Indian Journal of Dermatology
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CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 3  |  Page : 318-319
Concomitant segmental vitiligo and segmental morphea in an 8-year-old boy


1 From the Department of Dermatology and Venereology, Sapienza University of Rome, Rome, Italy
2 Molecular Medicine, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy

Date of Web Publication13-Jul-2021

Correspondence Address:
Francesca Magri
From the Department of Dermatology and Venereology, Sapienza University of Rome, Rome
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_247_20

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How to cite this article:
Magri F, Miraglia E, Soda G, Giustini S. Concomitant segmental vitiligo and segmental morphea in an 8-year-old boy. Indian J Dermatol 2021;66:318-9

How to cite this URL:
Magri F, Miraglia E, Soda G, Giustini S. Concomitant segmental vitiligo and segmental morphea in an 8-year-old boy. Indian J Dermatol [serial online] 2021 [cited 2021 Nov 27];66:318-9. Available from: https://www.e-ijd.org/text.asp?2021/66/3/318/321314




Sir,

Segmental vitiligo (SV) and segmental morphea are unilateral cutaneous disorders, with an autoimmune pathogenesis. The association of the two generalized forms has been largely reported, while the combination of the two segmental variants is uncommon.

An 8-year-old boy referred to our hospital for vitiligo and morphea lesions at his trunk. As referred by his parents, the SV lesion was present at birth and was stable. The morphea lesion appeared 2 years before, initially with peripheral lilac ring and previously treated with topical steroids. On physical examination, confluent achromic lesions, with jagged border, were present at the left scapular region, with dermatomal distribution, while a homolateral sclerotic patch was present at the left lumbar region [Figure 1]. A cutaneous biopsy of the sclerotic lesion was performed, histologically confirming the diagnosis of morphea: epidermal atrophy and dermal thickening and collagen hyalinization, with lymphocytic infiltration, were present [Figure 2]. Routine laboratory exams were in normal range; thyroid function tests and celiac disease antibodies tests were negative. The patient is under topical treatment with vitamin E cream, with partial improvement of the sclerotic lesion.
Figure 1: Vitiligo of the left scapular region and sclerotic patch of the left lumbar region

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Figure 2: Hematoxylin–eosin stain 10×; epidermal atrophy; thickening and collagen hyalinization in the deep dermis, with mild inflammatory infiltration of lymphocytes, which are distributed around blood vessels. Atrophy of adnexal structures, particularly the pilosebaceous units, with hypertrophic hair erector muscle. The vascular changes are thickening of the walls of small blood vessels and narrowing of their lumen

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Our case is of interest because of its rare association of these two autoimmune conditions. A study on 1098 vitiligo individuals has shown that 20% of patients presented comorbid autoimmune diseases. Between them, 0.2% of individuals had linear morphea.[1]

Many reports describe cases of concomitant generalized vitiligo and morphea. Instead, the association between SV and segmental morphea had been rarely reported[2],[3],[4] and is summarized in [Table 1].
Table 1: Reports of concomitant segmental vitiligo and segmental morphea

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The explanation of this association is still unclear.

SV is a localized vitiligo with typical distribution along dermatomes or Blaschko's lines. Both SV and classic vitiligo are autoimmune conditions with T-cell-mediated degeneration of melanocytes. Recent studies indicate that SV is due to an autoimmune attack against a mosaic region.[5] Also, morphea is considered an autoimmune disorder, with collagen density and sclerosis. Neurological, traumatic and infectious agents have been suggested as trigger factors of the autoimmune attack.

Differential diagnosis of both conditions is summarized in [Table 2]. In our patient, the vitiligo lesion was clearly identifiable, since its achromic macular aspect with jagged border, while the morphea lesion could be confused with other sclerotic conditions, including lichen sclerosus, radiodermatitis, and intralesional steroid-induced lipoatrophy. However, our patient did not report anamnestic data possibly related with the lesion, such as previous radiotherapy or injective treatments. Additionally, the histological exam ruled out other disorders.
Table 2: Main differential diagnosis of vitiligo and morphea

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In consideration of previous reports, and of the similar immunological pathogenesis, a common immunological pathophysiology is hypothesized. Concerning the segmental distribution, the currently accepted theory suggests that both diseases result from genetic mosaicism, with mutations in some ectodermal cells during the embryonic life.[2],[5] The disorder develops when cells resulting from mosaicism are exposed to a still unclear causative agent.[2],[5]

We report this case to add to the knowledge of the association of these two segmental disorders, both representing a condition of genetic mosaicism and suggesting common immunological pathogenic factors.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Gill L, Zarbo A, Isedeh P, Jacobsen G, Lim HW, Hamzavi I. Comorbid autoimmune diseases in patients with vitiligo: A cross-sectional study. J Am Acad Dermatol 2016;74:295-302.  Back to cited text no. 1
    
2.
Ubaldo HDC, Castro CCS. Coexistence of segmental vitiligo, scleroderma en coup de sabre and cleft lip on the same hemiface: Association with mosaicism? An Bras Dermatol 2019;94:248-50.  Back to cited text no. 2
    
3.
Yadav P, Garg T, Chander R, Nangia A. Segmental vitiligo with segmental morphea: An autoimmune link? Indian Dermatol Online J 2014;5(Suppl 1):S23-5.  Back to cited text no. 3
    
4.
Janowska M, Podolec K, Lipko-Godlewska S., Wojas-Pelc A. Coexistence of Parry-Romberg syndrome with homolateral segmental vitiligo. Postepy Dermatol Alergol 2013;30:409-11.  Back to cited text no. 4
    
5.
Van Geel N, Speeckaert R. Segmental vitiligo. Dermatol Clin 2017;35:145-50.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]



 

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