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Anaplastic large-cell lymphoma (ALCL) is a T-cell lymphoma characterized by large, CD30-positive lymphoid cells with abundant cytoplasm and pleomorphic nuclei. Cutaneous ALCL can be either a primary ALCL or a secondary presentation of systemic ALCL. Both types of cutaneous ALCL rarely involve the periocular skin, and are often underdiagnosed. We report a case of primary cutaneous ALCL involving the eyelid, which mimicked preseptal cellulitis.

A 29-year-old man presented with a 1-month history of progressive swelling and ulceration on his face.

On physical examination, he was found to have extensive ulceration and swelling of the right eyelid (Fig. 1). Following a diagnosis of preseptal cellulitis, he was treated intensively with intravenous antibiotics, but despite this treatment, the degree of periorbital and facial swelling increased.

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Figure 1. Right periorbital and ill-defined diffuse facial swelling, with a palpable eyebrow nodule, and multiple grouped vesicles, erosions and crusts.

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On histological examination of a biopsy taken from the periorbital area, large, multinucleated, pleomorphic cells with hyperchromatic nuclei were seen, which were CD30-positive. These cells were characteristic of ALCL (Fig. 2). The patient was transferred to the oncology department of our institution, where curative chemotherapy with a cyclophosphamide, doxorubicin, vincristine and prednisolone regimen was started.

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Figure 2. (a) Dense lymphohistiocytic and moderate eosinophilic infiltration (haematoxylin and eosin, original magnification × 400). (b) CD30-positive atypical lymphoid cell infiltration (original magnification × 400).

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The differential diagnosis of unilateral eyelid erythema and oedema is broad, and includes cellulitis, herpes zoster infection, insect bite, hordeolum, dacryocystitis, and malignant disorders such as lymphoma. Periorbital cellulitis is an uncommon, potentially devastating condition that can lead to serious sequelae, including blindness, intracranial infection and even death. Periorbital cellulitis is classified into preseptal or postseptal cellulitis depending on its relationship with the orbital septum. Compared with postseptal cellulitis, preseptal cellulitis is characterized by periorbital swelling and erythema without proptosis, ophthalmoplegia and loss of vision.

Primary cutaneous ALCL of the eyelid is very uncommon, and is often misdiagnosed owing to the similarity between the clinical features of ALCL and preseptal cellulitis. To our knowledge, only six cases of primary cutaneous ALCL of the eyelid have been reported previously (Table 1).[1-5] Almost all of the patients in these reports presented with an ulcerated inflamed nodule on the eyelid, and the initial diagnosis in all six cases was of preseptal cellulitis. As with our patient, the lesions in these patients failed to respond to systemic antibiotics. Following the diagnosis of ALCL, all patients underwent complete excision, along with chemotherapy or radiotherapy. They remained clinically and radiographically free of disease after treatment, and all had favourable outcomes.

Table 1. Clinical data of the six patients with primary cutaneous anaplastic large cell lymphoma on the eyelid previously reported in the English literature
PatientAge, years/ genderSymptoms (all on eyelid)TreatmentPrognosis
  1. CR, complete response.

118/MInflamed noduleObservationSpontaneous remission
245/FTender noduleLocal excision Spontaneous remission
310/MFirm ulcerated noduleLocal excision and chemotherapyCR (9-month follow-up)
431/FUlcerated skin lesionLocalized radiotherapyCR (6-month follow-up)
551/FSwollen and ulcerated skin lesionLocalized radiotherapyCR (6-month follow-up)
629/MUlcerated, nontender lesionLocalized radiotherapyCR (7-month follow-up)

The clinical features of primary cutaneous ALCL of the eyelid, which include painful inflamed periorbital swelling, can closely mimic those of preseptal cellulitis. The distinctive features of primary cutaneous ALCL include ulcerated nodules and a failure to respond to systemic antibiotics.

In conclusion, we report a patient with ALCL of the eyelid, initially diagnosed as preseptal cellulitis. Dermatologists need to be aware of this condition and perform adequate testing to ensure the correct diagnosis and treatment of potential cutaneous ALCL.

References

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  2. References
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    Koestinger A, McKelvie P, McNab A. Primary cutaneous anaplastic large-cell lymphoma of the eyelid. Ophthal Plast Reconstr Surg 2012; 28: e1921.
  • 2
    Koreen IV, Cho RI, Frueh BR, Elner VM. Primary cutaneous anaplastic large cell lymphoma of the medial canthus and orbit. Ophthal Plast Reconstr Surg 2009; 25: 635.
  • 3
    Sanka RK, Eagle RC Jr, 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: 34351.
  • 4
    Segal ZI, Cohen I, Szvalb S et al. Solitary extranodal anaplastic large cell lymphoma, Ki-1+, of the eyelid. Am J Ophthalmol 1997; 124: 1057.
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    Tanzi E, Edelman M, Rosenbaum PS. Ki-1-positive anaplastic large-cell lymphoma of the eyelid. Arch Ophthalmol 1999; 117: 9558.