PMID- 23858422
OWN - NLM
STAT- MEDLINE
DCOM- 20131017
LR  - 20131121
IS  - 1098-4275 (Electronic)
IS  - 0031-4005 (Linking)
VI  - 132
IP  - 2
DP  - 2013 Aug
TI  - Clonal cytophagic histiocytic panniculitis in children may be cured by 
      cyclosporine A.
PG  - e545-9
LID - 10.1542/peds.2012-3256 [doi]
AB  - Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis in childhood, 
      associated either with nonmalignant conditions or with subcutaneous 
      panniculitis-like T-cell lymphoma (SPTCL), and often also associated with 
      macrophage activation syndrome (MAS). Discriminating between these 2 conditions 
      is therapeutically important because nonmalignant CHP often improves under 
      cyclosporine and prednisone, whereas most cases of SPLT may be best treated with 
      more aggressive therapy. We report the cases of a 6-month-old boy and a 
      16-month-old girl who, after viral infection, developed multiple infiltrating 
      skin nodules on the limbs and face, associated with MAS. Histopathologic findings 
      for skin biopsy specimens revealed CHP associated with heavily cellular lobular 
      panniculitis. Hemophagocytosis and immunohistochemical staining features were 
      consistent with typical characteristics of in situ MAS in adipose tissue: the 
      lymphocytes were mostly TCD8+ cells with an activated phenotype (human leukocyte 
      antigen (HLA) -DR+) and expressed interferon-gamma; CD68+ macrophages expressed tumor 
      necrosis factor-alpha and interleukin-6. A monoclonal rearrangement of the T-cell 
      receptor gamma gene was present in skin tissue but not in peripheral blood or bone 
      marrow lymphocytes. Cyclosporine A treatment resulted in the complete remission 
      of cutaneous and systemic manifestations in both patients for 66 and 29 months, 
      respectively. This report suggests that the diagnosis of a reactive T-cell 
      lymphoproliferation should be the treatment of choice in young children with 
      severe CHP, even if there is a SPTCL-like aspect with an in situ T-cell 
      clonality. It also suggests that CSA is the optimal treatment of this condition 
      and postulates the possible pathologic process underlying this efficacy.
FAU - Bader-Meunier, Brigitte
AU  - Bader-Meunier B
AD  - Necker Hospital, Assistance Publique-Hopitaux de Paris, France. 
      brigitte.bader-meunier@nck.aphp.fr
FAU - Fraitag, Sylvie
AU  - Fraitag S
FAU - Janssen, Carl
AU  - Janssen C
FAU - Brochard, Karine
AU  - Brochard K
FAU - Lamant, Laurence
AU  - Lamant L
FAU - Wouters, Carine
AU  - Wouters C
FAU - Bodemer, Christine
AU  - Bodemer C
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20130715
PL  - United States
TA  - Pediatrics
JT  - Pediatrics
JID - 0376422
RN  - 0 (Immunosuppressive Agents)
RN  - 83HN0GTJ6D (Cyclosporine)
SB  - IM
MH  - Biopsy, Needle
MH  - Bone Marrow/pathology
MH  - Chickenpox/complications/immunology
MH  - Cyclosporine/adverse effects/*therapeutic use
MH  - Diagnosis, Differential
MH  - Female
MH  - Histiocytosis/diagnosis/*drug therapy/immunology/pathology
MH  - Humans
MH  - Immunosuppressive Agents/adverse effects/*therapeutic use
MH  - Infant
MH  - Lymphohistiocytosis, Hemophagocytic/diagnosis/*drug therapy/immunology/pathology
MH  - Lymphoproliferative Disorders/diagnosis/drug therapy/immunology/pathology
MH  - Macrophage Activation Syndrome/diagnosis/drug therapy/immunology/pathology
MH  - Male
MH  - Panniculitis/diagnosis/*drug therapy
MH  - Respiratory Tract Infections/complications/immunology
MH  - T-Lymphocytes/drug effects/immunology/pathology
OTO - NOTNLM
OT  - children
OT  - clonality
OT  - cytophagic histiocytic panniculitis
OT  - macrophage activation syndrome
EDAT- 2013/07/17 06:00
MHDA- 2013/10/18 06:00
CRDT- 2013/07/17 06:00
PHST- 2013/07/17 06:00 [entrez]
PHST- 2013/07/17 06:00 [pubmed]
PHST- 2013/10/18 06:00 [medline]
AID - peds.2012-3256 [pii]
AID - 10.1542/peds.2012-3256 [doi]
PST - ppublish
SO  - Pediatrics. 2013 Aug;132(2):e545-9. doi: 10.1542/peds.2012-3256. Epub 2013 Jul 
      15.