Granulomatous slack skin: clinical retrospective study of 8 cases of the Cutaneous Lymphoma French Study Group
Oral presentation
Granulomatous slack skin (GSS) is a rare variant of mycosis fungoides (MF) presenting clinically as pendulous and lax plaques or tumours and histologically as a dermal infiltrate of neoplastic T-cells with multinucleated giant cells, loss of dermal elastin fibres and sometimes elastophagocytosis and lymphophagocytosis. Because of its scarcity, GSS is poorly known. The aim of this study was to describe the clinical and evolutive features and the response to treatment of a GSS cohort.
In this retrospective multicenter study, we have identified the cases of GSS in the Cutaneous Lymphoma French Study Group database and member centers.
Eight cases (7 males, median age: 29 years (22-70)) were included. The most frequently affected areas were the inguinal folds (75%), axillary folds and gluteal area (50% for both cases) but involvement of all body segments was observed. The median skin area affected by GSS was 13% (range: 3 - 20). A folliculotropic or plaque/tumor presentation was associated with GSS in 1 (13%) and 2 (25%) patients respectively. Two patients (25%) had visceral involvement confirmed by histology (Stage IVB) (Table).
The median duration of treatment was 36 months (5-187) and 23 different treatments or combinations of treatments were used. In total, the efficacy of 30 lines of treatment could be characterized: at best, stable disease (SD) was achieved after 13 of the treatments lines (43%), partial response (PR) after 12 (40%), complete response (CR) after 2 (7%) and progression (PD) after 3 treatment lines (10%). The treatments for which response was assessed at least 2 times were methotrexate (MTX; 1 SD, 2 PR, 1 CR), bexarotene (1 SD, 2 PR, 1 PD), gemcitabine (2 PR), doxorubicin (2 PD), and brentuximab vedotin (1 SD, 1 PR). Three patients died (38%), 2 related to GSS (the 2 stage IVB patients, 7 (case 8) and 15 (case 2) years after diagnosis), and 1 (41 years, case 3) in the context of hemophagocytic lymphohistiocytosis (HLH), while he had been out of follow-up for 12 years. In these 3 patients, paraneoplastic events were reported (venous thromboembolic disease (cases 2 and 3), hypercalcemia (case 8), HLH (case 3).
We report the largest series of GSS published to date. Our results identify GSS as a difficult to treat entity. Most of the time, the treatment only allows to achieve SD. However, the clinical assessment of the treatment efficacy may be challenging, due to the trophic consequences of the loss of elastic fibers. Among the usual treatments for MF, MTX seems to have the best results. Although GSS progresses slowly, severe/lethal forms exist and seem to be associated with the occurrence of paraneoplastic events.
In this retrospective multicenter study, we have identified the cases of GSS in the Cutaneous Lymphoma French Study Group database and member centers.
Eight cases (7 males, median age: 29 years (22-70)) were included. The most frequently affected areas were the inguinal folds (75%), axillary folds and gluteal area (50% for both cases) but involvement of all body segments was observed. The median skin area affected by GSS was 13% (range: 3 - 20). A folliculotropic or plaque/tumor presentation was associated with GSS in 1 (13%) and 2 (25%) patients respectively. Two patients (25%) had visceral involvement confirmed by histology (Stage IVB) (Table).
The median duration of treatment was 36 months (5-187) and 23 different treatments or combinations of treatments were used. In total, the efficacy of 30 lines of treatment could be characterized: at best, stable disease (SD) was achieved after 13 of the treatments lines (43%), partial response (PR) after 12 (40%), complete response (CR) after 2 (7%) and progression (PD) after 3 treatment lines (10%). The treatments for which response was assessed at least 2 times were methotrexate (MTX; 1 SD, 2 PR, 1 CR), bexarotene (1 SD, 2 PR, 1 PD), gemcitabine (2 PR), doxorubicin (2 PD), and brentuximab vedotin (1 SD, 1 PR). Three patients died (38%), 2 related to GSS (the 2 stage IVB patients, 7 (case 8) and 15 (case 2) years after diagnosis), and 1 (41 years, case 3) in the context of hemophagocytic lymphohistiocytosis (HLH), while he had been out of follow-up for 12 years. In these 3 patients, paraneoplastic events were reported (venous thromboembolic disease (cases 2 and 3), hypercalcemia (case 8), HLH (case 3).
We report the largest series of GSS published to date. Our results identify GSS as a difficult to treat entity. Most of the time, the treatment only allows to achieve SD. However, the clinical assessment of the treatment efficacy may be challenging, due to the trophic consequences of the loss of elastic fibers. Among the usual treatments for MF, MTX seems to have the best results. Although GSS progresses slowly, severe/lethal forms exist and seem to be associated with the occurrence of paraneoplastic events.