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BMJ Case Rep. 2010; 2010: bcr07.2009.2064.
Published online Feb 19, 2010. doi:  10.1136/bcr.07.2009.2064
PMCID: PMC3029424
Unusual association of diseases/symptoms
Jessner’s lymphocytic infiltrate: a rare cause of lid ectropion
Segun Awotesu,1 Vincent Dubois,1 Nabil El-Hindy,2 and Mark Watts1
1Arrowe Park Hospital, Ophthalmology Department, Arrowe Park Road, Upton, Wirral CH49 5PE, UK
2Norfolk & Norwich, Ophthalmology Department, Colney Lane, Norwich NR4 7UY, UK
Correspondence to Segun Awotesu, doctorawo/at/googlemail.com
Abstract
Ectropion is a condition in which the lower eyelid turns outwards from the globe. It is commonly due to age related involutional laxity of the lid tissue. Ectropion may also be secondary to scarring or contracture of the periorbital skin and underlying tissues which pulls the eyelids outwards. Jessner’s lymphocytic infiltrate is a rare and enigmatic dermatological condition which is uncommon in the elderly. We describe a case of recurrent ectropion following redo lid surgery in which Jessner’s lymphocytic infiltrate was fortuitously diagnosed on skin biopsy. We are unaware of any report in the literature describing recurrent cicatrical ectropion due to this condition.
Lid ectropion is the abnormal eversion of the eyelid away from the globe. It may be caused by involutional, cicatricial paralytic or mechanical causes. The most common cause is due to age related involutional laxity of the lid tissue. The condition usually manifests with symptoms ranging from mild ocular irritation to conjunctival keratinisation. Treatment involves topical lubrication to alleviate discomfort and prevent corneal surface exposure. Surgical correction aims to correct the underlying horizontal lid laxity and usually achieves a good functional and cosmetic result.
Jessner’s lymphocytic infiltrate is a rare dermatological condition which infrequently presents in the elderly and has not previously been reported in the literature to cause cicatricial ectropion. We describe a case of recurrent bilateral lower lid ectropion after surgery in an elderly patient. Jessner’s lymphocytic infiltrate was subsequently diagnosed following skin biopsy. This case highlights the importance of considering alternative causes for ectropion in the elderly, including cicatrising dermatological conditions such as Jessner’s lymphocytic infiltration. These conditions carry a higher risk of surgical failure and patients should be counselled appropriately preoperatively.
A 78-year-old man was referred to the oculoplastics clinic with a 12 month history of bilateral painless runny eyes. His medical history included hypertension controlled on 75 mg irbesartan once daily. He was a non-smoker and did not consume alcohol. The family history was not significant.
Examination revealed bilateral anterior lid margin inflammation and right lower lid ectropion. Probing and irrigation of the nasolacrimal system revealed no obstruction. Surgical intervention was not discussed and the patient was discharged from the clinic with advice on how to tackle his lid margin inflammation by regular gentle cleansing and warm compresses.
The patient presented 9 months later with worsening symptoms. Examination revealed bilateral periorbital eczematous skin changes with pronounced contracture in the vertical meridian. Hydrocortisone ointment 1.0% three times daily was prescribed. Review 6 weeks later revealed no improvement and he subsequently underwent left sided ectropion repair with full thickness clavicular skin graft with a view to proceeding to the contralateral side at a later date. A dermatology referral was made for a co-existing eczematous non-malar facial rash unresponsive to a combined triamcinolone acetonide and nystatin preparation.
Investigations
Routine biochemistry and haematology blood tests were unremarkable. A punch biopsy was performed on one of the facial lesions and the resulting histology results were diagnostic of Jessner’s lymphocytic infiltrate. In view of this and the patient’s unremarkable medical history, further blood investigations were not requested.
Differential diagnosis
  • Involutional ectropion
  • Cicatrical ectropion
  • Eczema
  • Seborrhoiec dermatitis.
Treatment
Treatment with clobetasone butyrate ointment 0.05% twice daily and emollients resulted in pronounced clinical improvement.
The patient subsequently underwent right ectropion repair with skin graft. At his postoperative visit signs of early graft failure, including crusting and hypoperfusion, were noted on the left. Follow up at 3 months revealed symptomatic improvement, but bilateral lower lid ectropion due to contracture of the grafts. We believe that his subjective improvement is likely to be secondary to improved ocular surface integrity from applying topical lubricants.
The patient was not keen on further surgical intervention to address the lower lid malposition. He was prescribed Carbomer based ocular lubricants to prevent drying of the exposed conjunctiva and discharged.
The patient re-attended the oculoplastics clinic 18 months later complaining of worsening watering eyes and discomfort despite intensive ocular lubrication. Examination revealed facial eczema and considerable bilateral lower lid cicatrical ectropion (fig 1). The guarded surgical prognosis was fully explained and he is awaiting repeat ectropion repair surgery.
