Ofuji Papuloerythroderma Associated with Follicular Mucinosis in Mycosis Fungoides

Abstract

Ofuji papuloerythroderma is a distinctive clinical entity of unknown etiology, which may occasionally be associated with B cell and T cell lymphoma or visceral malignancy. We report a case of papuloerythroderma associated with follicular mucinosis in mycosis fungoides (MF) that raises the possibility of papuloerythroderma as a form of prelymphomatous skin eruption. This specific papuloerythroderma responded well to the Re-PUVA treatment, which is a combination of etretinate and PUVA photochemotherapy.

Papuloerythroderma 2009: Two New Cases and Systematic Review of the Worldwide Literature 25 Years after Its Identification by Ofuji et al.

“…UV treatment modalities, alone or in combination with CCS, seemed to achieve the best results [25]. Single case reports and small case series have also shown the efficacy of interferon α [15,44,75], etretinate [30], cyclosporin A [32,62,70,80,86], azathioprine [83], re-PUVA [24] and bath PUVA [68,82] in monotherapy.…”

“…Eleven cases showed histological features compatible with a diagnosis of cutaneous T-cell lymphoma (CTCL) [16,24,25,35,37,42,43,46,87], while in 2 cases this diagnosis was likely but could not be confirmed [4,75]. Six PEO patients developed a CTCL later on [12,13,25,43,71,98].…”

“…Six PEO patients developed a CTCL later on [12,13,25,43,71,98]. In 6 out of these 17 cases, the diagnosis of mycosis fungoides (MF) was further specified into that of folliculotropic MF [12,24,35,43,46,87]. …”

“…Seventy papers belonged to the English literature, 25 to the Asian literature and 17 to the non-English/non-Asian Western literature. One hundred twenty-five [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51], 25 [52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73], and 18 cases of PEO [74,75,76,77,78,79,80,81,82,83,84,85,86,87] were identified, respectively, for a grand total of 170 cases (including the present 2) as of July 31, 2009. Six case reports were excluded as redundancy with later articles was suspected [88,89<...>…”

Papuloerythroderma of Ofuji: A Report of Two Cases

“…Furosemid ve aspirin ile indüklenmiş olguların bildirilmesi üzerine ilaçların da etyolojide rol oynayabileceği düşünülmüştür (2,20). Sistemik veya kutanöz malin hastalıklara eşlik etmesi nedeniyle para- neoplastik sendrom olarak kabul edenler olduğu gibi, mikozis fungoides veya Sezary sendromundan farklı bir kutanöz T hücreli lenfoma ya da prelenfomatöz bir hastalık olarak kabul edenler de vardır (21,22). Ferahbaş (23) tarafından yayınlanan bir makalede, Ofuji'nin papuloeritrodermasının, mikozis fungoidesin klinik bir varyantı olduğu bildirilmiştir.…”