Dermatology Online Journal
Poikilodermatous mycosis fungoides
- Author(s): Farley-Loftus, Rachel
- Mandal, Rajni
- Latkowski, Jo-Ann
- et al.
Poikilodermatous mycosis fungoidesDepartment of Dermatology, New York University, New York, New York
Rachel Farley-Loftus MD, Rajni Mandal MD, Jo-Ann Latkowski MD
Dermatology Online Journal 16 (11): 8
Poikilodermatous mycosis fungoides is a rare form of cutaneous T-cell lymphoma that is characterized clinically by localized or diffuse patches, which consist of telangiectases, mottled hyper- and hypopigmentation, and atrophy. The immunophenotype of neoplastic cells is similar to that observed in classic mycosis fungoides. Therapeutic options used in poikilodermatous and classic mycosis fungoides include both skin-directed and systemic treatments. We present a case of poikilodermatous mycosis fungoides in a 53-year-old woman, who initially presented with erythroderma and who has failed multiple treatment modalities.
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A 53-year-old woman presented to the Dermatology Clinic at Bellevue Hospital Center in September, 1998, for evaluation of a chronic, generalized, erythematous and pruritic eruption of seven years duration. Several skin biopsy specimens performed at that time and until 2001 were non-diagnostic. Clinical and histopathologic diagnoses entertained during this period included lupus erythematosus, dermatomyositis, chronic actinic dermatitis, dermatophytosis, atopic dermatitis, and mycosis fungoides. Additional skin biopsies taken from the trunk and the extremities in 2001 disclosed the diagnosis for which this patient has been followed to date.
Previous treatments included narrow-band ultraviolet B phototherapy and oral bexarotene monotherapy; these treatments were tolerated poorly. From 2001 until 2005, the patient was maintained on acitretin 50 mg daily in combination with interferon-α six million units subcutaneously three to four times weekly, with stabilization of her disease. Acitretin was interrupted in June, 2005, due to persistent elevation of liver enzymes and was started again in December at a lower dose of 25 mg daily. In July, 2007, acitretin was stopped, and bexarotene 375 mg daily was initiated in combination with interferon-α, with subsequent clinical improvement. This regimen was discontinued in April, 2009, due to lack of a sustained response, and vorinostat 400 mg daily was started by Hematology/Oncology. She discontinued therapy after two months due to increased xerosis, alopecia, and irritation of her skin.
Past medical history included diabetes mellitus type II, anemia, depression, and hypothyroidism. Current medications include hydroxyzine hydrochloride, fenofibrate, levothyroxine, omega-3-acid ethyl esters, calcium, vitamin D, ferrous sulfate, niacin, solifenacin succinate, and sertraline. Current topical preparations include triamcionlone acetonide 0.1 percent ointment twice daily several days each week to the trunk and extremities, clobetasol propionate 0.05 percent ointment twice daily to the hands, and various topical emollients.
Generalized, erythematous and poikilodermatous patches and thin plaques with fine scale involved >80 percent of the body surface area and included the upper and lower extremities, trunk, and flexural surfaces. Diffuse, nonscarring alopecia with preservation of follicular ostia was noted on the scalp.
Multiple, 1-cm, mobile, nontender axillary and inguinal lymph nodes were palpable bilaterally.
The white-cell count was 3.9 x 109/L, hemoglobin 9.2 g/dL, mean corpuscular volume 76.1 fL, and platelets 216x109/L. A comprehensive metabolic panel was normal. The lactate dehydrogenase was 155 U/L in July, 2007. Flow cytometry analysis of peripheral blood, which was performed in September, 2005, and again in March and November, 2008, showed no immunophenotypic evidence of lymphoma or leukemia, with normal expression of B- and T-cell antigens and a normal CD4/CD8 ratio.Computed tomography scans of the chest, abdomen, and pelvis performed in November, 2008, showed stable, prominent, bilateral axillary, chest wall, and inguinal lymph nodes.
There is a superficial, perivascular, lymphocytic infiltrate. Some lymphocytes extend into the overlying epidermis where there is little associated spongiosis. There is mild, papillary dermal fibroplasia. Immunoperoxidase studies show that most of the epidermal lymphocytes are reactive for CD4, with rare epidermal lymphocytes reactive for CD8.
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. Neoplastic cells that result from the clonal expansion of CD4+ memory T-cells demonstrate a predilection for skin. Classic MF is characterized by erythematous, atrophic, and wrinkled patches, with fine scale on non-sun-exposed skin. Plaques may arise de novo or from the progression of patch-stage disease. Further progression may result in nodules and tumors, which often ulcerate. Clinical variants of MF are less common and include granulomatous slack skin, pagetoid reticulosis, hypopigmented MF, poikilodermatous MF, and folliculotropic MF [1, 2].
Poikilodermatous MF is a rare clinicopathologic variant, in which atrophy, telangiecatases, and reticulate or mottled hyper- and hypopigmentation are present in lesional skin [1, 3]. Originally termed poikiloderma atrophicans vasculare, it initially was considered to be a pre-mycotic eruption that would eventually progress to mycosis fungoides ; however, the current view is that this form represents a clinical variant and not a precursor to MF . Poikiloderma may be localized or diffuse and often is noted on the breasts, hips, buttocks, and flexural areas. It may coexist with patches of classic MF in some patients . Patches may be asymptomatic or mildly pruritic .
Histopathologic features of poikilodermatous MF include epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence. Epidermotropism of atypical lymphocytes often is observed although Pautrier microabsesses are not common . Immunophenotypic studies typically demonstrate a CD2+,CD3+, CD4+, CD7-, CD45RO+, CD8- pattern, which is consistent with a mature helper T-cell phenotype and is similar to that observed in classic MF [2, 6, 7]. Rarely, cases of MF are characterized by a CD8+ cytotoxic T-cell phenotype .
The differential diagnosis of poikilodermatous MF includes other conditions in which poikiloderma is prominent. These include large plaque parapsoriasis; connective-tissue diseases, such as lupus erythematosus and dermatomyositis; poikiloderma of Civatte; overuse of topical glucocorticoids; radiation dermatitis; graft-versus-host disease; and genodermatoses, such as Rothmund-Thompson syndrome [3, 6].
Treatment modalities for MF include both skin-directed and systemic therapies. Skin-targeted therapies include topical glucocorticoids, mechlorethamine, carmustine, topical bexarotene, phototherapy, photochemotherapy, and radiation (local or total skin electron beam). Systemic therapies employed for refractory, advanced, or systemic disease include oral bexarotene, interferon-α, extracorporeal photopheresis, denileukin diftitox, methotrexate, and single-agent chemotherapy [1, 8, 9]. A randomized, prospective study comparing aggressive modalities, which included electron-beam radiation and systemic chemotherapy, to sequential topical therapy showed no appreciable improvement in survival across groups after a median follow-up period of 75 months . Owing to this lack of long-term benefit after early aggressive therapy, treatment based on clinical staging is generally recommended [3, 8, 9].
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