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Keratosis lichenoides chronica (KLC) is a rare acquired disease of adulthood, of unknown etiology, characterized by keratotic parallel linear lesions, retiform plaques, and keratotic, often follicular papules, chronicity and lichenoid histopathologic features. KLC of pediatric onset is considered extremely rare. Its features and relationship to adult onset KLC are unknown. We studied 8 cases of pediatric-onset KLC in the literature and 6 personal cases and compared them with 40 reported adult-onset KLC patients. The following features characterize pediatric-onset KLC: familial occurrence; probable autosomal recessive inheritance; early or congenital onset with facial erythemato-purpuric macules; forehead, eyebrow, and eyelash alopecia; pruritus; and a low frequency of other cutaneous and systemic abnormalities. Pediatric-onset KLC may represent a different disease or a subset of adult-onset KLC, with special genetic and clinical characteristics. Determining its precise nosology will have prognostic and therapeutic implications.

Oral retinoids are molecules derived from vitamin A that represent one of the most important steps forward in dermatologic therapeutics in the present century. The treatment of acne, severe psoriasis, and severe disorders of keratinization, prevalent diseases in children and adolescents, have radically changed since the advent of oral retinoids. Like most highly-effective medications, oral retinoids also have important untoward effects. Specialists, and in particular, dermatologists and pediatricians should be prepared to maneuver the delicate balance between therapeutic efficacy and side effects in order to give the pediatric patient the maximum benefit with the lowest possible risk.

Background: Extensive epidermal necrosis in newborn infants is an unusual event of heterogeneous cause. Objective: The objective of this article is to describe what seems to be a previously unrecognized lethal disease. Methods: The clinical and histopathologic features of three premature infants, two of them nonidentical twins, and the autopsy findings of one of them were analyzed. Results: Intrauterine lethal epidermal necrosis with hair follicle calcification, except for the face, hands, feet, elbows, and knees, was present in all three patients. Some histopathologic features were suggestive of epidermal apoptosis. Conclusion: We propose that the clinicopathologic alterations in our patients represent a new condition that may be caused by massive epidermal apoptosis.(J Am Acad Dermatol 1998;38:712-5.)

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.