PMID- 22123268
OWN - NLM
STAT- MEDLINE
DCOM- 20120720
LR  - 20120327
IS  - 1533-0311 (Electronic)
IS  - 0193-1091 (Linking)
VI  - 34
IP  - 2
DP  - 2012 Apr
TI  - Treated Whipple disease with erythema nodosum leprosum-like lesions: cutaneous 
      PAS-positive macrophages slowly decrease with time and are associated with 
      lymphangiectases: a case report.
PG  - 182-7
LID - 10.1097/DAD.0b013e318221ba55 [doi]
AB  - Pathologically, Whipple disease (WD) is characterized by the accumulation of 
      myriad macrophages parasitized by Tropheryma whipplei (TW) bacilli denoted by 
      periodic acid-Schiff (PAS) positivity. These PAS+ macrophages are typically found 
      in the duodenum associated with lymphangiectasia. Recently, we reported the 
      presence of PAS+ macrophages and free TW in erythema nodosum leprosum (ENL)-like 
      lesions and normal skin in a patient with WD who suffered from the immune 
      reconstitution inflammatory syndrome (IRIS). We extend that report by describing 
      the clinical and pathologic findings over 5 years of follow-up. First, the IRIS 
      gradually diminished and abated over 18-month time. Second, at no point did WD 
      recur, and all duodenal and skin biopsies tested by polymerase chain reaction 
      were negative for TW DNA. Third, PAS+ macrophages were identified in 26 of 27 
      skin biopsies (96%) and decreased along with free TW over time. Fourth, ENL-like 
      lesions had significantly greater numbers of PAS+ macrophages than normal skin. 
      Moreover, normal abdominal skin (region of ENL-like lesions) had greater PAS+ 
      counts than arm skin (not a site of IRIS). Last, lymphangiectases, a histologic 
      sign of lymphostasis, was found in all skin biopsies. Overall, these findings 
      implicate bacillary burden as a factor in the immune tolerance to live TW in 
      active WD and the initiation of ENL-like nodules against dead/nonreplicative TW 
      in treated WD. In addition, poor lymphatic drainage is likely responsible for the 
      gradual clearance of TW from the skin and the impaired delayed-type 
      hypersensitivity reaction (absence of activated macrophages) against TW found in 
      WD, presumptively due to reduced/absent immune cell trafficking necessary for 
      lymphocyte-macrophage interactions and induction of adaptive immunity.
FAU - Paul, Joan
AU  - Paul J
AD  - Divisions of Dermatopathology and Dermatology, Department of Pathology, Albany 
      Medical College, Albany, NY, USA.
FAU - Schaller, Jorg
AU  - Schaller J
FAU - Rohwedder, Angela
AU  - Rohwedder A
FAU - Carlson, J Andrew
AU  - Carlson JA
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Am J Dermatopathol
JT  - The American Journal of dermatopathology
JID - 7911005
SB  - IM
MH  - Adult
MH  - Erythema Nodosum/immunology/microbiology/*pathology
MH  - Follow-Up Studies
MH  - Humans
MH  - Immune Reconstitution Inflammatory Syndrome/microbiology/pathology
MH  - Lymphangiectasis/microbiology/*pathology
MH  - Macrophages/immunology/pathology
MH  - Male
MH  - Whipple Disease/immunology/microbiology/*pathology
EDAT- 2011/11/30 06:00
MHDA- 2012/07/21 06:00
CRDT- 2011/11/30 06:00
PHST- 2011/11/30 06:00 [entrez]
PHST- 2011/11/30 06:00 [pubmed]
PHST- 2012/07/21 06:00 [medline]
AID - 10.1097/DAD.0b013e318221ba55 [doi]
PST - ppublish
SO  - Am J Dermatopathol. 2012 Apr;34(2):182-7. doi: 10.1097/DAD.0b013e318221ba55.