Mycosis Fungoides

Abstract: 65 year-old man with 14 year history of mycosis fungoides (MF).

HPI:  This 65 yo man has had a dermatitis around his hips since childhood.  In 1999, a biopsy was done that was reported as "very suggestive of MF."  He was treated with PUVA in 1999  and since then with topical corticosteroids (initially clobetasol and more recently fluocinonide). His disease is either quiescent or has not progressed since diagnosis.

O/E:  There are patches and plaques on the left and to a lesser extent right hips.  Some atrophy and "cigarette paper changes" are present on the left hip.  There is no to minimal involvement elsewhere.  He has no lymphadenopathy.

Clinical Photos:

Pathology:  Photomicrographs courtesy of Deon Wolpowitz, M.D., Boston University SkinPath

Focal parakeratosis, flattened epidermis, lymphocytic exocytosis and "haloed lymphocytes" lining up along the  dermal epidermal junction and forming small intraepidermal aggregates, and intermittent moderate band-like lymphohistiocytic infiltrate.  These changes are suggestive of CTCL, MF type.

Diagnosis:  CTCL, MF type. Patient has had a rash in the area since childhood.  Since the pathological diagnosis was first made in 1999 his disease has not progressed much and is still probably Stage IA.

Questions:  Do you think this is early M.F.?  Could the poikilodermatous change be due to proponged use of topical corticosteroids? Will treatment make a difference?  He has read about new treatments.  We discussed imiquimod. Does anyone have experience with that?  How would you approach this patient?

References:

1.  [This valuable reference was kindly suggested by Barry Ladizinski, M,D.]  Defining early mycosis fungoides.
Pimpinelli N, Wood GS; et. al. International Society for Cutaneous Lymphoma.
J Am Acad Dermatol. 2005 Dec;53(6):1053-63. Review.
Abstract: This editorial review summarizes the results of 5 meetings sponsored by the International Society for Cutaneous Lymphoma at which the clinicopathologic and ancillary features of early mycosis fungoides were critically examined. Based on this analysis, an algorithm was developed for the diagnosis of early mycosis fungoides involving a holistic integration of clinical, histopathologic, immunopathologic, and molecular biological characteristics. A novel aspect of this algorithm is that it relies on multiple types of criteria rather than just one, for example, histopathology. Before its finalization, the proposed diagnostic algorithm will require validation and possibly further refinement at multiple centers during the next several years. It is anticipated that a more standardized approach to the diagnosis of early mycosis fungoides will have a beneficial impact on the epidemiology, prognostication, treatment, and analysis of clinical trials pertaining to this most common type of cutaneous lymphoma. 

2.  Treatment of cutaneous T cell lymphoma: current status and future directions.  Apisarnthanarax N, Talpur R, Duvic M.  Am J Clin Dermatol. 2002;3(3):193-215. Abstract: The treatment of cutaneous T cell lymphoma (CTCL), which includes mycosis fungoides and Sezary syndrome, has been in a state of continual change over recent decades, as new therapies are constantly emerging in the search for more effective treatments for the disease...  Read full abstract to give an idea of how complicated this is.

3. Imiquimod in mycosis fungoides. Free Open Access

Martínez-González MC, et. al.  Eur J Dermatol. 2008;18(2):148-52.

Abstract:  Imiquimod is a topically active imidazoquinoline immunomodulator agent. It works as an indirect antiviral and antitumoral and stimulates the production of INF-alpha and various other cytokines. We assayed topical imiquimod in treating early stages of mycosis fungoides. We applied imiquimod 5% cream in four patients with multi-treatment resistant plaques of MF (stages IA and IIB). We applied it on one patient in association with systemic INFalpha-2a. We observed a complete clinical clearance of the lesions in all four patients. In three cases we achieved a complete histopathological clearance and in one case a partial histopathological clearance. The patient treated with imiquimod and systemic INFalpha-2a showed the most spectacular improvement with a rapid total response. We ascribe this improvement to a synergic effect of imiquimod and systemic INFalpha-2a treatment. Before the introduction of imiquimod, this patient had been treated for 2 years with systemic INFalpha-2a alone, without any evidence of clinical response. Imiquimod could be an effective therapy for early-stage disease of CTCL, used alone or in combination with systemic immunomodulatory therapy.