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Hand pain in the early diagnosis of lepra.

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calves and bluish discoloration of the left first toe occurred. Central and peripheral nervous system symptoms, visual disturbances, cardiovascular and gastrointestinal complaints, testicular pain, and Raynaud’s
phenomenon were absent. Physical examination in 1968
revealed findings consistent with livedo reticularis on the
back and lower extremities, and cyanosis of the left first
and all the right toes; no other abnormalities were
Laboratory data included: a moderate leukocytosis ranging from 11,000 to 17,000 WBC/mm3; polymorphonuclear leukocytosis (without eosinophilia); a
sedimentation rate of 27 mm/hr; and negative antinuclear antibodies, rheumatoid factor, LE cell preparations, and serum cryoglobulins. Blood chemistries, urinalysis, chest x-rays, electrocardiogram, and
electromyogram were normal. Calf muscle and skin
biopsies were compatible with polyarteritis. Synovial
biopsy revealed nonspecific synovitis.
Subsequently, treatment has consisted of varying
doses of prednisone, chlorambucil, salicylates, isoxsuprine HCI, and intraarticular corticosteroids. He has
suffered with persistent bilateral intermittent synovitis
with effusions of the knees, and discoloration, numerous
small infarctions, ulcerations, and localized areas of
gangrene on the toes of both feet. Large doses of oral
prednisone (up to 60 mg daily) have been associated
with dramatic resolution of vasculitis and its consequences. Despite these therapeutic measures, intermittent bilateral knee effusions recur, resulting in severe
pain and functional impairment, with restriction of ambulation.
Twelve aspirations of the right and fifteen aspirations of the left knee have been accomplished over a 30month time period (1975-1977); the mean volume of
synovial fluid removed was 3 1 ml, the maximum 70 ml.
Synovianalysis showed clear yellow fluid, with a good
string sign and viscosity, a white blood count of
2300/mm3, 90% polymorphonuclear leukocytes, 10%
lymphocytes, a normal glucose (87 mg%), and no crystals on microscopic examination of an unstained wetmount. Knee roentgenograms taken in September 1977
revealed no periarticular osteopenia or osseous cysts.
There was minimal narrowing of the medial joint space
with early spur formation, and irregularity of the patellar articular surface, more prominently on the right than
left. Clinical examination of the knees at that time revealed no instability, deformity, or limitation of motion,
and only questionable crepitation. No other joints have
been affected, nor has there been any clinical or laboratory evidence of internal organ involvement.
Discussion. The distribution, severity, chronicity,
and treatment of the articular disease manifest by our
patient were similar to that reported by Smukler and
Schumacher, as was the absence of internal organ involvement seen with systemic polyarteritis. In contrast
to their cases, however, our patient suffered more ominous evidence of cutaneous vasculitis including localized
areas of gangrene that required hospitalization, debridement, and the administration of large doses of systemic
corticosteroids and a cytotoxic agent. Surgical adiputation of the affected digits and distal foot was strongly
considered, but proved unnecessary since the patient
responded to stringent medical treatment. We concur
with the conclusions of the authors that the arthropathy
associated with cutaneous polyarteritis tends to be
chronic and recurrent, has a predilection for the knees,
and is inflammatory in nature, at times with prominent
effusion resulting in considerable symptomatology.
University of Miami School of Medicine
Miami. Florida 331.52
Hand Pain in the Early Diagnosis of Lepra
To the Editor:
Lepra reaction (type 2) is characterized by one or
more of the following: erythema nodosum leprosum,
intermittent fever, nerve swelling and pain, bone pain,
swollen joints, acute iridocyclitis, epistaxis, edema of
the glottis, lymphadenitis and mental depression. Also,
polyarthritis, myositis, and immune-complex glomerulonephritis have been described in a patient with
lepromatous leprosy (Iveson JMI, McDougall AC,
Leathem AJ, Harris HJ: Lepromatous leprosy presenting with polyarthritis, myositis and immune-complex
glomerulonephritis. Brit Med J 3:619-621, 1975).
