Giant congenital nevus with progressive sclerodermoid reaction in a newborn.

Giant congenital melanocytic nevi are a rare occurrence in the pediatric population. The risk of malignant transformation associated with these lesions has been well established; however, the management strategies for giant congenital nevi remain controversial. We report an unusual sclerodermoid reaction in a giant congenital nevus in a 6-week-old Caucasian girl. Given its abnormal clinical appearance, the entire lesion was excised. The histology was consistent with an atypical compound/sclerosing spindle and epithelioid cell congenital nevus. No evidence of malignant change was seen histologically. The incidence of malignant transformation in giant congenital nevi has been difficult to calculate. Review of the literature yields an incidence of between 4 and 9%, favoring surgical excision of these lesions where possible. Atypical presentations of giant congenital nevi are rare, and we have found no other reported cases with a stromal change similar to that seen in our patient. We hypothesize that this change may represent an atypical host reaction to the nevus cells.

Disposable baby wipes: efficacy and skin mildness.

The results of a series of four clinical studies demonstrated that disposable baby wipes were milder to the skin than use of a cotton washcloth and water, recognized as a "gold standard" for skin mildness. Importantly, the baby wipes caused no significant change from the baseline value in any of the skin parameters examined. This observation verified that the test wipes are minimally disruptive to the epidermal barrier and thus suitable for use on intact or compromised, irritated skin.

Argyria following the use of dietary supplements containing colloidal silver protein.

The onset of argyria following the use of dietary supplements containing colloidal silver protein is presented. The patient was using a silver-containing product for cold and allergy prophylaxis. We review the past and present medicinal roles of silver and include a differential diagnosis for argyria. The hyperpigmentation of argyria is usually permanent, and it follows a sun-exposed distribution. This case report highlights the potential for toxicity following the use of dietary supplements and demonstrates the importance of physician inquiry regarding alternative medicines. Finally, we examine the limited role of the Food and Drug Administration (FDA) in regulating alternative medicines marketed as dietary supplements.

Lymphomatoid granulomatosis in a 4-year-old boy.

Lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestructive infiltrative process involving primarily the lung, skin, central nervous system, and kidney. The incidence is highest in middle-aged men and is rare in children. We report a case of lymphomatoid granulomatosis involving both skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, which was unusual in light of recent evidence suggesting a B-cell origin in the majority of cases.

Pediatric skin tumors.

This article summarizes several malignant childhood neoplasms and benign tumors that can mimic malignancies. Because malignant skin tumors are rare in children, parents and physicians often are not sufficiently suspicious to ensure that an early diagnosis can be made. Many malignant skin tumors have features that suggest a vascular or hemangioma-like lesion. Because hemangiomas occur in 10% of infants, it is often considered prudent to adopt a wait-and-see attitude; however, if the lesion is too firm to be a hemangioma or its growth pattern does not follow that of a typical hemangioma, additional options should be considered. To manage childhood skin malignancies, one needs expert consultation, early biopsy, and correct histopathologic interpretation.

Basal cell carcinoma in children: report of 3 cases.

BACKGROUND: The peak incidence of basal cell carcinoma occurs in the seventh decade of life and is rare in children. When found in the pediatric age group, basal cell carcinoma is usually associated with a genetic defect, such as basal cell nevus syndrome, xeroderma pigmentosum, or nevus sebaceus. In areas of intense UV radiation exposure, such as the southwestern United States, children may be at increased risk of developing this malignancy de novo. OBSERVATIONS: Three children (2 boys, aged 8 and 16 years, and an 11-year-old girl) from Tucson, Ariz, with isolated basal cell carcinoma unassociated with any other disease or syndrome are described. CONCLUSIONS: Basal cell carcinoma in children is probably the result of a combination of UV radiation exposure and genetic background. Early recognition in children can prevent extensive tissue destruction and excess scarring after excision. A higher index of suspicion for basal cell carcinoma may also aid in prompt diagnosis of a possible genetic disorder, such as basal cell nevus syndrome.

Prepubertal hidradenitis suppurativa: two case reports and review of the literature.