Figure 1
Figure 1
Preoperative facial photograph showing bilateral lid ectropion and tarsal conjunctival exposure.
This rare skin condition was first described in 1953 by Jessner and Kanof.1 It is characterised clinically by flat erythematous plaques and papules commonly affecting the head and upper trunk. The lesions vary in size from 2 mm to 2 cm in diameter, and may be arranged in crescents or rings. These may be solitary or numerous and do not typically cause significant symptoms.2 The condition usually occurs in the fourth and fifth decade of life with lesions occurring slightly earlier in females.3
The condition may wax and wane with remissions and exacerbations. In some patients the lesions may persist for several years and eventually disappear without sequelae. The lesions have been known to reappear at the original site or in previously unaffected areas.
Histologically, the condition is characterised by the presence of a normal epidermis and perivascular dermal lymphocytic infiltrate (fig 2). Immunohistochemical studies have identified that the infiltrates consist of predominantly mature T lymphocytes4 (fig 3).
Figure 2
Figure 2
Skin biopsy: perivascular moderate chronic inflammatory cell infiltrate, with no involvement of the epidermis.
Figure 3
Figure 3
Perivascular CD3 positive T cells (brown cells) identified on immunochemistry.
The gold standard for diagnosis is a skin biopsy performed on a new untreated lesion. Photo-testing with ultraviolet (UV) A or UVB can elicit typical lesions in some patients with Jessner’s lymphocytic infiltrate and has led to the hypothesis that the condition may be a variant of lupus erythematosus.5
Ectropion typically occurs as a result of age related horizontal lid and canthal tendon laxity. Studies have shown a close correlation with increasing age with a prevalence as high as 17% in those aged 80 years or older.6
Current management of Jessner’s lymphocytic infiltrate includes topical and systemic steroids, oral antimalarials and cryotherapy of small solitary lesions. Successful treatment of a case using a pulsed dye laser has also been described.7
This case is most unusual as Jessner’s lymphocytic infiltration rarely presents in the elderly. A comprehensive personal and family history did not identify any other possible causes for his cicatricial skin changes. We were unable to identify any other reports in the literature of Jessner’s lymphocytic infiltration causing a recurrent bilateral lid ectropion.
Clinicians are advised to be aware of this possible presentation as the clinical appearance may mimic an age related involutional ectropion. Surgery for cicatricial ectropion carries a higher risk of surgical failure than for involutional ectropion, and thus patients should be counselled appropriately preoperatively.8
Learning points
  • Ectropion occurs commonly in the elderly, frequently as a result of age related involutional skin changes.
  • Surgical correction is usually successful with dramatic functional and cosmetic improvement.
  • Jessner’s lymphocytic infiltrate is a rare dermatological condition which can affect the periorbital skin and cause severe cicatrical ectropion. This may be mistaken for an age related ectropion, particularly in the elderly.
  • The surgical outcome in cases of cicatrising ectropion due to Jessner’s lymphocytic infiltrate is poorer than in age related ectropion, and clinicians managing such patients should provide appropriate counselling.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
1. Jessner M, Kanof NB. Lymphocytic infiltration of the skin. Arch Dermatol 1953; 68: 447–9.
2. White GM, Cox NH. Diseases of the skin: a colour atlas and text, 2nd ed Mosby Publishing, 2005: 178–79.
3. Toonstra J, Wildschut A, Boer J, et al. Jessner’s lymphocytic infiltration of the skin. A clinical study of 100 patients. Arch Dermatol 1989; 125: 1525–30. [PubMed]
4. Willemze R, Dijkstra A, Meijer C. Lymphocytic infiltration of the skin (Jessner): a T-cell lymphoproliferative disease. Br J Dermatol 1984; 110: 523–9. [PubMed]
5. Weber F, Schmuth M, Fritsch P, et al. Lymphocytic infiltration of the skin is a photosensitive variant of lupus erythematosus: evidence by phototesting. Br J Dermatol 2001; 144: 292–6. [PubMed]
6. Mitchell P, Hinchcliffe P, Wang JJ, et al. Prevalence and associations with ectropion in an older population: the Blue Mountains Eye Study. Clin Experiment Ophthalmol 2001; 29: 108–10. [PubMed]
7. Borges da Costa J, Boixeda P, Moreno C. Pulsed-dye laser treatment of Jessner lymphocytic infiltration of the skin. J Eur Acad Dermatol Venereol 2009; 23: 595–6. [PubMed]
8. Liebau J, Schulz A, Arens A, et al. Management of lower lid ectropion. Dermatol Surg 2006; 32: 1050–6. [PubMed]
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