Allergic contact dermatitis in children: a practical approach to management.

Allergic contact dermatitis (ACD) may account for at least 20% of all childhood dermatitis. Clinically, its morphology is identical to other forms of dermatitis in acute, subacute and chronic forms. A persistent or unusual and localized pattern is often the key to diagnosis. Treatment has centered around the use of corticosteroids, with the adjunct of antihistamines, wet dressings, and emollients for alleviation of symptoms. The newer topical immunosuppressives, tacrolimus and pimecrolimus, may also hold promise as alternative therapies, although they have not been well-studied in this regard. Allergen identification, sometimes through patch testing and allergen avoidance are the keys to preventing recurrences of this disease.

Unsuspected sporotrichosis in childhood.

We report 10 prepubertal girls with sporotrichosis who were misdiagnosed because they had solitary ulcerative skin nodules, rather than a "sporotrichoid" pattern of multiple linear nodules. All had positive cultures for Sporothrix schenckii. We urge clinicians to consider sporotrichosis in the differential diagnosis of a solitary skin nodule.

Kwashiorkor in the United States: fad diets, perceived and true milk allergy, and nutritional ignorance.

BACKGROUND: Kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme poverty in developing countries and with chronic malabsorptive conditions such as cystic fibrosis in developed countries. Rare cases of kwashiorkor in affluent countries unrelated to chronic illness have been reported. We present 12 cases of kwashiorkor unrelated to chronic illness seen over 9 years by pediatric dermatologists throughout the United States, and discuss common causative themes in this easily preventable condition. OBSERVATIONS: Twelve children were diagnosed as having kwashiorkor in 7 tertiary referral centers throughout the United States. The diagnoses were based on the characteristic rash and the overall clinical presentation. The rash consisted of an erosive, crusting, desquamating dermatitis sometimes with classic "pasted-on" scale-the so-called flaky paint sign. Most cases were due to nutritional ignorance, perceived milk intolerance, or food faddism. Half of the cases were the result of a deliberate deviation to a protein-deficient diet because of a perceived intolerance of formula or milk. Financial and social stresses were a factor in only 2 cases, and in both cases social chaos was more of a factor than an absolute lack of financial resources. Misleading dietary histories and the presence of edema masking growth failure obscured the clinical picture in some cases. CONCLUSIONS: Physicians should consider the diagnosis of kwashiorkor in children with perceived milk allergies resulting in frequent dietary manipulations, in children following fad or unorthodox diets, or in children living in homes with significant social chaos. The presence of edema and "flaky paint" dermatitis should prompt a careful dietary investigation.

Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry.

OBJECTIVE: To extend the information base on cutaneous manifestations of neonatal lupus erythematosus (NLE) with regard to maternal disease, sex of child, onset, localization, influence of UV light, prognosis, and recurrence rates in subsequent pregnancies. METHODS: Review of records from the Research Registry for Neonatal Lupus. RESULTS: The cohort includes 47 mothers (83% white) whose sera contain anti-SSA/Ro, anti-SSB/La, and/or anti-U1-ribonucleoprotein antibodies and their 57 infants (20 boys and 37 girls) diagnosed with cutaneous NLE (absent heart disease) between 1981 and 1997. At detection of the child's rash, 13 mothers were asymptomatic, 11 had an undifferentiated autoimmune syndrome (UAS), 9 had systemic lupus erythematosus (SLE), 7 Sjögren's syndrome (SS), 6 SLE/SS, and 1 rheumatoid arthritis/SS; 20 reported photosensitivity. Within 5 years, 7 asymptomatic mothers experienced disease progression: 1 developed photosensitivity, 2 SLE, 3 SS, 1 SLE/SS; in 2 mothers UAS progressed to SLE; and 2 mothers with SS developed SLE. The infant's rash often followed UV light exposure; mean age at detection was 6 weeks, and mean duration was 17 weeks. All had facial involvement (periorbital region most common) followed by the scalp, trunk, extremities, neck, and intertriginous areas. In 37, the rash resolved without sequelae, 43% of which were untreated. A quarter had residual sequelae that included telangiectasia and dyspigmentation. One child developed Hashimoto's thyroiditis, and 2 developed systemic-onset juvenile rheumatoid arthritis. Of 20 subsequent births, 7 children were healthy, 2 had congenital heart block (CHB) only, 4 CHB and skin rash, and 7 skin rash only. CONCLUSIONS: Future pregnancies should be monitored by serial echocardiograms, given the substantial risk for heart block. Affected children should be observed for later development of a rheumatic disease.

Benign cephalic histiocytosis with diabetes insipidus.

Benign cephalic histiocytosis is a rare skin condition consisting of small tan papules on the face and upper trunk that is believed not to be associated with internal organ involvement. The infiltrating histiocytes are not Langerhans' cells (LCs). We report a 5-year-old girl who presented with diabetes insipidus 1 year after developing multiple small brown asymptomatic skin papules. Histologic examination revealed a non-LC histiocytic proliferation in the dermis without epidermal invasion. She had infiltration of the pituitary stalk on brain imaging. Diabetes insipidus has heretofore been associated with LC histiocytosis and xanthoma disseminatum but not benign cephalic histiocytosis.

Allergic contact dermatitis.

Allergic contact dermatitis (ACD) in children is underrecognized. It is often confused with antibody-mediated allergies such as urticaria or allergic rhinitis, but the mechanism in ACD involves T lymphocytes and not antibody. Surprisingly, sensitization to common allergens is likely to occur in infancy. All contact allergens are weak allergens requiring repeated exposure over long periods of time. Detection of specific allergens is by epicutaneous (patch) testing and will provide the basis for allergen avoidance therapeutic strategies.

Childhood pernio and cryoproteins.

Childhood pernio is an uncommon condition described mainly through isolated case reports. We examined the cutaneous spectrum, clinical associations, presence of cryoproteins, and evolution of the condition in children, and performed a retrospective case series evaluation of children with pernio seen at a single ambulatory care university center over a 10-year period. Cases were drawn from a population of 3.2 million. Follow-up was at least 3 years. We found four boys and four girls with pernio. Distribution of skin lesions was on the fingers, toes, and ears. Four children had cryoglobulins or cold agglutinins, two had a positive rheumatoid factor, and none had a positive ANA or ANA profile. All eight cleared within 3 months and did not recur over at least a 3-year period. We concluded that childhood pernio is uncommon and may be associated with the presence of cryoproteins.

Examination of the oral mucosa and peripheral blood cells of patients with recurrent aphthous ulceration for human herpesvirus DNA.

OBJECTIVE: The purpose of this study was to exam the oral mucosa and peripheral blood cells of patients with recurrent aph-thous ulceration (RAU) for the presence of the following human herpesviruses: herpes simplex viruses 1 and 2, varicella zoster virus, Epstein-Barr virus, cytomegalovirus, human herpesvirus-6, and human herpesvirus-7. STUDY DESIGN: Fifty-eight subjects with RAU and 10 control subjects were recruited at an academic referral center and enrolled in this prospective, nonrandomized, case-controlled study. Each of the subjects with RAU was seen during an acute episode, and swab specimens from lesional (RAU-acute/lesion) and clinically normal (RAU-acute/normal) oral mucosa were obtained. Each of 2 subjects with RAU was evaluated during more than one acute episode. Three subjects with RAU were seen between active episodes, and swab specimens were taken from clinically normal (RAU-convalescent) oral mucosa. Swab specimens from clinically normal (control/normal) oral mucosa were obtained from the control subjects. Peripheral blood specimens were obtained from subjects with RAU and control subjects at the time the swab specimens were performed. Through use of polymerase chain reaction, all swab and peripheral blood specimens were examined for the presence of human herpesvirus DNA. Statistical significance was determined by means of chi(2) analysis. RESULTS: Herpes simplex virus and human herpesvirus-6 were found in a higher percentage of mucosal specimens from the control subjects (herpes simplex virus, 4/10; human herpesvirus-6, 5/9) than from the subjects with RAU (RAU-acute/lesion: 3/45 herpes simplex virus, 13/53 human herpesvirus-6; RAU-acute/normal: 7/48 herpes simplex virus, 9/53 human herpesvirus-6). No difference was demonstrated between RAU-acute/lesion, RAU-acute/normal, and RAU-convalescent mucosal specimens for any of the human herpesviruses. Different human herpesviruses were identified from individual subjects with RAU during subsequent episodes of disease. Epstein-Barr virus (6/35), human herpesvirus-6 (3/40), and human herpesvirus-7 (7/43) were detected in the peripheral blood mononuclear cells during acute RAU but not in RAU-convalescent or control peripheral blood mononuclear cells. CONCLUSIONS: The detection of human herpesvirus DNA from the oral mucosa and peripheral blood mononuclear cells of patients with RAU appears to represent normal viral shedding rather than a direct causal mechanism in this disorder.

Does sensitization to contact allergens begin in infancy?

OBJECTIVE: Because previous studies have found allergic contact sensitization common in children by 5 years of age, our aim was to determine the prevalence of positive epicutaneous test results in children <5 years of age and to determine whether sensitization to contact allergens was as common in infancy. METHODS: We recruited 95 asymptomatic children 6 months to 5 years of age from well-child visits at Denver area pediatric practices for epicutaneous patch testing using the T.R.U.E. Test system. Allergens were placed on the skin for 48 hours, and at a later follow-up visit, positive reactions were evaluated. RESULTS: A total of 85 patients completed the study. Of these, 20 (24.5%) had 1 or more positive reactions to the tested allergens. Positive reactors ranged from 6 to 65.5 months of age, with an average of 30.4 months of age. Of the children, 16 reacted to 1 allergen, and 4 reacted to 2. Eleven positive reactions were observed to nickel, followed by 8 to thimerosal. Other positive reactions were to neomycin, cobalt, and kathon CG. CONCLUSIONS: Children as young as 6 months of age may be sensitized to contact allergens. Within this pediatric population, the prevalence of sensitization is 24.5%. Sensitization to contact allergens may occur in infants.

Steroid rosacea in prepubertal children.

OBJECTIVE: To examine clinical associations, family history of rosacea, and response to treatment in prepubertal children with steroid rosacea. DESIGN: Retrospective case-series evaluation of children younger than 13 years with steroid rosacea seen over an 8-year period (1991-1998). SETTING: Ambulatory care university hospital. PATIENTS: Referral patients from pediatricians serving a population of 3.4 million. INTERVENTIONS: Abrupt cessation of topical corticosteroid use and initiation of treatment with oral erythromycin stearate for 4 weeks. MAIN OUTCOME MEASURES: Age at onset, class of topical corticosteroid used, family history of rosacea, location of lesions, treatment, and weeks to clearing. RESULTS: We evaluated 106 (46 boys and 60 girls) who developed steroid rosacea. Preceding steroids used were predominantly (54% of children) class 7 agents including 1% hydrocortisone and over-the-counter hydrocortisone preparations. Only 3% of children had used superpotent (class 1) topical corticosteroids. The mean age at onset was 7.04 years (range, 6 months to 13 years). Twenty-nine children were younger than 3 years. A family history of rosacea was found for 20% of the children. After abruptly stopping topical steroid use and starting treatment with oral erythromycin, 86% of children had complete clearing within 4 weeks and 100% by 8 weeks. Clearing within 3 weeks was observed in 22% of children. CONCLUSIONS: Abrupt discontinuation of topical corticosteroids and institution of oral antibiotics resulted in clearing within 4 weeks. This finding does not support the concept that prepubertal children with steroid rosacea need to continue low-strength steroids in a gradual withdrawal strategy. This conclusion is supported by the finding that 54% developed the steroid rosacea while being treated with the lowest-strength (class 7) topical corticosteroids. Even over-the-counter hydrocortisone preparations induced steroid rosacea in susceptible children. Susceptibility may be genetic as 20% of children had a first-degree relative with rosacea.

Overexpression of mutant ras in human melanoma increases invasiveness, proliferation and anchorage-independent growth in vitro and induces tumour formation and cachexia in vivo.

