Orbit, 2014; 33(5): 395–398 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2014.907813

C ASE REPORT

Lymphomatoid Papulosis Type C of the Eyelid in a Young Girl: A Case Report and Review of Literature Manju Meena1, Peter A. Martin1, Claire Abouseif2, and Peter Bullpitt2 1

Oculoplastic Unit, Department of Ophthalmology, Sydney Hospital and Sydney Eye Hospital, Macquarie Street, Sydney, NSW, Australia and 2South Eastern Area Laboratory Services, Prince of Wales Hospital, Sydney

ABSTRACT Purpose: To report an unusual presentation of a case of Lymphomatoid papulosis(LyP) in a young girl. Material and Methods: A 14-year-old female presented with a history of swelling of the left upper eyelid of two weeks duration. There was a history of trivial trauma prior to the swelling. The patient was diagnosed as having pre-septal cellulitis elsewhere and was put on oral antibiotics. The lesion was non-responsive to oral antibiotics. The patient was then referred to our hospital. Ocular examination revealed an elevated lesion measuring 15 mm  10 mm on the left upper eyelid, associated with pre-septal swelling and induration. Ocular movements were normal. The anterior and posterior segment examination was normal. Incision biopsy was done from the eyelid lesion. Multiple cutaneous lesions were also biopsied. Results: The histopathology examination confirmed the diagnosis of lymphomatoid papulosis type C. Dermatological and systemic evaluation ruled out the other aggressive forms of CD30(+) lymphoid proliferation. Conclusion: We report an unusual presentation of lymphomatoid papulosis(LyP) type C in a young girl. Complete systemic work up and histopathological evaluation is mandatory in cases of suspicious lesions, not responding to conservative treatment. Keywords: Histopathology, lymphomatoid, ocular, papulosis

INTRODUCTION

CASE REPORT

Lymphomatoid papulosis(LyP) is rhythmic paradoxical eruptions of erythematous papules and it has a benign clinical course.1 It was originally described by Macauley (1978) as a self-healing paradoxical eruption, histologically malignant but clinically benign.2 These lesions are usually symmetrically distributed and most commonly involve the trunk and extremities. We report a case of LyP type C of the eyelid presenting as pre-septal cellulitis in a young female. To the best of our knowledge, this is the first case report of LyP type C involving the eyelid region. The clinical, histopathological findings of LyP and a brief review of the literature will be discussed in this report.

A 14-year-old female presented with a history of painful non-resolving soft tissue swelling of the left upper eyelid of 2 weeks’ duration. There was a history of trivial trauma prior to the swelling. The provisional diagnosis of pre-septal cellulitis was made elsewhere. She was treated with oral Flucloxacillin 250 mg 4 times a day for 2 weeks. There was no subsequent improvement and the patient was referred. On examination the best corrected visual acuity(BCVA) was 20/20 in both eyes. There was an elevated lesion on the left upper eyelid, measuring 15  10 mm in dimensions, associated with surrounding pre-septal edema and induration (Figure 1). Ocular movements were full and the rest of the ocular examination was

Received 11 November 2013; Revised 8 March 2014; Accepted 20 March 2014; Published online 6 June 2014 Correspondence: Manju Meena, Oculoplastic Unit, Department of Ophthalmology, Sydney Eye Hospital, 8 Macquarie St., Sydney NSW 2000, Australia. E-mail: [email protected]

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FIGURE 1. Clinical photo shows an elevated lesion on the left upper eyelid, measuring 15  10 mm in dimensions, associated with surrounding pre-septal edema and induration.

unremarkable. An incisional biopsy of the lesion was performed in view of the unusual presentation and non-responsiveness to oral antibiotics. On microscopic examination the epidermis showed parakeratosis and basal lymphocytic epidermotrophism. The dermis showed heavy superficial and deep mixed infiltrate which includes numerous large atypical lymphoid cells. The nuclei were pleomorphic and bizzare with multinucleated forms. The infiltrate was both perivascular and interstitial and included moderate numbers of small- to medium-sized mildly atypical lymphoid cells. The large pleomorphic cells were positive for CD30 and CD45 and were ALK negative. CD 20 was negative and the CD3 and CD5 stained the background T lymphocytes and some of the large atypical cells were also stained with CD5. The CD68 stained background histiocytes and there were no plasma cells or eosinophils. There were scattered mitosis and Ki67 was 40% (Figures 2A–H). The T-cell receptor

