Lourdes Tamayo

Giant pigmented nevi: Clinical, histopathologic, and therapeutic considerations

Eighty pediatric patients with giant pigmented nevi more than 20 cm in their greatest diameter are reported. The incidence was 1 in 4150 general pediatric outpatients. The mode of inheritance of giant pigmented nevi is probably multifactorial; four second-degree relatives of our patients also had large nevi, and there was a 2:1 female predominance. Satellite nevi were present in 74% and nevi in mucous membranes in 31% of the patients. Eighty-six percent of nevi were pigmented and hairy. Benign nodules were observed in 19% of the patients and plexiform overgrowths in 6%. Nevi extensively involving the extremities resulted in reduced growth of the affected limb. Electroencephalograms showed abnormalities in 20% of the patients with giant pigmented nevi involving the head and upper portion of the trunk. Malignant transformation appeared in four patients and was fatal in three of them. Management consisted of observation only in 49%, surgery in 27.5%, chemical peel in 21%, and dermabrasion in 2.5% of the patients. The mean follow-up was 4.7 years.

Oral Retinoid (Ro 10-9359) in Children with Lamellar Ichthyosis, Epidermolytic Hyperkeratosis and Symmetrical Progressive Erythrokeratoderma

8 children with lamellar ichthyosis, 1 with epidermolytic hyperkeratosis and 5 with symmetrical progressive erythrokeratoderma were treated with a new aromatic retinoid (Ro 10-9359). Clinical improvement was dramatic. The children acquired an appearance never obtained before with other managements. The treatment had to be maintained to prevent recurrence. The tolerance to the drug was good. The side-effects were minimal and tended to disappear after several months of treatment. Our results suggest that because of its efficacy, good tolerance and easy administration, the oral retinoid Ro 10-9359 is at present the treatment of choice for the great ichthyotic disorders of children.

Pityrosporum ovale in infantile seborrheic dermatitis.

Abstract: The presence of Pityrosporum ovale was investigated in four groups of infants age 1 to 24 months, 15 with infantile seborrheic dermatitis, 15 with infantile atopic dermatitis, 15 with other infantile dermatoses, and 15 healthy infants. Samples were taken from the scalp, face, presternal area, and inguinal area. Pityrosporum ovale was detected by smears and/or cultures in 73% of infants with seborrheic dermatitis, 33% with atopic dermatitis, 33% with other dermatoses, and 53% of healthy infants. The percentages of positive smears and/or cultures from four body sites in each patient group were 42% for seborrheic dermatitis, 20% for atopic dermatitis, 20% for other infantile dermatoses, and 23% for healthy infants. The majority of infants with positive cultures or positive direct examination for P. ovale were between 1 and 8 months of age. The organism was isolated in 28% of samples taken from the scalp, 32% from the face, 30% from the presternal area, and 15% from the inguinal area. Patients with infantile seborrheic dermatitis were treated with 2% topical ketoconazole cream for two weeks. Eleven of these children were clinically cleared and 13 became mycologically negative.

Erythrokeratodermia progressiva symmetrica

10 cases of erythrokeratodermia progressiva symmetrica are described; 6 were familial, with autosomal-dominant inheritance. The disease started in early childhood, progressed during 1 or 2 years and then remained stationary with sharply marginated plaques remarkably symmetrical, usually sparing the thorax and abdomen. Histologically the picture was psoriasiform. The best therapeutic control was obtained with oral aromatic retinoids. The differential diagnosis is discussed.

Retinoids in disorders of keratinization: their use in children.

During the last 10 years we treated 39 children with severe keratinization disorders with the aromatic retinoid etretinate. Six of these children were followed-up for 8-9 years. Mucocutaneous serum enzymatic and lipid side effects of etretinate were mild, transient and well tolerated. Osseous side effects were present after 4-6 years in all our 6 patients on prolonged retinoid therapy. Asymptomatic osseous neoformation and osseous reabsorption in the absence of calcium, phosphate, and alkaline phosphatase serum alterations have been observed. The growth and development curves and the sexual development of our patients (with exception of a patient with Ruds syndrome) have been normal. Osteoporosis and slender diaphysis were often present at initiation of therapy. On the basis of our findings and recent reports of the literature we suggest restricting retinoid therapy of keratinizing disorders in children to conditions severe enough to be physically, psychologically or socially incapacitating. In an attempt to reduce the risk of chronic toxicity and possibly to allow regression of initial bone alterations, intermittent therapy and combination therapy are recommended.

Focal dermal hypoplasia

Focal dermal hypoplasia in five sisters and their mother is presented. The lesions present in this family and in eight additional Mexican cases are reviewed. Fourteen out of 15 cases were females and two were males. All cases had cutaneous lesions, clinically typical of focal dermal hypoplasia; these were confirmed histologically. Osseous changes and syndactyly were the more frequently associated lesions. The resemblance of this syndrome to incontinentia pigmenti and to aplasia cutis congenita is stressed. The study of this family, together with five families reported in the literature, suggests X‐linked dominant inheritance with lethality in males. The known male cases could represent occasional survival or genetic heterogeneity.

Incontinentia pigmenti achromians(Ito's hypomelanosis)

ABSTRACT: A 2‐year‐old Mexican girl had hypochromic lesions characteristic of incon‐tinentia pigmenti achromians on the right half of the body. Extra cutaneous alterations were: dacryostenosis, bilateral genu valgus, ataxic gait, language retardation, hydrocephaly and abnormal electroencephalogram. Electron microscopy showed numerous Langerhans cells in the epidermis.

Staphylococcal blistering dactylitis: report of two patients.

Abstract: Two children with staphylococcal blistering distal dactylitis (BDD), a condition usually caused by streptococci, are reported. Etiologic distinction in BDD can be made only bacteriologically. Staphylococci are increasingly the cause of bacterial cutaneous infections.

NEUROICHTHYOSIS WITH HYPOGONADSSM (RUD'S SYNDROME)

ABSTRACT: Ruds syndrome is a neuroichthyosis with hypogonadism, associated with mental deficiency and epilepsy. Short stature is a frequent component of the syndrome. The primary genetic defect and the pattern of inheritance have not yet been determined. A typical patient is presented, with mental deficiency, short stature, hypoacusia, muscular atrophy, tylosis, pseudoacanthosis nigricans and endocrine disturbances. The neuroichthyosis with hypogonadism must be considered Ruds syndrome. A classification of neuroichthyosis is proposed. In a first group is neuroichthyosis wilh hypogonadism, in the second group is neuroichthyosis with spasticity and in the third group, neuroichthyosis without hypogonadism or spasticity.

DIFFUSE DERMOGRAPHIC MASTOCYTOSIS WITHOUT VISIBLE SKIN LESIONS

ABSTRACT: The case is presented of a 3‐year‐old girl with urticaria and pressure dermographism. The condition began the age of one year. A skin biopsy confirmed the suspected diagnosis of diffuse mastocytosis.