Childhood pemphigus.

BACKGROUND: Five children with pemphigus are reported: three with pemphigus vulgaris, one with pemphigus vegetans, and one with pemphigus foliaceus. Only one case of juvenile pemphigus vegetans has been published in the literature. MATERIALS AND METHODS: All three patients with pemphigus vulgaris were treated with oral corticosteroid; in two cases, azathioprine was added for steroid-sparing effect. The patient with pemphigus vegetans had a clinical presentation resembling pemphigus vulgaris, but the lesions in the perianal area healed as hypertrophic granulation tissue. He was treated with oral corticosteroid, azathioprine, and intralesional corticosteroid. The patient with pemphigus foliaceus presented with exfoliative dermatitis, and was treated with oral corticosteroid; methotrexate was added later for steroid-sparing purposes RESULTS: The patients were followed up for 1-4 years; the prognosis of childhood pemphigus is good. CONCLUSIONS: Long-term follow-up is needed to detect flaring of the disease and the side-effects of immunosuppressive drugs.

Pediatric sarcoidosis presenting with hypertensive encephalopathy.

A 3-year-old girl with simultaneous presentation of hypertensive encephalopathy, subcutaneous nodules, hepatosplenomegaly and proteinuria was presented. The sarcoidosis was confirmed by histologic demonstration of non-caseating granulomas in the liver, left kidney, a cervical lymph node and subcutaneous nodules. The proteinuria resolved spontaneously. During the six-month period of steroid therapy, the renal mass and hepatosplenomegaly were dissolved but the cervical lymphadenopathy still persisted. The hypertensive state was difficult to control which required a multiple antihypertensive drug regimen.

Cutaneous manifestations of childhood systemic lupus erythematosus.

We assessed the mucocutaneous signs in 57 children with classical systemic lupus erythematosus seen during a 6 year period. The female:male ratio was 4.2:1. Ages ranged from 4 to 15 years (mean 11.9 years) at the time of diagnosis. Cutaneous manifestations (77%) were the second most common finding, next to renal involvement (84%). The skin changes noted were malar rash (74%), oral ulcer (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid lupus erythematosus (LE) (19%). All 11 discoid LE patients were girls. Periungual erythema, Raynaud's phenomenon, periungual gangrene, nail involvement, and subacute LE were rare. Antinuclear antibody reaction was positive in 93% and anti-dsDNA was positive in 46%. Eight patients died, six from severe infection and two from renal failure.

Henoch-Schönlein purpura in Thailand: review of 41 children.

The clinical features of 41 Thai children with Henoch-Schönlein purpura were presented, with particular emphasis on the cutaneous features and date of onset. There were 20 boys and 21 girls, ages ranged from 2 to 15 years. All patients had palpable purpura on the lower limbs. Palpable purpura were also present on forearms and pinna in 25 (61%) and 5 (12%) respectively. Scalp edema was found in five patients. Hemorrhagic vesicles and bullae were found in only one patient. Twenty six patients (63%) had skin lesions as the first sign. Thirty four patients (83%) had skin lesions in the first four days of the clinical course. Other dominant features included were abdominal pain, arthralgia and nephritis 73, 66 and 39 per cent respectively. The pertinent literature is reviewed.

Calcinosis cutis presenting years before other clinical manifestations of juvenile dermatomyositis: report of two cases.

Calcinosis cutis in dermatomyositis is dystrophic calcification appearing late in the course of the disease. Two cases are reported here of calcinosis cutis that presented years before other clinical manifestations of juvenile dermatomyositis. The first case was a 14-year-old Thai girl who had asymptomatic subcutaneous nodules that spontaneously ruptured, exuding a chalky discharge and healing with an atrophic scar 8 years before the onset of other clinical manifestations of juvenile dermatomyositis; that is, Gottron's papules, proximal muscle weakness grade IV/V with atrophy, slightly elevated serum creatinine phosphokinase level and an abnormal electromyogram compatible with myopathy. The second case was a 15-year-old Thai boy who had calcinosis cutis 3 years before the onset of other clinical manifestations of juvenile dermatomyositis, and the calcinosis cutis was so severe that it interfered with the movement of his extremities. Both cases responded well to aluminium hydroxide therapy.

Henoch-Schonlein purpura presenting as hemorrhagic vesicles and bullae: case report and literature review.

