10.5826/dpc.1301a3
text/xml
2160-9381
2189
en
Said Amal
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Association Between Lipoid Proteinosis And Coeliac Disease
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Commentary
2023-01-31
2021-11-20
2023-01-31
2023-02-27
e2023003
Dermatol Pract Concept
Said Amal
2023/01/31
10.5826/dpc.1301a3
e2023003
Department of Dermatology, Faculty of Medicine and Pharmacy, Mohammed VI University Hospital, Cadi Ayad University, Marrakech, Morocco
2160-9381
en
e2023003
Tecellioglu M, Kamisli O, Acar C, et al. Lipoidproteinosis and epilepsy: Molecularanalysis. Ann Med Res. 2019;(0):1. doi:10.5455/annalsmedres.2019.02.100
Association Between Lipoid Proteinosis And Coeliac Disease
https://dpcj.org/index.php/dpc/article/download/2189/1967
                 Association Between Lipoid Proteinosis
                           and Coeliac Disease
                       Layla Bendaoud1, Ihssane Bigjoine1, Ouafa Hocar1, Said Amal1


1 Department of Dermatology, Faculty of Medicine and Pharmacy, Mohammed VI University Hospital, Cadi Ayad University, Marrakech,
  Morocco



Citation: Bendaoud L, Bigjoine I, Hocar O, Amal S. Association between lipoid proteinosis and coeliac disease. Dermatol Pract Concept.
2023;13(1):e2023003. DOI: https://doi.org/10.5826/dpc.1301a3
Accepted: July 9, 2022; Published: January 2023
Copyright: ©2023 Bendaoud et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding Author: Layla Bendaoud, Resident in the Department of Dermatology, Rouidate, Marrakech, Telephone: +212678817589,
Email: laylabendaoud@gmail.com




Lipoid proteinosis, also known as Urbach-Wiethe disease,                 Test IgA anti-transglutaminases were highly positive at low
is a rare autosomal recessive condition, characterized by a              titer. The diagnosis of coeliac disease was established. The
mutation of extracellular matrix protein 1(ECM1), leading                workup for other disease locations of lipoid proteinosis was
to deposition of collagenous material in the skin and tissues            negative. The therapeutic approach was to put the patient
[1]. This disease may be associated with diabetes and other              on a gluten-free diet and acitretin for his skin lesions. After
abnormalities. We report a case of lipoid proteinosis associ-            3 months of treatment with acitretin, the dermatological ex-
ated with celiac disease in a 23-year-old man.                           amination noted a minor improvement of the edema. The cu-
    A 23-year-old patient was admitted to our department                 taneous lesions were still present, without new lesions. Then,
for facial edema evolving for 1 year, with no other associated           he was lost to follow-up.
signs. The dermatological examination revealed an infiltrated                 Lipoid proteinosis, also called hyalinosis cutis muco-
edema of the face, predominantly on the forehead, associ-                sae, is a rare, non-life-threatening condition and was first
ated with discrete 3 mm yellow papules in the ear pavilion               described in 1929 by a dermatologist and an otorhinolar-
(figure 1), without mucosal involvement. The histological                yngologist, E. Urbach and C. Weithe [2]. Around 300 cases
study of the papule showed a homogeneous, eosinophilic,                  have been reported in the literature with higher prevalence in
PAS-stained dermal deposit of hyaline substance, in favor of             South Africa and Sweden.
lipoid proteinosis. Laboratory tests revealed hypochromic                     Hoarseness of the voice is usually the first sign of this
microcytic anemia (hemoglobin of 9 g/dl), ferritin 7ng/ml,               disease that appears in childhood. The dermatological man-
hypocholesterolemia 0.7 mmol/l with normal albumin and                   ifestations include vesiculobullous lesions, hyperkeratotic
protein. The patient was further evaluated with an upper                 plaques on the extensor surfaces of elbows, knees, and hands,
endoscopy. The histological examination of the duodenal                  yellow papules, and multiple acneiform and pox-like scars
biopsy revealed atrophic pangastritis with villous atrophy.              that are seen on the face [3]. Moniliform blepharitis is the




Commentary | Dermatol Pract Concept. 2023;13(1):e2023003                                                                                 1
                                 Figure 1. Yellowish-white papules of the ear.



characteristic ocular involvement, affecting the eyelid mar-          References
gins. This disease may affect all organ systems of the body.
    Histologic features consist of thick homogeneous, eosin-           1. Parida JR, Misra DP, Agarwal V. Urbach-Wiethe s­ yndrome. BMJ
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    Some cases in the literature have mentioned the as-                   ficiency: A Case Report. AMJ. 2011;4(3):155-159. doi:10.4066
sociation of lipoid proteinosis with diabetes [5], and with               /AMJ.2011.635
­epilepsy [6]. This is the first report describing an association      3. Natarajan B, Prathap P, Asokan N, Balakrishnan S.
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                                                                          ­
                                                                          ­presentation. JSSTD. 2020;2:56-58. doi:10.25259/JSSTD_9_2020
    Currently, there is no effective treatment. Several
                                                                       4. Giandomenico SD, Masi R, Cassandrini D, et al. ­Lipoidproteinosis:
molecules have been used with different results, such as
                                                                           case report and review of the literature. :6.
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inhibitory effect on collagen. Also, CO2 laser, dermabrasion,              ­Lipoidproteinosis: a case seriesfrom Istanbul. International Jour-
and chemical peeling represent other therapeutic means for                nal of Dermatology. 2007;46(10):1011-1016. doi:10.1111/j.13
the management of cutaneous lesions [3].                                  65-​4632.2007.03115.x
                                                                       6. Tecellioglu M, Kamisli O, Acar C, et al. Lipoidproteinosis
    Knowledge of this rare disease will aid health profession-
                                                                          and epilepsy: Molecular analysis. Ann Med Res. 2019;(0):1.
als in diagnosing it early, and in providing the appropriate
                                                                          doi:10.5455/annalsmedres.2019.02.100
treatment to improve the quality of life.




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