Hidradenitis suppurativa (HS) is a chronic suppurative scarring disease of apocrine sweat gland-bearing skin in the axillary, anogenital, and, rarely, the breast and scalp regions. Females are more commonly affected than males and it is usually seen at puberty or later. We report two girls with prepubertal hidradenitis suppurativa whose initial presentation predated any signs of puberty. This early onset is very rare and its etiology remains unknown. Severe disease can be seen in prepubertal children and surgical intervention is effective in these cases.

Toddler-age nodulocystic acne.

Toddler-age nodulocystic acne is a rare disorder for which the etiology remains unknown. We report 2 cases and discuss the safety and efficacy of oral isotretinoin therapy. Oral isotretinoin is safe and effective in treating this condition but requires close monitoring.

Dermatologic manifestations of rheumatic diseases.

Cutaneous manifestations, potential sources of major discomfort, anxiety, and concern often coexist with or precede symptoms of systemic features of rheumatic diseases in children and adolescents. This chapter identifies skin features that may indicate rheumatologic disorders and describes various treatments and management approaches for cutaneous features.

Pediatric psychocutaneous disorders.

Psychosocial dysfunction in dermatologic disorders is more frequent than commonly recognized. This review discusses skin changes that may suggest a psychological problem and a screening survey (the Pediatric Symptom Checklist) that facilitates office assessment of the degree of psychosocial dysfunction present.

Congenital cutaneous candidiasis: case report and review.

This report describes a preterm infant with congenital cutaneous candidiasis associated with paronychia, dystrophy of the nail plates, and marked, transient leukocytosis.

Further delineation of the epidermal nevus syndrome: two cases with new findings and literature review.

"Epidermal nevus syndrome" ("ENS") is a neurocutaneous disorder in which epidermal nevi are associated with other abnormalities, most commonly of the skeletal and central nervous systems. We present two cases of epidermal nevus syndrome (ENS) with very different clinical findings. The first case is a newborn with multiple linear epidermal nevi of the trunk and limbs, and several other anomalies, including bony duplications of the lower limbs and hypoplastic left heart syndrome. The second patient, a 6-year-old boy, has a linear nevus sebaceous of the scalp with severe CNS involvement, including generalized seizures, moderate mental retardation, microcephaly, and a left hemiparesis. He also has genitourinary, cardiac, and skeletal defects. These two patients exhibit several abnormalities not previously recognized and illustrate the wide clinical spectrum of "epidermal nevus syndrome." We present a review of the clinical findings in 74 cases of "ENS." Correlation was noted between the presence of skin lesions located on the head and CNS involvement. The wide clinical spectrum of "ENS" as illustrated by these two patients suggests that "ENS" is a causally heterogeneous group of disorders.

Benign pigmented apocrine vulvar hamartomas.

Pigmented vulvar lesions were observed in a child during a sexual abuse evaluation. Gross examination of the lesions appeared most consistent with bowenoid papulosis; however, biopsy confirmed the lesions to be pigmented apocrine hamartomas. To our knowledge, these rare and benign tumors have never been described as pigmented, but should be added to the differential diagnosis of pigmented vulvar lesions.

Phototoxic coumarins in limes.

Coumarins in the rind and pulp of Persian and Key limes were quantified. In the rind of Persian limes, coumarin concentrations were in the order: limettin > bergapten > isopimpinellin > xanthotoxin > psoralen. In the rind of Key limes, psoralen and xanthotoxin were analytically absent; limettin was 10 times more concentrated than either bergapten or isopimpinellin, which were equal in concentration. Coumarin content in Persian lime pulp was in the order: isopimpinellin > limettin > bergapten > xanthotoxin > psoralen. For Key lime pulp, the concentrations of limettin, isopimpinellin and bergapten were equal; psoralen and xanthotoxin were not detected. Coumarins in lime pulp were 13 to 182 times less concentrated than those in the peel. Based on the amounts and types of coumarins, Persian limes appear to be potentially more phototoxic than Key limes. Although bergapten may be the main component of limes responsible for phytophotodermatitis, dermatological interaction assays with psoralen, bergapten, xanthotoxin and limettin should be conducted.