10.5826/dpc.1204a187
none
text/xml
2160-9381
2235
en
Mohamed Denguezli
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
immune compromised district concept
Unilateral Rosacea in a patient with multiple sclerosis
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Letter to the Editor
2022-10-31
2021-12-10
2022-10-31
2022-12-02
e2022187
Dermatol Pract Concept
none
Mohamed Denguezli
2022/10/31
10.5826/dpc.1204a187
e2022187
a:1:{s:5:"en_US";s:68:"Department of Dermatology, Military Hospital of Instruction of Tunis";}
2160-9381
immune compromised district concept
en
e2022187
Piccolo V, Ruocco V, Russo T, Ruotolo F, Piccolo S, Baroni A. Unilateral rosacea in patients with facial nerve palsy: a mere example of immunocompromised district. J Dermatol2013;40:850.
Unilateral Rosacea in a patient with multiple sclerosis
https://dpcj.org/index.php/dpc/article/download/2235/1809
                     Unilateral Rosacea in a Patient With
                              Multiple Sclerosis
          Mariem Tabka1, Rima Gammoudi1, Refka Frioui1, Nadia Fetoui1, Sana Mokni1,
                Amina Ounallah1, Colondane Belajouza1, Mohamed Denguezli1


1 Department of Dermatology, Farhad Hachad Hospital of Sousse, Sousse, Tunisia



Citation: Tabka M, Gammoudi R, Refka F, et al. Unilateral Rosacea in a patient with multiple sclerosis. Dermatol Pract Concept.
2022;12(4):e2022187. DOI: https://doi.org/10.5826/dpc.1204a187
Accepted: March 17, 2022; Published: October 2022
Copyright: ©2022 Tabka 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: Refka Frioui, MD, Department of Dermatology, Farhat Hachad Hospital, Ibn Jazzar,4000 Sousse, Tunisia.
Phone: +216 29320235; Email: rafkouna1993@gmail.com
Written Consent from the patient: The authors certify that they have obtained all appropriate patient consent forms, in which the patient
gave his consent for images and other clinical information to be included in the journal. The patient understands that his name and initial
will not be published and due effort will be made to conceal his identity, but that anonymity cannot be guaranteed.




Introduction                                                             compatible with rosacea. The unilateral distribution of the
                                                                         dermatosis was suggestive of pre-existing neurological le-
Rosacea is a common and chronic inflammatory skin                        sion. We referred the patient for additional testing, including
­condition of clinical heterogeneity and intriguing pathophys-           an MRI of the brain, which demonstrated a demyelinating
iological mechanisms. Herein, we report a case of unilateral             plaque at the trigeminal root entry zone consistent with tri-
rosacea in a patient with multiple sclerosis.                            geminal neuralgia secondary to multiple sclerosis (Figure 2).
                                                                         She was put on natalizumab and bolus steroid therapy. The
                                                                         pain has decreased. However, the rosacea got worse, proba-
Case presentation                                                        bly because of corticosteroids.
A 37-year-old woman presented to the dermatology
­department with a 2-year history of facial unilateral redness           Conclusion
and paroxysmal pain. Over the last 2 years, she received
multiple treatments, such as ivermectin cream, doxycycline               Despite its high prevalence, the underlying pathophysiology
and metronidazole gel, without any benefit. Her facial der-              of rosacea remains unclear. This observation highlights the
matosis presented as unilateral persistent erythema with                 complexity of the disease contributing mechanisms. Indeed,
­multiple papules and pustules strictly confined to the right            it describes a distinct variant of the disease denominated
side ­(Figure 1). The skin biopsy revealed a perivascular and            neurogenic rosacea, presented with unilateral arrangement
perifollicular inflammatory infiltrate consisting of lym-                and associated with an autoimmune disease. The patho-
phocytes, neutrophils with the presence of demodex mites,                physiological mechanisms implicated in the development of



Letter to the Editor | Dermatol Pract Concept. 2022;12(4):e2022187                                                                            1
Figure 1. (A) Unilateral rosacea confined to the right hemiface. (B) Right lateral aspect evidencing erythema and papulopustules. (C)_Slight
erythema on the left side.