FIGURE 2. (A-H) A) The epidermis shows parakeratosis and basal lymphocytic epidermotrophism. The dermis shows heavy superficial and deep mixed infiltrate which includes numerous large atypical lymphoid cells (H&E  20). B) The dermal infiltrate of atypical large lymphoid cells with pleomorphic nuclei including horseshoe and reniform forms (H&E  40). C-H). On immunohistochemistry, the large pleomorphic cells show positive staining for CD30, LCA and negative for ALK. The CD3 and CD5 stained the background T lymphocytes and some of the large atypical cells also stained with CD5. The Ki 67 index was 40%. Orbit

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FIGURE 3. The dermatological examination shows multiple pink-purple papulomatous cutaneous lesions involving the upper limbs.

analysis suggested the presence polyclonal T-cell receptor gene re-arrangement. The history was reviewed which revealed the presence of similar multiple lesions all over the body for about one year, with waxing and waning course. Dermatological examination showed multiple pink-purple papulomatous cutaneous lesions involving the upper, lower limbs and trunk (Figure 3). The systemic work-up was unremarkable. The patient was referred to a dermatologist, who performed a punch biopsy of the calf lesion that corresponded with the histopathological findings of the eyelid biopsy. Therefore, the diagnosis of Lymphomatoid Papulosis Type C was confirmed. The benign nature and prognosis of the disease was explained to the parents. The patient was then referred to a hematologist for further treatment.

DISCUSSION Cutaneous CD30+ lymphoid proliferations represents a distinct form of T-cell lymphoma which is characterized by the morphology (large and anaplastic) and immunophenotype (CD30+) of the tumor cells. They present as a spectrum of diseases composed of clinically indolent lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (cALCL), and aggressive systemic ALCL. Excluding classic mycosis fungoides (MF), primary cutaneous CD30+ lymphoid proliferations comprise approximately 30% of all cutaneous T cell lymphomas. CD30+ lymphoid proliferations of the ocular adnexa are rare as 1–3% of adnexal lymphomas are of nonB-cell type.3–5 LyP is classified according to the World Health Organization (WHO)-European Organization for !

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Research and Treatment of Cancer (EORTC) as a CD30+ lymphoproliferative disorder.6 Although it is not an aggressive malignant process, patients with LyP have an increased risk for developing lymphoma (10–20%) or a non-lymphoid tumour.7,8 LyP is characterized by cyclic eruptions of erythematous papulo-nodular lesions that are usually 510 mm in size with occasional central ulceration, followed by spontaneous healing and scar formation. It can affect patients of any age, the median age at presentation is 45 years.9 These lesions are most commonly located at the trunk and extremities.3 LyP lesions spare the mucous membrane and usually present in clusters, but may be single or disseminated. Spontaneous regression of the lesions occurs after 4 to 6 weeks and a hyper or hypo- pigmented scar may remain. LyP is usually not treated, although psoralen plus ultraviolet A or Low-dose methotrexate has been used for aggressive disease. LyP exhibits four histological types, and affected patients may simultaneously have more than one present.3,10,11 Type A is usually dense, wedge-shaped, and resembles Hodgkin’s disease with scattered infrequent large atypical CD30+ cells resembling Reed-Sternberg cells. These cells are surrounded by eosinophils and neutrophils, with the neutrophils present within vascular spaces. The epidermis is usually sparsely infiltrated and may be ulcerated. Type B is band-like or nodular and resembles MF with cerebriform cells. Neutrophils and eosinophils may be infrequently present. Type C resembles ALCL with clusters and sheets of CD30+ cells in the upper dermis with no fatty tissue infiltration. Type D simulates histologically an aggressive epidermotropic CD8-positive T-cell lymphoma. There are infiltrates of atypical lymphoid cells extending throughout the dermis and massive epidermotropism displaying a pagetoid reticulosis-like pattern and a CD8(+)CD30(+) cytotoxic T-cell phenotype.11 Recently, Kempf et al.12 described a newer variant called Type E LyP based on angiocentric and angiodestructive infiltrate of small-sized to mediumsized atypical lymphocytes expressing CD30+ and frequently CD8. Primary ocular adnexal CD30(+)lymphocytic infiltrates, are extremely rare. There have been four previously reported patients with LyP of the eyelids.13–16 And another study suggesting that a patient experienced a hemi-retinal vein occlusion secondary to systemic LyP.17 The unusual findings in our case were the presentation as pre-septal cellulitis, rare location of the lesion, younger age and rare variant of Lymphomatoid papulosis. The CD30(+) lymphoid proliferations represent a spectrum of conditions therefore, interpretation of the pathologic findings in CD30(+) lymphoid proliferations is based in part on clinical findings. We recommend that ocular

