An 88 y.o. presents with reddened itchy skin. What is the problem?

 hint the peripheral smear has unusual cells pictured below.

hint the peripheral smear has unusual cells pictured below.

sezary1.JPG

Our patient had Sezary syndrome.  This is a type of cutaneous lymphoma occurring in 3% of skin lymphomas. It was first described by Albert Sezary in 1938.  It is an accumulation of T cells and therefore a T cell lymphoma where the T cells invade the skin causing intense pruritus.  The cells can also appear in the blood where they appear to be “cerebriform” lymphocytes. The disease can be associated with mycosis fungoides and is associated with lymphadenopathy in the later stages. Clinically,  it is associated with redness of the skin, hyperkeratosis of the palms and  and occasionally lagophthalmos.  ( inability to close the eyelids;referring to the myth that rabbits sleep with their eyes open)

 mycosis fungoides with typical plaques

mycosis fungoides with typical plaques

Mycosis fungoides  is the most common form of cutaneous T-cell lymphoma. It is an unusual expression of CD4-T cells. These lymphocytes form dense plaques in the skin with lymphoid cells lining  up along the epidermal basal layer.  Mycosis fungoides is considered a less virulent lymphoma than Sezary syndrome and often patches in the skin can be dormant for years. They may begin as simply hypopigmented dermal thickening.  In the final stages tumors may develop in the patches.

mf lymphoskinbx.JPG

Treatment consists of radiation of the skin with electron beam or UVB light and psoralen with UVA therapy.  Recently imiquimod has been used in treatment of skin lesions. Imiquimod increases cytokine release  and inflammatory reaction.

Our patient received a retinoic acid compound and light treatments.  Because of the overall poor prognosis of Sezary syndrome, he was made DNR/DNI.  

 

Sezary A, Bouvrain Y. Erhthrodermie avec presence de cellules monstrueuses dans le derme et le sang circulant. Bulletin dela Societe francaise de dermatologie et de syphiligraphie, Paris, 1938;45:254-260.

Thangavelu M, Finn WG, Yelavarthi KK,  et al. Recurring structureal chromosome abnormalities in peripheral blood lymphocytes of patients with mycosis fungoides/Sezary syndrome Blood 89(9)3371-7.

Jawed S. Hyskowski P, et al. Primnary cutaneous T-cell lymphoma ( mycosis fungoides and Sezary syndrome. 2014. Journal of the American Academy of Dermatology 70(2) 223. E1-223e17.

Yamashit T, Abbade L, Marques M, Marques S.  Mycosis fungoides and Sezary syndrome: clinical histopathological and immunohistochemical review and update. An Bras Dermatol 2012Nov-Dec 87(6 ) 817-830.