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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.85 no.6 Rio de Janeiro Nov./Dec. 2010
WHAT IS YOUR DIAGNOSIS?
Jonas RibasI; Antonio Pedro Mendes SchettiniII; Carla Barros da Rocha RibasIII; Clarisse de Albuquerque CorrêaIV
IHead of the Department of Dermatology, School of Medicine, Federal University of Amazonas (UFAM), Manaus, Amazonas, Brazil
IIDermatologist, Master's degree in Tropical Medicine, Dermatologist, Alfredo da Matta Foundation (FUAM), Manaus, Amazonas, Brazil
IIICoordinator of the medical residency in Dermatology, Alfredo da Matta Foundation (FUAM), Manaus, Amazonas, Brazil
IVPhysician, Brazilian Airforce, Dermatology Department, Airforce Hospital, Manaus, Amazonas, Brazil
An 18-year old female patient presented with a single, erythematous, desquamative plaque. The clearly outlined lesion was situated in the abdominal region. The patient reported that it had been present for the past year and that she had used no previous medication. Histopathology showed lymphocytic infiltration with atypia, principally affecting the superficial dermis with epidermotropism and mild spongiosis. Immunophenotyping revealed a predominance of CD3-positive cells, confirming the diagnosis of mycosis fungoides.
Keywords: Diagnosis; Cutaneous T-cell lymphoma; Mycosis fungoides
HISTORY OF THE DISEASE
ECSS, an 18-year old female born and living in Manaus in the Brazilian state of Amazonas presented with a single, well-defined erythematous, desquamative plaque with a maximum diameter of 4 cm, situated in the abdominal region, which had been present for the past year (Figure 1). The patient stated that she had not used any medication previously. A skin biopsy was performed for diagnostic evaluation.
At histopathology, the hematoxylin-eosinstained sections showed the presence of a group of mononuclear cells containing atypical lymphocytes and mild spongiosis. In the papillary dermis an infiltrate of perivascular lymphoid cells was found reaching the epidermis. No further alterations were identified in the rest of the dermis or hypodermis (Figures 2 and 3). The paraffin block was sent for immunohistochemical evaluation, which showed that the majority of the lymphoid cells present were CD3-positive in addition to being CD20 negative; CD45RO-positive; CD30-negative; and AE1/AE3-negative. The set of histopathological and immunohistochemical findings were conclusive for a diagnosis of unilesional mycosis fungoides.
The patient was submitted to laboratory evaluation (full blood count, glucose and clinical chemistry) and systemic evaluation (chest x-ray, bone xray, a magnetic resonance imaging scan of the head and computed tomography). No abnormalities were found.
The authors opted for complete surgical removal of the lesion. At a follow-up visit twelve months later the patient was asymptomatic and there was no recurrence of the lesion.
Mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) of primary cutaneous origin. Classically, it is characterized by an initial stage during which lesions are nonspecific, generally erythematous, squamous plaques. In the second stage, the initial lesions become infiltrated and new lesions appear. In the third stage, reddish-brown tumors appear that may progress to ulceration. 1 Histologically, mycosis fungoides is characterized by an infiltrate of atypical lymphoid cells with T-helper immunophenotype and accentuated epidermotropism forming an intraepidermal collection of cells known as Pautrier's microabscess. 2 In 1939, Woringer and Kolopp described a variant of this classic form in which skin involvement was limited to one or a few lesions. The presence of atypical mononuclear cells with a pagetoid appearance, marked epidermotropism and sparse dermal involvement was shown histologically. 3
Other clinical forms with limited skin involvement have been described, including unilesional mycosis fungoides, recognized by its clinical, histological and progressive characteristics. Clinically, it presents as a lesion in a plaque with an eczematous, psoriasis-like or poikilodermal appearance, while histological evaluation reveals lymphoid cells with atypia that are always limited to the papillary dermis or epidermis. Immunohistochemical studies show a predominance of CD4 over CD8 cells. 5-7 The condition progresses with no tendency towards becoming systemic. Treatment with aggressive methods of radio or chemotherapy is not justified, since a good response is obtained with local therapies such as PUVA therapy, localized electron beam, topical nitrogen mustard, topical corticoids, topical bexarotene or surgical removal of the lesion. 8
In addition to topical therapy with imiquimod, photodynamic therapy, a new, effective treatment for precancerous lesions and non-melanocytic skin cancer, is currently being used with success for the treatment of mycosis fungoides. 9,10
In conclusion, attention should be paid to recognizing this clinical variant of mycosis fungoides, since its characteristics of a favorable outcome, absence of systemic involvement and good response to local therapies permit less aggressive management of these patients and provide more accurate data regarding prognosis.
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4. Oliver GF, Winkelmann RK. Unilesional mycosis fungoides: a distinct entity. J Am Acad Dermatol. 1989;20:63-70. [ Links ]
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7. Glusac EJ, Shapiro PE, McNiff JM. Cutaneous T-Cell Lymphoma. Refinement in the aplication of controversial histologic criteria. Dermatol Clin. 1999;17:601-14. [ Links ]
8. Sanches J, Moricz C, Neto C. Processos linfoproliferativos da pele. Parte 2 - linfomas cutâneos de células T e de células NK. An Bras Dermatol. 2006;81:7-25. [ Links ]
9. Ardigo M, Cota C, Berardesca E. Unilesional mycosis fungoides successufully treated with imiquimod. Eur J Dermatol. 2006;16:446. [ Links ]
10. Recio ED, Zambrano B, Alonso ML, de Eusebio E, Martín M, Cuevas J, et al. Topical 5-aminolevulinic acid photodynamic therapy for the treatment of unilesional mycosis fungoides: a report of two cases and review of the literature. Int J Dermatol. 2008;47:410-3. [ Links ]
Approved by the Editorial Board and accepted for publication on 01.07.2009. * Study conducted at the Federal University of Amazonas, (UFAM), Manaus, Amazonas, Brazil
Conflict of interest: None
Financial funding: None
Approved by the Editorial Board and accepted for publication on 01.07.2009.
* Study conducted at the Federal University of Amazonas, (UFAM), Manaus, Amazonas, Brazil