Stage IIB Mycosis Fungoides

摘要

Seema Daulat is from Dallas, Texas and completed her undergraduate studies in Finance and Spanish at the University of Texas at Austin. She then obtained her MD from University of Texas Southwestern Medical School in Dallas. She completed a preliminary internship in Internal Medicine at the University of Washington in Seattle. She is currently a dermatology clinical research fellow studying cutaneous T-cell lymphoma under Madeleine Duvic at MD Anderson Cancer Center in Houston, Texas. Introduction Mycosis Fungoides (MF), the most common variant of cutaneous T-cell lymphoma (CTCL), has a wide spectrum of disease as evidenced by the staging system. In early disease, normal life expectancy and even complete remission are prevalent. However, in later stage disease, success in therapy is often difficult to achieve. Typically, MF manifests as patches, plaques, nodules, and tumors. However, there are some cases with clinically and histologically distinct patterns including ichthyotic MF, adnexotropic (including syringotropic and folliculotropic) MF, and granulomatous MF. We present a patient with MF stage IIB folliculotropic syringotropic and granulomatous MF with patches of alopecia who failed multiple treatment modalities, yet achieved complete remission with combined modality therapy. Initial Presentation A 59-year-old Caucasian man presented in 2005 with a ten-year history of pink plaques that started on his right forehead. He was treated with topical steroids with only minor improvement. Biopsy was reportedly “inconclusive.” The patient continued to develop similar red patches and plaques on the face and scalp with patches of scarring alopecia. Five years after onset, he was seen at a university hospital and given the diagnosis of discoid lupus. For the next five years, he was treated with various modalities for discoid lupus including sun avoidance, prednisone, and hydroxychloroquine without benefit. He also received methotrexate, topical steroids, and intralesional triamcinolone.When the patient was seen at another university hospital in 12/2004, two biopsies were consistent with patch or early plaque MF with an atypical lymphoid infiltrate and prominent epidermotropism. He tried various treatment modalities, including a regimen of psoralen plus ultraviolet-A (PUVA) thrice weekly and topical bexarotene to spot-treat lesions, followed by a regimen comprised of nitrogen mustard (topical mechlorethamine), topical steroids, and low-dose methotrexate.He presented to our clinic to discuss other treatment options. Physical Examination and Workup He had 6/10 pruritus. Atrophic patches of hair loss and several ulcerated tumors (see Figure 1) were present on his scalp. Large erythematous plaques were present on the face. A large atrophic plaque was present on the right cheek. A large hyperpigmented patch was present at the base of the neck. Scattered on the back were smaller 0.5 to 1.5 cm annular plaques as well as several larger plaques and ulcerated tumors. The largest lesion was a 5.5 x 3.5 cm annular red plaque on the central upper abdomen surrounded by scattered annular plaques measuring 1-2 cm. In the inguinal area was a patch of alopecia in the pubic region that measuring 4 cm. On the extremities were scattered 1-2 cm pink plaques and atrophic pink patches. The feet and hands were completely spared. The patient's body surface area was 21.7%, including 7.4% patches, 12.8% plaques and 1.5% tumors.