LETTERS NOTES

& COMMENTS

Recurrent episodes of neuro-Sweet syndrome in a Caucasian patient To the Editor: We read with interest the article by Rochet and colleagues1 concerning a study of 77 patients with Sweet syndrome (SS). It exhaustively describes the cutaneous manifestation and triggering infections, drugs, and malignancies, but there is no reference to the extracutaneous involvement, including effect on the central nervous system (CNS). To the best of our knowledge, 58 cases of SS with neurologic features have been reported.2 When CNS symptoms are referred, neuro-Sweet disease (NSD) should be investigated to avoid misdiagnosed meningoencephalitis, multiple sclerosis, neuro-Behc¸et disease, CNS lupus, or brain tumors. NSD may manifest with encephalitis, meningitis, headaches, consciousness disturbance, epilepsy, hemiparesis or tetraparesis, psychiatric disorders, or dyskinesia. These manifestations usually follow, but may precede, appearance of the skin lesions and are generally benign, transient, and rarely recurrent.3,4 If NSD is diagnosed, a more careful management in terms of therapy and follow-up is necessary, as in the case we report herein. A 45-year-old Caucasian woman with a 2-yearhistory of recurrent skin eruptions and arthralgias presented with erythematous plaques on her face, trunk, and extremities (Fig 1). She was not febrile. Laboratory tests showed high C-reactive protein (CRP) (19.8 mg/L) and fibrinogen (4.09 g/L) values; viral-bacterial serology test results were negative. Skin biopsy revealed neutrophilic dermatosis. Full-body computed tomography scan results were negative. Prednisone 35 mg/day was introduced, and the patient’s symptoms, as well as CRP and fibrinogen values, improved. After 4 years of intermittent exacerbations with inconstant neutrophilia, prednisone was suspended for decreased efficacy and adverse effects. Other treatments were used (colchicine, dapsone, methotrexate, anakinra, interferon- , leukapheresis) without benefit. Conversely, treatment with cyclosporine A (CyA) 200 mg/day resolved the cutaneous and arthralgic manifestations, but after 2 months, fever and right arm dysesthesias/paresthesias suddenly developed. Because an infectious inflammatory pathogenesis or a drug neurotoxicity was suspected, CyA was discontinued. Laboratory test results were normal. Brain magnetic resonance imaging showed multiple 192

JULY 2014

Fig 1. Sweet syndrome. Erythematous plaques and nodules on trunk and extremities.

T2-flair cortical-subcortical lesions in the left hemisphere and brainstem with slight contrast enhancement. Cerebrospinal fluid-analysis disclosed neutrophil hypercellularity. Microbiological serology and typing for HLA-B51/B54/Cw1 results were negative. According to clinical history, neurologic presentation, and neuroimaging, NSD was diagnosed. Histopathologic, clinical, and laboratory features excluded neuro-Behc¸et disease. Clinical and radiologic improvement was seen after a 3-day treatment with methylprednisolone boluses (1 g/day). Four months later, CyA was reintroduced because other drugs did not prevent cutaneous relapses. During the following year, the patient experienced several neurologic relapses (right arm involuntary movements and paresthesias). An electroencephalogram revealed focal epilepsy while the brain magnetic resonance imaging showed partial regression of the previous lesions and new left occipital-parietal lesions (Fig 2). A drug-induced neurotoxicity was considered because of time correlation with CyA. CyA can damage the parieto-occipital-temporal lobes through an alteration of the blood-brain barrier, causing generalized seizures, endocranial hypertension, and visual disturbances. Remission usually occurs when discontinued.5 Because our patient showed diffuse brain involvement with a stable lesion load even after CyA suspension, drug-induced neurotoxicity was unlikely. These clinical and radiologic findings suggest that CyA alone, although effective on the skin, is insufficient to prevent NSD relapses. Conversely, neurologic remission was obtained during steroid administration that alone was not able to prevent cutaneous relapses. In fact, corticosteroids might be protective of the CNS by reducing the inflammatory J AM ACAD DERMATOL

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Fig 2. Neuro-Sweet disease. A, Flair hyperintensities bilaterally in cortical-subcortical areas. B, T1-weighted gadolinium-enhanced magnetic resonance image shows contrast-enhancement positivity in left occipital cortex. These images are suggestive of diffuse CNS involvement (A) with evidence of active lesions (B).

activity of neutrophils and stabilizing the bloodbrain barrier. Our case underlines the importance of detecting neurologic involvement in SS in order to achieve the best therapeutic solution and avoid neurologic misdiagnosis. Francesco Drago, MD,a Giuseppe Ribizzi, MD,b Giulia Ciccarese, MD,a Arianna Fay Agnoletti, MD,a and Aurora Parodi, MDa DISSAL, Section of Dermatology, IRCCS Azienda Universitaria Ospedaliera, San Martino-IST,a and Department of Neurology, San Martino Hospital,b Genoa, Italy Funding sources: None. Conflicts of interest: None declared. Correspondence to: Dr Arianna Fay Agnoletti, MD, DISSAL, Section of Dermatology, IRCCS Azienda Universitaria Ospedaliera, San Martino-IST, Genoa 16132, Italy E-mail: [email protected] REFERENCES 1. Rochet NM, Chavan RN, Cappel MA, Wada DA, Gibson LE. Sweet syndrome: Clinical presentation, associations, and response to treatment in 77 patients. J Am Acad Dermatol 2013;69:557-64. 2. Hisanaga K, Iwasaki Y, Itoyama Y. Neuro-Sweet Disease Study Group. Neuro-Sweet disease: clinical manifestations and criteria for diagnosis. Neurology 2005;64:1756-61. 3. Maxwell G, Archibald N, Turnbull D. Neuro-Sweet’s disease. Pract Neurol 2012;12:126-30.

4. Makimoto G, Manabe Y, Yamakawa C, Fujii D, Ikeda-Sakai Y, Narai H, et al. Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation. Neurol Int 2012;4:e5. 5. Erbetta A, Salmaggi A, Sghirlanzoni A, Silvani A, Potepan P, Botturi A, et al. Clinical and radiological features of brain neurotoxicity caused by antitumor and immunosuppressant treatments. Neurol Sci 2008;29:131-7. http://dx.doi.org/10.1016/j.jaad.2013.10.067

Let’s put an expiration date on the current approach to drug expiration dates To the Editor: The dermatoethics case scenario addressing the inadvertent dispensing of an expired topical medication by Drs Wang and Keller eloquently met its intent of reminding us to be accountable for our mistakes and to pursue patient-physician healing.1 However, among potential problems associated with such an error, their statement that ‘‘possible consequences include an adverse reaction’’ merits further discussion. Clinicians can take a degree of comfort in the knowledge that there are no reports in the literature of ingestion, injection, or topical application of current drug formulations used beyond their expiration date that have resulted in human toxicity.2 A half century ago, renal tubular damage was reported from a degraded tetracycline formulation that is no longer on the market.3 While loss of effectiveness is a valid concern, 90% of potency is retained for at least 5 years (sometimes much longer) after the label’s expiration date listed on many unopened agents that

Recurrent episodes of neuro-Sweet syndrome in a Caucasian patient.

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