyperkeratosis, follicular keratosis, appearance of the granular layer, epidermal thickness, tonofilament clumps, epidermal vacuolation, spongiosis, bullae and dyskeratosis, appearance of the basal layer, inflammation, mitoses, and adnexae. A detailed description of the histological features of each type of ichtnyosis studied is presented. Some ichthyoses can be recognized on routine hematoxylin and eosin staining (bullous ichthyosiform erythroderma, Netherton's syndrome, and neutral lipid storage disease); some forms require frozen sections to demonstrate fat (neutral lipid storage disease) or enzyme activity (Sjögren-Larsson syndrome). Protein electrophoresis and enzymology are necessary for X-linked recessive ichthyosis. A close liaison with the clinicians is essential for the diagnosis of all types of ichthyosis, and combined studies including routine histopathology, electron microscopy, and frozen sections may be required for the diagnosis.
Sjögren-Larsson syndrome is a rare autosomal recessive neurocutaneous disorder in which the combination of spasticity, ichthyosis, and mental retardation commonly result in patients being wheelchair bound. We reviewed a cohort of children with Sjögren-Larsson syndrome who were successfully managed with early physiotherapy and later soft-tissue surgery. The favourable outcome seen in these patients should encourage orthopaedic intervention in suitable cases.