Pediatric Dermatology Vol. 21 No. 5 558–560, 2004
Unilesional (Segmental) Mycosis Fungoides Presenting in Childhood
Blackwell Publishing, Ltd.
Qasem A. Alsaleh, M.D.,* Arti Nanda, M.D., N.B.E.,* Hisham Baker, M.D., D.M.R.T., Ph.D.,† Humood Al-Sabah, M.D.,* and Eduardo Calonje, M.D.‡ *As’ad Al-Hamad Dermatology Center and †Kuwait Cancer Control Center, Al-Sabah Hospital, Kuwait; and ‡Department of Dermatopathology, St. John’s Institute of Dermatology, St. Thomas’ Hospital, London, England
Abstract: Mycosis fungoides is rare in children, and a unilesional presentation is also rare. A 13-year-old Kuwaiti boy with unilesional mycosis fungoides is described. Clinically he had a single indurated large plaque on the left shoulder with histopathologic features typical of cutaneous T-cell lymphoma. The diagnosis was further supported by the presence of a T-cell clone discovered through molecular biology studies of paraffin-embedded material. No other lesions were detected. The lesion showed a favorable response to local radiotherapy.
Unilesional cutaneous T-cell lymphoma refers to a rare variant of cutaneous T-cell lymphoma that is clinically characterized by a solitary lesion with histopathologic features indistinguishable from those of mycosis fungoides (MF). Limited involvement of skin with a single lesion has been described as Woringer-Kolopp disease (1–3). Woringer-Kolopp disease is a distinct entity that differs from classic MF and from unilesional MF by the presence of striking epidermotropism and a paucicellular dermal infiltrate (4–11). Unilesional MF has only rarely been reported in children (12). We report a child with unilesional MF who had unique clinical and histopathologic features. CASE REPORT A 13-year-old Kuwaiti boy presented to the outpatient dermatology department of As’ad Al-Hamad Dermatology Center in February 2001, with a 10-year history of a hyperpigmented, indurated plaque measuring 8 cm × 10 cm on the left scapular area and extending onto the left Address correspondence to Qasem A. Alsaleh, M.D., P.O. Box: 17296, Khaldeyah 72453, Kuwait, or e-mail:
[email protected].
558
upper arm (Fig. 1). The lesion began as an erythematous scaly plaque that slowly increased in size and became hyperpigmented. At presentation he also had two small mobile palpable axillary lymph nodes (1.5–2 cm in diameter) on the ipsilateral side. The clinical differential diagnosis included morphea and Becker nevus. A skin biopsy was performed and scanning magnification of the specimen showed mild psoriasiform hyperplasia of the epidermis with focal interface change and a superficial and deep perivascular and interstitial cell infiltrate. Of interest is that the low-power appearance of the dermal infiltrate was reminiscent of granuloma annulare. Closer examination revealed focal migration of lymphocytes into the epidermis with a predilection for the basal cell layer (Fig. 2). In the latter, the lymphocytes had a focal linear arrangement. Spongiosis was minimal and the infiltrating lymphocytes displayed some degree of nuclear irregularity. The dermal infiltrate consisted of lymphocytes, occasional larger lymphoid cells with irregular nuclei, histiocytes, and occasional plasma cells (Fig. 3). Giant cells and necrobiosis were not seen.
Alsaleh et al: Unilesional Mycosis Fungoides 559
Figure 1. An indurated, hyperpigmented plaque affecting the left scapular area and adjacent upper arm.
Figure 3. A marked infiltrate of abnormal lymphocytes extends into the lower dermis in a perivascular and interstitial pattern (hematoxylin-eosin; magnification 40×).
The plaque was treated with local radiotherapy (40 Gy × 20 fractions) and became progressively less indurated and cleared completely after a few weeks. The patient continues to remain clear, with no evidence of a recurrence of lesions more than 1 year after treatment. DISCUSSION
Figure 2. Abnormal lymphocytes are seen in the upper dermis, within the epidermis, and along the basal cell layer (hematoxylin-eosin; magnification 40×).
Immunohistochemical studies revealed that most of the cells in the infiltrate stained with pan-T-cell markers, including CD2, CD3, CD5, and CD7. Most of these T cells were positive for CD4, indicating a helper phenotype. Only occasional cells stained for CD8, and staining for CD20 and CD30 was negative. Polymerase chain reaction (PCR) analysis of DNA obtained from the paraffin-embedded tissue demonstrated a T-cell clone (V3 clone). One of the enlarged axillary lymph nodes was excised and histologic examination showed follicular hyperplasia only. A bone marrow biopsy specimen was normal. Various other investigations, including complete blood counts, serum biochemistry, human immunodeficiency virus (HIV) serology, hepatitis screening, serum immunoelectrophoresis, computerized tomography (CT) scan of chest and abdomen, and total body gallium scan, were all normal or negative. We made the final diagnosis of unilesional MF in a segmental distribution.
