Cutaneous T-cell lymphoma

Case history #1

A 55-year-old man presents with a 5-year history of a gradually worsening, asymptomatic skin eruption that has not responded to treatment for dermatitis. On examination, he has asymmetrical, erythematous scaly patches with signs of poikiloderma (mild atrophy, telangiectasia, and pigmentation) affecting his buttocks, hips, flanks, and upper thighs. There is no evidence of enlarged peripheral lymph nodes, liver, or spleen. Routine full blood count and biochemistry are normal.

Case history #2

A 69-year-old woman presents with a 12-month history of a generalised erythematous rash associated with intense pruritus that is disturbing sleep and affecting her quality of life. She reports weight loss, fatigue, and shivering. She has no prior history of skin disease. On examination, more than 90% of her skin surface is diffusely thickened, demonstrating erythema, oedema, scaling, and fissures. Numerous excoriation marks are visible, and there is keratoderma of the palms and soles. Moderately enlarged lymph nodes are palpable in the axillae and groins.

Other presentations

The development of tumour lesions, erythroderma, lymphadenopathy, and visceral and facial involvement occurs in the later stages of mycosis fungoides (MF), while systemic features (constitutional symptoms, hepatomegaly) and erythroderma are common initial findings associated with Sézary syndrome (SS). Involvement of the nails (onychodystrophy), hair (alopecia), face (leonine facies), and eyes (ectropion) may also occur in SS.

Atypical clinical presentations of MF are not uncommon. These include hypopigmented MF, particularly seen in black skin and in childhood and adolescence; follicular MF, in which the infiltrate predominantly affects pilosebaceous units leading to follicular mucinosis and hair loss; and unilesional MF (Worringer-Kollop disease), most commonly on acral sites.

Rarely, presentations of MF include bullous, granulomatous, ichthyosiform, and purpuric clinico-pathological forms.