Mycobacterial disease in patients with chronic granulomatous disease: A retrospective analysis of 71 cases.

BACKGROUND: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by inborn errors of the phagocyte nicotinamide adenine dinucleotide phosphate oxidase complex. From the first year of life onward, most affected patients display multiple, severe, and recurrent infections caused by bacteria and fungi. Mycobacterial infections have also been reported in some patients. OBJECTIVE: Our objective was to assess the effect of mycobacterial disease in patients with CGD. METHODS: We analyzed retrospectively the clinical features of mycobacterial disease in 71 patients with CGD. Tuberculosis and BCG disease were diagnosed on the basis of microbiological, pathological, and/or clinical criteria. RESULTS: Thirty-one (44%) patients had tuberculosis, and 53 (75%) presented with adverse effects of BCG vaccination; 13 (18%) had both tuberculosis and BCG infections. None of these patients displayed clinical disease caused by environmental mycobacteria, Mycobacterium leprae, or Mycobacterium ulcerans. Most patients (76%) also had other pyogenic and fungal infections, but 24% presented solely with mycobacterial disease. Most patients presented a single localized episode of mycobacterial disease (37%), but recurrence (18%), disseminated disease (27%), and even death (18%) were also observed. One common feature in these patients was an early age at presentation for BCG disease. Mycobacterial disease was the first clinical manifestation of CGD in 60% of these patients. CONCLUSION: Mycobacterial disease is relatively common in patients with CGD living in countries in which tuberculosis is endemic, BCG vaccine is mandatory, or both. Adverse reactions to BCG and severe forms of tuberculosis should lead to a suspicion of CGD. BCG vaccine is contraindicated in patients with CGD.

Atypical cutaneous lesions of Penicillium marneffei infection as a manifestation of the immune reconstitution inflammatory syndrome after highly active antiretroviral therapy.

Penicillium marneffei infections normally manifest as molluscum contagiosum like skin lesion in HIV-infected persons. We report a case with verrucous lesions over nose and face due to Penicillium marneffei infection after HAART treatment. A 28-year-old man presented, after two weeks of HAART treatment, with multiple erythematous, scaly, papules and nodules with central necrosis predominantly in face and both extremities and scrotum. Microbiological investigations confirmed the diagnosis of Penicillium marneffei infection. This is perhaps the first case report with such manifestation due to Penicillium marneffei infection.

Keratitis.

Corneal inflammation or keratitis is a significant cause of ocular morbidity around the world. Fortunately, the majority of the cases are successfully managed with medical therapy, but the failure of therapy does occur, leading to devastating consequences of either losing the vision or the eye. This review attempts to provide current information on most, though not all, aspects of keratitis. Corneal inflammation may be ulcerative or nonulcerative and may arise because of infectious or noninfectious causes. The nonulcerative corneal inflammation may be confined to the epithelial layer or to the stroma of the cornea or may affect both. For clarity, this section has been divided into nonulcerative superficial keratitis and nonulcerative stromal keratitis. While the former usually includes hypersensitivity responses to microbial toxins and unknown agents, the latter can be either infectious or noninfectious. In the pathogenesis of ulcerative keratitis, microorganisms such as bacteria, fungi, parasites (Acanthamoeba), or viruses play an important role. Approximately, 12.2% of all corneal transplantations are done for active infectious keratitis. Available world literature pertaining to the incidence of microbial keratitis has been provided special place in this review. On the other hand, noninfectious ulcerative keratitis can be related to a variety of systemic or local causes, predominantly of autoimmune origin.

Corneal ulcer caused by Bipolaris hawaiiensis.

Following an injury to the right eye, a corneal ulcer with hypopyon developed in a leprosy patient. Direct examination of the corneal scrapings on three occasions showed septate, branched, dematiaceous hyphal elements. When scrapings were cultured on Sabouraud's glucose and brain heart infusion agars. Bipolaris hawaiiensis was isolated repeatedly. The patient responded successfully to treatment with nystatin ointment, although the central opacity of the cornea remained and visual acuity did not improve.

Mycotic granuloma caused by Phialophora repens.

This is the first reported infection by the saprophytic fungus, Phialophora repens. The infection was a solitary granulomatous nodule in the scalp of a Zaïrian man with advanced lepromatous leprosy. The patient was being treated by long-term prednisolone therapy. In tissue sections there were nonpigmented microcolonies composed of irregularly branched septate hyphae. A darkly pigmented fungus was isolated on Sabouraud's medium. The mycologic features of the etiologic agent were typical of P. repens. The infection was treated successfully by excision of the nodule.