Case for diagnosis

Ribas, Jonas; Schettini, Antonio Pedro Mendes; Ribas, Carla Barros da Rocha; Corrêa, Clarisse de Albuquerque

doi:10.1590/S0365-05962010000600028

Abstracts

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.

Diagnosis; Cutaneous T-cell lymphoma; Mycosis fungoides

Paciente do sexo feminino, 18 anos, apresentando única placa eritemato-descamativa, bem delimitada, na região abdominal com evolução de 1 ano e sem uso anterior de medicação. O histopatológico demonstrou infiltrado linfocítico com atipias acometendo principalmente derme superficial com epidermotrifismo e discreta espongiose. E a imunofenotipagem demonstrou predomínio de células CD3-positivo, confirmando o diagnóstico de Micose Fungóide.

Diagnóstico; Linfoma cutâneo de células T; Micose fungóide

WHAT IS YOUR DIAGNOSIS?

^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

Mailing address

ABSTRACT

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.

COMMENTS

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. ¹Histologically, 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. ²In 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. ^5-7The 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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