Unilesional MF was first described in 1981 (4) and since then a small number of reports have appeared in the literature describing this presentation as a rare and distinct variant of MF that differs from Woringer-Kolopp disease both clinically and histologically (4–12). Clinically Woringer-Kolopp disease usually presents as a single verrucous plaque often arising in the extremities. Histologically it is characterized by a significant epidermotropism of atypical lymphocytes with a relative paucity of dermal infiltrate. Unilesional MF, in contrast, has diverse clinical presentations, including a psoriasiform plaque, an indurated plaque, an eczematous lesion, a lesion with prominent follicular involvement, a poikilodermatous patch, and a hypopigmented macule. Any part of the body may be affected and the histologic features are indistinguishable from those of classic MF. Our patient presented with an indurated plaque on the scapular area and histologic features were very suggestive of MF. The finding of a clonal population of T cells by PCR further supported the diagnosis. The presence of a superficial and deep infiltrate containing atypical lymphocytes in a perivascular and interstitial pattern reflected the marked clinical induration of the lesion. The low-power appearance of this infiltrate was reminiscent of granuloma annulare. This histopathologic pattern, although rare, has been well described in MF (13). Our case is also unusual because the disease presented in a
560 Pediatric Dermatology Vol. 21 No. 5 September/October 2004
13-year-old child in which the lesion had been present since the age of 3 years. MF is rare in children (14) and unilesional MF is even more rarely reported in children (12). Interestingly, juvenile MF often has a CD8+ phenotype (14,15), in contrast to our patient, who had a CD4+ phenotype. The pathogenesis of localized forms of cutaneous T-cell lymphoma is not known. In Woringer-Kolopp disease it has been proposed that the disease arises from the proliferation of an abnormal clone within the epidermis, while solitary MF results from the invasion of the epidermis by atypical lymphocytes (4,6,7). Microdissection studies of early classical MF, however, have suggested that clonal lymphocytes may develop within the epidermis in the presence of a polyclonal population of dermal T lymphocytes (16). Unilesional MF appears to run an indolent course related to small tumor burden and by the presence of an antitumor response (11,17). The treatment of choice in unilesional MF is either local superficial radiotherapy or excision. As in our patient, the majority of those affected respond well to treatment, with no evidence of recurrence, but follow-up is often limited (11,12,18,19). It is reasonable to assume that the behavior of unilesional MF is similar to that of most cases of patch stage MF with an indolent course. In summary, our patient is an example of childhoodonset cutaneous T-cell lymphoma, a rare and distinct variant of MF. REFERENCES 1. Lever WF. Localized mycosis fungoides with prominent epidermotropism: Woringer-Kolopp disease. Arch Dermatol 1977;113:1254 –1256. 2. Mandojana RM, Helwig EB. Localized epidermotropic reticulosis (Woringer-Kolopp disease): a clinicopathologic study of 15 new cases. J Am Acad Dermatol 1983;8:813– 829. 3. Burns MK, Chan LS, Cooper KD. Woringer-Kolopp disease (localized pagetoid reticulosis) or unilesional mycosis fungoides? An analysis of 8 cases with benign disease. Arch Dermatol 1995;131:325 –329. 4. Russell JR, Chu A. Pagetoid reticulosis and solitary mycosis fungoides: distinct clinicopathological entities. J Cutan Pathol 1981;8:40 –51.
5. Rigel E, Medenica M, Lorinz A. Localized mycosis fungoides not manifesting as Woringer-Kolopp disease. Arch Dermatol 1983;119:756–760. 6. Oliver GF, Winkelmann RK, Banks PM. Unilesional mycosis fungoides: clinical, microscopic and immunophenotypic features. Australes J Dermatol 1989;30:65–71. 7. Oliver GF, Winkelmann RK. Unilesional mycosis fungoides: a distinct entity. J Am Acad Dermatol 1989;20:63–70. 8. Evans LT, Mackey SL, Vidmar DA. An asymptomatic scaly plaque. Unilesional mycosis fungoides (MF). Arch Dermatol 1997;133:234. 9. Yerret JL, Rousselet MC, Peria P. Unilesional plaque-type mycosis fungoides: 3 cases. Ann Dermatol Venereol 1997;124:527–530. 10. Marzano AV, Berti E, Lupia L, Alessi E. Unilesional follicular mycosis fungoides. Dermatology 1999;199:174–176. 11. Heald PW, Glusac EJ. Unilesional cutaneous T-cell lymphoma: clinical features, therapy, and follow-up of 10 patients with a treatment-responsive mycosis fungoides variant. J Am Acad Dermatol 2000;42:283–285. 12. Hodak E, Phenig E, Amichai B, et al. Unilesional mycosis fungoides: a study of seven cases. Dermatology 2000;201:300–306. 13. Shapiro PE, Pinto FJ. The histologic spectrum of mycosis fungoides/Sézary syndrome (cutaneous T-cell lymphoma). A review of 222 biopsies, including newly described patterns and the earliest pathologic changes. Am J Surg Pathol 1994;18:645–667. 14. Whittam LR, Calonje E, Orchard G, et al. CD8-positive juvenile onset mycosis fungoides: an immunohistochemical and genotypic analysis of six cases. Br J Dermatol 2000;143:1199–1204. 15. Shabrawi-Caelen LE, Cerroni L, Medenos LJ, McCalmont TH. Hypopigmented mycosis fungoides: frequent expression of a CD8 positive T-cell phenotype. Am J Surg Pathol 2002;26:450–457. 16. Carroni J, Arzherger E, Ardigo M, Putz B, Kerl H. Monoclonality or intraepidermal lymphocytes in early mycosis fungoides detected by molecular analysis after laser-beam based microdissection. J Invest Dermatol 2000;114:1154– 1157. 17. Berger CL, Wang N, Christensen I, Longley J, Heald P, Edelson RI. The immune response to class I associated tumor-specific cutaneous T-cell lymphoma antigens. J Invest Dermatol 1996;107:392–397. 18. Wilson LD, Kancinski BM, Jones GW. Local superficial radiotherapy in the management of minimal stage A cutaneous T-cell lymphoma (mycosis fungoides). Int J Radiat Oncol Biol Phys 1998;40:109–115. 19. Micaily B, Miyamoto C, Kantor G, et al. Radiotherapy for unilesional mycosis fungoides. Int J Radiat Oncol Biol Phys 1998;42:361–364.
