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Discrete (‘acral nonpapular’) localised lichen myxedematosus
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  1. Jeave Reserva1,
  2. Rachel Marchalik2,
  3. Marylee Braniecki3
  1. 1Department of Dermatology, Georgetown University/MedStar Washington Hospital Center, Washington DC, USA
  2. 2Georgetown University School of Medicine, Washington DC, USA
  3. 3Department of Pathology, University of Illinois at Chicago College of Medicine, Chicago, Illinois, USA
  1. Correspondence to Dr Jeave Reserva, jeave.reserva{at}gmail.com

https://doi.org/10.1136/bcr-2015-209462

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    Description

    Localised lichen myxedematosus (LM) is a chronic idiopathic papular mucinosis that can be divided into five subtypes: a discrete papular (DPLM) form, acral persistent papular mucinosis (APPM), papular mucinosis of infancy, a nodular form and self-healing cutaneous mucinosis.1

    DPLM presents as a chronic eruption of red or flesh-coloured 2–5 mm papules, symmetrically involving the limbs and trunk. It is seen equally in both genders and is histologically characterised by diffuse mucin deposition in the upper and mid-dermis, with slight lymphocytic infiltration and fibroblast proliferation. APPM is much more commonly seen in women and presents as white to flesh-coloured papules exclusively on the dorsal hands and extensor forearms. Its histopathology shows only focal dermal mucin deposition with sparing of the subepidermal zone (Grenz zone) and no increase in fibroblasts.1–3

    We report a case of a healthy patient who presented with a 7-year history of scaly plaques on the dorsal hands with finger swelling. Physical examination revealed multiple 5–10 mm pink scaly plaques overlying the metacarpophalangeal and proximal interphalangeal joints, and dorsal hands (figure 1). A rheumatological evaluation revealed no evidence of arthritis. Clinical differential diagnosis included cutaneous mucinosis and amyloidosis.

    Figure 1
    Figure 1

    Pink plaques with fine scales located on the metacarpophalangeal and proximal interphalangeal joints with swelling of the proximal digits.

    A punch biopsy revealed mild superficial perivascular lymphocytic infiltrate without evidence of interface dermatitis (figure 2) and widened dermal spaces between collagen bundles (figure 3). Alcian blue highlighted dermal mucin deposition without sparing of the subepithelial Grenz zone (figure 4). An increased number of fibroblasts was noted on anti-CD34 immunostaining (figure 5).

    Figure 2
    Figure 2

    Mild superficial perivascular lymphocytic infiltrate.

    Figure 3
    Figure 3

    Wide dermal spaces in between thickened collagen bundles.

    Figure 4
    Figure 4

    Alcian blue stain revealing stromal mucin deposition with involvement of the subepithelial Grenz zone.

    Figure 5
    Figure 5

    Anti-CD34 immunostaining demonstrating fibroblast proliferation.

    Learning points

    • The presence of fibroblast proliferation and stromal mucin deposition in the subepithelial Grenz zone suggests the diagnosis of discrete papular lichen myxedematosus (DPLM) over acral persistent papular mucinosis (APPM). However, the presence of lesions on the dorsum of the hands is characteristic of APPM.

    • DPLM can rarely present with plaques in place of papules.

    • Rare equivocal cases with features of DPLM as well as APPM may be suggestive of a novel variant of discrete (acral nonpapular) localised lichen myxedematosus.

    Acknowledgments

    The authors would like to thank Dr Liborka Kos, MD, for providing the clinical photos for this case.

    References

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      1. Concheiro J,
      2. Perez-Perez L,
      3. Peteiro C, et al
      . Discrete papular lichen myxoedematosus: a rare subtype of cutaneous mucinosis. Clin Exp Dermatol 2009;34:e608–10. doi:10.1111/j.1365-2230.2009.03294.x
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    2. ↵
      1. Luo DQ,
      2. Wu LC,
      3. Liu JH, et al
      . Acral persistent papular mucinosis: a case report and literature review. J Dtsch Dermatol Ges 2011;9:354–9. doi:10.1111/j.1610-0387.2010.07594.x
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      1. Harris JE,
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      . Acral persistent papular mucinosis. J Am Acad Dermatol 2004;51:982–8. doi:10.1016/j.jaad.2004.07.002
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    View Abstract

    Footnotes

    • Contributors JR, RM and MB drafted and revised the paper.

    • Competing interests None.

    • Patient consent None.

    • Provenance and peer review Not commissioned; externally peer reviewed.