Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome.

BACKGROUND: In adults, erythema multiforme (EM) is thought to be mainly related to herpes infection and Stevens-Johnson syndrome (SJS) to drug reactions. AIMS: To investigate this hypothesis in children, and to review our experience in the management of these patients. METHODS: A retrospective analysis of 77 paediatric cases of EM or SJS admitted to the Children's Hospital in Bordeaux between 1974 and 1998. RESULTS: Thirty five cases, inadequately documented or misdiagnosed mostly as urticarias or non-EM drug reactions were excluded. Among the remaining 42 patients (14 girls and 28 boys), 22 had EM (11 EM minor and 11 EM major), 17 had SJS, and three had isolated mucous membrane involvement and were classified separately. Childhood EM was mostly related to herpes infection and SJS to infectious agents, especially Mycoplasma pneumoniae. Only two cases were firmly attributed to drugs (antibiotics). No patient died. EM and SJS sequelae were minor and steroids were of no overall benefit. CONCLUSION: In paediatric practice EM is frequently misdiagnosed. The proposal that SJS is drug related in adults does not apply to children, and in our recruitment EM and SJS are mostly triggered by infectious agents. The course of both diseases, even though dramatic at onset, leads to low morbidity and mortality when appropriate symptomatic treatment is given.

Congenital cutaneous defects as complications in surviving co-twins. Aplasia cutis congenita and neonatal volkmann ischemic contracture of the forearm.

BACKGROUND: During twin pregnancies, several complications may result in the death of a co-twin depending on the date of death. We describe herein 2 infant survivors of monozygotic twin pairs with 2 distinct possible complications: a aplasia cutis congenita and Volkmann ischemic contracture. OBSERVATIONS: One infant had extensive aplasia cutis congenita with an associated monozygotic co-twin who died at 3 months of gestation, and the other child had a localized arm defect due to Volkmann ischemic contracture and brain damage, with a co-twin who died at approximately 6 weeks of gestation. CONCLUSIONS: Congenital cutaneous defects may result in the death of a co-twin. The most common of these defects is aplasia cutis congenita associated with a fetus papyraceus or a dead fetus related to ischemic/thrombotic events in the placenta and fetus. Volkmann ischemic contracture is rare in the newborn but can cause neonatal cutaneous defects. The cause of Volkmann ischemic contracture in newborns is unknown; however, our second observation suggests the possible role of a dead fetus.

[Faun tail and sacral hemangioma associated with occult spinal dysraphism]. / Appendice caudal et hémangiome sacré révélateurs d'une dysraphie occulte.

INTRODUCTION: Hemangiomas are the most frequent skin tumor of childhood. Usually, a "wait and see" policy is adopted. However, a sacral hemangioma may reveal occult neurodysraphism. CASE REPORT: MRI discovered lipomyelomeningocele and a tethered spinal chord in an asymptomatic 4-month-old boy with sacral hemangioma and faun tail. Because of absence of neurological defect only surgery of the caudal appendage was performed for the moment. DISCUSSION: Midline lombosacral lesions, as well as lipomas, hirsutism or pilonidal cyst, may be associated with occult spinal defect; the most severe is tethered chord. Renal or ano-genital anomalies can be also associated. CONCLUSION: MRI is necessary in case of midline sacral hemangioma to detect underlying anomalies.

Erythema nodosum in children: a study of 27 patients.

Erythema nodosum (EN) seems to occur in children more rarely than in adults. It still remains the most frequent acute panniculitis, for which the diagnosis is almost always clinical. In a retrospective study of 27 pediatric patients, we have attempted to clarify the clinical spectrum and prognosis of this disease and discuss the differential diagnosis of nodular eruptions on the lower limbs of children. In almost half the patients of our series, the cause of EN remained undetermined. Streptococcal infections (usually of the pharynx) were the most common cause of EN in children (22% of patients in our series), followed by Yersinia infection in about 15% of patients. Tuberculosis, an important cause in the past, was never found, but must always be excluded. A benign course was noted in all patients. Erythema nodosum is easily recognized clinically, but other subcutaneous lesions, especially nodular vasculitis and Schönlein-Henoch purpura, have to be excluded by pathologic study, in cases of atypical presentation or long duration.

