Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
Brief Report
Case Report
Editorial
Focus
Images/Instrument in Dermatology/Dermatosurgery
Letter to the Editor
Living Legends
Looking back in history
Original Article
Perspective
Resident Forum
Residents Forum
Review Article
Spot the Diagnosis
Tropical Dermatology
Visual Treats in Dermatology
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
Brief Report
Case Report
Editorial
Focus
Images/Instrument in Dermatology/Dermatosurgery
Letter to the Editor
Living Legends
Looking back in history
Original Article
Perspective
Resident Forum
Residents Forum
Review Article
Spot the Diagnosis
Tropical Dermatology
Visual Treats in Dermatology
View/Download PDF

Translate this page into:

Visual Treats in Dermatology
2022
:2;
66
doi:
10.25259/CSDM_82_2022

Beaded papules of eyelids

Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
Corresponding author: Aravind Sivakumar, Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India. aravinddermat@gmail.com
Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Sivakumar A, Kalimuthu A. Beaded papules of eyelids. CosmoDerma 2022;2:66.

A 12-year-old adolescent boy presented with multiple skin colored papules of both eyelids since 5 years of age [Figure 1]. He also complained of verrucous plaques over the extensors of both elbow and knee joints associated with occasional itching. He was born of third degree consanguineous marriage with negative family history in other siblings. His mother gave the history of fluid filled vesicles during early childhood which healed with scarring. Examination revealed the presence of beaded papules arranged linearly along the margin of both eyelids symmetrically consistent with moniliform blepharosis evident on dermoscopy [Figure 2]. There was also the presence of pock like scarring over the face and extremities on a background of waxy thickened skin. Hoarseness of voice was present which was confirmed by direct laryngoscopy showing thickened vocal cords. There were no neurological manifestations except for paroxysms of anger episodes. A skin biopsy from the lesion revealed the presence of homogenous hyaline eosinophilic material in the dermis staining positively for periodic acid Schiff stain thus confirming a diagnosis of lipoid proteinosis.

Figure 1:: Multiple beaded papules arranged linearly over the margin of the eyelid (moniliform blepharosis).
Figure 2:: Dermoscopy highlighting the linearly arranged closely set beaded monomorphic papules (DermLite DL4, Polarized mode, ×10).

Lipoid proteinosis also referred to as Hyalinosis cutis et mucosae or Urbach Wiethe disease is an autosomal recessive disorder characterized by a defect in the gene ECM1 coding for extracellular matrix glycoproteins important for structural support and scaffold for the dermis and basement membrane. Skin manifestations can occur as early as infancy in the form of blistering. The skin then becomes thickened and waxy with verrucous and psoriasiform plaques mainly over the frictional sites. Similar thickening can also occur in the vocal cords leading to progressive hoarseness of voice. Neurological manifestations range from seizures to behavioral disorders and neuropsychiatric disturbances. A computed tomogram of the brain reveals bean-shaped temporal calcifications bilaterally. A skin biopsy can be done to rule out other differentials such as amyloidosis, porphyria, and scleromyxedema.[1]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

  1. , , . Lipoid proteinosis. JAMA Dermatol. 2018;154:1479-80.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
247

PDF downloads
9
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections