ARTICLE
Auteur(s) : Eleni
Mylona1, Chariklia Vadala1, Vasilios
Papadakos2, Dionysios Loverdos1, Michael
Samarkos1, Athanasios Skoutelis1
15th Department of Internal Medicine,
Evangelismos Hospital, 45-47 Hipsilantou Str., Kolonaki,
GR-106 76 Athens, Greece
2Department of Rheumatology, Evangelismos Hospital,
Athens, Greece.
accepté le 23 Mai 2009
Adult onset Still’s disease (AOSD) is a systemic inflammatory
disorder, the diagnosis of which is based on certain criteria [1].
The presence of the typical Still’s rash is considered to be a
major criterion for AOSD diagnosis [1]. However, over the last few
years, there have been several atypical cutaneous findings reported
in patients with AOSD [2]. We report the case of patient with AOSD
who is unique in having developed cutaneous polyarteritis nodosa
(PAN) during the active phase of her disease.
Case report
A 29-year-old woman, previously healthy, was admitted to our
department with a two week history of high spiking fever, headache
and polyarthralgia. On admission the patient was normotensive with
a regular heart rate of 78 beats per minute when afebrile. Physical
examination revealed a red maculopapular rash over the interior
surface of her knees, as well as painless inguinal and maxillary
lymphadenopathy. Laboratory evaluation disclosed: leukocytes 14 ×
109/L (91% neutrophils), erythrocyte sedimentation rate
(ESR) 108 mm/L, C-reactive protein 31.3 mg/dL
(< 0.5 mg/dL), normal renal and liver function. Serum
ferritin was 18 ng/mL (26-300 ng/mL). Serum immunoglobulins,
complement levels, rheumatoid factor, ANA, antineutrophil
cytoplasmic antibodies (ANCA) and ASO were negative.
Ophthalmological and neurological examinations, as well as
cerebrospinal fluid findings, were normal. A chest computed
tomography (CT) scan demonstrated bilateral infiltrates with
moderate bilateral pleural effusions, while echocardiography showed
small pericardial effusions. Abdominal CT scan revealed
hepatosplenomegaly. Transbronchial biopsies via fiberoptic
bronchoscopy revealed mild, non-specific inflammation without
evidence of granulomatous disease, vasculitis or infection. Fifteen
days after the first chest CT scan and while administrating no
treatment, a second chest CT scan was performed, which revealed a
remission of the pleuropericardial effusions and disappearance of
the infiltrates. Based on the diagnostic criteria of Yamaguchi
et al. [1] and after having excluded other infectious,
neoplastic and rheumatic diseases, the diagnosis of AOSD was made.
The patient was treated with prednisolone 30 mg daily, with
satisfactory clinical and laboratory responses. In the second week
of her treatment, the patient developed small painful nodules on
her lower legs (figure
1A) with arthralgias at her wrists and ankles. On
examination, the nodules were found to be in different stages of
development with 5-6 days duration of eruption. Their biopsy
revealed vasculitis of the small and medium-sized muscular arteries
of the deep dermis and subcutaneous fat, with fibrinoid necrosis,
consistent with the cutaneous variant of polyarteritis nodosa (figure 1B).
Hydroxychloroquine 400 mg/daily and methotrexate
12.5 mg/weekly were added to the patient’s daily treatment
with prednisolone 30 mg. About one month after methotrexate
initiation, the nodules disappeared and the arthralgias subsided.
The prednisolone dose was decreased by 2.5 mg every two weeks.
One year after the discharge, the disease has been fully controlled
and the patient’s symptoms and cutaneous findings have not
recurred.
Discussion
Because of the diverse presentation and the lack of specific
laboratory and pathological manifestations, different
classification schemes have been developed to help standardize the
diagnosis of AOSD. The most widely accepted criteria set, as
presented by Yamaguchi et al., is a compilation of major
(fever ≥ 39 °C lasting one week or longer, arthralgia lasting
≥ two weeks, typical rash, leukocytosis ≥ 10,000/mm3
including ≥ 80% granulocytes) and minor (sore throat,
lymphadenopathy and/or splenomegaly, liver dysfunction defined as
an abnormally elevated level of transaminases and/or lactate
dehydrogenase, negative RF and ANA) criteria with the exclusion of
infections, malignancies and other rheumatic or systemic diseases
[1]. Our patient fulfilled 3 major and 2 minor of the
aforementioned criteria and, moreover, she suffered from serositis
and pneumonitis which, although not included in the diagnostic
criteria, are thought to be significantly specific for AODS [1].
Cutanous PAN is one of the three entities that are included in
the term PAN, together with classic systemic PAN and microscopic
PAN (microscopic polyangeiitis) [3]. The distinction of cutaneous
PAN from classic systemic PAN is still a matter of debate, as the
cutaneous variant may be regarded either as a separate dermal
disease or an initial manifestation of systemic disease [4].
Moreover, even if considered as a separate dermal disease,
constitutive symptoms like fever, neuropathy and muscle involvement
as well as arthralgias, resembling AOSD, are not uncommon [5].
Although our patient fulfilled the diagnostic criteria of AOSD,
the coexistence of a necrotizing vasculitis imposes the
re-evaluation of PAN vs AOSD as the diagnosis. However, the patient
had no indication of involvement of the characteristic organs
usually implicated in systemic PAN (kidney, gastrointestinal tract,
central or peripheral nervous system). Moreover, she developed
pneumonitis, while PAN is known to spare the lungs [6]. The
occurrence of pulmonary lesions in systemic vasculitis shifts the
differential diagnosis in favor of microscopic polyangeiitis (MPA).
However, MPA is characterized by small vessel vasculitis which
predominantly affects muscular arteriole, capillaries and venules.
The absence of involvement of capillaries and venules and the
localization of the affected vessels in the deep dermis or/and
subcutaneous fat in PN is a major point of distinction from MPA [3,
7]. Moreover, the latter belongs to the ANCA positive vasculitides,
and cutaneous nodules do not appear to be features of this disease
due to the absence of involvement of larger vessels [7]. The
attribution of high fever and arthralgias to the systemic
manifestations of cutaneous PAN could not explain the rest of our
patient’s symptoms (serositis, pneumonitis, lymphadenopathy) since
these are not considered to belong to the constitutive symptoms of
cutaneous PAN [5].
Subcutaneous nodules in the course of AOSD, without histological
examination, have been described in the past [8]. To the best of
our knowledge, no previous cases of AOSD associated with cutaneous
variant of PAN have been reported.
Acknowledgements
Conflict of interest: none. Financial support: none.
References
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