Autosomal dominant granulomatous arthritis, uveitis, skin rash, and synovial cysts.

In 1985, Blau reported a family with 11 members in four generations affected by granulomatous arthritis, iritis, skin rash, and periarticular synovial cysts. We report a second family with these abnormalities, thereby confirming this syndrome as a distinct familial entity with transmission compatible with autosomal dominant inheritance. Affected members in our family included a mother and two daughters. Disease onset was at 10 months to 8 years of age. Each had uveitis, symmetric polyarthritis, and synovial cysts overlying the ankle and wrist joints. In addition, both daughters had an intermittent generalized erythematous papular rash that on biopsy revealed noncaseating granulomatous infiltration. All three patients improved during alternate-day steroid therapy. Recognition of this disorder as distinct from other, more common causes of arthritis is important because of the apparent autosomal dominant transmission and because of the excellent responses to low-dose steroid therapy.

Panic attack syndrome.

Panic attack syndrome in four generations of a family and in six additional pediatric patients is reported. The syndrome appears to have an autosomal dominant mode of inheritance. Diagnosis of panic attack syndrome in children has not been reported previously, but the existence of this disorder has been noted for at least 100 years under various synonyms. There is substantial evidence that the syndrome has an endogenous origin and is therefore a biochemical illness. The triggering effect of sodium lactate infusion and the alleviation of symptoms by use of monoamine oxidase inhibitors or beta-blocking agents support this view. Imipramine hydrochloride, propranolol hydrochloride, phenelzine sulfate, and alprazolam are often useful in the treatment of panic attacks, and except for propranolol, any of them may be effective against depression also.

Carpal-tarsal osteolysis.

A mother-daughter kindred with carpal-tarsal osteolysis and evidence of renal involvement is presented. There was a similarity between the early clinical manifestations of this syndrome and those of rheumatoid arthritis. The distinction made in previous reports between the dominantly inherited form and the sporadic form with nephropathy is questioned.