Neonatal Malassezia furfur pustulosis.

BACKGROUND: Papulopustular eruptions of the face in neonates are frequently referred to as neonatal acne or sebaceous miliaria. Our findings suggest that there is an association between this type of eruption and Malassezia furfur infection. OBSERVATIONS: Direct examination of pustule smears showed M furfur yeasts in eight of 13 cases involving neonates with erythema and papulopustules of the face, neck, and scalp (mean age at onset, 22 days [range, 7 to 30 days]). The pustules were predominantly neutrophilic. Treatment with 2% ketoconazole cream applied topically twice daily was effective in 1 week. CONCLUSION: Malassezia furfur is frequently associated with a common nonfollicular pustulosis of the newborn, probably improperly termed neonatal acne.

[Localized granuloma annulare in children: outcome in 30 cases]. / Granulome annulaire localisé chez l'enfant: évolution de 30 cas.

BACKGROUND: The outcome of granuloma annulare in childhood is not well defined. POPULATION AND METHODS: A questionnaire was sent to the family of 40 children under 15 years of age examined for granuloma annulare from 1987 to 1992. Thirty of them answered, permitting a retrospective study. RESULTS: The sex-ratio F:M was 1.3:1. Ages ranged from 1 to 13 years (mean: 4.5 years). Lesions developed before the age of 5 years in 76.7% of cases. Involved sites were essentially the back of hands and feet; lesions were unique in half of the cases. No association with diabetes mellitus was found. Three familial cases were observed. Duration of lesions varied from 6 months to 7 years (mean: 2.5 years). Age at onset, sex, biopsy and treatment had no influence on outcome. CONCLUSION: Granuloma annulare in children is a benign disorder but its course may last up to several years.

[Lymphomatoid papulosis in a child]. / Papulose lymphomatoïde chez un enfant.

BACKGROUND: Lymphomatoid papulosis (LyP), uncommon in children, has a benign clinical course in contrast with a malignant histology. CASE REPORT: A 9-year old boy developed nodular skin lesions for 6 months on the scalp and penis and a papule skin on his trunk. Surgical excision of scalp nodules was performed to prevent necrosis and ulceration of the ear. The biopsy showed a dense mixed cellular infiltrate including histiocytes, lymphocytes and numerous atypical large cells of which a minority expressed the CD30 antigen. PCR showed an oligoclonal rearrangement of the TCR gamma chain gene. Physical examination, bone marrow aspirate and thoracoabdominal CT scans were normal. Three years after surgery, no other lesions occurred. DISCUSSION: Differential diagnosis of LyP is difficult with non Hodgkin's lymphoma, especially CD30+ lymphoma. Prognosis remains the major problem in LyP. Ten to 20% of cases are associated with lymphomas in adults.

[Treatment of severe cervicofacial hemangioma with interferon alpha-2b]. / Traitement d'un hémangiome cervicofacial grave par interféron alpha-2b.

BACKGROUND: Indications for active treatment of hemangiomas are those lesions which, by virtue of their size and site, compromise vital structures. The treatment of choice is oral or intravenous corticosteroids, but interferon alpha may represent alternative therapy. CASE REPORT: A 15-day-old girl was admitted for congenital hypothyroidism. She had a large cervicofacial hemangioma extending to periorbital and laryngeal areas. The patient was given systemic prednisone (2 mg/kg/day) and L-thyroxin without success. An episode of acute respiratory distress a few weeks later required tracheostomy while prednisone dosage was increased to 4 mg/kg/day. This drug was not tolerated and the hemangioma was treated by particle embolization that was followed by a partial decrease in the volume of the hemangioma. A second episode of cardiorespiratory distress at 7 1/2 months of age required interferon alpha, 3 million units/m2/day, that was progressively effective. After 11 months of treatment, the hemangioma disappeared, without relapse 6 months later. CONCLUSION: Interferon alpha is an interesting alternative therapy of infantile hemangiomas when they are resistant to steroid treatment.

[Diffuse cutaneous mastocytosis without permanent lesion]. / Mastocytose cutanée diffuse sans lésion permanente.

