Nomenclature of the primary telangiectasias.
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Biomedical subjects
Publications and source records attributed to M J Rothe.
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The mainstays of treatment of cutaneous lupus erythematosus (LE) are topical and intralesional steroids, sunscreen, and the antimalarials. However, the practitioner is often faced with the challenge of cutaneous disease unresponsive to these conventional measures. We present our approach to the management of cutaneous LE and review the literature regarding therapy of refractory disease.
Antimalarial compounds have been demonstrated to be both safe and efficacious in the treatment of lupus erythematosus as well as several other dermatologic conditions. It is imperative, however, that physicians familiarize themselves with the potential complications associated with the use of these agents. Furthermore, it is essential that a successful collaborative relationship be developed between the dermatologist and the ophthalmologist who are to treat and monitor the patient to avoid any untoward complications that might afflict the patient. It is important to stress that patients on antimalarial drug therapy be evaluated by an ophthalmologist prior to starting treatment and every 4 to 6 months thereafter. The use of Amsler's grids in compliant patients is recommended. If the patient develops retinal changes consistent with the diagnosis of premaculopathy, then careful consideration should be given to the availability and use of alternative agents.
Ocular and cutaneous stigmata are hallmarks of the heritable disorders of collagen and elastic tissue. Awareness of the oculocutaneous features is essential both to aid diagnosis and to direct management of affected patients. Although the angioid streaks of PXE, blue sclerae of OI, ocular fragility of EDS and ectopia lentis of Marfan syndrome are the best-known ocular signs of these disorders, other relevant ophthalmologic symptoms often accompany such classic findings.
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A patient experienced rapidly proliferating, multiple cutaneous hemangiomas subject to significant hemorrhage, with associated asymptomatic pulmonary involvement. The dramatic features of his cutaneous disease, an overview of the spectrum of neonatal hemangiomatosis, and recent therapeutic options are reviewed.
Oral acyclovir recently was approved for the treatment of herpes zoster and has been shown to be effective in the treatment of varicella. Studies of the use of high-dose oral acyclovir in the management of varicella-zoster infections in immunocompetent pediatric patients are reviewed. Guidelines are provided for the drug's use in immunocompetent children and adolescents, pregnant patients, and pediatric atopic patients receiving systemic steroids.
Allergists and dermatologists both care for patients with atopic dermatitis, and their roles may be complementary. Questions regarding food allergy, aeroallergens, and the interpretation of skin and RAST testing are answered by an allergist.
The presence of mast cells, often in an activated state or increased numbers, has been noted in various cutaneous disorders. Recent studies suggest that mast cells are of primary importance in these conditions and their presence does not merely represent a secondary event. This review focuses on atopic dermatitis, contact hypersensitivity reactions, bullous pemphigoid, fibrosing conditions, neurofibromatosis, wound healing, polycythemia vera, and psoriasis, in which there is significant evidence to indicate a pathophysiologic role for mast cells.
Ichthyosis follicularis (IF) is a rare disorder of keratinization that has been described primarily in males and proposed as a possible X-linked disorder. We report two black girls with nonscarring alopecia; photophobia; follicular hyperkeratoses; hyperkeratosis of the extensor aspects of the hands, knees, and elbows; fixed, erythematous, perineal plaques; and angular cheilitis who seem to fit the clinical criteria for IF. One girl also had gingival hypertrophy and a hearing deficit. One child's father had identical symptoms. We propose that these girls may have a variant of IF that is inherited as an autosomal dominant trait.
Calcinosis cutis is a common clinical feature of dermatomyositis and scleroderma but is only rarely reported in association with systemic lupus erythematosus (SLE). We describe three patients with long-standing systemic lupus erythematosus in whom extensive calcinosis cutis developed. We identify characteristics our patients share in common with 23 previously described patients.
A case of scleromyxedema with associated rhabdomyolysis is reported. This appears to be a well-documented, although rare complication of scleromyxedema. This case is unique in that mucin was demonstrated in the muscle as well as the skin, and spontaneous resolution of the rhabdomyolysis occurred with the implementation of IV fluids and bed rest.
We have measured oestrogen and progesterone binding in specimens of histopathologically confirmed lentigo maligna (LM) lesions excised from five elderly white men, using a dextran-coated charcoal method. Oestrogen binding was observed in four of the five specimens, and progesterone binding in all five. Marginal normal skin showed only non-specific binding for oestrogen and progesterone. The presence of steroid hormone receptors in LM suggests the possibility that hormone responsiveness may be relevant in the transformation of a lentigo maligna into malignant melanoma.
We have measured the plasma levels of somatomedin-C (SM-C) or insulin-like growth factor I (IGF-I) in 13 patients with progressive systemic sclerosis (PSS) and age and sex matched healthy controls. We found the plasma SM-C levels to be within normal limits in all the patients. Thus, if somatomedin-C plays a role in the pathogenesis of PSS, it is more likely to be at the fibroblast receptor level or in the synthetic response of fibroblasts to SM-C.
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