                                                                        often in patients with neurological or neuropsychiatric con-
                                                                        ditions, including complex regional pain syndrome, essential
                                                                        tremor, depression and obsessive-compulsive disorder [2].
                                                                        Dysesthesia secondary to neuronal injury was commonly re-
                                                                        ported and was associated with classical rosacea signs [2].
                                                                        It is well known that patients suffering from rosacea have
                                                                        an increased of developing a number of auto-immune dis-
                                                                        eases, including multiple sclerosis. Yet, it is also important
                                                                        to notice that trigeminal neuralgia, attributed to multiple
                                                                        sclerosis, may explain the neurogenic inflammation leading
                                                                        to such skin condition as well as the unilateral arrangement
                                                                        of the lesions. Our case may also reflect a regional desta-
                                                                        bilization of the neuroimmunocutaneous system induced by
                                                                        multiple sclerosis. This hypothesis fully represents the con-
                                                                        cept of immuno-compromised district (ICD) [3,4]. ICD, a
                                                                        newly introduced pathogenic concept, stipulates that several
                                                                        different factors are likely to create a privileged cutaneous
                                                                        district, which explains the segmental presentation of many
                                                                        skin disorders, including bullous pemphigoid, pemphigus,
                                                                        lichen planus, discoid lupus erythematosus, drug eruptions
Figure 2. Neuroimaging findings in our patient with                     and acne [4-6]. Trigeminal neuralgia attributed to multiple
gadolinium-enhanced T1-weighted image on the axial plane that           sclerosis, in analogy with the aforementioned facial nerve
shows a hyperintense pontine lesion at the right trigeminal nerve       palsy, could locally alter the immune response and induce
zone.
                                                                        the occurrence of rosacea following the neurologically im-
                                                                        paired facial side.
rosacea include dysregulation of the innate immune system,                  Regardless of the etiopathogenic mechanisms of ­rosacea,
imbalance of commensal skin microbiota, and abnormal                    the interaction between the skin and the immune and ner-
neurovascular signaling [1]. Neurogenic rosacea, a recently             vous systems is currently well established, with rosacea
described rosacea subtype, demonstrates the role of local               being one of the established examples involving these var-
neural-associated mediators dysregulation in the patho-                 ious systems. The systematized arrangement of a dermato-
physiology of the dermatosis [2]. Moreover, it occurs more              sis that is strictly confined to a specific area may refer to



2                                                                        Letter to the Editor | Dermatol Pract Concept. 2022;12(4):e2022187
the ­“immunocompromised district” concept, and thus pro-             2. Scharschmidt TC, Yost JM, Truong SV, Steinhoff M, Wang KC,
moting investigations into possible immunocompromising                  Berger TG. Neurogenic Rosacea: A Distinct Clinical Subtype
                                                                        Requiring a Modified Approach to Treatment. Arch Dermatol.
­factors. Further research is clearly needed to better describe
                                                                        2011;147(1):123-126. DOI: 10.1001/archdermatol.2010.413.
the underlying patho-physiologic characteristics and to iden-
                                                                        PMID: 21242409. PMCID: PMC3692271.
tify additional effective treatment methods”.                        3. Egeberg A, Hansen PR, Gislason GH, Thyssen JP. Clustering of
                                                                        autoimmune diseases in patients with rosacea. J Am Acad Derma-
Acknowledgment: We thank the patient for granting permis-               tol. 2016;74(4):667-672.e1. DOI: 10.1016/j.jaad.2015.11.004.
sion to publish this information (the patient in this manu-             PMID: 26830864.
script has given written informed consent to publication of          4. Ruocco V, Ruocco E, Piccolo V, Brunetti G, Guerrera LP, Wolf
                                                                        R. The immunocompromised district in dermatology: A unify-
her case details). We are also indebted to B
                                           ­ adreddine Sriha,
                                                                        ing pathogenic view of the regional immune dysregulation. Clin
MD, PhD, Department of Pathology, Farhat Hached
                                                                        Dermatol. 2014;32(5):569-576. DOI: 10.1016/j.clinderma-
Hospital, University of Sousse, for his interpretation of
­                                                                       tol.2014.04.004. PMID: 25160098.
the skin biopsies. He received no compensation for his               5. Piccolo V, Russo T, Baroni A. Unilateral bullous pemphigoid
contributions.                                                          in hemiplegic patients: An instance of immunocompromised
                                                                        district. J Dermatol. 2013;40(1):64-65. DOI: 10.1111/j.1346-
                                                                        8138.2012.01647.x. PMID: 22901292.

References                                                           6. Piccolo V, Ruocco V, Russo T, Ruotolo F, Piccolo S, Baroni
                                                                        A. ­Unilateral rosacea in patients with facial nerve palsy: a
 1. Choi JE, Di Nardo A. Skin Neurogenic inflammation. Semin            mere example of immunocompromised district. J Dermatol.
    ­Immunopathol. 2018;40(3):249-259. DOI: 10.1007/s00281-             2013;40(10):850. DOI: 10.1111/1346-8138.12208. PMID: 239​
     018-0675-z. PMID: 29713744. PMCID: PMC6047518.                     57626.




Letter to the Editor | Dermatol Pract Concept. 2022;12(4):e2022187                                                                  3