Henoch-Schonlein purpura is a syndrome characterized by nonthrombocytopenic purpura accompanied by arthralgia, gastrointestinal symptoms, or renal involvement. We report a 5.5-year-old boy with high fever and hemorrhagic vesicles and bullae varying in size from 2 to 50 mm in diameter, on both pinnas, the hard palate, gums, dorsa of the hands, buttocks, and both legs, and review the pertinent literature.

Eczematous skin reaction from patch testing with aeroallergens in atopic children with and without atopic dermatitis.

To determine whether aeroallergens could induce eczematous lesions, 30 patients with atopic dermatitis were studied in comparison with 30 patients with respiratory atopy without atopic dermatitis. All patients were between 2 and 14 years of age. Patch testing with five aeroallergens--housedust, mite, cockroach, mold mix, and grass mix--was done on skin that was stripped by 10 applications of adhesive tape. Intradermal tests with the same antigens were done on the forearm. In 27 (90%) children with atopic dermatitis, patch testing with aeroallergens induced eczematous lesions at one or more sites. Mite, cockroach, house dust, mold mix, and grass mix caused reactions in 21 (70%), 21 (70%), 19 (63%), 15 (50%), and 13 (43%) patients, respectively. Three patients had a dermatitis flare at the antecubital and popliteal fossae during testing. Only three (10%) atopic children without atopic dermatitis had eczematous lesions, which was significantly different from children with atopic dermatitis (P < 10(-6)). Intradermal skin tests in both groups were not significantly different. This study supports previous reports that aeroallergens plays an important role in causing eczematous skin lesions.

Collodion baby: the out-come of long-term follow-up.

About 10 Thai collodion babies were treated at the Department of Pediatrics, Faculty of Medicine, Chulalongkorn University from 1970 to 1990. Most of them were premature and small for gestational age infants. The sex incidence of males to female was 3:2. Only half of the cases had a family history of ichthyosis. The severity of thickness and tightness of the collodion membranes varied from mild to severe. The clinical and histopathologic findings revealed 5 cases of lamellar ichthyosis (50%), of which one case had episodes of pustular psoriasis. One case evolved to be ichthyosis vulgaris 10 per cent, one case had palmoplantar hyperkeratosis (10%), one case had been apparently normal up to 18 years old, one died of respiratory infection and another case was lost from follow-up. All cases of lamellar ichthyosis were associated with mental retardation with some degree of slow physical development. One severe collodion baby suffered from congenital absence of distal phalanges of toes with increased thickness of the finger nails.

Corticosteroid treatment of extensive hemangiomas: analysis of 22 cases in children.

During the past 16 years, 22 Thai infants with extensive hemangiomas which impaired bodily functions and were life-threatening, especially in Kasabach-Merrit Syndrome have been treated. The incidence of female-to-male ratio was 8:3, all of the hemangiomas appeared between birth and 3 months of age. Among the 22 infants, 11 (50%) had hemangiomas on the head and the neck, and 2 had laryngeal hemangiomas. Two cases of Kasabach-Merrit Syndrome, located at the shoulder and the thigh, were complicated with severe bleeding, infection and congestive heart failure. Ocular impairment, malocclusion, and cutaneous distortion were the most important functional problems. Three cases were associated with other congenital anomalies: intracranial angioma, Dandy Walker Syndrome and Klippel-Trenauncy Syndrome. Prednisone (2-4 mg/kg/d) was used in all cases with other symptomatic treatment. Antiplatelet aggregating and antithrombotic drugs were useful in Kasabach-Merrit Syndrome. Definite response was noted in 72.72 per cent of the patients, doubtful response in 23.32 per cent, and no response in 4.54 per cent which was better than in other reports. There were no serious side-effects of the corticosteroid treatments reported in our careful students.

Concomitant disseminated varicella and generalized staphylococcal scalded skin syndrome in a leukemic patient.

We report a case of nosocomial fatal varicella infection in a 13-year-old boy with acute lymphoblastic leukemia complicated with staphylococcal scalded skin syndrome. His underlying leukemia, immunosuppressive drugs, disseminated varicella, S. aureus colonization, and acute renal insufficiency were all contributing factors that were pathogenetically linked in the development of his generalized SSSS.