
	Version PDF

Syringotropic cutaneous T cell lymphoma treated with PUVA therapy

European Journal of Dermatology. Volume 15, Number 4, 262-4, July-August 2005, Therapy

Summary

Author(s) : Alessandro Venturini, Cristina Zane, Rosita Rodella, Carla Leali, Piergiacomo Calzavara Pinton, Fausto Zorzi , Department of Dermatology, Spedali Civili, p.le Spedali Civili, 1 25123 Brescia, ItalyFax: (+39) 030 399 5015., Department of Pathology, Poliambulanza, Brescia, Italy.

Summary : Syringotropic cutaneous T cell Lymphoma (SCTCL) is a rare localized variant of CTCL. It is characterized by erythematous papules that, at histological examination, show dense dermal infiltrates of atypical T cells, that are preferentially located around hyperplastic eccrine sweat glands and ducts, with absent or minimal epidermotropism. Its relationship with mycosis fungoides and other CTCLs is not clarified and is still under discussion. Several treatment approaches have been suggested, but therapeutic results are often disappointing. We report the case of a patient with typical clinical and histopathological features of SCTCL and an excellent response to PUVA therapy.

Keywords : PUVA therapy, syringotropic cutaneous T-cell lymphoma

Pictures

ARTICLE

Auteur(s) :, Alessandro Venturini^1,*, Cristina Zane¹, Rosita Rodella¹, Carla Leali¹, Piergiacomo Calzavara Pinton¹, Fausto Zorzi²

¹Department of Dermatology, Spedali Civili, p.le Spedali Civili, 1 25123 Brescia, ItalyFax: (+39) 030 399 5015.
²Department of Pathology, Poliambulanza, Brescia, Italy

accepté le 11 Mars 2005

A 62-year-old caucasian man had asymptomatic erythematous papular lesions on the arms and legs.The lesions appeared progressively during the last 12 months and a prolonged therapy with topical steroids (clobetasol 0.05% cream b.i.d) was unsuccessful.At dermatological examination ( (figure 1) ) follicular, erythematous, horny papules were seen on the lower and upper limbs. Papules were isolated or confluent in roundish plaques of 4-5 cm in size.Superficial lymph-nodes were neither visible nor palpable.Physical examination was otherwise normal.Blood cell count and blood chemistries were within normal ranges. Radiological investigation of the chest and ultrasonography of the abdomen gave normal findings.Histopathologic examination of a biopsy of the lesional skin ( (figure 2A) ) showed a dense lymphoid infiltrate surrounding hyperplastic and hypertrophic eccrine sweat glands.The upper part of the infiltrate was close to the dermo-epidermal junction, without evidence of epidermotropism.In addition, sparse lymphoid infiltrates were seen around some hair follicles.Perivascular lymphoid infiltrates and fibrinoid necrosis of the vessel walls of small arterioles were also present.The T cell immunophenotype of the infiltrate was: CD3+ ( (figure 2B) ), CD4+, CD5+, CD45R0+, CD57– and CD56–.An 8,18 keratin stain for eccrine glandular cells cytoplasm clearly showed the surrounding lymphocytic infiltrate (( figure 2B, figure 2C) ).The patient was treated with PUVA therapy. According to an aggressive protocol, 8 MOP was administered at the dose of 0.6 mg/kg body weight, the initial UVA dose was established on the basis of the individual photochemotoxic threshold, the weekly exposures were four and the UVA increments were delivered twice weekly.Complete resolution of the skin lesions was observed after 26 treatments (UVA cumulative dose 77 J/cm²).After 1 year, the patient is still in clinical remission.

Discussion

Syringotropic cutaneous T cell lymphoma (SCTCL) is a rare disease: only 19 cases with compatible clinical and histological features have been reported [1-14].

In addition, two patients with cutaneous T cell Lymphoma with combined folliculotropic/syringotropic features have been described [15].

SCTCL is characterized by multiple localized erythematous papules, representing the clinical counterpart of histological findings of hyperplastic eccrine glands and ducts surrounded by a dense syringotropic lymphocytic infiltrate. Alopecia, if present, is due to the involvement of hair follicles.

The neoplastic nature of the infiltrate remained controversial, until recent gene rearrangement studies have demonstrated the monoclonality of the lymphocytic infiltrate in most cases [5-8, 13, 14].

Eccrine hyperplasia seems to represent the exclusive histological hallmark of this disease, although it could be related to a secondary reaction to the inflammation due to the surrounding lymphocytes, and an attempt to regeneration [6, 8, 9, 12].

SCTCL should be considered as a distinct clinicopathological entity [5, 14], although its relationship with mycosis fungoides and syringolymphoid hyperplasia with alopecia (SLHA) is not fully clarified.

SLHA is another rare condition [14]. Clinical and histological findings are very similar to those of SCTCL, and there is a general agreement that they could represent the same pathological entity [14].

The relationship of SCTCL with MF is debated.

