Download Corneal Involvement In A Case Of Mycosis Fungoides

Brief Report
Sanitha Sathyan, Aneeta Jabbar
Corneal Involvement In A Case Of
Mycosis Fungoides - Case Report
A 42 year old gentleman, who was diagnosed as Mycosis
fungoides in 2006, currently undergoing radiotherapy,
presented to our OPD with complaints of pain, watering and
defective vision in left eye of 1 month duration. He was under
the care of a local ophthalmologist and was on systemic
and topical acyclovir, topical antibiotics, cycloplegics and
preservative free lubricants for 1 week.
On examination, he had generalized hyperpigmented
plaques over the body and face including the periorbital
region. His visual acuity was: OD: 6/6, OS: 6/18 . OD: upper
lid showed an ulcerative plaque like lesion 2 mm from the
anterior lid margin with localized keratinization of the skin.
Anterior segment and fundus were otherwise unremarkable.
fig:3 showing partly epithelialised lesion in OS
Fundus examination in both eyes were within normal limits.
Our diagnosis was OD: Infiltration of upper lid secondary to
Mycosis fungoides, OS : Herpes Simplex Virus epitheliopathy
Corneal infiltration secondary to Mycosis fungoides was also
considered as a differential diagnosis.
He was continued on Acyclovir eye ointment for 2 weeks ,
Prednisolone acetate 1% eye drops QID and preservative
free lubricant eye drops. Since his was a biopsy proven case
of Mycosis fungoides, futher biopsy from the lid lesion was
deferred.
fig: 1 showing the lid lesion in OD
OS: There was a linear epithelial defect in the inferior
paracentral cornea simulating a dendrite. Stroma surrounding
the epithelial lesion was edematous, with cellular reaction
and Descemet’s level folds. There was stromal scarring
nasally with punctate keratitis There was no vasularization.
At one week review, the corneal lesion was resolving with
mid stromal scar and mild punctate keratitis. Acyclovir
eye ointment was stopped after 3 weeks and topical
steroids tapered. At one month follow up, the lesion healed
completely with mid stromal scaring.
Discussion
Mycosis fungoides is a malignant cutanoeus T- cell lymphoma
characterized by clonal expansion of T helper cells and rarely
T-suppressor or natural killer cells, that usually present as a
widespread chronic cutaneous eruption, which progresses
to form plaques and tumours. Eventually non-cutaneous
involvement supervenes with infiltration of lymph nodes and
visceral organs[1].
fig:2 showing the linear lesion, partly scarred in OS
Ocular involvement may occur in up to a third of advanced
cases of Mycosis fungoides and most frequently presents
as tumours or infiltration of the eyelids [2] Less frequent
manifestations include involvement of the caruncle[3] the
cornea[2] the sclera[4] the optic disc[5] and optic nerve[6]
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Address for Correspondance: Cataract and Cornea Services, Little Flower Hospital and Research Centre, Angamaly
Sanitha Sathyan et al - Case Report - Mycosis Fungoides
and the orbit[7].Intraocular involvement has been reported
only rarely[5,8].
pointed more towards a self resolving variant of corneal
Corneal involvement is a rare occurrence in Mycosis
fungoides. This was first reported by Besnier and Hallopeau
in (1892) and histologically confirmed by Herman in 1951.
Occasionally this may be the first evidence of the disease, but
usually it is seen in well developed cases and occasionally as
a terminal event.
clinical suspicion is needed in the diagnosis of the unusual
Corneal manifestations vary. They may appear as a regular
linear infiltrate, somewhat resembling a dendrite, which
progresses to an ulcerative keratitis involving the stroma and
Descemets membrane.Alternatively progressive and usually
heavily vascularised stromal infiltrates appear which may
suffer acute exacerbations and may breakdown to ulceration
or undergo spontaneous resolution.
Pathological examinations have demonstrated the typical
infiltration characterized by large mononuclear cells of the
primitive lymphoblastic type.
This case was reported because of the rare corneal
presentation of Mycosis fungoides. Antivirals were used
in the treatment keeping in view of the more common
herpetic keratitis. But the lesion lacked the typical dendritic
borders and terminal bulbs. Reference to the older literature
involvement in Mycosis fungoides. Therefore a high index of
presentation of this rare disease.
References
1. Lever WF, Schaumberg-Lever G. Histopathology of skin.
Philadelphia: Lippincott, 1990: 819-27.
2. Stenson S, Ramsay DL. Ocular findings in mycosis fungoides. Arch
Ophthalmol 1981; 99: 272-7.
3. Fradkin AH, Ruiz RS, Sloane JA. Mycosis fungoides involving the
caruncle. AmJ Ophthalmol 1969;
68: 719-22.
4. Wolter JR, Leenhouts TM, Hendrix RC. Corneal involvement in
mycosis fungoides. AmJ Ophthalmol 3; 55: 317-22.
5. Keltner JL, Fritsch E, Cykiert RC, Albert DM. Mycosis fungoides:
intraocular
and
central
nervous
system
involvement.
Arch
Ophthalmol 1977; 95: 645-60.
6. Pariser DM. Mycosis fungoides involving the brain and optic nerves.
Arch Dermatol 1978; 114: 397-9.
7. Zucker JL, Doyle MF. Mycosis fungoides metastatic to the orbit.
Arch Ophthalmol 1991; 109: 688-91.
8. Forster HC. Mycosis fungoides with intraocular involvement.
TransAmAcad Ophthalmol Otolaryngol 1960; 64: 308-13.
Dr Sanitha Sathyan has finished her DNB at Little Flower Eye Hospital, Angamaly
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