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Advances in dermatology and venereology Acta Dermato-Venereologica
Imatinib Treatment of Lymphomatoid Papulosis Associated with Myeloproliferative Hypereosino philic Syndrome Presenting the FIP1L1-PDGFRA Fusion Gene
Pablo GARCÍA-MARTÍNEZ 1 , Dolors SITJAS 2 , Enric LLISTOSELLA 2 , Blanca ESPINET 3 , María RODRÍGUEZ-RIVERA 3 , Maria I . HERNANDEZ-MUÑOZ 4 , Fernando GALLARDO 1 and Ramon M . PUJOL 1 * Departments of 1 Dermatology and 3 Pathology , 4 Institut Mar d ’ Investigacions Mèdiques ( IMIM ), Universitat Autònoma de Barcelona , Hospital del Mar-Parc de Salut Mar , Passeig Marítim 25 – 29 , ES-08003 Barcelona , and 2 Department of Dermatology , Hospital Josep Trueta , Girona , Spain . * E-mail : 93329 @ parcdesalutmar . cat Accepted Apr 3 , 2017 ; Epub ahead of print Apr 4 , 2017
Peripheral blood eosinophilia has been reported to occur in a wide range of haematological malignancies including primary cutaneous lymphomas . The concurrence of lymphomatoid papulosis ( LyP ) with peripheral blood eosinophilia seems to be an uncommon phenomenon . Atypical lymphocytes in LyP display the phenotype of activated T-helper cells , consistently express CD30 antigen , have a Th2 cytokine profile , and secrete eosinophilstimulating cytokines . Cases of LyP have been reported in association with myeloproliferative hypereosinophilic syndrome ( M-HES ) presenting the Fip1-like 1 / plateletderived growth factor receptor-α ( FIP1L1-PDGFRA ) fusion gene . In such cases , imatinib treatment may lead to a complete and persistent resolution of LyP lesions .
CASE REPORT
A 25-year-old man was referred to our Department in November 2011 for evaluation of a 3-year history of recurrent crops of self-healing erythematous papules and nodules with central ulceration on the lower extremities , oral mucosa and face . Biopsy had established the diagnosis of LyP . The patient reported periodical appearance of lesions that regressed spontaneously leaving residual scars . His past medical history revealed childhood-onset asthma , allergic conjunctivitis and rhinitis . Peripheral eosinophilia of 1,200 / mm 3 had also been detected and was related to the allergic symptoms along with progressive cough and discrete dyspnoea .
Physical examination disclosed a firm , well-demarcated , painless , ulcerated nodule , 1.2 cm in diameter on the right aspect of the chin . Several erythematous papules and residual scars on the lower extremities were also observed . A solitary nodule with a central crust was noted on the left leg ( Fig . 1a , b ). No enlarged lymph nodes were detected .
Two 4-mm punch biopsies from different lesions disclosed a dense , dermal , wedge-shaped infiltrate with clusters of large atypical lymphoid cells admixed with small lymphocytes , histiocytes , neutrophils and eosinophils ( Fig . 1c – f ). Neoplastic cells were CD30 + and expressed mature T helper cell markers ( CD3 , CD2 , CD5 and CD7 , CD4 ). No expression of ALK , c-KIT or CD56 was detected . Neoplastic cells were also MUM-1 + and 60 % of cells expressed Ki-67 antigen . In both biopsy specimens , an identical clonal rearrangement of the T-cell receptor ( TCR ) γ and β chains was detected . No T-cell clonality was detected in peripheral blood samples .
Laboratory tests disclosed massive peripheral eosinophilia ( 6,120 / mm 3 ), normal IgE levels , elevated tryptase ( 15.5 ng / ml ) ( NV < 13.5 ) and vitamin B12 serum levels (> 2,000 ng / ml ) ( NVs : 197 – 866 ). Stools for ova and parasites were negative . Body computed tomography ( CT ) showed bronchial dilatation with peribronchial thickening and confluent centrolobulillar nodules forming a ground glass pattern . Electrocardiogram , echocardiogram and ophthalmological examination showed no abnormalities .
A bone marrow biopsy was obtained showing hypercellular marked eosinophilia , atypical spindle-shaped mast cells and increased reticulin fibrosis . Interstitial deletion of the 4q12
Fig . 1 . Clinical and histochemical characteristics . ( a ) Ulcerated inflammatory nodule on the right side of the patient ’ s chin . ( b ) Necrotic erythematous infiltrated plaque on the leg . ( c ) Histopathology revealing a wedge-shaped dense inflammatory infiltrate extending from papillary to reticular dermis ( haematoxylin and eosin staining ( H & E ); original magnification × 40 ). ( d ) Atypical , enlarged lymphocytes admixed with smaller lymphocytes , neutrophils and eosinophils ( H & E × 400 ). ( e ) The infiltrate showed positive CD4 immunoreactivity ( CD4 stain ; × 40 ). ( f ) Atypical , large lymphocytes corresponded with CD30 + cells ( CD30 stain ; × 400 ). ( g ) Detection of the ( 4q12 ) deletion by interphase fluorescence in situ hybridization ( FISH ) in peripheral blood cells . Loss of red signal indicating fusion of FIP1L1-PDGFRA . ( h ) FIP1L1-PDGFRA fusion gene was not detected in T cells from cutaneous LyP lesion by FISH .
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2669 Acta Derm Venereol 2017 ; 97 : 855 – 857