Granulomas in common variable immunodeficiency: A diagnostic dilemma

Abstract

A 60-year-old man with common variable immunodeficiency presented with a 7-year history of violaceous plaques and papules on the thighs, arms and trunk. In the preceding 2 years he had developed new lesions on both hands. He had been previously diagnosed with sarcoidosis on the basis of skin and visceral histology, but subsequent opinion was that these were sarcoid-like granulomas rather than being representative of true sarcoidosis. Biopsy of the hand lesions showed necrotizing granulomas, and a single acid-fast bacillus (AFB) was identified on Wade-Fite stain. Subsequent repeat tissue biopsies for histology, culture and polymerase chain reaction testing failed to confirm the presence of mycobacterial organisms and it was felt that the organism was a contaminant introduced during tissue processing. The hand lesions responded well to intralesional injections of triamcinolone acetonide 10 mg/mL and oral tetracycline 500 mg b.d. was later introduced with a good clinical response. The diagnostic dilemma of finding granulomatous inflammation in a patient with common variable immunodeficiency, and the significance of a single AFB on histology are discussed. The treatment of sarcoid-like granulomas with tetracycline therapy is also commented on.

Etanercept treatment of cutaneous granulomas in common variable immunodeficiency

“…9 Necrobiotic and tuberculoid granulomas have also been described in patients with CVID. 10 The granulomas of patients with CVID usually affect the lymph nodes, spleen, liver, and gastrointestinal tract, but cutaneous granulomas in CVID are not uncommon. Krupnick et al 11 reviewed 15 patients with cutaneous granulomas and CVID.…”

Granulomatous disease in common variable immunodeficiency

“…Granulomatous disease raises the question of mycobacterial infection as an etiology, but granulomata in CVID are typically noncaseating, and mycobacteria have not been cultured from these patients [23,24]. There is one case report of a 12-year-old previously healthy girl who developed CVID associated with granulomatous lymphoproliferation following infection with Toxoplasma gondii [25].…”

The granulomatous reaction pattern

“…687,688 In chronic granulomatous disease, despite the name, true granulomas are not common in cutaneous lesions. [698][699][700][701][702][703][704][705] This disease, which has multiple phenotypes, is the most commonly diagnosed primary immunodeficiency disease in adults, after selective IgA deficiency. 686,[689][690][691][692][693][694][695] Carriers of X-linked chronic granulomatous disease (OMIM 306400) may present with lupus erythematosus-like lesions.…”