Dr Dinesh Selva Oculoplastic and Orbital Unit Department of Ophthalmology Royal Adelaide Hospital North Terrace Adelaide 5000 South Australia Australia Tel: + 61 8 8222 5222 Fax: + 61 8 8222 5221 Email: Awestwoo@mail.rah.sa.gov.au
Purpose: To present a patient with rosacea lymphoedema of one upper eyelid resulting in unilateral complete ptosis.
Methods: A 51-year-old white man presented with a 12-month history of progressive painless swelling of the left upper eyelid. An incisional biopsy of the upper eyelid was performed.
Results: The biopsy showed dermal oedema with lymphangiectasia and telangiectasia, accompanied by a mild to moderate mixed chronic inflammatory infiltrate of lymphocytes, histiocytes, plasma cells and rare eosinophils. Stains for fungi and mycobacteria were negative. The lack of lichenoid reaction, dermal mucin or lip swelling indicated a lymphoedematous manifestation of rosacea. The patient was treated with minocycline and prednisolone with no effect.
Conclusion: Rosacea lymphoedema involving the eyelid, as in our case, is a rare complication and can present diagnostic and therapeutic challenges to the ophthalmologist.
Acne rosacea is a chronic idiopathic dermatosis typically affecting the central region of the face in fair-skinned individuals. The peak prevalence is in the fourth decade, with women being more commonly affected than men (Berg & Linden 1989). It is characterized by erythema, telangiectasia, papules, pustules, chronic facial oedema and sebaceous gland hyperplasia. We describe a patient with rosacea lymphoedema of one upper eyelid, resulting in unilateral complete ptosis, in contrast to two previously reported periocular cases with predominantly lower eyelid involvement (Bernardini et al. 2000).
A 51-year-old white man presented with a 12-month history of progressive painless swelling of the left upper eyelid. Past medical history included rosacea of granulomatous type, depression and bilateral nephrocalcinosis. Systemic review was negative for past ocular trauma, angio-oedema, erysipelas, systemic lupus erythematosus, myxoedema, dermatomyositis, thyroid disease or sarcoidosis. The patient had been intermittently treated with oral minocycline for forehead rosacea with good effect prior to presentation.
Examination revealed near complete left ptosis (interpalpebral fissure of 2 mm), with erythema and non-tender, non-pitting oedema affecting the left upper eyelid (Fig. 1). Extraocular muscle function was normal bilaterally. Best spectacle corrected visual acuity was 20/30 in the right eye and 20/200 in the left (known amblyopia). Intraocular pressure was normal in both eyes, as well as the anterior ocular segment. Fundoscopic examination was unremarkable. Central forehead rosacea was evident. There were no orbital signs. Laboratory studies revealed normal full blood examination, erythrocyte sedimentation rate, C-reactive protein and electrolytes except for a chronically raised urea and creatinine level secondary to bilateral nephrocalcinosis. Computerized tomography and magnetic resonance imaging of the orbits showed preseptal oedema but no evidence of orbital pathology. An incision biopsy of the forehead revealed some plugged follicular ostia, mild sebaceous hyperplasia, superficial dermal telangiectatic vessels and a perivascular and perifollicular mixed inflammatory infiltrate of lymphocytes and plasma cells. A few perifollicular histiocytes and giant cells were noted as changes characteristic of rosacea. An incision biopsy of the left upper eyelid showed less distinct follicular changes (Fig. 2). Instead, dermal oedema with lymphangiectasia and telangiectasia was noted, accompanied by a mild to moderate mixed chronic inflammatory infiltrate of lymphocytes, histiocytes, plasma cells and rare eosinophils. A couple of ill-defined perivascular granulomas were detected and stains for fungi and mycobacteria were negative. Taken in conjunction with the forehead biopsy findings, lack of lichenoid reaction, dermal mucin or lip swelling, a lymphodermatous manifestation of rosacea was suggested. The patient was trialed on prednisolone 50 mg daily for 1 month with no effect and then elected to have no further treatment as he was not troubled by the ptosis. At follow-up, 6 months later, there was no change in the clinical findings.
Ocular involvement in acne rosacea is common, affecting 3−58% of patients with rosacea, depending on the series. Of these, 20% experience ocular manifestations as the initial presentation of rosacea (Browning & Proia 1986). Blepharitis and conjunctivitis are the most common presentations. Rosacea lymphoedema involving the eyelid, as in our case, is a rare complication that may occur at any stage. It usually manifests as an insidiously progressive non-pitting swelling, having a symmetrical or, less commonly, an asymmetrical distribution, with variable degrees of accompanying erythema and telangiectasia. In the dermatologic literature, this condition is referred to as ‘solid facial oedema of rosacea’ or ‘persistant facial oedema of rosacea’ as a new nomenclature in dermatology literature for rosacea lymphoedema (Holzle et al. 1995; Uhara et al. 2000).
The pathogenesis of lymphoedema remains unclear. One of the theories is based on the chronic inflammatory process that accompanies this disease. As a result of the inflammatory process there may be destruction of elastin around blood vessels, resulting in transudation of fluids, or it may lead to fibrosis and permanent obstruction of deep dermal lymphatic vessels with fluid accumulation, congestion and lymphoedema of the underlying tissue (Wilkin 1994).
The histologic findings of rosacea lymphoedema have not been well described in the literature. Reported histologic findings include dilated blood vessels, perifollicular fibrosis, perivascular and perifollicular lymphocytes, histiocytes, neutrophils and stromal oedema (Wilkin 1994; Chen & Crosby 1997; Bernardini et al. 2000). Our case had striking vascular and lymphangiectasia with a mixed chronic inflammatory cell infiltrate. We did not identify a neutrophilic component or fibrosis, but lymphocytoclasis in swollen lymphatic channels and a couple of perilymphatic poorly formed granulomas are likely contributors to lymphoedema in this case.
The differential diagnosis of chronic facial oedema is wide and may include several groups of diseases (Bernardini et al. 2000). The possible aetiologies are inflammatory (acne vulgaris, systemic lupus erythematosus, angio-oedema, allergic dermatitis, dermatomyositis), infectious (erysipelas, herpes zoster), congenital (facial hemiatrophy, Apert's syndrome), malignant (angiosarcoma, lymphoma, mycosis fungoides, Kaposi's sarcoma), hypothyroidism and nephrotic syndrome.
Recommended medical treatments for acne rosacea include topical metronidazole and oral macrolide such as doxycycline or minocycline. The use of systemic corticosteroids or isotretinoin may be required in severe and resistant cases (Bernardini et al. 2000). However, these treatments are generally ineffective in rosacea-induced chronic lymphoedema (Bernardini et al. 2000), as in our patient, who failed to respond to oral prednisolone. The successful use of surgical debulking has been reported in two cases of periocular rosacea lymphoedema (Bernardini et al. 2000). It is interesting to mention a recent case report which describes successful treatment with quinolones (fleroxacin) in a patient with rosacea, resulting in marked reduction in both solid facial oedema and periorbital oedema (Uhara et al. 2000).
In conclusion, rosacea lymphoedema is a rare complication of acne rosacea that may affect the upper eyelid and that presents both diagnostic and therapeutic challenges to the ophthalmologist.