Mycosis fungoides plantaris

ARTICLE

Auteur(s) : Gemma Márquez Balbás, Angeles Sola Casas, Maribel Iglesias Sancho, Manuel Sánchez-Regaña, Loida Galvany Rossell, Elisabet Dilmé Carreras, Joaquim Sola Ortigosa, Pablo Umbert Millet

Dept of Dermatology, Dermatology Univesitary Hospital Sagrat cor, Viladomat street 288, 08029 Barcelona, Spain

Mycosis fungoides palmaris en plantaris (MFPP) is a variant of mycosis fungoides (MF) that presents primarily on the palms and soles [1], and it has been infrequently described. Herein we report three new cases of MF plantaris, each with a different clinical form.

Case 1. A 63 year-old female presented a 1 year history of multiple hyperkeratotic and fissured plantar plaques (figure 1A). She was treated for plantaris hyperkeratosis resembling keratoderma with topical corticosteroids and keratolytics, and oral acitretin, with partial response. One 4 mm punch biopsy specimen was obtained from the right foot, and it showed psoriasiform epidermal hyperplasia and atypical lymphocytes with cerebriform nuclei (figure 1B). The immunohistochemical study showed positivity for CD4 (> 70%).

Case 2. A 25-year-old male presented some brownish and purple plaques, a little desquamative, on both feet (figure 1C). A punch biopsy from the right foot was diagnostic for MF plantaris. The skin biopsy showed a psoriasiform epidermal hyperplasia and focal epidermotropism of cerebriform lymphocytes and Pautrier’s micro-abscesses in some sections (figure 1D). Immunohistochemical findings showed the infiltrate to be composed mostly of CD4.

Case 3. A 43-year-old female presented a 2-year history of an eczematous plantar plaque. It was pruritic and had not responded to emollients and topical keratolytics (figure 1E). A biopsy specimen from the lesion showed psoriasiform epidermal hyperplasia, a band-like infiltrate containing numerous cerebriform lymphocytes in the papillary dermis, and a single Pautrier’s micro-abscess (figures 1F, G). The immunohistochemical study showed a marked predominance of CD4.

In all three cases a diagnosis of MF plantaris was established. No other cutaneous involvement was observed, besides the plantar lesions. In addition, extension studies were negative in all patients. The first case is currently receiving treatment with re-PUVA (acitretin 25 milligrams per day, and topical 8-metoxipsoralen and UVA), with a poor response after three months of treatment. The second case presented clinical remission after two months of treatment with topical clobetasol propionate 0.05% and oral acitretin 25 mg/day. The third case also presented a complete response with topical clobetasol propionate 0.05% after six months of treatment. The last two cases have had a follow-up period of almost 4 years.

MFPP is infrequent, about 0.6% of all MF [2]. Clinically, MFPP mimics many other dermatoses. Cutaneous lesions may present as multiple or single lesions, scaly and erythematous patches, hyperkeratotic and dyshidrotic plaques [3, 4]. A wide clinical spectrum is the reason why diagnosis of this entity is often delayed. When the differential diagnosis is difficult, T cell-receptor (TCR) gene rearrangement study is one of the most useful methods for diagnosing MF [1]. In addition, clinical and histological findings should be considered.

It is important to distinguish it from palmo-plantar involvement in the course of a generalized MF, which is not uncommon. The histopathological findings in a skin biopsy are indistinguishable between these two entities. In single lesions, the most important differential diagnosis is Woringer-Kolopp disease, which is characterized by the presence of one or several scaly patches and plaques with acral distribution. It is a benign T-cell lymphoproliferative process with certain histological and phenotypical similarities both to early epidemotropic mycosis fungoides-type cutaneous T-cell lymphoma and other T-cell lymphoproliferations.

In MFPP, various therapeutic modalities have been described with PUVA [5], narrow band UVB [5], excision of solitary lesions [5], high potency topical corticoids and intralesional corticoids, methotrexate [5], bexarotene [5], radiotherapy [6], and electron beam therapy [6], CO2 laser and topical nitrogen mustard [5].

This form of MF typically has a long benign course [4], no case of extracutaneous spread has been described [5]. A skin biopsy should be included in the study of all recalcitrant palmo-plantar dermatoses, and MFPP considered in the differential diagnosis.

Acknowledgements

References

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