Granulomatous Slack Skin Responds to UVA1 Phototherapy

Abstract

Granulomatous slack skin (GSS) is an extremely rare disorder within the group of cutaneous T cell lymphomas (CTCL). Ultraviolet A1 (UVA1) phototherapy has previously been reported to be useful in the treatment of CTCL such as mycosis fungoides. We report a 35-year-old Caucasian male with GSS treated with UVA1 phototherapy starting at 20 J/cm² UVA1 3 times a week and subsequently increased in increments of 5 J/cm² to a medium-range dose of 50 J/cm² per session. The patient underwent a total of 45 sessions with a cumulative dose of 1,495 J/cm² UVA1 without any adverse events. At the conclusion of UVA1 phototherapy, a decrease in erythema and skin thickness was observed which was most prominent in the periphery of the lesion in the right groin area. A follow-up 12 months after phototherapy showed continued treatment benefit. To our knowledge, this is the first report describing the successful use of UVA1 (340–400 nm) phototherapy in a patient with GSS.

Granulomatous slack skin presenting as diffuse poikiloderma and necrotic ulcers, with features of granulomatous vasculitis and response to oral prednisone, acitretin, and oral psoralen plus ultraviolet light therapy—A case report

“…There is good evidence that re-PUVA is effective for clearing skin lesions, particularly in early stages of the disease, and long-term remissions have been reported 2, 27, 28, 32, 33. Our patient exhibited a significant decrease in size and induration of the folds and a decrease in indurated plaques on the neck and trunk and further clearance of poikiloderma with re-PUVA.…”

“…Our patient continues to respond to oral PUVA and topical corticosteroids and is being monitored closely 2, 27, 28, 29, 30, 31, 32, 33, 36…”

Ultraviolet A1 phototherapy for mycosis fungoides

“…In 2009, Oberholzer presented a patient with a diagnosis of granulomatous slack skin MF, who received a total dose of 1495 J/cm 2 in 45 sessions (Table 1). One year after completion of therapy, the patient was asymptomatic 13 . There is also an interesting case of a 9‐year‐old child with MF treated successfully with UVA1, 14 who was followed up for 4 years.…”

Phototherapy of mycosis fungoides

“…Comprehensive reviews on ECP have been published recently and the treatment will not be covered here (20,21). Other modalities where only small case series have been published to date include photodynamic therapy, excimer laser, and UVA1 (22)(23)(24)(25)(26)(27)(28)(29). These treatments have not found widespread application in MF because they are either limited to the irradiation of very limited skin areas, or the equipment is expensive and not widely available, or both.…”