Desmoplastic hairless hypopigmented naevus: a variant of giant congenital melanocytic naevus.

Desmoplasia has been described in melanoma and Spitz naevus but not in giant congenital melanocytic naevus (GCMN). In melanoma desmoplasia is associated with a better survival. Four paediatric patients with hard, ligneous, progressively hypopigmented and alopecic GCMN were seen among 143 cases of GCMN at the Department of Dermatology of the National Institute of Paediatrics, Mexico City. Clinically, induration was progressive in three patients and regressive in one. Pigmentation was regressive in all. Histopathologically, all four patients showed intense dermal fibrosis, scarce naevus cells, and hypotrophic or absent hair follicles. Follow-up and serial biopsies in three patients documented the progressive nature of fibrosis and naevus cell depletion. No evidence of malignant transformation was found. Naevus cell depletion resulted in pigment loss and may have reduced the risk of malignant transformation. Although the cause of fibrosis is unknown, the possibility of an immune reaction to naevus cells is postulated.

Treatment of 18 children with scabies or cutaneous larva migrans using ivermectin.

In addition to onchocerciasis and other filarial diseases, ivermectin has been used for the treatment of scabies, head lice, larva migrans and gnathostomiasis. However, there is concern regarding the safety of its use in children under 5 years of age or weighing less than 15 kg. We present our experience in 18 children (aged 14 months to 17 years), with scabies or cutaneous larva migrans successfully treated with ivermectin. They included four cases of crusted scabies associated with immunosuppression and seven cases of common scabies four of whom had associated clinical mental retardation, immunosuppression or hypomobility. A further seven patients had cutaneous larva migrans. Fifteen patients were cured with a single dose of ivermectin, and three patients with crusted scabies required a second dose. None of our patients suffered significant adverse effects. We believe that ivermectin is a safe and effective alternative treatment of cutaneous parasitosis in children.

Unusual leukoderma after erythema multiforme: a case report.

Erythema multiforme is an inflammatory disorder of the skin that usually fades without sequelae. It is well known that after inflammatory events, hyper- or hypochromic spots can remain, especially in skin types III-VI, but achromia is very rare. We report a case of residual leukoderma after erythema multiforme.

Circle hairs: a clinical curiosity.

A case of circle hairs is reported and the literature on the subject reviewed. Differential diagnoses are discussed as well as some theories on the nature of circle hairs.

The use of retinoids in the pediatric patient.

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.

Cleansers, moisturizers, and talcs: patterns of use among children of low socioeconomic level.

The use of cosmetics (cleansers, moisturizers, and talcs) was investigated in 200 infants and children of low socioeconomic level with and without skin disease. For children with atopic dermatitis, mothers preferred to use unscented soap. Lubricating or hydrating creams were widely used in children without skin disease. Talc and mineral oil were significantly more frequently used in infants than in children. Both of these products are potentially harmful and represent an unnecessary expense for families with a survival-level budget. Dermatologists should discourage their use.

Intrauterine epidermal necrosis: report of three cases.

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.

Acute annular urticaria in infants and children.

Characteristic features of acute annular urticaria in 34 infants and small children were large, erythematous annular and polycyclic lesions with violaceous centers, eyelid, hand, and foot edema, absence of angioedema of the airway, absence of pruritus, spontaneous resolution in 8 to 10 days, and frequent history of furazolidone medication for diarrhea. Nondermatologists often misdiagnose acute annular urticaria as erythema multiforme and unnecessarily overtreat patients. The differential diagnosis with other conditions presenting with annular lesions in children is discussed.

Giant congenital melanocytic nevi, neurocutaneous melanosis and neurological alterations.

BACKGROUND: Magnetic resonance imaging (MRI) findings suggestive of neurocutaneous melanosis (NCM) have been reported in asymptomatic patients with giant congenital melanocytic nevi (GCMN). OBJECTIVE: To investigate the presence of NCM and the clinical neurologic status of patients with GCMN involving the head an neck. METHODS: Thirteen patients with GCMN involving the head and neck were clinically examined by pediatric specialists in dermatology, ophthalmology and neurology. Electroencephalograms, noncontrasted and contrasted computerized tomography (CT) scans and MRI were performed. RESULTS: Eleven of 13 patients with GCMN of the head and neck previously considered asymptomatic were found to present mild but evident neurologic alterations. No signs of NCM were found in the CT scans or in the MRI. CONCLUSIONS: Patients with GCMN of the head and neck may have associated neurologic alterations not related to the presence of neurocutaneous melanosis.

Proteus syndrome: new findings in seven patients.

Seven children with Proteus syndrome (PS) are reported. The majority of clinical findings coincide with what is reported in the literature. New findings were blue sclerae, telecanthus, epiblepharon, endotropy, hemimegaly of the optic nerve, occipital dysmyelination and compression of the corpus callosum, craneosynostosis, decalcification and thinning of the cortical layer of long bones, and talipes equinus. The clinical findings, possible etiology, differential diagnosis, and treatment of PS are discussed.

Keratitis, ichthyosis, and deafness (KID syndrome): review of the literature and proposal of a new terminology.

