The eyes do not see what mind doesn't know

Clinical scenario:

                          A 61 years old male patient  was referred to our clinic complaining of generalized itchy skin rashes , and swelling of the both lower limbs and disfigurement of the face . 

He had no other associated symptoms. The condition had stared 2 years ago with generalized itching without any skin lesions and patient received medication from a dispensary as a case of allergy,  then 4 month later he developed itchy skin lesions on trunk and extremities with  redness and scaling that  varied in size (non sun exposed areas) and his condition became worse .Gradually skin started to become thick with appearance of subcutaneous  nodules (Fig . 1) mainly on the trunks, face and body folds (axillae and groins) .

Fig 1 Leionine facies 

Fig 2 Lesions on the back 

He   lost his eye  brows. Skin biopsy was done in some local hospital and he was diagnosed as a case of lepromatous leprosy (acute lepra reaction) and the patient received medication as a case of leprosy for about 6 months without any improvement. 

Past history :No  history of epistaxis  No history of numbness, weakness or ulcerations  

Patient is non diabetic and has no  history of hypertension , bronchial  asthma . 

No drug history apart from medications given  during his present condition.

Examination : 

    On examination patient was conscious oriented hemodynamically stable with no  jaundice.There was generalised lymphadenopathy (cervical sub mental , axillary and in inguinal areas ) the lymph nodes were rubbery , mobile and not matted . The size of lymph nodes  varied from 1-3 cms . He had painless enlargement of these nodes 

Systemic examination :   He had splenomegaly . Chest and CVS  examination were normal.  

None of his peripheral nerves were palpable and neurological examination was normal. 

Skin examination : 

Thickening and infiltrating plaques of facial skin with loss of eye brows  leading to characteristic leonine facies . (Fig1 )No ulceration of the nasal mucosa or nasal septum was noted.

Evaluation: The Hemogram showed  marked Eosinophilia and Erythrocyte sedimentation rate (ESR )was elevated .Peripheral blood film showed atypical lymphocytes . 29% Immature lymph. ??? lymphoma for    immunopheno    typing . The Lymphocytes had nuclei that were  hyper chromatic  cerebriform Fig 2 

  

Fig3 Atypical Lymphocytes in the peripheral film

    

The renal and liver function tests were normal . Apart from splenomegaly the ultrasound abdomen was unremarkableThe skin biopsy :The biopsy  showed diffuse infiltration of lymphocytes and scattered histiocytes in the upper and mid dermis (Fig 3)features of T cell lymphoma  and immunocytopathology showed CD+3 phenotype of mature helper T cell .Keeping in view dermatological findings abnormal peripheral blood film and generalized lymphadenopathy he was diagnosed as Sézary syndrome (SS) 

Fig 3 Skin biopsy showing infiltration of Atypical lymphocytes 

Management : 

Patient was managed with low dose of Methotrexate   and anti Leprosy medications were stopped .He is following our clinic .Take home message : Sézary syndrome (SS) has been defined historically by the triad of  erythroderma, generalized lymphadenopathy, and the presence of neoplastic T cells (Sézary cells) in skin, lymph nodes, and peripheral blood. Generalized erythroderma,Superficial lymphadenopathy, Atypical cells in circulating blood .Erythroderma from onset with leonine facies, Alopecia, palm and sole hyperkeratosis The condition is difficult  to treat and has median  survival of 3 years . Low dose methotrexate has reasonable response rate of 50%. Other treatment option are photophoresis, Local Retinoid, interferon alfa and low dose chlorabucil. Systemic chemotherapy even is given in selected cases 

This case was Contributed by 

Dr Ahmed Abdel Razik Khamis M.D

Consultant Dermatologist

King Abdul Aziz Specialist Hospital Taif Saudi Arabia 

Further reading :    Cutaneous Lymphoma