Malignant melanoma is the deadliest form of skin cancer. Previous studies have shown that the incidence of ras mutation increases with progression of melanoma, but that such mutations may not be present in the earliest radial growth phase melanomas. Recently it has been proposed that introduction of ras mutations into cells deficient in tumour suppressor genes such as p16 (INK4a) is sufficient to induce characteristics of cellular transformation such as anchorage-independent growth and tumour formation in vivo. To test this hypothesis in human melanoma, mutant N-ras, mutant H-ras or wild-type H-ras genes were transfected by electroporation into WM35 cells, a p16-deficient human melanoma cell line of low invasive potential. Increased expression of mutant ras p21 enhanced anchorage-dependent cell growth on tissue culture plastic. In addition, overexpression of mutant N-ras and H-ras, but not of wild-type H-ras, increased the experimental invasive potential, inducing anchorage-independent growth in soft agar, increasing cell motility measured by time-lapse video microscopy, and increasing invasiveness through reconstituted basement membranes. Finally, overexpression of mutant H-ras in melanoma cells was shown to increase tumorigenicity and to induce cachexia when H-ras transfected cell lines were injected subcutaneously in severe combined immunodeficiency (SCID) mice. Thus the addition of activating ras mutations to a melanoma cell line already deficient in p16 leads to enhanced proliferation, survival and migration in vitro and to enhanced subcutaneous tumour formation in vivo. This phenotype is typical of the behaviour of vertical growth phase (VGP) melanoma, and we propose that activation of the ras signalling pathway in the presence of deletions in p16 or related tumour suppressors can induce the VGP melanoma phenotype.

Childhood immunobullous disease following a second organ transplant.

Immunobullous diseases are quite unusual in children. We report two children who developed immunobullous disease shortly after a second solid organ transplantation. One child had IgA pemphigus vegetans, the other bullous pemphigoid. We hypothesize that the repeat organ transplantation served as a "booster" immunization for autoantibody production. Both children developed their immunobullous condition while being treated with immunosuppressive drugs that are used to treat immunobullous disorders.

The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus.

BACKGROUND: Cutaneous neonatal lupus erythematosus (NLE) is an uncommon disease described mainly through isolated case reports. OBJECTIVE: Our purpose was to examine the cutaneous spectrum, clinical associations, and course of disease in babies with anti-Ro-positive NLE. METHODS: This is a retrospective case series evaluation of newborns with anti-Ro-positive NLE seen at a single ambulatory care university center over a 20-year period. Cases were drawn from a population of 3.2 million. Follow-up was at least 3 years. RESULTS: Four boys and 14 girls were included in our evaluation. Distribution of skin lesions in 18 babies was as follows: face, 17; periorbital "owl-eye" or "eye mask" facial rash, 14; scalp, 15; arms and legs, 13; trunk and groin, 6. Crusted lesions were predominant in 3. Photosensitivity was seen in 12, and features of cutis marmorata telangiectasia congenita were observed in 4. In 17 neonatal lupus was not suspected until the dermatology consultation. Noncutaneous manifestations included thrombocytopenia in 4, cholestatic hepatitis in 3, and congenital heart block in 3. Four patients had residual telangiectasia that persisted for 3 or more years but eventually cleared in 2 patients. Three babies had dyspigmentation that spontaneously cleared within 22 months. None had atrophy or scarring. CONCLUSION: Periorbital, scalp, and extremity lesions are common in cutaneous NLE. Crusted lesions predominated in male infants. In children selected by cutaneous involvement, thrombocytopenia and hepatic disease were present as frequently as cardiac disease and occurred more frequently in male babies with crusted skin lesions. Children with cutaneous NLE should be evaluated for hematologic and hepatic as well as cardiac involvement.

Fixed drug eruptions in children.

To determine the anatomic location and offending drug in fixed drug eruptions (FDE) in children, we performed a 5-year retrospective analysis. Thirty-five children with FDE were evaluated. The most common cause of FDE was the combination drug trimethoprim-sulfamethoxazole.