The so-called KID (keratitis, ichthyosis, deafness) syndrome is a congenital disorder of ectoderm that affects not only the epidermis, but also other ectodermal tissues such as the corneal epithelium and the inner ear. Sixty-one patients who fulfill the criteria for this syndrome were identified in a review of the literature through December 1993. All had cutaneous and auditory abnormalities, and 95% also had ophthalmologic defects. The most frequent clinical features were neurosensory deafness 90%, erythrokeratoderma 89%, vascularizing keratitis 79%, alopecia 79%, and reticulated hyperkeratosis of the palms and soles 41%. All of these findings constitute the major criteria for the diagnosis. The KID acronym does not accurately define this entity since the disorder is not an ichthyosis, because scaling is not the main cutaneous feature and not all patients have keratitis early in the course. We suggest that this syndrome should be included under the general heading of congenital ectodermal defects as a keratodermatous ectodermal dysplasia (KED).

Encephalocraniocutaneous lipomatosis: an uncommon neurocutaneous syndrome.

We describe a 2 1/2-year-old girl with encephalocraniocutaneous lipomatosis. The dysmorphologic manifestations in the skull, brain, skin, and eyes associated with a normal karyotype suggested the diagnosis of this rare neurocutaneous syndrome.

Edematous, scarring vasculitic panniculitis: a new multisystemic disease with malignant potential.

BACKGROUND: Hydroa vacciniforme (HV) is a disease of unknown origin characterized by erythema, vesicles, necrosis, and varicelliform scars in light-exposed skin. Systemic involvement is absent. A few patients have been reported with "severe HV" with systemic involvement, development of non-Hodgkin's lymphoma, and a poor prognosis. OBJECTIVE: Our purpose was to characterize and differentiate our patients' disease from HV. METHODS: We performed a retrospective clinicopathologic study of 14 children previously diagnosed as having "severe HV." RESULTS: The extension and severity of the cutaneous lesions, fever, wasting, failure to thrive, hepatosplenomegaly, vasculitis, panniculitis, and potential development of lymphoma are features that clearly differentiate edematous scarring vasculitic panniculitis from HV. CONCLUSION: Edematous scarring vasculitic panniculitis is a novel multisystemic disease with malignant potential that is not related to classic HV.

[Tuberous sclerosis. Early diagnosis with autonomic nervous system responses in hypopigmented skin]. / Esclerosis tuberosa. Diagnóstico precoz mediante respuestas de sistema nervioso autónomo en piel hipopigmentada.

The earliest clinical sign for the diagnosis of tuberous sclerosis (TS) is the presence of cutaneous hypopigmented macules. However, there are no clinical, histopathological or functional criteria to discriminate between hipopigmented macules of TS and those without associated pathology. In this prospective study, the responses of the autonomous nervous system, erythema and sweating induced through iontophoresis with pilocarpine, were studied in three groups of patients (20 with TS, 10 with hipomelanosis of Ito, and 10 with hipopigmented macules without associated pathology). In hipopigmented macules without associated pathology, the responses were similar to those observed in normal skin. In TS erythema and sweating were significantly diminished (p = < 0.001). In hypomelanosis of Ito the decrease in erythema and sweating were not statistically significant. In TS the degree of decrease of erythema and sweating correlated positively with the severity of the neurological alterations. Light and electronmicroscopic studies of the hypopigmented macules in the three groups showed morphologically normal sweat glands and nerves. The latter suggests a disfunction of the sweat glands in TS as a cause of their abnormal behavior. We conclude that sweat testing in hypopigmented macules is a useful mean for the early diagnosis of ET.

Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital.

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.

Trichoepitheliomatous infiltration of the skin simulating leprosy.

A 13-year-old girl had a six-year history of infiltrated erythematous plaques on the face, alopecia of the eyebrows, diffuse alopecia of the scalp, and absence of body hair. Histologically, the lesions on the face and body corresponded to trichoepitheliomas. The lesions on the face clinically simulated lepromatous leprosy. This case probably represents an entity not previously described.

Dermoid cyst connected with the lacrimal canaliculum.

An infant had a 3 x 1.5-cm congenital, slow-growing, lobulated tumor below the right inferior eyelid. The mass transilluminated, and needle aspiration yielded tears. Dacryocystography showed a large cystic area connected with the lower lacrimal canaliculum. Histologically, the tumor proved to be a dermoid cyst. This association has not been reported previously. Surgical excision and suture of the pedicule resulted in permanent cure.

[Epidemiology of skin diseases in 10,000 patients of pediatric age]. / Epidemiología de las enfermedades de la piel en diez mil pacientes en edad pediátrica

Ten thousand new patients were seen between January 1971 and January 1975 at the Department of Pediatric Dermatology, "Hospital del Niño IMAN" in Mexico City. The patients were between 0 and 18 years old. They represented 10.4% of the entire pediatric population attending the general out-patient clinic of the hospital. The results of our study showed no significant difference among the sexes of patients. The highest proportion of patients in the sample were under one year of age. The most frequently observed groups of dermatoses were: parasitic, cutaneous reactions, viral and bacterial. The ten diseases most frequently observed were: papular urticaria (16.3%); atopic dermatitis (12.9%); scabies (10.4%); viral warts (8.4%); impetigo (6.8%); pitiriasis alba (6.6%); vitiligo (2.6%); acne (2.5%); dermatophytosis (2.4%) and numular eczema (2.3%). The 20 skin diseases most frequently recorded accounted for 85.8% of the sample. The ten most frequent skin diseases in each group of age were also recorded. The monthly and annual frequency for the more frequent dermatoses was studied. The present study provides epidemiological information for a rational development of programs on care, education and investigation in pediatric dermatology in Mexico.