Published Web Locationhttps://doi.org/10.5070/D39md3v1b6
From the Ronald O. Perelman Department of Dermatology, New York University
Alexandra Brecher MD PhD
Dermatology Online Journal 9(4): 23
The case of a 46-year-old woman with poikiloderma vasculare atrophicans is discussed. It is a rare clinical form of patch-stage mycosis fungoides characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangiectases.
History.—A 46-year-old-woman had an 8-year-history of a dermatosis involving the trunk, extremities, and face. The patient presented to the Bellevue Hospital Center dermatology clinic with an 8-year history of a generalized eruption. She reported worsening of symptoms with exposure to direct sunlight. She denied any new systemic or topical medications prior to onset of the eruption. Review of systems was negative for rheumatologic and neurologic symptoms. Past medical history includes left nephrectomy.
She was treated with topical glucocorticoids and antihistamines with little improvement. Flow cytometry demonstrated no evidence of lymphoproliferative disease. Immunophenotyping and gene rearrangement studies were negative. The patient underwent short trials of UVB phototherapy but was unable to tolerate treatment. She was placed on methotrexate with some improvement. The regimen was changed to acitretin and interferon-α. The pruritus resolved, and the erythema has improved.
Physical examination.—Generalized erythema with desquamation was present on the trunk, extremities, and face. On the anterior chest and breasts, there were atrophic patches, mottled erythematous patches, and telangectases. There was no lymphadenopathy or hepatosplenomegaly.
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Laboratory data.—The white-cell count was 6.7 x 109/L, hemoglobin 11.7 mg/dl, hematocrit 35.1 percent, platelet count 224 x 109/L, and lactic dehydrogenase 131 U/L. Anti-nuclear antibody was negative. A basic metabolic panel, liver function tests, lipid profile, and urinalysis were normal. Computed tomography scans of the chest, abdomen, and pelvis were normal.
Histopathology.—There is a superficial perivascular and bandlike infiltrate of lymphocytes that extend to the overlying focally atrophic epidermis where there is minimal spongiosis. The lymphocytes arrange as linear array and in few small collections at the dermoepidermal junction. Some lymphocytes have slightly enlarged nuclei. There is an associated papillary dermal fibroplasia. A few macrophages containing melanin are admixed with the dermal lymphocytic infiltrate.
Mycosis fungoides is a malignant neoplasm of T-lymphocyte origin, most commonly a memory CD4+ T cell. In most cases, it is a chronic, slowly progressive disease that usually begins as erythematous scaly patches that over time may evolve into more infiltrated plaques or even tumors. A wide range of atypical presentations has been described including erythrodermic, poikilodermatous, verrucous, hyperkeratotic, hypopigmented, vesicular, bullous, and pustular.
Poikiloderma vasculare atrophicans (parapsoriasis variegata) represents a variant of mycosis fungoides that is characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangectases . Lesions typically occur on the trunk and flexural areas of middle-aged patients, with a male predilection . The patches may be asymptomatic or mildly pruritic and are usually stable or gradually increase in size. Histologically, these lesions show focal parakeratosis with hyperkeratosis and acanthosis. Poikilodermatous patches show an atrophic epidermis with dilated dermal blood vessels and macrophages with melanin. Numerous atypical lymphocytes are observed around dermal blood vessels and some epidermotropism is observed, but Pautrier microabcesses are usually absent. Immunohistologic studies have shown similar phenotypic findings in classic cases of mycosis fungoides, namely, a CD2+, CD3+, CD4+, CD8-, CD45RO+ pattern with a minority of CD7+ cases [3, 4].
References1. Dougherty J. Poikilodermatous atrophicans vasculare. Arch Dermatol 103:550, 1971.
2. Howard MS, Smoller BR. Mycosis fungoides: Classic disease and variant presentations. Semin Cut Med Surg 19:91, 2000.
3. Lindae ML, et al. Poikilodermatous mycosis fungoides and atrophic large plaque parapsoriasis exhibit similar abnormalities of T cell antigen expression. Arch Dermatol 1988;124:366.
4. Kikuchi A, et al. Parapsoriasis en plaques: Its potential for progression to malignant lymphoma. J Am Acad Dermatol 1993;29:419.
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