Cutaneous mastocytosis usually includes objective skin signs such as pigmented maculopapulae or skin infiltration. We report an unusual case of cutaneous mastocytosis without systemic involvement in a 9-month old infant. Clinical expression was entirely functional (pruritus, urticaria) with no permanent lesions. There were 22 times more mastocytes found in the skin biopsy than in similar biopsies obtained in healthy control subjects of the same age, which corresponds to the counts found in cutaneous mastocytosis. Diffuse cutaneous mastocytosis with no permanent lesions is an exceptional form of mastocytosis (3 cases reported in the literature). The long-term outcome is unknown. This syndrome should be distinguished from idiopathic anaphylaxis by quantification of the dermal mastocytes.

[Microsporum langeronii dermatophytosis in a newborn infant contaminated in France]. / Dermatophytie à Microsporum langeronii chez un nouveau-né contaminé en France.

Dermatophytosis due to Microsporum langeronii was observed in a 21-day neonate born in Bordeaux, France to a mother of West African origin. A typical misleading manifestations were: non alopecic squamation of the scalp with seborrhoea associated with circineous vesiculo-squamous lesions of the forehead. The source of the contamination was undoubtedly the mother who presented squamation without alopecia of the scalp. M. langeronii were observed in the scales and the hair. Epidemiological search for tinea should be carried out in the family as well as in schools attended by the brothers and sisters. Familial contamination is more frequent.

[Erythromelalgia: a familial case. Discussion on the role of mercury]. / Erythromélalgie: une observation familiale. Discussion sur le rôle du mercure.

Erythromelalgia is an acrosyndrome characterized by paroxysmal manifestations associating erythema, local sensations of warmth and pain which improve with exposure to cold. Childhood forms, exceptional and usually primary, are quite severe and particularly resistant to treatment. The search for a causal agent is most often negative and the pathogenesis remains to be determined. We report a case of erythromelalgia observed in a 4-year-old girl, her father and her younger sister. This case was particular due to an association with mercury poisoning. The symptomatology was improved after different therapeutic attempts including Clomipramide and, particularly effective, rerigerating socks (D(r) Comet, CNES, Toulouse). In the literature we were unable to find any case of erythromelalgia related to mercury poisoning. The cases of familial erythromelalgia reported suggest X-linked dominant transmission. Finally, this case demonstrated the difficulties in diagnosing and treating erythromelalgia, especially in the child.

Treatment of port-wine stains with the 585-nm flashlamp-pulsed tunable dye laser: a study of 74 patients.

Over 20 months, we have treated 74 patients (59 children less than 12 years of age) with port-wine stains (PWS) using a 585-nm flashlamp-pulsed dye laser (SPTL-1, Candela Corp., Wayland, Mass., USA) after topical anesthesia with Emla cream. A 5-point reference color scale was used to evaluate the results. 45 patients had at least one treatment on the entire surface of the lesion. A mean of 88 impacts per session was delivered. There was a significant decrease in color in around two thirds of the cases after one complete treatment with a gradual tendency to improvement after subsequent treatments. Younger age at the beginning of treatment was not found to be predictive of a better outcome after the first treatment. In around one third of the cases, positive test site treatment was not correlated with significant improvement after one treatment. Lesions situated on the limbs were less responsive than those on the head and neck. Except for problems due to absence of general anesthesia in young children enduring repeated stressful and sometimes painful procedures, the overall impression is that early treatment of PWS is possible with very limited risks of scarring using this technique.

[Epidemiology of urticaria in infants]. / Epidémiologie de l'urticaire chez l'enfant.

Epidemiology of childhood urticaria and especially that of infants is badly understood because of a lack of studies devoted to this age group. A retrospective study of 40 infants that were hospitalised for urticaria has been made to clarify the factors that produce urticaria at that age. Certain clinical signs seem to be more frequent at this age, such as the bruised appearance of the lesions. Acute urticaria is the most frequent form. An etiology has been confirmed or suspected in 2/3 cases: intolerance of cow milk proteins before 6 months, drug and infection causes after 6 months. Prospective studies of infantile urticaria are necessary.