398 M. Meena et al. adnexal lesions with unusual clinical features should undergo incisional biopsy for correct diagnosis. Histopathological analysis plays an important role in differentiating LyP from more aggressive forms of CD30(+) lymphoid proliferation.

DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

REFERENCES 1. Kunishige JH, McDonald H, Alvarez G, et al. Lymphomatoid papulosis and associated lymphomas: a retrospective case series of 84 patients. Clin Exp Dermatol 2009;34:576–581. 2. Macaulay W L. Lymphomatoid papulosis. Int J Dermatol 1978;17:204–212. 3. Droc C, Cualing HD, Kadin ME. Need for an improved molecular/genetic classification for CD30+lymphomas involving the skin. Cancer Contr 2007;14:124–132. 4. Assaf C, Hirsch B, Wagner F, et al. Differential expression of TRAF1 aids in the distinction of cutaneous CD30positive lymphoproliferations. J Invest Dermatol 2007;127: 1898–1904. 5. Coupland SE, Foss HD, Assaf C, et al. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases. Ophthalmology 1999;106: 2109–2120. 6. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105: 3768–3785.

7. Cabanillas F, Armitage J, Pugh WC, et al. Lymphomatoid papulosis: A T-cell dyscrasia with a propensity to transform into malignant lymphoma. Ann Intern Med 1995;122: 210–217. 8. Wang HH, Myers T, Lach LJ, et al. Increased risk of lymphoid and nonlymphoid malignancies in patients with lymphomatoid papulosis. Cancer 1999;86:1240–1245. 9. Kunishige JH, McDonald H, Alvarez G, et al. Lymphomatoid papulosis and associated lymphomas: a retrospective case series of 84 patients. Clin Exp Dermatol 2009;34:576–581. 10. LeBoit PE. Lymphomatoid papulosis and cutaneous CD30+ lymphoma. Am J Dermatopathol 1996;18:221–235. 11. Cardoso J, Duhra P, Thway Y, Calonje E. Lymphomatoid papulosis type D: a newly described variant easily confused with cutaneous aggressive CD8-positive cytotoxic T-cell lymphoma. Am J Dermatopathol 2012;34: 762–765. 12. Kempf W, Kazakov DV, Scha¨rer L, et al. Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas. Am J Surg Pathol 2013;37:1–13. 13. Boulton JE, Chesterton JR, McGibbon DH. Symmetrical lymphomatoid papulosis masquerading as pyoderma chancriformis of the eyelids [letter]. Br J Ophthalmol 1995; 79:391–392. 14. Lemagne JM, Croughs P, Roumanos N, Maldague P. Lymphomatoid papulosis of the eyelid [inFrench]. J Fr Ophtalmol 1987;10:247–252. 15. Sanka RK, Eagle Jr RC, Wojno TH, et al. Spectrum of CD30+ lymphoid proliferations in the eyelid lymphomatoid papulosis, cutaneous anaplastic large cell lymphoma, and anaplastic large cell lymphoma. Ophthalmology 2010; 117:343–351. 16. Cloke A, Lim LT, Kumarasamy M, et al. Lymphomatoid papulosis of the eyelid. Semin Ophthalmol 2013;28:1–3. 17. Ramaesh K, Monaghan H, Kemmett D, et al. Retinal vein occlusion in lymphomatoid papulosis. Eur J Ophthalmol 2002;12:542–546.

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Lymphomatoid papulosis type C of the eyelid in a young girl: a case report and review of literature.

To report an unusual presentation of a case of Lymphomatoid papulosis(LyP) in a young girl...
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