Subject(s)
Acrodermatitis/pathology , Erythema/pathology , Leg Dermatoses/pathology , Leg/pathology , Lyme Disease/pathology , Acrodermatitis/drug therapy , Acrodermatitis/microbiology , Atrophy , Chronic Disease , Diagnosis, Differential , Erythema/drug therapy , Erythema/microbiology , Humans , Leg/microbiology , Leg Dermatoses/drug therapy , Leg Dermatoses/microbiology , Lyme Disease/complications , Lyme Disease/drug therapy , Male , Middle AgedABSTRACT
UNLABELLED: Congenital 'self-healing' Langerhans' cell histiocytosis of Hashimoto-Pritzker is a rare disease occurring mainly in the neonatal period. CASE REPORT: We report on the case of a newborn with widespread eruption since birth, consisting of nodules, papulonodules, sometimes with ulcerations and scabs, concerning all the body, with a predilection for the cephalic area and the scalp, without general abnormalities. The clinical examination, histopathological data, immunohistochemistry, and the benign evolution in nine-, 18- and 24-month periods without particular treatment define the diagnosis of congenital self-healing Langerhans' cell histiocytosis of Hashimoto-Pritzker. CONCLUSION: The position of this disease among the Langherans' cell histiocytoses is probably situated at the benign pole. This is a benign self-healing disease restricted to the skin and the prognosis is good (self-involution). It is important to eliminate a malignant form of other Langerhans' cell histiocytosis such as Letterer-Siwe disease by a checkup searching for a visceral disease. The good prognosis should not lead to forget the possibility of error or forms of relapses; it is therefore imperative to have a rigorous, regular and especially long-term follow-up.
Subject(s)
Histiocytosis, Langerhans-Cell/pathology , Infant, Newborn, Diseases/pathology , Humans , Immunohistochemistry , Infant, Newborn , Male , Prognosis , Skin Diseases/pathologyABSTRACT
OBJECTIVES: To establish the clinical, etiological, and prognostic features of acute urticaria in infancy and early childhood and to define its optimal management. DESIGN: Prospective study. The inception cohort was collected from April 1, 1992, through March 31, 1994. After initial evaluation, the course of the disease was assessed at 2 months and after 1 to 2 years. SETTING: Emergency department of a regional teaching pediatric hospital (referral center), which is also the only pediatric hospital for the general community in the city (population, 600,000 inhabitants). PATIENTS: Fifty-seven consecutive infants, aged 1 to 36 months, hospitalized with a final diagnosis of acute urticaria. Follow-up at 1 to 2 years was available in 40 of 57 patients. INTERVENTION: Oral antihistamines (dexchlorpheniramine maleate, terfenadine, or hydroxyzine hydrochloride) for 2 weeks. MAIN OUTCOME MEASURES: Recurrence and chronicity. RESULTS: Annular or geographic papules and plaques with hemorrhagic lesions were seen in 28 patients (49% of cases) and angioedema in 34 patients (60% of cases). An underlying cause was suspected or identified in 52 patients (91% of cases). Infection, either associated or not with drug intake, was the cause in 46 patients (81%) and foods were the cause in 6 (11%). Parasitic infestations were noncontributory. Hemorrhagic lesions and association with articular symptoms were statistically more frequent in urticaria caused by infections. Atopy in the patient or family was associated in 33 patients (58% of cases), and particularly atopic dermatitis was associated with urticaria caused by food. At 1- to 2-year follow-up, 12 (30%) of 40 patients surveyed had chronic or recurrent urticaria. CONCLUSIONS: Causative factors in urticaria are dominated by benign viral illnesses, often associated with antibiotic drug therapy. In most patients, laboratory investigations are not required. Twenty percent to 30% of cases evolve into chronic or recurrent disease.