The lymphocytic infiltrate is phenotypically similar, but it displays affinity for the eccrine gland and duct with minimal epidermotropism in SCTCL, whereas it concerns epidermis with possible focal adnexotropism in MF [16, 17].

Therefore we could hypothesize that SCTCL represents a form of CTCL with a peculiar lymphocytic homing pattern, leading to selective syringotropism [15].

SCTCL usually runs as a chronic disease, strictly limited to skin involvement [13, 14], but in a single case, progression to a systemic disease has been described [1].

Several treatment approaches have been suggested, but therapeutic results were often disappointing.

In the present patient topical steroids were not effective, thus confirming previous data in the literature [10, 13].

We can hypothesize that topical therapies are usually ineffective, due to the lack of penetration into the deep dermis where the infiltrates are located.

Surgical excision [5] has been suggested for patients with single or few lesions.

Radiotherapy [6, 14] and single [9] or multi-agent [12] chemotherapy may be effective [14], but they have several contraindications, and the hazards of severe local and systemic adverse effects are well known.

Phototherapy and photochemotherapy associated to various systemic therapies have been employed as well.

High dose UVA1 phototherapy [10] was found ineffective.

PUVA and Bath PUVA therapies have been used with contrasting results.

The treatment failure observed with BathPUVA [8] can be related to the lack of penetration of topically applied psoralen.

No significant improvement was seen in a patient treated with PUVA [14] and in 2 patients after PUVA plus oral retinoids [12, 14], whereas, in a recent report [15], PUVA associated with IFN alpha was found effective.

However, in the present patient, PUVA allowed for a complete and persistent resolution of skin lesions.

Differences in efficacy could be related to differences in treatment protocols, but unfortunately previous reports [12, 14, 15] do not describe them in detail.

In conclusion, the optimal treatment for SCTCL is still unknown.

Photochemotherapy may be effective, but an aggressive and individualized protocol is recommended.

References

1 Sarkany I. Patchy alopecia, anhidrosis, eccrine gland wall hypertrophy and vasculitis. Proc R Soc Med 1969; 62: 157-9.

2 von Steigleder GK, Bauermeister-Jasso K. Lymphomatoid granulomatosis with epithelial islands. Z Hautkr 1981; 56: 851-61.

3 Vakilzadeh F, Brocker EB. Syringolymphoid hyperplasia with alopecia. Br J Dermatol 1984; 110: 95-101.

4 Berger TG, Goette DK. Eccrine proliferation with follicular mucinosis. J Cutan Pathol 1987; 14: 188-90.

5 Burg G, Schmockel C. Syringolymphoid hyperplasia with alopecia-a syringotropic cutaneous T-cell lymphoma? Dermatology 1992; 184: 306-7.

6 Zelger B, Sepp N, Weirer K, et al. Syringotropic cutaneous T-cell lymphoma: a variant of mycosis fungoides? Br J Dermatol 1994; 130: 765-9.

7 Tomaszewski MM, Lupton GP, Krishnan J, et al. Syringolymphoid hyperplasia with alopecia. J Cutan Pathol 1994; 21: 520-6.

8 Esche C, Sander CA, Zumdick M, et al. Further evidence that syringolymphoid hyperplasia with alopecia is a cutaneous T cell lymphoma. Arch Dermatol 1998; 134: 753-4.

9 Tannous Z, Baldassano MF, Li VW, et al. Syringolymphoid hyperplasia and follicular mucinosis in a patient with cutaneous T-cell lymphoma. J Am Acad Dermatol 1999; 41: 303-8.

10 Haller A, Elzubi E, Petzelbauer P. Localized syringolymphoid hyperplasia with alopecia and anhidrosis. J Am Acad Dermatol 2001; 45: 127-30.

11 Garcovich A, Garcovich S, Massi G. An unusual variant of granulomatous adnexotropic cutaneous T-cell lymphoma. Br J Dermatol 2003; 148: 363-5.

12 Ah-Weng A, Howatson SR, Goodlad JR, et al. Erythrodermic syringotropic cutaneous T-cell lymphoma. Br J Dermatol 2003; 148: 349-435.

13 Hobbs JL, Chaffins ML, Douglass MC. Syringolymphoid hyperplasia with alopecia: two case reports and review of the literature. J Am Acad Dermatol 2003; 49: 1177-80.

14 Thein M, Ravat F, Orchard G, et al. Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases. Br J Dermatol 2004; 151: 216-26.

15 Jacobs MA, Kocher W, Murphy GF. Combined folliculotropic/syringotropic cutaneous T-cell lymphoma without epidermal involvement: report of 2 cases and pathogenic implications. Hum Pathol 2003; 34: 1216-20.

16 Rongioletti F, Smoller B. The histologic value of adnexal (eccrine gland and follicle) infiltration in mycosis fungoides. J Cutan Pathol 2000; 27: 406-9.

17 Hitchcock MG, Burchette JL, Olsen EA, et al. Eccrine gland infiltration by mycosis fungoides. Am J Dermatopathol 1996; 18: 447-53.