Jane M. Grant-Kels

Drug-induced toxic epidermal necrolysis treated with cyclosporin.

A 35‐year‐old woman developed toxic epidermal necrolysis secondary to phenytoin. Because the life‐threatening eruption was resistant to prednisone and high‐dose methylprednisolone therapy, cyclosporine therapy was initiated. Within 24–48 hours, the eruption stabilized and the patient improved.

Cutaneous Manifestations of Cystic Fibrosis

Abstract:u2002 Cystic fibrosis is an autosomal recessive disease reported in 1 in 2500 live births in Northern American and Northern European Caucasian populations. Classic disease findings include chronic bacterial infection of airways and sinuses, malabsorption of fat, infertility in men, and elevated concentrations of chloride in sweat. Less well‐recognized findings associated with cystic fibrosis include cutaneous findings, which can be primary or secondary manifestations of the disease process. Patients demonstrate more atopic and drug hypersensitivity reactions than the general population, but have similar rates of urticaria compared with the general population. In atypical presentations of cystic fibrosis, the nutrient deficiency dermatitis of the disease may aid with diagnosis, and notably can be the presenting sign. Other dermatologic manifestations of cystic fibrosis include early aquagenic skin wrinkling and cutaneous vasculitis, which can be associated with arthralgias. Familiarity with the nutrient deficiency dermatitis of this entity may play a role in the timely diagnosis of the disease, and the other cutaneous findings add to our understanding of the protean nature of its manifestations.

Controversy: are systemic steroids indicated in the treatment of erythema multiforme?

Abstract: The treatment of erythema multiforme major with systemic steroids became established during the 1950s. Recently, two retrospective case reviews comparing steroid‐treated and nonsteroid‐treated groups of patients with erythema multiforme found that these agents may be associated with complications. As a result, many clinicians have become uncertain as to the appropriate therapy of this disease entity. We successfully treated the condition with steroids in two children and one adolescent. The controversy over the potential efficacy of such therapy for erythema multiforme persists, however.

Childhood subungual melanoma in situ in diffuse nail melanosis beginning as expanding longitudinal melanonychia

Abstract:u2002 We report a rare childhood occurrence of melanoma in situ presenting as diffuse nail pigmentation resulting from expanding longitudinal melanonychia, and discuss factors that should come into play when considering a possible nail matrix biopsy.

Multiple Agminate Spitz Nevi

Abstract: A five‐year‐old boy had multiple agminate Spitz nevi arising in an area of hyperpigmentation that developed concurrently with the nevi. Multiple Spitz nevi occurring in a single group are rare, and their presence on a hyperpigmented background is extremely uncommon. Very few such cases have been described, and all of those leslons arose on a congenital hyperpigmented patch. In contrast, the nevi in our patient appeared to arise on an acquired hyperpigmented patch that was not present at birth, and histologically did not demonstrate features of a congenital nevus. Accurate diagnosis is necessary to avoid unnecessary radical therapy.

PRIMARY TELANGIECTASIA OF CHILDHOOD

Telangiectasias are dilated blood vessels appearing on the skin and mucous membranes. Their presence may be insignificant, as in the syndrome of hereditary benign telangiectasia, or they may indicate serious disease, as in ataxia-telangiectasia (A-T). HOW do primary care physicians and dermatologists, who are often the first to observe such lesions, distinguish whether or not a given telangiectasia is a significant clinical finding. How do they decide how many can be present on a normal child? Characteristics of telangiectasias helpful in making this distinction include morphology, distribution, number, associated abnormalities, pattern of inheritance, tendency for progression or spontaneous regression, and age of onset.

ECCRINE SWEAT GLAND DISORDERS: PART I — NEOPLASMS

Many eccrine gland neoplasms can be separated into categories based upon anatomic appearance atid microscopic differentiation as tbese cbaracteristics pertain to normal eccrine gland arcbitecture (Table 1). Eccrine lobules are cotnposed of glands and ducts (Fig. 1). The glandular or secretory portion is a coiled tubule located in tbe dertnis or upper portion of tbe subcutaneous fat. Tbe secretory segment differentiates into tbree cell types: clear (serous) cells, dark (mucous) cells, and tnyoepitbelial cells. The ducts are located witbin tbe dermis and epidertnis. Tbe intradermal segtnent is cotnposed of a coiled portion tbat is continuous witb botb tbe coiled tubule of tbe gland and the straight segmetit of tbe intradermal duct. Tbis segtnent ascends tbe entire lengtb of tbe reticular and papillary dertnis and crosses tbe epidermis to open tbrough tiny pores onto tbe skin surface in a spiral fasbion. Tbe intraepidermal portion of tbe eccrine duct is called tbe acrosyringium.^

Lymphotropic Adamantinoid Trichoblastoma

Abstract:u2002 We report a lymphotropic adamantinoid trichoblastoma in a 14‐year‐old girl. This is a rarely reported skin adnexal tumor in adolescent patients. The clinical, histologic, and immunohistochemical features of this tumor are described.

Xanthomas and the inherited hyperlipoproteinemias in children and adolescents.

Abstract: Early recognition of the hyperlipoproteinemias is a crucial element in preventing premature coronary artery disease. Xanthomas provide a cutaneous marker of the silent, underlying pathology. Identifying them, and understanding their relation to the inherited hyperlipoproteinemias may facilitate early diagnosis of hyperlipoproteinemias in pediatric patients. The dermatologist plays an important role in identifying these lesions and in subsequently facilitating prompt referral and treatment.

Allergy Consultation in the Management of Atopic Dermatitis

Abstract: 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.