Poikilodermatous mycosis fungoides
Published Web Locationhttps://doi.org/10.5070/D321k491p5
Poikilodermatous mycosis fungoidesThe Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
Bradley Bloom MD, Shari Marchbein MD, Max Fischer MD MPH, Hideko Kamino MD, Rishi Patel MD, Jo-Ann Latkowski MD
Dermatology Online Journal 18 (12): 4
Poikilodermatous mycosis fungoides (MF) is a variant of MF, formerly referred to as poikiloderma vasculare atrophicans. The lesions are classically characterized by large plaques of hypopigmentation and hyperpigmentation with atrophy and telangiectases. The plaques may be asymptomatic or mildly pruritic and typically involve the major flexural areas and trunk. Poikilodermatous MF has an early stage (IA-IIA) at diagnosis and a male predominance. Poikilodermatous MF shows an atypical T-cell infiltrate in the papillary dermis with evidence of epidermotropism, epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence. Recent studies suggest a predominance of a CD8+, CD4- immunophenotype. Treatment modalities are similar to classic MF with phototherapy being the most common first-line therapy. Poikilodermatous MF has an excellent prognosis.
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A 23-year-old woman presented to the Dermatology clinic at Bellevue Hospital Center with a ten year history of pink patches on her lower extremities. She reported that the eruption began on her lower legs and slowly spread to her buttocks. The patches deepened in color from pink to red as they increased in size, although they remained asymptomatic throughout progression of her disease.
A biopsy specimen obtained elsewhere in 2003 showed a lichenoid lymphohistiocytic infiltrate with numerous extravasated red blood cells and siderophages. Thereafter she was lost to follow up until 2008, when she presented to a dermatologist in the Dominican Republic. A repeat biopsy was non-diagnostic.
Past medical history included asthma. She takes no oral medications.
Two punch biopsy specimens were obtained from representative lesions on the left lower extremity and buttock.
Confluent, erythematous and poikilodermatous patches were present on the lower extremities and buttocks. Patches showed mottled hyperpigmentation and hypopigmentation with atrophy and telangiectases. The popliteal fossae and anterior aspects of the knees were spared.
A comprehensive metabolic panel, including basic metabolic panel and liver function tests, was normal. Lactate dehydrogenase was mildly elevated at 242 U/L (upper limit of normal 225 U/L). Flow cytometry of the peripheral blood was normal and the CD4/CD8 ratio was normal. A positron emission tomography/computed tomography scan was without evidence of lymph node or visceral involvement.
There is a perivascular and band-like infiltrate of lymphocytes, some of which extend to an overlying atrophic epidermis, where there is vacuolar alteration of the basal layer with scattered necrotic keratinocytes. Intraepidermal lymphocytes have hyperchromatic nuclei with irregular contours. There are telangiectases, focal fibrosis, and scattered melanophages. Lymphocytes express CD3, CD4, CD5, and diminished CD7.
Poikilodermatous mycosis fungoides is a rare clinical variant of mycosis fungoides (MF). The term poikiloderma atrophicans vasculare was introduced in 1908 to describe what we now recognize as poikilodermatous MF . The clinical presentation of this clinical MF variant is characterized by large patches or plaques of hypopigmentation and hyperpigmentation with atrophy and telangiectases . The patches or plaques may be asymptomatic or mildly pruritic and typically involve the major flexural areas and trunk . Patients with poikilodermatous MF often describe a stinging sensation rather than pruritus. Similar to classic MF, poikilodermatous MF presents as an early stage (IA-IIA) at diagnosis with a male predominance . However, poikilodermatous MF patients usually are younger than are classic MF patients and a recent article delineating this clinical variant of MF described the frequent association with lymphomatoid papulosis .
Histopathologic features of poikilodermatous MF show an atypical T-cell infiltrate in the papillary dermis with evidence of epidermotropism, epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence [3, 4]. Pautrier microabscesses rarely are present [1, 2, 3]. The atypical lymphocytes are commonly CD4+, which is similar to classic MF [2, 3, 5, 6]. However, more recent studies suggest a predominance of a CD8+, CD4- immunophenotype .
The differential of diagnosis of poikilodermatous MF comprises any diseases with clinical manifestations of poikiloderma, including large plaque parapsoriasis, connective- tissue diseases (e.g., lupus erythematosus and dermatomyositis), corticosteroid induced poikiloderma, radiation dermatitis, and graft-versus-host disease [2, 3].
Treatment for poikilodermatous MF is similar to that of classic MF . Initial management is dependent on the stage, age, and general health of the patient [2, 7, 8]. As in classic MF, treatment for poikilodermatous MF is generally skin-directed. Narrowband ultraviolet B phototherapy is the first-line therapy for poikilodermatous MF . Other skin-directed treatments include topical glucocorticoids, topical retinoids, topical cytotoxic agents, and radiation therapy. Topical glucocorticoids are not so effective in poikilodermatous MF as they are in classic MF . If the patient fails skin-directed treatments, systemic therapies such as oral retinoids and alpha interferon are effective. In poikilodermatous MF, some of the skin changes, such as telangiectases, may be permanent depending on the length and extent of the disease. Poikilodermatous MF is one of few clinical variants of MF in which a biopsy may be warranted to assess the efficacy of the treatment modality.
Similar to classic MF, poikilodermatous MF has an excellent prognosis. In a recent retrospective study, 96 percent of the study patients with poikilodermatous MF remained at the same stage over a mean follow-up period of more than 11 years .
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