Letters to the Editor

ment (8). active disease. following days a dramatic clinical response,

In conclusion, this is the first case reporting Our patient had a 10-day history of high including remission of skin vasculitis, was
the successful treatment of GBS following fever (> 39.5ºC), diffuse arthalgias and observed. By the seventh week he had
gold therapy with high dose IVIG. myalgias, and an evanescent salmon pink resumed all of his daily activities, treatment
rash prior to admission, as well as a one was discontinued at the 14th month and he
M.G. TEKTONIDOU, MD, Consultant month history of sore throat, low-grade did extremely well during the following 6
F.N. SKOPOULI, MD, Professor fever, mild abdominal pain and weight loss months. Laboratory examinations, includ-
Department of Internal Medicine, Euroclinic of 5 kg. Remarkably, at the age of 6 and 8 ing repeated determination of cryoglobu-
Hospital, Athens, Greece. years he had two episodes of high fever and lins, were unremarkable.
Address correspondence and reprint requests arthralgias which fully resolved after a 3- Clearly, our patient fulfilled the diagnostic
to: Dr. F.N. Skopouli, 29 Vournazou Street, month treatment with high doses of aspirin. criteria of ASD (1, 3, 4), which was con-
115 21 Athens, Greece. On admission he also had hepatosplen- firmed during the 20-month follow-up.
E-mail: fskopouli@[Link] omegaly, anemia, leukocytosis, strikingly Because of his childhood episodes of fever
elevated C-reactive protein and fer ritin lev- and arthralgias the diagnosis of Still’s dis-
References els, mild liver dysfunction and normal renal ease of childhood onset is tempting; howev-
1. ENDTZ LJ: Complications nerveuses du traite- function. Serum immunoglobulins IgG and er, since he denied any similar episodes for
ment aurique. Rev Neurol (Paris) 1958; 99: IgA were elevated, IgM was normal, while the next 40 years, he was classified as hav-
395-410. complement levels were at the lower limit ing an adult-onset disease (1, 6, 7). We sys-
2. SERRE H, MORLOCK G, SANY J, DUBOIS A, of normal. Rheumatoid factor, antinuclear tematically excluded viral infections, sep-
NALET B : Syndrome de Guillain-Barré après
antibodies, antineutrophil cytoplasmic anti- sis, tuberculosis, sarcoidosis, malignancies,
chrysothérapie. Rhumatologie 1975; 27: 367-
bodies and an extensive work-up for infec- systemic connective tissue diseases (1, 2),
72.
3. BONTOUX D, LEFEVRE JP, MEDEJEL A, DA- tion were all negative. Of note, cryoglobu- and Schnitzel’s syndrome (8), not only
BAN M : Neurologic complications of chryso- lins were detectable as polyclonal IgG, IgA, because ASD is a relatively rare disease, but
therapy. Apropos of 2 cases, 1 with Guillain- and IgM. also because of the atypical coexistence of
Barré syndrome. Rev Rhum Mal Osteoartic On the second day of hospitalization a mild both skin and renal involvement. Cutaneous
1974; 41: 48-51. migratory, itchy, maculopapular rash ap- vasculitis and glomerulonephritis in the
4. SCHLUMPF U, MEYER M, ULRICH J , FRIEDE peared on the head, upper trunk, back, but- course of ASD have been described only
R: Neurologic complications induced by gold tocks and groins. The rash evolved into pur- once and twice in the past, respectively (9,
treatment. Arthritis Rheum 1983; 26: 825-31. puric plaques after two days and disap- 10). To the best of our knowledge no previ-
5. DICK DJ , RAMAN D . The Guillain-Barré syn -
peared soon after. On the fifth day several ous cases of ASD associated with mixed
drome following gold therapy. Scand J
Rheumatol 1982; 11: 119-20. annular purpuric plaques on the ankles and cryoglobulinemia have been reported. Per -
6. VERNAY D, DUBOST JJ, THEVENET JP, dorsal area of the feet and a symmetric con- haps clinicians are overlooking a possibly
SAUVEZIE B, RAMPON S : “Chorée fibrillair e fluent purpuric plaque on the shins were not uncommon aspect of this disease, since
de Mor van” followed by Guillain-Barré syn- observed (Fig. 1). Skin biopsy findings were during the course of a severe, acute inflam-
drome in a patient receiving gold therapy. compatible with leukocytoclastic vasculitis. matory response such as ASD transient
Arthritis Rheum 1986;1413-4. In the following days his general condition cryoglobulin formation is not unexpected.
7. ROQUER J, HERRAIZ J, MAYMO J, OLIVE A, deteriorated to the point that he was unable This case suggests that ASD should be
CARBONELL J: Miller–Fisher syndrome to walk, cutaneous lesions became fixed, included in the list of systemic conditions
(Guillain-Barré syndrome with ophthalmople- and renal dysfunction demonstrated by the that may be complicated with mixed cryo-
gia) during treatment with gold salts in a
presence of granular casts, mild pyuria and globulinemia.
patient with rheumatoid arthritis. Arthritis
Rheum 1985; 28: 838-9. hematuria and elevated serum urea, was
8. PLASMA E XCHANGE/S ANDOGLOBULINE observed. Renal biopsy revealed rare vessel A.V. ELEZOGLOU, MD
GUILLAIN BARRÉ SYNDROME TRIAL GROUP: hyaline deposits, mesangial alterations and E. GIAMARELOS-BOURBOULIS, MD,
Randomised trial of plasma ex-change, intra- mild hypercellularity with C3 and IgM Lecturer of Internal Medicine
venous immunoglobulin, and combined treat- deposits on immunofluorescence. Bone mar- N. KATSILAMBROS MD, Professor
ments in Guillain Barré syndrome. Lancet row and liver biopsy findings were unre- P. P. SFIKAKIS, MD, FACR, Assistant Prof.
1997; 349: 225-30. markable. At the 10th day methylpred- First Dept. of Propedeutic Medicine, Athens
nisolone (72 mg/day) and methotrexate University Medical School, Laikon Hospital,
(17.5 mg/week) were started (5); during the Ag. Thoma Str., Athens 11527, Greece.
Cutaneous vasculitis associated Please address correspondence to: P.P.
with mixed cryoglobulinemia in Sfikakis, MD, 3, Amaryllidos Str, 154 52,
adult Still’s disease Athens, Greece. E-mail:
psfikakis@[Link]
Sirs,
Adult Still’s disease (ASD) is a systemic References
inflammatory disorder of unknown origin, 1. CUSH JJ, MEDSGER JR TA, CHRISTY WC et
characterized by high spiking fever, arthral- al.: Adult-onset Still’s disease: Clinical
gias or arthritis, sore throat, and an evanes- course and outcome. Arthritis Rheum 1987;
cent rash often occurring during the fever 30: 186-94.
spikes (1-3). We describe a 47-year-old man 2. LARSON EB : Adult Still’s disease. Evolution
of a clinical syndrome and diagnosis, treat-
fulfilling the diagnostic criteria of ASD (1,
Fig. 1. Demarcated annular purpuric plaques ment and follow-up of 17 patients. Medicine
3, 4) who is unique in having developed
with a palpable border and clearing in the center, (Baltimore) 1984; 63: 82-91.
mixed cryoglobulinemia, leukocytoclastic 3. HUNDER GC : Vasculitis. B. Clinical and lab-
cutaneous vasculitis and mild mesangial without necrosis or ulceration; smaller 3-5 mm
purpuric papules surround the plaques. oratory features. In KLIPPEL JH (Ed.) Primer
glomerulonephritis during the phase of on the Rheumatic Diseases. 11th ed. 1997,

