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A Case Report of Pterygium Inversum Unguis Associated with Systemic Lupus Erythematosus

Zhang, Xiao-Li1,#; Teng, Yan1,2,#; Chen, Hao3; Ju, Mei4; Liu, Yi1; Liu, Mu-Sang5,∗

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International Journal of Dermatology and Venereology: September 2020 - Volume 3 - Issue 3 - p 182-183
doi: 10.1097/JD9.0000000000000034
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Abstract

Introduction

Pterygium inversum unguis (PIU), also known as ventral pterygium, is a rare abnormality characterized by adherence of the distal nail bed to the ventral surface of the nail plate, resulting in obliteration of the distal groove. PIU was first described by Caputo and Prandi in 1973 in a woman who developed ventral pterygium on multiple fingers of both hands without definite cause.1 This condition, which have been reported in few cases, can be congenital, idiopathic, or secondary to systemic connective tissue diseases or other. We herein report a case of acquired PIU associated with systemic lupus erythematosus (SLE), which is rare of its kind in the Chinese dermatologic literature.

Case report

A 33-year-old woman presented with a 2-year history of multiple telangiectasias in the abdomen of the fingers in May 2018. The telangiectasias were scattered between bright red or purple patches. She was initially treated for frostbite with topical heparin ointment. One month before the current visit, she presented with proximal muscular weakness, a malar rash, photosensitivity, knee and ankle arthritis, and PIU of the fingers. She complained for mild to moderate pain while clipping her fingernails. She was then admitted to our hospital.

Physical examination of the fingernails (excluding the thumbs) revealed marked subungual keratotic thickening between the distal hyponychium and ventral nail plate. The distal portion of the nail bed was adherent to the ventral surface of the nail plate, obliterating the nail groove (Fig. 1). The patient had no family history of a similar nail abnormality. The laboratory findings were consistent with the diagnosis of SLE (positive for ANA, Ro/SSA, La/SSB, U1-SnRNP antibody, anti-dsDNA, and Smith antibody).

Figure 1
Figure 1:
Fingers (excluding the thumbs) showing the adherence of the distal nail bed to the ventral surface of the nail plate, resulting in obliteration of the distal groove.

The patient was treated with methylprednisolone (40 mg/day), mycophenolate mofetil (1.0 g twice a day), and hydroxychloroquine (0.2 g twice a day). In addition, her nails were treated by topical application of tretinoin 0.025%. Approximately 2 months after the beginning of treatment, most of the SLE symptoms had disappeared or improved; however, the nail disorder has not been improved. The patient had no adverse or unanticipated reactions and was satisfied with the effect.

Regular follow-up has been undergoing until the time of this writing. The patient continued to use topical agent of tretinoin 0.025% at the sites of pterygium intermittently and attend the clinic regularly. The SLE symptoms were well controlled, while PIU was not significantly aggravated or alleviated. Without obvious discomfort, the patient refused other possible treatment options and gave the informed consent for publishing her case.

Discussion

We have herein described a case of a 33-year-old woman with a recent diagnosis of SLE, in whom the distal portion of the nail bed of the bilateral fingers (excluding the thumbs) was adherent to the ventral surface of the nail plate, obliterating the groove. The patient in the present case had an acquired form of PIU affecting all of the fingernails (excluding the thumbs) without involvement of the toenails.

Acquired PIU is reportedly associated with connective tissue diseases such as systemic sclerosis2 or other conditions such as stroke, onychophagia, allergic dermatitis, or use of gel polish, and after allogeneic haematopoietic stem cell transplantation.3–6 Because of the rarity of this condition, its exact origin remains speculative. In order to better recognize the origin and pathology of this infrequent disease, Zaias et al.7 classified it into three categories: congenital aberrant hyponychium, acquired irreversible PIU, acquired reversible extended hyponychium. Acquired form of the disorder may be linked to abnormal distal circulation or exposure to certain chemical stimuli, which leads to destruction of the matrix, reactive hyperkeratosis and thus pterygium formation.4,7 This may explain the pathogenesis of our patient, who could be categorized into acquired irreversible PIU, secondary to SLE.

The management of PIU is not well defined. Different treatments, including topical retinoids, hydroxypropyl chitosana, and electrodissection, can reportedly produce a good response.8-9 The most effective strategy involves treatment of the underlying cause of acquired PIU. In our case, however, the PIU did not improve after 2 months of treatment of SLE and use of topical retinoids. The patient was still undergoing follow-up at the time of this writing.

In conclusion, patients with PIU must be evaluated to rule out associated causes, and these patients require long-term follow-up examinations to detect the development of connective tissue diseases such as SLE.

References

[1]. Caputo R, Prandi G. Pterygium inversum unguis. Arch Dermatol 1973;108(6):817–818.
[2]. Marie I, Gremain V, Nassermadji K, et al. Nail involvement in systemic sclerosis. J Am Acad Dermatol 2017;76(6):1115–1123. doi: 10.1016/j.jaad.2016.11.024.
[3]. Vadmal M, Reyter I, Oshtory S, et al. Pterygium inversum unguis associated with stroke. J Am Acad Dermatol 2005;53(3):501–503. doi: 10.1016/j.jaad.2005.04.057.
[4]. Ficicioglu S, Korkmaz S. Onychophagia induced melanonychia, splinter hemorrhages, leukonychia, and pterygium inversum unguis concurrently. Case Rep Dermatol Med 2018;2018:3230582. doi: 10.1155/2018/3230582.
[5]. Cervantes J, Sanchez M, Eber AE, et al. Pterygium inversum unguis secondary to gel polish. J Eur Acad Dermatol Venereol 2018;32(1):160–163. doi: 10.1111/jdv.14603.
[6]. Szlauer-Stefanska A, Kaminska-Winciorek G, Giebel S. Onychoscopy of non-infectious nail abnormalities in patients after allogeneic haematopoietic stem cell transplantation. J Eur Acad Dermatol Venereol 2019;33(4):637–642. doi: 10.1111/jdv.15357.
[7]. Zaias N, Escovar SX, Zaiac MN, et al. Hyponychium abnormalities congenital aberrant hyponychium vs. acquired pterygium inversum unguis vs. acquired reversible extended hyponychium: a proposed classification based on origin, pathology and outcome. J Eur Acad Dermatol Venereol 2015;29(7):1427–1431. doi: 10.1111/jdv.12632.
[8]. Jang YH, Jung HJ, Lee JS, et al. Idiopathic pterygium inversum unguis: response to topical retinoid. Korean J Dermatol 2012;50(12):1092–1093.
[9]. Lee JH, Kim M, Park HJ, et al. A case of idiopathic pterygium inversum unguis: response to electrodissection. Clin Exp Dermatol 2015;40(4):441–442. doi: 10.1111/ced.12513.
Keywords:

pterygium inversum unguis; systemic lupus erythematosus; case report

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