Case Report: A case of hypertrophic lupus erythematosus with negative CD123 staining and absence of transepidermal elimination of elastin.

We report the case of a 49-year-old male with clinical and histological findings consistent with hypertrophic lupus erythematosus (HLE). HLE must be clinically and histologically differentiated from keratoacanthoma, hypertrophic lichen planus, squamous cell carcinoma and plaque type psoriasis. CD123 positivity and transepidermal elimination of elastin have recently been reported as tools to distinguish HLE. Interestingly, in this case, biopsies of two separate lesions failed to reveal these two features. The etiology of this discrepancy is unknown and further studies are needed to clarify the utility of CD123 positivity and transepidermal elimination of elastin in the diagnosis of hypertrophic lupus erythematosus.

Introduction

Hypertrophic lupus erythematosus (HLE) is a rare subset of discoid lupus erythematosus, characterized by erythematous, indurated, verrucous papules and nodules located on sun-exposed areas. HLE must be clinically and histologically differentiated from keratoacanthoma, hypertrophic lichen planus, squamous cell carcinoma and plaque type psoriasis. CD123 positivity and transepidermal elimination of elastin have recently been reported to distinguish HLE [1, 2].

Report of case

A 49-year-old, unemployed, white male presented with a three-year history of an expanding “rash”. He reported no constitutional symptoms. He had previously been treated with oral prednisone and an unknown topical steroid without improvement and was off all medications at our initial visit. The patient had a past medical history of hepatitis C. He denied a family history of skin or autoimmune diseases. Laboratory work-up was significant for positive anti-nuclear antibodies and anti-Ro antibodies. Physical exam revealed multiple hyperkeratotic, verrucous papules and nodules with white, scaly, cribriform centers overlying patches of depigmentation, erythema and atrophy on his bilateral arms ( Figure 1) and anterior legs. His face and scalp had several atrophic, depigmented patches. Two punch biopsies were obtained from separate lesions. Histological sections demonstrated an interface inflammatory pattern with deep peri-vascular and peri-appendageal lymphocytic infiltrate and rare plasma cells ( Figure 2). A diagnosis of HLE was made. The patient was prescribed clobetasol ointment 0.05% twice daily. At the three month follow-up, there was improvement of the hypertrophic lesions. The patient was subsequently lost to follow-up.

Figure 1.

Clinical photo of hypertrophic lupus erythematosus.

Hypertrophic lupus erythematosus presenting as a verrucous plaque on the patient’s elbow.

Figure 2.

Histological photo of hypertrophic lupus erythematosus.

Hypertrophic lupus erythematosus displays epidermal acanthosis and expansion of follicular ostia with a superficial and deep perivascular and periappendageal intradermal lymphocytic infiltrate (hematoxylin and eosin, 40× magnification).

Discussion

HLE was first described by Bechet in 1940 [3]. Clinical diagnosis can be challenging as HLE can mimic psoriasis or even squamous cell carcinoma. Uitto et al. described two histological patterns of HLE One resembled hypertrophic lichen planus, while the other was similar to keratoacanthoma [4]. Daldon et al. found that transepidermal elimination of elastin was present in 14 cases of HLE [1]. Recently, Ko et al. reported that a band of CD123 positive cells at the dermal-epidermal junction was characteristic of five cases of HLE [2].

In this patient, we examined these two recently described histologic features of HLE. Interestingly, both CD123 positivity and transepidermal elimination of elastin were not present in this case ( Figure 3). However, the histological and clinical findings were most consistent with HLE. The etiology of this discrepancy is unknown and further studies are needed to clarify the utility of CD123 positivity and transepidermal elimination of elastin in the diagnosis of hypertrophic lupus erythematosus.

Figure 3.

( A): Example of positive CD123 immunohistochemical stain from tonsil control tissue (200× magnification). ( B): CD123 immunohistochemical stain on the biopsy of hypertrophic lupus erythematosus from the reported patient. Only rare plasmacytoid dendritic cells are seen in the dermis. The stain is negative for the dense band of CD123 positive cells that Ko et al. described in hypertrophic lupus erythematosus (100× magnification).

There is no definitive treatment for HLE. Options include topical or intralesional steroids, hydroxychloroquine, topical calcineurin inhibitors, topical or oral retinoids, thalidomide and surgical excision [5, 6]. Winchester et al. reported on the efficacy ustekinumab, an inhibitor of IL-12 and IL-23 [7].

This case highlights the discrepancies of CD 123 positivity and absence of transepidermal elimination of elastin in HLE.

Consent

Written informed consent for publication of clinical details and clinical images was obtained from the patient.

The authors have now addressed all the concerns.

I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.

The authors successfully addressed the reviewer comments in this case report.

I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.

This article is well written but needs a few additions to improve it:

Please elaborate on what CD 123 is, and how it is used for diagnosing HLE. It is not common knowledge and should be expanded upon.

It might also be worth commenting on this paper by Miyashita A in which the authors showed patients with CD123 positive cells responded better to treatment than patients with low CD123. Do you think any of this could be supported or refuted by your cases?

I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.

This report describes a case of hypertrophic lupus erythematosus based on clinical and histopathologic criteria that is negative for CD123 and elastin elimination. Negative data is important. However, as the emphasis is on the lack of CD123 and the lack of transepidermal elastin elimination, it would be good to show the negative results. For the CD123 stain, it would be good to show a positive control to make sure that the antibody really worked.

I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above.

This is a case of hypertrophic lupus erythematosus that is described as unusual in pathologic presentation.

The title needs to indicate absence of transepidermal elimination of elastin. It is currently unclear if transepidermal elmination of elastin was present.

The order of treatment described for HLE is confusing.  One would start with hydroxychloroquine, then add quinacrine to hydroxychloroquine, with topicals as adjunctive therapy. Oral retinoids,  thalidomide, and immuosuppressives would be options. Given that frequently there are multiple lesions that may actually koebnerize in a surgical scar, one would not include surgical excision as an option.

The report cited in favor of TNF-alpha inhibitor is on ustekinumab, which is not a TNF inhibitor. This needs revision.

Information about the antibody used for CD123 staining, as well as whether frozen or fixed tissue was used, is important. Anti-CD123 staining is not as good on fixed tissue. Were there any positive controls stained simultaneously?

I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above.