Mycosis fungoides mimicking erythema gyratum repens: an additional variant?

ARTICLE

Auteur(s) : Amilcare Cerri¹, Pamela Vezzoli², Stefano M Serini², Carlo Crosti², Emilio Berti³, Angelo V Marzano²

¹Dipartimento di Medicina, Chirurgia e Odontoiatria, Polo San Paolo, Università degli Studi di Milano, Milan, Italy
²U.O. Dermatologia, Fondazione IRCCS Ca’ Granda - Ospedale Maggiore Policlinico Dipartimento di Anestesiologia, Terapia Intensiva e Scienze Dermatologiche, Università degli Studi di Milano, Via Pace 9- 20122 Milan, Italy
³Università degli Studi di Milano-Bicocca

Mycosis fungoides (MF), is the most common cutaneous T-cell lymphoma (CTCL) that, in addition to its classic presentation, may show atypical features defining many clinical variants of the disease [1]. We report a MF mimicking erythema gyratum repens, in a male patient with a five-year history of classic MF, which evolved into an erythrodermic picture, associated with disseminated nodules and plaques, leading to the patient's death. The possible link between this unique clinical presentation and the unfavourable course of the disease in our patient is discussed.

A 61-year-old, otherwise healthy man presented in April 2000 for classic clinico-pathological and immunophenotypical aspects of MF. Molecular biology investigations proved a monoclonal rearrangement of the T-cell receptor (TCR)-γ chain. Haematological staging ruled out any extracutaneous involvement. At that time, and subsequently, the patient was treated with cycles of PUVA-therapy in combination with topical corticosteroids, achieving partial clinical remission. In April 2005, concentric, slightly infiltrated, erythematous annular lesions (figures 1A, B) were seen on the chest and upper limbs of the patient, together with new erythematous plaques on the forehead, periorbital areas, intergluteal region and legs. Histopathological and immunohistochemical examinations of upper limb biopsy specimens from the edge of a ring confirmed MF (figures 1C, D). Subsequently, cutaneous spreading was characterized by disseminated and confluent lesions with a figurate and circinnate border, closely resembling EGR (figure 1E). Microscopy examination of scratched skin samples and cultural analyses from erythema gyratum repens-like lesions excluded mycotic infection. Molecular biology investigations confirmed a monoclonal rearrangement of the TCR-γ chain, while a repeated staging ruled out extracutaneous disease and other possible neoplasms. The patient again had PUVA therapy, and then with oral bexarotene, with good but transient responses. Unfortunately, his disease evolved into an erythrodermic picture with widespread nodules and plaques, without peripheral blood or visceral involvement. After a trial with forodesine, which induced a partial remission, in June 2009 his general condition rapidly deteriorated and he died of septicaemia resistant to various systemic antibiotic regimens, in the absence of extracutaneous spreading or high grade transformation.

MF is a highly polymorphic epidermotropic T-cell lymphoma that can mimic many dermatoses; this makes the diagnosis more difficult but also may influence the disease prognosis, since some authors report that certain subtypes of MF seem to be linked with a worse prognosis [2]. Annular forms of MF are considered uncommon, since there are few cases reported in the literature [3-6]. Annular presentation did not seem to be linked to a more aggressive course of the disease. In our patient, however, the onset of annular lesions, closely resembling those of so-called erythema gyratum repens, preceded disease progression, indicating the possible selection of new T-cell clones responsible for the unfavourable and bizarre clinical presentation of the disease. EGR is a rare dermatosis characterized by mobile concentric erythematous wave-like bands, which produce a pattern resembling wood grain, usually involving the trunk [7]. It is often associated with malignancies, although it has also been reported in the absence of any underlying tumour. In our patient, the absence of internal malignancies during a long follow-up, associated with the clear results of histology and molecular biology investigations, proved that erythema gyratum repens-like lesions were a unique presentation of MF. Jouary et al. [8] reported an eruption mimicking erythema gyratum repens in a patient with stage III MF receiving polychemotherapy, which turned out to be a dermatophytic superinfection. In our patient, repeated microscopy examination of scratched skin samples and cultural analyses excluded any role for a mycotic superinfection.

In conclusion, this is the first report of MF presenting with unique clinical features mimicking EGR, a dermatosis that may thus be added to the imitation repertoire of this highly polymorphic CTCL.

Acknowledgment

References

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