Acute febrile neutrophilic dermatosis (Sweet's syndrome) in neuro-Behçet's disease

doi:10.1016/0303-8467(93)90109-T

Clinical Neurology and Neurosurgery

Volume 95, Issue 4, December 1993, Pages 319-322

https://doi.org/10.1016/0303-8467(93)90109-T Get rights and content

Abstract

A case of Behçet's disease associated with Sweet's syndrome is presented. The onset was neurological symptoms and oral aphthae. Subsequently, genital ulcers developed. Coexistence of Sweet's syndrome and Behçet's disease has been reported only in few patients. On reviewing the literature, we propose that Sweet's syndrome is one of the associated diseases of Behçet's disease.

References (17)

J.D. O'Duffy et al.
Neurologic involvement in seven patients with Behçel's disease
Am. J. Med.
(1976)
International Study Group for Behçet's disease
Criteria for diagnosis of Behçet's disease
Lancet
(1990)
H. Behçet
Über rezidivierende, aphtose, durch ein Virus verursachte Geschwüre im Mund, am Auge und an den Genitalien
Dermatol. Wochenschr.
(1937)
S.M. Wolf et al.
Involvement of nervous system in Behçet's syndrome
Arch. Neurol.
(1965)
T. Cahjek et al.
Behçet's disease: report of 41 cases and a review of the literature
Medicine
(1975)
R.A. Shakir et al.
Neurological presentation of neuro-Behcet's syndrome: clinical categories
Eur. Neurol.
(1990)
M.Y. Aminof
Neurology and General Medicine
(1989)
B. Wechsel et al.
Cerebral venous thrombosis in Behçet's disease: clinical study and longterm follow up of 25 cases
Neurology
(1992)

There are more references available in the full text version of this article.

Cited by (20)

