CORRECT DIAGNOSIS:
Mycosis Fungoides – Poikiloderma Atrophicans Vasculare
DISCUSSION:
Mycosis Fungoides is the most common form of Cutaneous T-cell Lymphoma. We present a patient with an uncommon variant. Poikiloderma vasculare atrophicans is a reaction pattern characterized by mottled pigmentation, atrophy, and telangiectasia. The skin has been described as having a “cigarette paper” like quality. Poikilodermatous changes have been seen with other forms of cutaneous t-cell lymphoma, connective tissue disease, and certain genodermatoses. However, poikilodermatous findings on non-sun exposed areas should be considered MF until proven otherwise. Though historically considered either a separate entity or a premalignant condition, poikiloderma vasculare atrophicans is now recognized as a clinical variant of patch stage MF.
Histologically, poikilodermatous patches may show epidermal atrophy with infiltration of atypical lymphocytes. Dilated dermal vessels with a perivascular lymphocytic infiltrate may also be noted. Epidermotropism may be seen with or without pautrier microabscesses. Immunohistochemical findings will help differentiate poikilodermatous findings associated with MF from other diagnoses. Our patient’s biopsy is more reflective of the classic mycosis histology as it was taken from a more typical plaque.
TREATMENT:
Treatment of MF in the early stages focuses on skin-directed therapies. Based on the clinical exam, our patient was staged at IB(T2N0M0). Reasonable therapy at this stage includes the use of topical steroids, UVB, PUVA, nitrogen mustard, bexarotene gel, or 1,3-bis(2-chloroethyl)-l-nitrosourea(carmustine). Our patient was started on combination therapy with high potency topical steroids and narrowband UVB. To date, he is less symptomatic and has some regression of his plaques. If fails to respond adequately, other therapies as above may be considered. However, the patient’s financial constraints will likely preclude the use of other more expensive and less readily available treatments.
REFERENCES:
James, W., Berger, T., & Elston, D. (2006). Andrews’ diseases of the skin: Clinical dermatology (10th ed.). Canada: Saunders Elsevier.
Kreuter, A., Hoffmann, K., & Altmeyer, P. (2005). A case of poikiloderma vasculare atrophicans, a rare variant of cutaneous T-cell lymphoma, responding to extracorporeal photopheresis. Journal of the American Academy of Dermatology, 52(4), 706-708. https://doi.org/10.1016/j.jaad.2004.11.010 [PMID: 15763693]
Pimpinelli, N., & et al. (2005). Defining early mycosis fungoides. Journal of the American Academy of Dermatology, 53(6), 1053-1063. https://doi.org/10.1016/j.jaad.2005.06.031 [PMID: 16388246]
Elder, D. E., & et al. (2005). Lever’s histopathology of the skin (10th ed.). Philadelphia: Lippincott Williams & Wilkins.
