PMID- 20411809
OWN - NLM
STAT- MEDLINE
DCOM- 20100601
LR  - 20190917
IS  - 0009-918X (Print)
IS  - 0009-918X (Linking)
VI  - 50
IP  - 4
DP  - 2010 Apr
TI  - [Successful treatment of a case of steroid-dependent neuro-Sweet disease with 
      dapsone].
PG  - 257-61
AB  - A 35-year-old Japanese man was admitted to our hospital with recurrent 
      meningoencephalitis of unknown etiology. He presented with fever, convulsions and 
      loss of consciousness, which started at age 33. We diagnosed him with neuro-Sweet 
      disease (NSD) based on human leukocyte antigen (HLA) B-54/Cwl positivity and 
      neutrophilic infiltration into the dermis in a biopsied skin plaque. Intravenous 
      methylprednisolone and oral prednisolone markedly improved his fever and CSF 
      pleocytosis. Five years later he was again admitted to our hospital with high 
      fever, oral aphthae and dull-red edematous plaques on the face and body. He was 
      conscious, but he had neck stiffness, mild hyperreflexia in all limbs and an 
      extensor plantar response. Laboratory tests revealed increased white blood cell, 
      erythrocyte sedimentation rate (ESR) and C-reactive protein level. CSF analysis 
      indicated mild pleocytosis. A skin biopsy from an edematous plaque revealed 
      neurotrophils infiltrating the upper dermis. We treated him with intravenous 
      methylprednisolone (1 g/day) for 3 days, followed by oral prednisolone (50 
      mg/day). His symptoms improved remarkably; however, he had recurrence of 
      symptoms, such as fever, meningial irritation and oral aphtae, with attempted 
      taper of prednisolone. We started treatment with dapsone (75 mg/day) in addition 
      to prednisolone, and could taper oral prednisolone, without a relapse. However, 
      because some mildly recurred with the tapering of dapson, we maintained dapsone 
      treatment at 75 mg daily, added colchicine (1 mg/ day) and tapered only 
      prednisolone. His symptoms were improved and no relapse has been observed. NSD is 
      characterized by neurotrophic hyperactivation and infiltration of tissues. It is 
      highly responsive to systemic corticosteroid therapy; however, some cases show 
      frequent recurrences on tapering of corticosteroids. Dapsone is considered to 
      prevent neurotrophic overactivity. In this case, dapsone was supposed to be 
      effective to prevent reccurence of NSD upon tappering corticosteroids. Dapsone 
      should be a therapeutic options for steroid-dependent NSD showing frequent 
      recurrence.
FAU - Shibata, Ken-Ichi
AU  - Shibata K
AD  - Department of Neurology, Neurological Institute, Graduate School of Medical 
      Sciences, Kyushu University.
FAU - Tateishi, Takahisa
AU  - Tateishi T
FAU - Yamasaki, Ryo
AU  - Yamasaki R
FAU - Ohyagi, Yasumasa
AU  - Ohyagi Y
FAU - Kira, Jun-Ichi
AU  - Kira J
LA  - jpn
PT  - Case Reports
PT  - English Abstract
PT  - Journal Article
PL  - Japan
TA  - Rinsho Shinkeigaku
JT  - Rinsho shinkeigaku = Clinical neurology
JID - 0417466
RN  - 8W5C518302 (Dapsone)
RN  - 9PHQ9Y1OLM (Prednisolone)
RN  - SML2Y3J35T (Colchicine)
RN  - X4W7ZR7023 (Methylprednisolone)
SB  - IM
MH  - Adult
MH  - Colchicine/administration & dosage
MH  - Dapsone/*administration & dosage
MH  - Dermis/pathology
MH  - Drug Therapy, Combination
MH  - Humans
MH  - Male
MH  - Methylprednisolone/administration & dosage
MH  - Neutrophils/pathology
MH  - Prednisolone/administration & dosage
MH  - Pulse Therapy, Drug
MH  - Secondary Prevention
MH  - Sweet Syndrome/diagnosis/*drug therapy/pathology
MH  - Treatment Outcome
EDAT- 2010/04/24 06:00
MHDA- 2010/06/02 06:00
CRDT- 2010/04/24 06:00
PHST- 2010/04/24 06:00 [entrez]
PHST- 2010/04/24 06:00 [pubmed]
PHST- 2010/06/02 06:00 [medline]
AID - 10.5692/clinicalneurol.50.257 [doi]
PST - ppublish
SO  - Rinsho Shinkeigaku. 2010 Apr;50(4):257-61. doi: 10.5692/clinicalneurol.50.257.