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Repeated furunculosis in adult male with abnormal

neutrophil activity
W. Carvalho Neves Forte, A. M ontaje Noyoya, F. Ferreira de Carvalho Jr. and S. Bruno

Immunology Section of Santa Casa M edical School and Hospital, São Paulo, Brazil.

oxigenase system and phagocytic function w ith


SUM M ARY normal B and T cell function. Clinically, there are
serious infections, w hich usually start during the first
A 21 years old male suffered from repeated year of life, w ith abscess formation especially in
furunculosis in different regions of the body over the lungs, lymph nodes, skin and liver. There are case
last tw o years. This coincided w ith the start of reports w here diagnosis w as made as late as the
professional activities in hospital surroundings. The sixth decade of life (3). The infections are usually
purulent secretions all show ed grow th of caused by a relatively narrow spectrum of catalase
Staphylococcus aureus. All laboratory tests w ere positive micro-organisms like Staphylococcus
normal except for a decrease of the neutrophil aureus, Serratia marcenscens, Escherichia coli,
phagocytic ingestion phase. Pseudomonas and Aspergillus species. The
Before the diagnosis of defective phagocytosis prognosis of CGD is related to early diagnosis and
w as made, antibiotic treatment w as started about 4 treatment w ith antibiotics, sulfametoxazol-
to 5 days after the appearance of the infectious trimethopim and antifungus itraconazol if necessary
process and the furunculosis led to abscess and gamma interferon and bone marrow
formation w ith difficult healing and cellulitis. After the transplantation, if possible (4-11).
diagnosis of defective phagocytosis ingestion phase, Few case reports of adults w ith deficient ingestion
personal hygiene w as intensified during and after phase or defective phagocytic digestion have been
w ork shifts at the hospital and antibiotic treatment described in the literature (12).
w as started at the first signs of folliculitis, w hich
show ed healing.
CASE REPORT
Key w ords: Furunculosis. Defective phagocytosis.
Hospital. Staphylococcus aureus. A 21 year old male, w ithout previous recurrent
infection, w ho presented recurrent furunculosis over
Allergol et Immunopathol 2000;28:328-31. the last tw o years. The first lesion developed in the
nasal region and progressed to facial cellulitis. Tw o
months later, a similar infection developed in the
INTRODUCTION right calf region w ith a similar lengthy duration. At
this stage, immunologic investigation show ed
Defective phagocytic activity by neutrophils is deficiency in the phogocytic ingestion phase (table I)
usually diagnosed in children. The most frequently w ith normal nitroblue tetrazolin (NBT) test (table II).
encountered is chronic granulomatous disease (CGD) After identification of the alteration in
w ith an incidence of 1 in 181.000 live births (1, 2). phagocytosis, the furunculosis developed in the left
The deficiency in CGD is at the neutrophil superior eyelid, the right and then the left inguinal
digestion phase. CGD is a genetic syndrome region and the left leg (tw ice). Culture in all these
characterized by increased susceptibility to serious infections demonstrated Staphylococcus aureus as
infections associated to dysfunction of the NADPH causative agent. Antibiotics w ere administered

Allergol et Immunopathol 2000;28(6):328-331


W. Carvalho Neves Forte, A. Montaje Noyoya, F. Ferreira de Carvalho Jr., S. Bruno.— REPEATED FURUNCULOSIS
IN ADULT MALE WITH ABNORMAL NEUTROPHIL ACTIVITY 329

