JRSM >
Volume 96, Number 3 >
Pp. 127-129
Department of Medicine, Aga Khan University, Stadium Road, PO Box 3500,
Karachi 74800, Pakistan
1 Department of Pathology, Aga Khan University, Stadium Road, PO Box 3500,
Karachi 74800, Pakistan
Correspondence to: Dr Ikram A Burney E-mail: ikram.burney{at}aku.edu
Pathergy is the development of a papulopustular lesion around a puncture site on the skin, 24-48 hours after the injection of a sterile substance1. The pathergy test is used in some parts of the world as a diagnostic criterion for Behçet's disease2.
CASE HISTORY
A man aged 24 reported low-grade fever for eight months, pain and swelling in the right leg and knee for about six months and pustular lesions over the lips, arms and genitalia for the past three months. During this period he had been in Saudi Arabia and had been admitted three times for the treatment of fever and abscesses. Culture of aspirates from the right knee joint had been negative; 40% of the white cells were lymphocytes, 60% neutrophils; protein 53 g/L. On admission to our hospital he was febrile and tachycardic. There was no palpable lymphadenopathy. Over the left forearm and right elbow there were raised ulcers with undermining edges, exuding yellowish discharge; there were also multiple lesions on the lips and genitalia. Haemoglobin was 5.9 g/dL, white cell count 7.2 x 109/L (72% neutrophils, 20% lymphocytes, 2% eosinophils, 7% monocytes) and platelet count 43 x 109/L. Renal function was within normal limits, but there was evidence of liver dysfunction in a serum albumin of 1.9 g/dL and a prothrombin time of 19 s (control 12 s); bilirubin, alanine aminotransferase and alkaline phosphatase were normal.
The patient was treated with cloxacillin, amikacin, ceftazidime and metronidazole but did not respond. During the first 48 hours new eruptions developed at the site of insertion of intravenous cannulae and venepunctures. Figure 1 shows the lesion over the forearm. The clinical diagnosis was Behçet's disease. Antinuclear factor, anti-smooth-muscle antibody and antimitochrondrial antibody were all negative, as was a test for human immunodeficiency virus infection. A bone marrow aspirate yielded only hypocellular marrow, but a bone trephine biopsy revealed diffuse fibrosis and stained positively with the reticulin stain. Also, there was an abnormal population of heterogeneous mononuclear cells. A lesion similar to the other lesion developed around the site of insertion of the Jamshidi needle. The presumptive diagnosis was then myelofibrosis with secondary pyoderma gangrenosum.
|
Figure 2a shows the
histopathology of the skin biopsy. A diffuse dense lymphocytic infiltrate is
visible at the dermoepithelial junction and in the periadnexal and
perivascular area. There were atypical lymphoid cells, which were focally
infiltrating into the epidermis. The nuclei were pleomorphic and
hyperchromatic. The neoplastic cells were also infiltrating the vessel wall.
Both the skin lesion and bone biopsy were positive for leucocyte common
antigen (LCA) and T cell surface markers (i.e. UCHLA1 and CD3) but were
negative for CD20, a B-cell marker (Figure
2b). Monoclonality at the DNA level in the T cell
population was confirmed by T cell receptor 
chain gene rearrangement
assay. DNA was extracted from a formalin-fixed paraffin-embedded skin biopsy
specimen by use of a Nucleon genomic DNA extraction kit (Nucleon Biosciences,
USA). The polymerase chain reaction was performed as described by McCarthy
et al.3 on
an automated thermal cycler (Perkin Elmer, Model 9600, USA).
|
The patient was diagnosed as having a non-Hodgkin lymphoma stage IV and was started on combination chemotherapy. He went into partial clinical remission after a month and was then referred to a centre near his home.
COMMENT
A positive pathergy test is one of the diagnostic criteria for Behçet's disease2. The initial reaction to sterile puncture is a rapid accumulation of neutrophils along the needle track4. The neutrophils and the vascular endothelium are abnormally sticky5. The endothelial cells express ICAM-1 and E-selectin1. Rapid accumulation of neutrophils is followed by a sharp decline in their numbers, such that after 48 h they constitute only 5% of the infiltrating cells1,4. Hyperchemotaxis attracts a dense mononuclear cell infiltrate consisting predominantly of monocytes, macrophages, and CD4+ lymphocytes1. As well as in Behçet's disease, pathergy has been described in pyoderma gangrenosum, in hairy cell leukaemia and in chronic myeloid leukaemia treated with interferon alpha6,7. The lesions in our patient were characteristic of pathergy, but the final diagnosis was T-cell non-Hodgkin lymphoma. To our knowledge, this is the first such case presenting with a pathergy reaction.
REFERENCES
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  M. Melikoglu, S. Uysal, J. G. Krueger, G. Kaplan, F. Gogus, H. Yazici, and S. Oliver Characterization of the Divergent Wound-Healing Responses Occurring in the Pathergy Reaction and Normal Healthy Volunteers J. Immunol., November 1, 2006; 177(9): 6415 - 6421. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
