Preschool sarcoidosis.

Sarcoidosis is a multisystemic granulomatous disorder of unknown etiology, most commonly affecting young adults and presenting most frequently with bilateral hilar lymphadenopathy, pulmonary infiltration, and skin or eye lesions. Sarcoidosis in children under the age of 4, preschool sarcoidosis, is rare and has very different clinical mainfestations characterized by the typical triad of skin, eye and joint involvement without lung disease. Diagnosis is established when compatible clinical findings are supported by histological evidence of widespread noncaseating epithelioid-cell granulomas in more than one organ or a positive Kveim-Siltzbach skin test. This report describes a 2-year-old Thai girl with typical clinical manifestations of preschool sarcoidosis. The histopathological studies of tissue specimens and the laboratory investigations support the diagnosis of sarcoidosis. The patient responded to systemic corticosteroid therapy.

Analysis of skin diseases in a referral pediatric dermatology clinic in Thailand.

The retrospective study of skin diseases in children less than 13 years old was performed at the referral Pediatric Dermatology Clinic, Siriraj Hospital, Thailand. It included 4,265 visits made by 2,361 patients. The prevalence and demographic data of all diagnoses were analysed. In children, eczematous dermatitis was the most common (41.2%), followed by skin infections (21.9%), pigmentary disorder (7.0%), hypersensitivity skin diseases (4.1%), and others. Atopic dermatitis was the most common type of eczema in children (6.0%). The entity of contact dermatitis, scabies, vitiligo, tinea capitis, alopecia areata, papular urticaria, impetigo and urticaria represented 4.9, 4.1, 4.1, 3.3, 2.4, 2.3, 2.3 and 2.2 per cent, respectively. These data may be useful in planning the dermatologic education and health care program for Thai children in the future.

Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford progeria syndrome is an extremely rare condition of premature aging. It is characterized by growth retardation and accelerated degenerative changes of cutaneous, musculoskeletal and cardiovascular systems. The pathogenesis of the disease is unknown. The patients usually appear normal at birth. Typical manifestations develop gradually and are evident by the first or second year of life. They have a remarkably similar physical appearance consisting of short stature, alopecia, craniofacial disproportion, micrognathia, hypoplastic mandible, beak-like nose, decreased subcutaneous fat, atrophic skin, sclerodermoid lesion, mottling hyperpigmentation, prominent scalp veins, prominent eyes, protruding ears with absence of earlobes, faint midfacial cyanosis, delayed closure of fontanelles and sutures, delayed dentition, horse-riding stance, thin limbs with prominent stiff joints, coxa valga, skeletal hypoplasia and dysplasia, dystrophic nails and high-pitched voice. Laboratory investigations are unremarkable. Metabolic, endocrine, serum lipid and immunologic studies show no uniform abnormalities. Typical radiographs demonstrate evidence of resorption of the distal ends of clavicles, attenuation of the terminal phalanges, diffuse osteopenia, and fishmouth vertebral bodies. In this report, a 3-year-old Thai girl with typical characteristics of Hutchinson-Gilford progeria syndrome is described.

Hydroa vacciniforme with ocular involvement.

A 7-year-old Thai boy had several episodes of hydroa vacciniforme which were accompanied by an anterior uveitis with corneal clouding and stellate keratic precipitates. Wearing sunglasses prevented additional eye symptoms despite recurrence of the skin lesions. Repetitive UVA phototesting reproduced the typical skin lesions with fever and malaise. No reproduction of skin lesions was revealed by repetitive UVB phototesting. One should be aware of eye involvement in hydroa vacciniforme, and those who experience the eye involvement should be advised to wear protective sunglasses.

Successful treatment of cytophagic histiocytic panniculitis by cyclosporin A: a case report.

This is a report of a case, 7 1/2 year-old-boy having chronic febrile and recurrent crops of painful subcutaneous nodules on lower extremities, which had previously been diagnosed as Weber-Christian disease, which progressed to have cytophagic histiocytic activity in the skin, bone marrow with abnormal liver function and hemorrhagic diathesis. He was subsequently treated with corticosteroid without good response. After he was diagnosed as having cytophagic histiocytic panniculitis, cyclosporin A was administered intravenously in an initial dosage of 1 mg/kg/day and in oral maintenance dose of 10 mg/kg/day with a successful response and the patient completely recovered within 6 months with mild hypertension as an adverse effect.

Bullous pemphigoid in an infant: a case report and literature review.

Bullous pemphigoid is an autoimmune bullous disease that is rare in children and infants. It seems indistinguishable from the disease in adults although mucous membrane, palms and soles involvement appear more commonly in childhood bullous pemphigoid. There is no association with malignancy. The most reliable diagnostic criterias are the linear deposition of IgG and C3 along the basement membrane zone and the presence of circulating IgG antibasement membrane zone antibodies. The literature of bullous pemphigoid is reviewed and a case of a 7-month-old girl with typical clinical manifestations and immunofluorescence studies is reported. She responded very well to a high dose of systemic corticosteroid. The disease can be spontaneously resolved and the prognosis for children is good in most cases.