Neutrophilic dermatosis and hidradenitis suppurativa in patients with Behçet's disease: A neutrophilic disease in the spectrum of autoinflammatory syndromes
2023, Seminars in Arthritis and Rheumatism
Association of neutrophilic dermatosis (ND), hidradenitis suppurativa (HS) and Behçet's disease (BD) and shared efficacy of TNFα axis blockade suggests common physiopathology.
To investigate the clinical features and therapeutic response of ND and HS associated with BD.
We identified 20 patients with ND or HS associated with BD among 1462 patients with BD.
We analysed 20 (1.4%) patients diagnosed with ND or HS associated with BD: 13 HS, 6 pyoderma gangrenosum (PG), and 1 SAPHO. Our 6 PG cases over 1462 BD patients accounts for 400/100 000 prevalence. Thirteen had bipolar aphthosis, 6 vascular, 5 neurologic, and 4 ocular involvements. All PG occurred on limbs and had typical histology with constant dermal neutrophilic infiltrate. All HS had the classical axillary-mammary phenotype. Sixty-nine percent (69%) of HS were Hurley 1 stage. Treatment consisted mainly in colchicine (n = 20), glucocorticoids (n = 12), and anti-TNFα (n = 9). Interesting results with complete or partial responses were obtained with anti-TNFα (9 cases), ustekinumab (3 cases) and tocilizumab (1 case) to treat refractory ND or HS associated with BD.
PG seems overrepresented in patients with BD. Biotherapies such as anti-TNFα, ustekinumab and tocilizumab appear to be promising to treat refractory ND or HS associated with BD.
Successful treatment of relapsing neuro-Sweet's disease with corticosteroid and dapsone combination therapy
2007, Clinical Neurology and Neurosurgery
Sweet's disease with central nervous system involvement, tentatively named neuro-Sweet's disease, has rarely been reported. Although systemic corticosteroid therapy is highly effective for neurologic symptoms in neuro-Sweet's disease, relapse is common. Here, we describe the case of a 38-year-old Japanese man who presented with relapsing neuro-Sweet's disease that was successfully treated with a combination of corticosteroid and dapsone. Dapsone should be considered as a therapeutic option for neuro-Sweet's disease patients showing relapse.
Sweet's syndrome in Behçet's disease<sup>{lozenge, open}</sup>{lozenge, open}Pour citer cet article, utiliser ce titre en anglais et sa référence dans le même volume de Joint Bone Spine.
2007, Revue du Rhumatisme (Edition Francaise)
L'association syndrome de Sweet et maladie de Behçet est peu rapportée dans la littérature.
Une femme âgée de 47 ans aux antécédents d'aphtose bipolaire récidivante négligée depuis cinq ans. Elle fut hospitalisée pour des lésions cutanées érythémateuses, douloureuses à type de papule pseudovésiculeuse siégeant au visage, à la paume des mains, au cou et aux jambes associées à des polyarthralgies et un amaigrissement chiffré à 6 kg en un mois. L'examen clinique a révélé en plus des lésions cutanées une fièvre à 38,5 °C, une conjonctivite et une douleur à la mobilisation des articulations sans synovite. Son bilan biologique a objectivé un syndrome inflammatoire, une hyperleucocytose à 12,5 10³ avec 74 % de polynucléaires neutrophiles. La biopsie cutanée a mis en évidence des lésions caractéristiques du syndrome de Sweet et le test pathergy était positif. Le traitement a consisté en une corticothérapie à la dose de 40 mg/j associée à de la colchicine. L'évolution était satisfaisante.
Les manifestations cliniques du syndrome de Sweet et de la maladie de Behçet se ressemblent. À la lumière des données de la littérature, les auteurs pensent que la maladie de Behçet est une des affections qui peut être associée au syndrome de Sweet.
Sweet's syndrome in Behçet's disease
2007, Joint Bone Spine
Citation Excerpt :
A few cases of Sweet's syndrome have been reported in patients with Behçet's disease. Uysal et al. [12] suggested three possible relationships between the two diseases: Sweet's syndrome may be the acute phase of Behçet's disease; Sweet's syndrome and Behçet's disease may be separate conditions, particularly as the clinical similarities between the skin lesions mask markedly different histological abnormalities; or Behçet's disease may be one of the conditions with Sweet's syndrome. Mizoguchi et al. [4] studied human leukocyte antigens in 28 patients with Sweet's syndrome (including seven with symptoms of Behçet's disease) and 49 with Behçet's disease.
Few cases of Sweet's syndrome have been reported in patients with Behçet's disease.
A 47-year-old woman with five year history of oral and genital ulcers that had not been investigated previously was admitted for an eruption of tender, erythematous, vesicle-like papules over the face, neck, palms, and legs. She reported polyarthralgia and weight loss of 6 kg over the previous month. At admission, she had a fever of 38.5 °C and conjunctivitis. Joint tenderness to mobilization without evidence of synovitis was noted. Laboratory tests showed inflammation and leukocytosis (12.5 × 10⁹/L) with 74% neutrophils. The skin biopsy was typical for Sweet's syndrome, and the pathergy test was positive. Glucocorticoid therapy 40 mg/day and colchicine were effective in alleviating the manifestations.
Overlap exists between the clinical manifestations of Sweet's syndrome and Behçet's disease. Data from the literature suggest that Behçet's disease may be among the conditions that underlie Sweet's syndrome.
Idiopathic hypertrophic cranial pachymeningitis associated with Sweet's Syndrome
2002, European Journal of Radiology
A case of hypertrophic cranial pachymeningitis associated with Sweet's Syndrome is presented. Both entities have been described in association with several other chronic systemic inflammatory diseases and autoimmune conditions. To our knowledge the coexistence between Sweet's Syndrome and hypertrophic cranial pachymeningitis has not been reported up to date. We suggest a possible autoimmune or dysimmune mechanism in the pathogenesis of these two entities.
Sweet's syndrome associated with encephalitis
2001, Journal of the Neurological Sciences
The involvement of the central nervous system (CNS) in Sweet's syndrome (acute febrile neutrophilic dermatosis) is rare. We report a 47-year-old woman who presented with acute encephalitis and was subsequently diagnosed as having Sweet's syndrome. She developed altered consciousness following fever and erythematous skin plaques in the extremities. Cerebrospinal fluid (CSF) examination disclosed neutrophilic pleocytosis without decreased glucose level. Brain magnetic resonance imaging (MRI) showed abnormal signal intensity lesions in the basal ganglia and the hippocampus. Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet's syndrome. Treatment with acyclovir and antibiotics failed, but the subsequent corticosteroid therapy was effective. Awareness of neurological complication in Sweet's syndrome may avoid unnecessary empiric therapy for meningoencephalitis and will lead to a successful treatment with corticosteroids.

View all citing articles on Scopus

View full text

Case reportAcute febrile neutrophilic dermatosis (Sweet's syndrome) in neuro-Behçet's disease

Abstract

Am. J. Med.

Lancet

Über rezidivierende, aphtose, durch ein Virus verursachte Geschwüre im Mund, am Auge und an den Genitalien

Dermatol. Wochenschr.

Involvement of nervous system in Behçet's syndrome

Arch. Neurol.

Behçet's disease: report of 41 cases and a review of the literature

Medicine

Neurological presentation of neuro-Behcet's syndrome: clinical categories

Eur. Neurol.

Neurology and General Medicine

Cerebral venous thrombosis in Behçet's disease: clinical study and longterm follow up of 25 cases

Neurology

Case report
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in neuro-Behçet's disease