We had the opportunity to diagnose lepra reaction in a very bizarre case: a 48-year-old woman, who
came to Israel from Poland in 1957, was hospitalized
because of paroxysmal atrial fibrillation and symmetrical convulsions of short duration in all extremities. History included bronchial asthma since 1959 and allergic
reaction to different drugs. I n 1969 there was one episode of edema of the larynx. In 1970 she underwent
explorative laparotomy because of acute pain in the
abdomen, and acute pancreatitis was found. Since then,
she has been hospitalized six more times because of pain
in the right hypochondrium, vomiting, and fever, but no
definite diagnosis could be made. During one hospitalization elsewhere (in 1974) she also had a maculopapular eruption all over the body. In 1974 she underwent
drainage of a perianal abscess, and thereafter she was
hospitalized for prolonged fever of unknown origin with
abdominal pain. A short time after this she was admitted to a psychiatric hospital for depression. In the last
month she suffered pain in both hands, and these complaints prevailed during this last entire hospitalization.
On examination her general condition was good, blood
pressure was 130/80. Her temperature was subfebrile
(37-38°C) during many weeks. Occasional expiratory
wheezing was heard over both lungs.
The main complaint during this hospitalization
was pain in both hands accompanied by fever. There
were no signs of arthritis, and x-rays of the joints were
normal. A complete laboratory examination on admission to the rheumatology department showed no special
changes, except for a transient leukopenia (3000/mm3)
and thrombocytopenia (60,000/mm3). A connective tissue disorder was suspected, but no definite diagnosis
could be made.
Later on, during her hospitalization peripheral
neuropathy appeared. A thorough checkup ruled out
many causes of peripheral neuropathy, including metal
poisoning, vitamin deficiency, and diabetes. Repeated
neurological examinations disclosed bilateral distal hypoalgesia of the hands (gloves-form, without sharply
defined borders). The ulnar nerves on both sides and the
greater auricular nerve on the right side seemed to be
thickened and were tender on palpation. On the forehead a faint reddish infiltrate was visible; the right earlobe was slightly reddish and swollen, and there was
partial alopecia of the eyebrows. I n both legs and forearms there was an inability to discriminate sharp from
blunt and changes of temperature or touch. Both optic
discs were normal. X-ray of skull and EEG were normal. A skin biopsy of the ear lobule and a nasal smear
did not reveal acid-fast bacili. EMG revealed decreased
conduction in the right ulnar nerve at the elbow region.
Surgical decompression of the right ulnar nerve at this
site was performed. During this procedure, a biopsy was
done from a minor branch of this nerve, which microscopically showed signs of nerve fiber degenerations (including hydropic degeneration) and groups of acid-fast
Thus, lepra reaction of Hansen’s disease could be
diagnosed. After a short course of thalidomide therapy
her pain disappeared. Later she was transferred to a
leprosarium for further treatment. She was seen again, 1
year after the treatment with thalidomide was started;
she felt generally well, had no more pain, though she
complained again of paresthesias in both hands.
One can wonder whether all the details of her
past medical history belong to lepra, and in fact, without
doubt, m a n y symptoms and signs were similar to those
of lepra, for example, edema of the glottis, intermittent
fever, typical neurological findings, and bone pain.
There was the fever and .pain in her hands which,
eventually, has led us to the diagnosis of lepra.
Department of Medicine “ A and
Rheumatology Service
Hasharon Hospital
Petah-Tiqva. Israel
Reiter’s Syndrome: Occurrence in
T o the Editor:
I n March 1976 a 27-year-old white male welder,
JH, was seen on referral from a neurosurgeon for evaluation of back pain of 5 months’ duration, pain in the
right heel, swelling of the right ankle, right shoulder
pain, and morning stiffness. Range of motion was limited in the lumbar spine. Swelling was noted in the right
ankle with tenderness and swelling over the first left
metatarsal phalangeal joint. Slight limitation of motion
of the right shoulder was present. Pelvic x-ray demonstrated erosions of the sacroiliac joints. Laboratory data
included a Westergren sedimentation rate of 49 mm/hr,
negative RA test, and a positive HLA B27. Indocin
therapy was started, and over the next several months
his symptoms improved, with arthritis and back symptoms almost totally resolved by December 1976.
Approximately one year later, a 30-year-old
white male lumber salesman, WL, JH’s ex-roommate,
sought treatment for a 6-month history of swelling in the
right knee, low back pain, right shoulder pain, podagra.
swelling and pain of the left heel, and morning stiffness
of 2 hours’ duration. Unlike the previous patient, WL
gave a history of having a urethral discharge prior to the
onset of the arthritis and, more recently, a mild burning
in his eyes. On physical examination a slightly warm,
right swollen knee was noted. A tender, swollen left
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pain, hands, lepra, diagnosis, early
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