Panniculitis in childhood and adolescence.

BACKGROUND: The purpose of the present paper was to describe the clinical manifestations and treatment of patients with panniculitis. METHODS: From January 1983 to December 2002, 4294 patients were treated for pediatric rheumatological diseases at Pediatric Rheumatology Unit, University of São Paulo, Brazil. Of these, 35 children and adolescents (0.8%) presented with panniculitis: erythema nodosum (EN) or Weber-Christian disease (WCD). Clinical characteristics, laboratory exams, biopsy of the lesion, treatment and clinical course were studied. RESULTS: Of the 35 patients, 29 presented with EN and six with WCD, one of these with cytophagic histiocytic panniculitis. Mean age at symptom onset was 85 months (6-204 months) and the mean duration of follow up was 55 months (1-144 months). All the patients presented with inflammatory subcutaneous nodules. The patients with WCD presented with systemic manifestations and cutaneous atrophy. The principal etiologies of EN were streptococcal infection (42%), undetermined (13.5%), pulmonary tuberculosis (10%), and acute rheumatic fever (10%). Biopsy of the nodules indicated septal panniculitis in 14 patients with EN and lobular panniculitis without vasculitis in the patients with WCD, one of which had cytophagic histiocytic panniculitis. There was recurrence in 11 patients (38%) with EN and in all those with WCD. Non-steroidal anti-inflammatory drugs were used in 15 patients with EN and corticosteroids and/or immunosuppressive drugs in the six patients with WCD. Three patients died. CONCLUSIONS: EN is the most frequent panniculitis, with a benign course and is mainly associated with infections. WCD is a severe disease, with systemic involvement, that proceeds with cutaneous atrophy and requires the use of corticosteroids and or immunosuppressive drugs.

[Reflex sympathetic dystrophy]. / Distrofia simpático-reflexa.

OBJECTIVE: To describe eight patients with reflex sympathetic dystrophy in terms of clinical and laboratory characteristics and treatment. DESCRIPTION: Eight children (four girls) with reflex sympathetic dystrophy were retrospectively analyzed. The diagnosis of reflex sympathetic dystrophy was based on the presence of pain in the distal extremities, local edema, vasomotor instability and impairment of sensibility. Two patients had associated systemic lupus erythematosus, one had juvenile idiopathic arthritis and one had Glanzmanns thrombasthenia. Mean age was 11.5 years. Most of the patients had lower extremity involvement (7/8). The most important clinical signs were pain, edema and vasomotor instability in the affected extremity (8/8), functional impairment (7/8), and impaired sensibility (3/7). The erythrocyte sedimentation rate was abnormal in three patients and the bone scans in five. All patients received non-steroidal anti-inflammatory drugs and physical therapy with improvement of the symptoms in seven patients, until six months of treatment. Three patients were submitted to acupuncture with good response. One patient had a severe disease and received tricyclic antidepressants, with improvement more than one year after. COMMENTS: Reflex sympathetic dystrophy should be included as part of the differential diagnosis of limb pains of childhood, so that physicians can make an earlier diagnosis and prevent functional impairment.

Distrofia simpático-reflexa / Reflex sympathetic dystrophy

OBJETIVO: Descrever as características clínicas, laboratoriais e terapêuticas de oito crianças com distrofia simpático-reflexa. DESCRIÇAO: Foram analisados retrospectivamente oito casos de distrofia simpático-reflexa. O diagnóstico foi baseado na presença de dor no segmento distal de um membro, edema local, alterações vasomotoras e de sensibilidade. Dentre os oito pacientes com distrofia simpático-reflexa, dois eram portadores de lúpus eritematoso sistêmico, um de artrite idiopática juvenil e um de trombastenia de Glanzmann. Quatro eram meninas, e a média de idade foi de 11,5 anos. Os membros inferiores foram acometidos na maioria dos pacientes (7/8), e as características clínicas mais marcantes foram dor, edema e alterações vasomotoras da extremidade afetada (7/8), incapacitação funcional (7/8) e alterações de sensibilidade (3/8). A velocidade de hemossedimentação esteve alterada em três pacientes, e a cintilografia músculo-esquelética em cinco. Todos os pacientes receberam antiinflamatórios não-hormonais e fisioterapia, com melhora clínica observada em sete pacientes em até 6 meses de tratamento. Três pacientes foram submetidos à acupuntura, com boa resposta. Um paciente teve curso de difícil controle, tendo recebido antidepressivos tricíclicos e apresentando melhora após mais de 1 ano de tratamento. COMENTARIOS: A distrofia simpático-reflexa é uma doença que deve ser incluída no diagnóstico diferencial das dores em membros na infância, para que se estabeleça o diagnóstico precocemente, evitando-se, assim, prejuízo funcional a médio e longo prazo.