Table I Our results show a decrease in the ingestion phase


Results of chemotaxis and phagocytosis evaluation of of phagocytosis, w ith normal oxidative metabolism of
polymorphonuclear neutrophils polymorphonuclear neutrophils (17, 18).
These findings are coherent w ith the clinical
Neutrophils Chemotaxis Phagocytosis observation of more frequent infections by
Staphylococcus aureus and the progress of folliculitis
Control 28 micra 21% to abscesses and cellulitis, w hich occurred in this
With homologous serum 69 micra 53%
patient.
With autologous serum 73 micra 55%
Polymorphonuclear neutrophils are important cells
in the defense against pyogenic bacteria like
Staphylococcus aureus. They represent the cells of
the initial defense against these microorganisms.
immediately after the first signs of folliculitis, w hich Neutrophils, in the presence of infectious agents,
resulted in fast and uncomplicated resolution of the start expressing greater numbers of adhesive
infections. molecules. Under these circumstances, they leave
the center of blood vessels and start moving more
slow ly at the periphery of these vessels (they
DISCUSSION marginate). After the union betw een adhesion
molecules of neutrophils (LFA-1 and VLA-1) and their
Zimosan particle phagocytosis used in our receptors on endothelial cells (ICAM -1, ICAM -2 and
methodology, assesses the ingestion phase by VCAM ), neutrophils leave the bloodstream and enter
neutrophils and is similar to that used previously for tissues w here immunologic defense occurs.
monocytes (13), seeing that monocytes and In the presence of chemotactic agents like
neutrophils present analogous receptors for lipopolysaccharides of bacterial w alls and cytokines
phagocytosis. Incubation w ith serum allow s (IL-8) and leukotriene B4 produced by monocytes and
compliment activation by the zimosan, resulting in macrophages, chemotaxis of neutrophils occurs to
C3b and C5b component activation w hich unite to the infection sites.
these particles, allow ing ingestion to take place. The When confronted w ith an infectious agent, the
first control verifies spontaneous cell migration polymorphonuclear neutrophil attempts to phogocytize
w ithout chemotactic stimuli. The fact of there not it. At an initial stage, ingestion of the agent occurs
being any significant difference betw een the second through receptors forming a phagocytic vacuole w hich
test (cells incubated w ith Zy and human “ pool” contains the antigen. The neutrophil’s cytoplasmatic
serum) and the third test (cells, Zy and patient’s granules are then throw n into the phagocytic vacuole
serum) in the presence of normal complement level forming a phogosome, w here digestion w ill occur.
in this patient, indicates that the decrease in When the first phase of phagocytosis is deficient,
phagocytosis is due to an intrinsic problem of the that is, the ingestion phase, the w hole process of
neutrophils. phagocytosis w ill be impaired even in the presence
Nitroblue tetrazolium, a soluble yellow dye, captures of a normal digestion phase and there w ill be
liberated electrons during oxidative neutrophil recurrent infections by microorganisms dependent of
metabolism. After reduction, the particles become dark polymorphonuclear neutrophil defense. The O2 to O2-
blue, called formazana, w hich are deposited inside the reduction is an important intracellular reaction in the
neutrophil cytoplasm. The NBT test becomes more digestion of the ingested bacteria (NADPH oxigenase
evident w hen stimulated by substances like system). In CGD there is inability to form O2- and H in
lipopolysaccharides. This test can evaluate electron phagocytosis, so there is no digestion of bacteria, fungi
liberation w hich occurs during the digestion phase by and especially catalase-positive microorganisms. These
oxidative metabolism of neutrophils (14-16). microorganisms remain alive w ithin phagocytes,
leading to granuloma formation (19, 20).
This patient started to present folliculitis after
Table II
initiating w ork at a hospital. Classical treatment of
Result of NBT evaluation folliculitis w ith topical antibiotics w as adopted at first.
As there w as no improvement, systemic antibiotics
Nitroblue tetrazolium (NBT) test w ere prescribed w ith unsatisfactory outcome and
progress of infection to facial cellulitis and large
Spontaneous: 6% .
With bacterial lipopolysaccharide: 13% .
abscess formation. Culture of secretion revealed
Staphylococcus aureus, the same agent encountered

Allergol et Immunopathol 2000;28(6):328-331


W. Carvalho Neves Forte, A. Montaje Noyoya, F. Ferreira de Carvalho Jr., S. Bruno.— REPEATED FURUNCULOSIS
330 IN ADULT MALE WITH ABNORMAL NEUTROPHIL ACTIVITY

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W. Carvalho Neves Forte, A. Montaje Noyoya, F. Ferreira de Carvalho Jr., S. Bruno.— REPEATED FURUNCULOSIS
IN ADULT MALE WITH ABNORMAL NEUTROPHIL ACTIVITY 331

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