405
Letters to the Editor
Arthritis Foundation, Atlanta, Georgia. cal hallmark of spondyloathritis (SpA) (1- (Fig. 1). Ultrasonography showed a moder-
4. YAMAGUCHI M, OHTA A,TSUNEMATSU T et 3). Although more frequent in psori at i c ate diffuse thickening of the left Achilles
al.: Preliminary criteria for classification of arthritis (PsA) (1, 4), dactylitis has been tendon together with an enlarged bursa. The
adult Still’s disease. J Rheumatol 1992; 19: observed in all forms of SpA including the patient was given methotrexate at a dose of
424-30.
undifferentiated forms (1-3). Recent studies 10 mg/day and diclofenac at a dose of 100
5. AYDINTUG AO, D’CRUZ D, CERVERA R;
KHAMASHTA MA, HUGHES GR: Low dose
using ultrasound and magnetic resonance mg/day with good results.
methotrexate treatment in adult Still’s dis- imaging (MRI) have established that The present r eport emphasizes that dactyli-
ease. J Rheumatol 1992; 19: 431-5. dactylitis is due to flexor tenosynovitis and tis may simultaneously involve most of the
6. OHTA A, YAMAGUSHI M, KANEOKA H et arthritis of the interphalangeal and metacar- fingers.
al.: Adult Still’s disease:Review of 228 cases pophalangeal (or metatarsophalangeal)
from the literature. J Rheumatol 1987; 14: joints is not a condition sine qua non for the I. OLIVIERI1, MD, Consultant
1139-46. “sausage-shaped” feature (5-7). Dactylitis
7. CABANE J, MICHON A,ZIZA JM et al.: Com-
E. SCARANO2, MD, Researcher
is usually asymmetric and involves few fin-
parison of long term evolution of adult onset A. PADULA1, MD, Researcher
gers and/or toes.
and juvenile onset Still’s disease, both fol-
We have recently come across a 37-year-old V. GIASI1, MD, Researcher
lowed up for more than 10 years. Ann Rheum 1
Rheumatology Department of Lucania, San
Dis 1990; 49: 283-5. man suffering from PsA and showing
dactylitis of most of his fingers. His family Carlo Hospital, Potenza and Madonna delle
8. TOMKOVA H, SHIRAFUJI Y, ARATA J : Grazie Hospital, Matera, and the 2Radiology
Schnitzler’s syndrome versus adult onset history was negative for SpA and psoriasis.
Department, San Carlo Hospital, Potenza,
Still’s disease. Eur J Dermatol 1998; 8:118- His medical history revealed that he had Italy.
21. been suffering from psoriasis for 18 years.
9. BAMBERY P, THOMAS RJ, MALHORTA HS Address correspondence to: Dr. Ignazio
Four months before the consultation he
et al.: Adult Still’s disease: clinical experi- Olivieri, Rheumatology Department of
developed “sausage-like” swelling of most Lucania, San Carlo Hospital, Contrada
ence with 18 patients over 15 years in north - fingers together with Achilles enthesitis.
ern India. Ann Rheum Dis 1992; 51: 529-32. Macchia Romana, 85100 Potenza, Italy.
Physical examination disclosed dactylitis of E-mail: ignazioolivieri@[Link]
10. WENDLING D, HORY B, BLANC D: Adult
Still’s disease and mesangial glomerulo- the second, third and fourth fingers of the
nephritis. Report of two cases. Clin Rheum- right hand and of the first, third, fourth and References
atol 1990; 9: 95-9. fifth left fingers. There was also a soft tissue 1. GLADMAN DD: Psoriatic arthritis. In KHAN
swelling along his left Achilles tendon and MA (Ed.): Ankylosing Spondylitis and Relat -
at his calcaneal insertion. The only aspect ed Spondyloarthropathies. Spine:State of the
of the laboratory evaluation worthy of note Art Review. Philadelphia,Hanley and Belfus,
Inc., 1990; 637-56.
Dactylitis involving most of the was a C-reactive protein level of 20.6
2. KHAN MA, VAN DER LINDEN SM : A wider
fingers mg/liter (normal < 5). HLA typing showed spectrum of spondyloarthropathies. Semin
A2, A30, B38, and B51. Magnetic reso- Arthritis Rheum 1990; 20: 107-13.
Sirs, nance imaging revealed fluid in the syn- 3. OLIVIERI I, PADULA A,PIERRO A, FAVARO L,
Dactylitis or “sausage-like” digit is a clini- ovial sheaths of all the dactylitic fingers ORANGES GS, FERRI S: Late onset undiffer-
entiated seronegative spondyloarthropathy. J
Rheumatol 1995; 22: 899-903.
4. SALVARANI C, CANTINI F, OLIVIERI I et
al.: Isolated peripheral enthesitis and/or dac-
tylitis:A subset of psoriatic arthritis. J Rheu-
matol 1997; 24: 1106-10.
5. OLIVIERI I, BAROZZI L, FAVARO L et al.:
Dactylitis in patients with seronegative spon-
dylarthropathy: assessment by ultrasonogra-
phy and magnetic resonance imaging. Arthri -
tis Rheum 1996; 39: 1524-8.
6. OLIVIERI I, BAROZZI L, PIERRO A, DE MAT-
TEIS M, PADULA A, PAVLICA P: Toe dactyli-
tis in patients with spondyloarthropathy:
Assessment by magnetic resonance imaging.
J Rheumatol 1997; 24: 926-30.
7. OLIVIERI I, SALVARANI C, CANTINI F et al.:
Fast spin echo-T2 sequences with fat satura-
tion in dactylitis of spondylarthrtis. Arthritis
Rheum 2002; 46: 2964-7.

(a) (b)
Fig. 1. (a) T2-weighted image at the level of the proximal interphalangeal joints showing fluid in the
synovial sheaths of the 3rd, 4th and 5th left fingers and in the 2nd, 3rd and 4th right fingers. (b) T2-
weighted image at the level of the metacarpophalangeal joints showing also the involvement of the 1st
left finger (arrow).

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