Indian Journal of Dermatology
CORRESPONDENCE
Year
: 2021  |  Volume : 66  |  Issue : 3  |  Page : 318--319

Concomitant segmental vitiligo and segmental morphea in an 8-year-old boy


Francesca Magri1, Emanuele Miraglia1, Giuseppe Soda2, Sandra Giustini1,  
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

Correspondence Address:
Francesca Magri
From the Department of Dermatology and Venereology, Sapienza University of Rome, Rome
Italy




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;66:318-319


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 Sep 24 ];66:318-319
Available from: https://www.e-ijd.org/text.asp?2021/66/3/318/321314


Full Text



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}{Figure 2}

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}

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}

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

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3Yadav 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.
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