Infantile-Onset Multisystem Inflammatory Disease

Background

IOMID is Infantile-Onset Multisystem Inflammatory Disease, a rare disease occurring predominately in infants and young children. It is characterized by eye involvement and neurologic symptoms including chronic meningitis, macrocephaly, and developmental delay. There is also a distintive deforming arthropathy with unique epiphyseal radiologic findings preferentially affecting the larger joints.

SYNONYMS IOMID

LABORATORY/RADIOLOGIC/OTHER CHARACTERIZATION

Infantile-onset multisystem inflammatory disease: radiologic findings.

Kaufman RA, Lovell DJ.

Radiology 1986 Sep;160(3):741-6 Abstract quote

Infantile-onset multisystem inflammatory disease (IOMID) is a newly recognized disease that is similar to systemic-onset juvenile rheumatoid arthritis (JRA). The clinical symptoms of IOMID include rash, fever, lymphadenopathy, chronic meningitis, uveitis, and a distinct arthropathic condition. Skeletal involvement is surprisingly constant. The long bones become short, bowed, and widened. The metaphyses flare and simulate rickets. Grotesque epiphyses appear fragmented early in the course of the disease but are gradually incorporated into the widened shafts.

We observed these and other radiologic findings in the four cases described in the present study. The findings are distinctive and allow for a specific diagnosis.

CLINICAL VARIANTS CHARACTERIZATION

Infantile multisystem inflammatory disease: a specific syndrome?

Yarom A, Rennebohm RM, Levinson JE.

J Pediatr 1985 Mar;106(3):390-6 Abstract quote

We report two patients with infantile onset of evanescent rash, fever, arthropathy with severe deformities, periosteal changes, chronic meningitis, hydrocephalus, convulsions, developmental delay, papilledema, unusual uveitis, and lymphadenopathy. A few patients with similar findings have been previously reported. Although some similarity exists between findings in these patients and in others with systemic juvenile rheumatoid arthritis, they appear to differ both in regard to the nature and severity of the clinical and pathologic features.

We suggest that this group of patients has a separate rheumatic disorder not yet included in the standard classifications of the childhood rheumatic diseases.

Infantile-onset arthritis and multisystem inflammatory disease: "a new syndrome".

Mallouh A, Abu-Osba YK, Talab Y.

J Pediatr Orthop 1987 Mar-Apr;7(2):227-30 Abstract quote

A case is presented with early-onset polyarthritis involving both large and small joints, prolonged fever, skin rash, hepatosplenomegaly, persistent cerebro-spinal fluid pleocytosis, brain atrophy, macrocephaly with ventricular dilatation, a persistently open fontanelle, lymphadenopathy, subcutaneous nodules, developmental delay, failure to thrive, persistent hypochromic microcytic anemia, leukocytosis with shift to the left, early thrombocytopenia followed by thrombocytosis, high erythrocyte sedimentation rate, elevated immunoglobulin level, and vasculitis involving several organs. Thirteen cases have been previously reported under different names.

A unified name is needed; we suggest "infantile-onset arthritis and multisystem inflammatory disease."

Infantile-onset multisystem inflammatory disease: a differential diagnosis of systemic juvenile rheumatoid arthritis.

De Cunto CL, Liberatore DI, San Roman JL, Goldberg JC, Morandi AA, Feldman G.

Pediatric Rheumatology Section, Hospital Italiano, Buenos Aires, Argentina.

J Pediatr 1997 Apr;130(4):551-6 Abstract quote

We describe four unrelated children with neonatal maculopapular rash, fever, arthritis, hepatosplenomegaly, lymphadenopathy, eye involvement, and neurologic symptoms. Radiographs of the joints were surprisingly similar, showing an abnormal epiphyseal and metaphyseal appearance.

These clinical and radiologic findings allowed us to include these children in a very peculiar syndrome described as infantile-onset multisystemic inflammatory disease. A chondrosarcoma developed in one of our patients.

HISTOLOGICAL TYPES CHARACTERIZATION

General J Rheumatol 1995;22:1171-1173
Neutrophilic eccrine hidradenitis

VARIANTS

Radiology 1986;160:741-746

Commonly Used Terms

Skin

Last Updated 11/4/2001

HISTOLOGICAL TYPES	CHARACTERIZATION
General	J Rheumatol 1995;22:1171-1173 Neutrophilic eccrine hidradenitis
VARIANTS