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Mycosis fungoides bullosa: a case report and review of the literature
© Kneitz et al; licensee BioMed Central Ltd. 2010
Received: 30 March 2008
Accepted: 3 March 2010
Published: 3 March 2010
Mycosis fungoides, the most common type of cutaneous T-cell lymphoma, can manifest in a variety of clinical and histological forms. Bulla formation is an uncommon finding in mycosis fungoides and only approximately 20 cases have been reported in the literature.
We present a case of rapidly progressive mycosis fungoides in a 68-year-old Caucasian man who initially presented with erythematous plaques characterised by blister formation.
Although mycosis fungoides bullosa is extremely rare, it has to be regarded as an important clinical subtype of cutaneous T-cell lymphoma. Mycosis fungoides bullosa represents a particularly aggressive form of mycosis fungoides and is associated with a poor prognosis. The rapid disease progression in our patient confirms bulla formation as an adverse prognostic sign in cutaneous T-cell lymphoma.
Mycosis fungoides, the most common type of cutaneous T-cell lymphoma, can manifest in a variety of clinical and histological forms, but blistering is not a feature normally associated with the condition. Indeed, of the many variants that have been reported in the literature, approximately 20 cases of the bullous variant have been described.
Initial treatment of our patient with electron beam irradiation led to complete clearance, but after 1 year, generalised erythematous plaques of mycosis fungoides bullosa appeared on his trunk and extremities without lymph node and visceral involvement. Further electron beam irradiation was carried out.
The most common causes of acquired bulla formation on inflamed skin areas are acute contact dermatitis as well as infections with Staphylococci, or viruses of the herpes group. Bullous lichen planus or bullous lupus erythematosus are rare diseases, whereas bullous pemphigoid is the most common autoimmune bullous disorder .
In mycosis fungoides, bulla formation is very uncommon. Approximately 20 cases have been reported in the literature [2, 3]. Mycosis fungoides bullosa is largely restricted to older patients without predominance of gender. Predilection sites are the trunk and limbs. Vesicles and blisters usually arise in typical plaques and tumours but also in normal-appearing skin [2, 4].
An association with concomitant bullous pemphigoid or previous treatment with psoralen UVA photochemotherapy has been reported . Bowman et al. proposed the following criteria for diagnosis of mycosis fungoides bullosa : (1) clinically apparent vesiculobullous lesions; (2) typical histologic features of mycosis fungoides (atypical lymphoid cells, epidermotropism, Pautrier's microabscesses) with intra-epidermal or subepidermal blisters; (3) negative immunofluorescence ruling out concomitant autoimmune bullous diseases; and (4) negative evaluation for other possible causes of vesiculobullous lesions (for example, medications, bacterial or viral infection, porphyria, phototherapy).
The pathological mechanism underlying blister formation has not been elucidated. Confluence of Pautrier's microabscesses in mycosis fungoides lesions may lead to intra-epidermal bulla formation . Alternatively, proliferation of neoplastic lymphocytes may result in a loss of coherence between basal keratinocytes and basal lamina  or the cohesion of keratinocytes may be affected by the release of lymphokines by atypical lymphocytes .
Although mycosis fungoides bullosa is extremely rare, it has to be regarded as an important clinical subtype of cutaneous T-cell lymphoma. Mycosis fungoides bullosa represents an especially aggressive form of mycosis fungoides associated with a poor prognosis. Approximately 50% of patients die within 1 year after the appearance of the blistering of the lymphoma plaques [1, 9].
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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