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Blepharochalasis
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Blepharochalasis

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Contributors: Sid Schechet MD, D. Chimene Richa MD
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Synopsis

Blepharochalasis : Eyelid edema, Recurring episodes
Blepharochalasis is a rare inflammatory syndrome consisting of episodic, painless bouts of eyelid edema and erythema lasting a few days at a time. Quiescence can last as long as a couple of years between active phases, but patients usually experience a couple of active "attacks" per year. Long-term changes include increased eyelid folds, eyelid atrophy, pigmentary changes, and thinning. Blepharochalasis usually presents in adolescence and typically affects both upper eyelids, but unilateral and lower eyelid cases have been reported. Reports suggest a female predominance.

The exact pathogenesis of blepharochalasis is unknown, but there have been hereditary and immunologic links. Hormonal changes occurring during puberty may be a factor in the development of blepharochalasis. Motegi et al reported histological examination revealing perivascular and interstitial infiltration of lymphocytes in the dermis with a marked decrease of elastic fibers. Immunofluorescence analyses suggested that MMP-3 and MMP-9 might play a role in the development of blepharochalasis as well. Others have shown increased immunoglobulin A (IgA) activity.

While blepharochalasis is usually idiopathic and sporadic, a link has been suggested to the rare Ascher syndrome (swollen upper eyelids and lips, occasionally in conjunction with a euthyroid goiter).

No specific causative triggers or exposures have been reported, but patients may report preceding allergy symptoms, crying, fevers, bee stings, stress, or physical activity.

Codes

ICD10CM:
H02.30 – Blepharochalasis unspecified eye, unspecified eyelid

SNOMEDCT:
47704002 – Blepharochalasis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Dermatochalasis – While this disease entity sounds like blepharochalasis, it is quite different and refers to age-related laxity and redundant upper eyelid skin. This is also much more common in older individuals.
  • Preseptal / orbital cellulitis – This would be associated with fevers, skin warmth, and possible globe involvement (ocular motility restriction, relative afferent pupillary defect, etc). Blepharochalasis does not affect the globe.
  • Floppy eyelid syndrome – This typically occurs in overweight individuals with obstructive sleep apnea. Further, floppy eyelid syndrome is constant and does not "come and go" like blepharochalasis.
  • Thyroid eye disease – This is associated with proptosis, scleral show, globe involvement, and enlargement of extra-ocular muscles on imaging.
  • Idiopathic orbital inflammation (IOI) – While this can have a relapsing and remitting course, usually other parts of the orbit are affected. IOI responds to steroids while blepharochalasis has not been reported to. 
  • Hereditary angioedema – Typically affects other parts of the body.
  • Contact dermatitis
  • Lacrimal gland inflammation (dacryoadenitis) – Signs are more focal to the lacrimal gland area, while blepharochalasis typically involves the entire eyelid.
  • Lacrimal gland tumor

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:08/31/2017
Last Updated:02/06/2020
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Blepharochalasis
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A medical illustration showing key findings of Blepharochalasis : Eyelid edema, Recurring episodes
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