Annals of Oncology Advance Access originally published online on June 4, 2008
Annals of Oncology 2008 19(7):1360-1361; doi:10.1093/annonc/mdn387
letters to the editor |
Coincidential successful treatment of Jessner–Kanof disease with chemotherapy
A 43-year-old woman was referred to our clinic with the diagnosis of left-sided breast cancer in 1999. Her past medical history was significant for papular skin rash of the face, chest wall, forearms and lower legs of 7 years duration. A previous biopsy had showed lymphocytic infiltration compatible with Jessner–Kanof disease (JKD). Of note is that the patient had been treated for JKD with steroids and thalidomide twice in the past but after complete regression she suffered recurrence both times.After the diagnosis of breast cancer, the patient had undergone a left modified radical mastectomy with ipsilateral axillary lymph node dissection. Pathology revealed a grade3 pT3,pN1 infiltrating ductal carcinoma of the breast. Staging evaluation was negative for metastatic disease. She then received six cycles of adjuvant chemotherapy with the combination 5-fluorouracil, epirubicin, cyclophosphamide which she tolerated well. Since the first cycle of chemotherapy, the patient noticed a slight regression of the papular rash; after the second cycle, the rash was almost fading and it completely disappeared after cycle 3. Now, 9 years after her treatment with chemotherapy, the patient remains not only breast cancer free but also without signs of JKD recurrence.
JKD is a disorder characterised clinically by recurrent asymptomatic papules and plaques and histopathologically by lymphocytic infiltration of the skin. It is mainly a disease of male adults, is less common in females and is extremely rare in children.
The aetiology of the disease is unknown. Borrelia Burgdorferi infection has been reported to be associated with JKD [1], as well as with other cutaneous conditions. Medications have also been reported to cause skin rash resembling JKD. In a case report, the drug glatiramer acetate (GLAT) caused a biopsy-proven lymphocytic infiltration (T-cell pseudolumphoma). GLAT is an immunomodulatory drug for the treatment of multiple sclerosis [2]. An eruption clinically resembling JKD associated with angiotensin converting enzyme (ACE) inhibitors [3] has been reported as well.
The most common site affected by the disease is the head and neck region but the trunk and the limbs may also be involved. Some investigators suggest that involvement of a region not including the head and neck area represents a different clinical entity and should be called palpable migratory acriform erythema [4].
The diagnosis is established by biopsy which shows lymphocytic infiltration of the skin. Flow cytometry analysis reveals the polyclonality of this mixed T-cell population where T8 lymphocytes predominate. Differential diagnosis includes the indolent mucosa-associated lymphoid tissue (MALT) lymphomas; of interest is the fact that some cases of JKD and gastric MALT lymphomas have been associated with infectious agents, Borellia burgdorferi and Helicobacter pylori, respectively.
The natural history of the disease follows a waxing and waning course and may last for many years but does not evolve into a more aggressive form.
Current treatment options include topical or systemic corticosteroids and thalidomide. Our patient had been treated for JKD in the past but she only achieved a complete and sustained regression after chemotherapy was offered for another reason (her breast cancer). The chemotherapy regimen included three drugs, two of them being drugs of choice for the treatment of lymphomas. Epirubicin, an anthracycline, is included in combination regimens for the treatment of aggressive lymphomas while cyclophosphamide, an alkylating agent, is active in indolent (and aggressive) lymphomas, many times given as single agent. It is also used as immunosuppressant in autoimmune disorders and after bone marrow and solid organ transplantation. Recently, its suppressive action on CD4+CD25+ regulatory T cells has been elucidated [5].
What causes JKD essentially remains unknown, but it seems that a dysregulation of the immune system may play a role, probably an intermediate, between the true cause and the manifestation of the disorder. The association with B. burgdorferi infection and the drug GLAT which is an immunomodulator supports this hypothesis.
Considering all the above and having a great experience in the use of cyclophosphamide, we suggest that clinicians treating patients with JKD could consider this drug for the treatment of refractory or relapsing disease. The toxicity of cyclophosphamide when given in low doses and for short periods of time is minimal. A short course may be an appropriate option for patients who do not respond or relapse after thalidomide therapy.
1 First Department of Medical Oncology, St Savvas Anticancer Hospital, Athens, Greece
2 Department of Oncology, Nicosia General Hospital, Nicosia, Cyprus
3 Private practice, Athens, Greece
* (E-mail: georgeorphanosvp{at}hotmail.com)
References
1. Bagot M, Revuz J. Jessner-Kanof lesion and Borellia infection. J Am Acad Dermatol (1990) 23(4 Pt1):772–773.[Web of Science][Medline]
2. Noden S, Casper C, Kuhn A, Petereit HF. Jessner-Kanof lymphocytic infiltration of the skin associated with glatiramer acetate. Mult Scler (2005) 11(2):245–248.
3. Schepis C, Lentini M, Siragusa M, Batolo D. ACE inhibitor-induced eruption resembling lymphocytic infiltration (of Jessner-Kanof) and lupus erythematosus tumidus. Dermatology (2004) 208(4):354–355.[CrossRef][Web of Science][Medline]
4. Abeck D, Ollert MW, Eckert F, et al. Palpable migratory acriform erythema. Clinical morphology, histopathology, immunohistochemistry and response to treatment. Arch Dermatol (1997) 133(6):763–766.
5. Ghiringhelli F, Menarg C, Puig PE, et al. Metronomic cyclophosphamide regimen selectively depletes CD4+CD25+ regulatory T cells, restores T, NK effector functions in end stage cancer patients. Cancer Immunol Immunother (2007) 56(5):641–648.[CrossRef][Web of Science][Medline]
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