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Case Reports
. 2017 Nov-Dec;8(6):482-484.
doi: 10.4103/idoj.IDOJ_422_16.

Mutilating Keratoderma with Concomitant Alopecia and Keratoses Follicularis Spinulosa Decalvans: X-Linked Olmsted Syndrome and its Response to Isotretinoin

Gunjan Verma  1 , Kabir Sardana  1 , R K Gautam  1
Affiliations
Case Reports

Mutilating Keratoderma with Concomitant Alopecia and Keratoses Follicularis Spinulosa Decalvans: X-Linked Olmsted Syndrome and its Response to Isotretinoin

Gunjan Verma et al. Indian Dermatol Online J. 2017 Nov-Dec.

Abstract

We report a case of mutilating keratoderma with alopecia and keratoses follicularis spinulosa decalvans (KFSD), which was initially diagnosed as ectodermal dysplasia and Olmsted syndrome but was revisited as a case of X-linked Olmsted (XLO) syndrome. We focus on this uncommon entity (XLO) to highlight the differentials of alopecia with palmoplantar keratoderma.

Keywords: Keratoderma; X linked Olmsted; olmsted syndrome.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Atrichia of eyelashes and eyebrows, follicular papules, and pits on malar prominences. (b) Follicular papules on scalp with alopecia
Figure 2
Figure 2
(a) Mutilating transgradient keratoderma restricted to palm. (b) Posttreatment 1 month. (c) Mutilating keratoderma of soles. (d) Post-isotretinoin therapy marked improvement in PPK
See this image and copyright information in PMC

References

    1. Sardana K, Mahajan S, Sarkar R, Mendiratta V, Bhushan P, Koranne RV, et al. The spectrum of skin disease among Indian children. Pediatr Dermatol. 2009;26:6–13. - PubMed
    1. Haghighi A, Scott CA, Poon DS, Yaghoobi R, Saleh-Gohari N, Plagnol V, et al. A missense mutation in the MBTPS2 gene underlies the X-linked form of Olmsted syndrome. J Invest Dermatol. 2012;133:571–3. - PubMed
    1. Castori M, Valiante M, Ritelli M, Preziosi N, Colombi M, Paradisi M, et al. Palmoplantar keratoderma, pseudo-ainhum, and universal atrichia: A new patient and review of the palmoplantar keratoderma-congenital alopecia syndrome. Am J Med Genet A. 2010;152A:2043–7. - PubMed
    1. Wilson N, Milstone L, Kiszewski, Hansen CD, O’Toole EA, Schwartz ME, et al. Expanding the Phenotypic Spectrum of Olmsted Syndrome. J Invest Dermatol. 2015;135:2879–83. - PMC - PubMed
    1. Duchatelet S, Hovnanian A. Olmsted syndrome: Clinical, molecular and therapeutic aspects. Orphanet J Rare Dis. 2015;10:33. - PMC - PubMed

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