An Bras Dermatol. 2011;86(4Supl1):S46-9.
46
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Challenges in diagnosis and treatment of a case of SAPHO
syndrome in childhood *
Desafios no diagnóstico e tratamento de um caso de síndrome SAPHO na infância
Patricia Craveiro Gomes da Silva1Elisa Fontenelle de Oliveira2
Andréa Valentim Goldenzon3Pâmela Craveiro Gomes da Silva 4
Marta Cristine Felix Rodrigues5
Abstract: The authors report a case of SAPHO Syndrome, in pediatric age, with a dermatological focus.
This entity should be considered in patients who have pain in the anterior chest wall or other muscu-
loskeletal symptoms, accompanied by palmoplantar pustulosis and acne fulminans. The specific cuta-
neous manifestations, diagnosis and the treatment will be presented.
Keywords: Acne Vulgaris, Osteitis, Psoriasis, Synovitis
Resumo: Os autores relatam um caso clínico sobre Síndrome SAPHO, na faixa etária pediátrica, com
enfoque dermatológico. Essa entidade deve ser considerada, nos pacientes que tenham dor na parede torá-
cica anterior ou outros sintomas musculoesqueléticos, acompanhados por lesões dermatológicas, como
pustulose palmoplantar e acne fulminans. As manifestações cutâneas específicas, diagnóstico e o tratamen-
to utilizado serão apresentados.
Palavras-chave: Acne Vulgar, Osteíte, Psoríase, Sinovite
Received on 03.08.2010.
Approved by the Advisory Board and accepted for publication on 25.10.2010.
* Work carried out at the Pediatric Dermatology Clinic from Jesus Municipal Hospital – Rio de Janeiro (RJ), Brazil.
Conflict of interest: None / Conflito de interesse: Nenhum
Financial funding: None / Suporte financeiro: Nenhum
1Pediatrician, ex- resident from Jesus Municipal Hospital, Rio de janeiro (RJ), Brazil.
2Dermatologist, Pediatric Dermatology Clinic at Jesus Municipal Hospital, - Rio de Janeiro (RJ), Brazil.
3Master’s degree in Public Health from Fundação Oswaldo Cruz (Fiocruz) – Pediatric Rheumathologist – Pediatric Rheumatology Clinic at Jesus Municipal
Hospital - Rio de Janeiro (RJ), Brazil.
4Post-graduate student of the Dermatology service from the University Hospital “Gaffré e Guinle” – The Federal University of Rio de Janeiro (HUGG – UNIRIO)
– Rio de Janeiro (RJ), Brazil.
5Master’s decree in Pediatrics from the Federal University of Rio de Janeiro (UFRJ) - Pediatric Rheumathologist - Pediatric Rheumatology Clinic at Jesus
Municipal Hospital - Rio de Janeiro (RJ), Brazil.
©2011 by Anais Brasileiros de Dermatologia
CASE REPORT
INTRODUCTION
SAPHO Syndrome is a chronic disease, occa-
sionally self-limited, composed of synovitis, acne, pus-
tulosis hyperostosis and osteitis.1In general, the
development of the disease is prolonged, with
episodes of remission and exacerbation without
response to antibiotics.
The association of palmoplantar pustulosis with
arthro osteits of the anterior chest wall was first
described in Japan, in 1967. In 1968, Kato and cols.
described the first case of bilateral clavicular
osteomyelitis with palmoplantar pustulosis.2In 1974,
Sonozaki and cols. reported a sero-negative rheuma-
toid syndrome with sternoclavicular hyperostosis
associated with palmoplantar pustulosis. Kahn and
cols. noticed that arthro-osteitis sterile was the com-
mon denominator of a great number of patients with
pain in the anterior chest wall or other musculoskele-
tal symptoms, carriers of palmoplantar pustulosis and
acne fulminans.² So, in 1984, they characterized the
SAPHO Syndrome with three criteria . It was enough,
for the diagnosis of the syndrome ,the presence of
only one of these characterisitcs: multifocal osteitis
with or without cutaneous manifestations; sterile junt
inflammation, acute or chronic, associated with pus-
tule or palmoplantar psoriasis, acne or hidradenitis;
sterile osteitis in presence of one of the skin manifes-
tations mentioned above.
CASE REPORT
The ambulatory care and hospitalizations of a
male mullato pediatric patient, with pregnancy and
child birth without complications, normal growth and
development and no family history will be described.
The infant, when he was three months old, had
pnemonia and recurrent pyoderma. When he was 11
months old he was hospitalized for the first time due
