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Case Report

A case of hypertrophic lupus erythematosus with negative CD123 staining and transepidermal elimination of elastin

[version 1; peer review: 2 approved with reservations]
PUBLISHED 21 Mar 2014
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Abstract

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 HLE1,2.

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.

dcc007be-2037-4119-aff6-767d3a70bf3e_figure1.gif

Figure 1. Clinical photo of hypertrophic lupus erythematosus.

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

dcc007be-2037-4119-aff6-767d3a70bf3e_figure2.gif

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 19403. 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 keratoacanthoma4. Daldon et al. found that transepidermal elimination of elastin was present in 14 cases of HLE1. Recently, Ko et al. reported that a band of CD123 positive cells at the dermal-epidermal junction was characteristic of five cases of HLE2.

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. 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.

There is no definitive treatment for HLE. Options include topical or intralesional steroids, topical or oral retinoids, topical calcineurin inhibitors, thalidomide, hydroxychloroquine and surgical excision5,6. Winchester et al. reported on the efficacy TNF-alpha inhibitors7.

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

Consent

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

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Version 2
VERSION 2 PUBLISHED 21 Mar 2014
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how to cite this article
Hughes M, Gardner JM and Gao L. A case of hypertrophic lupus erythematosus with negative CD123 staining and transepidermal elimination of elastin [version 1; peer review: 2 approved with reservations]. F1000Research 2014, 3:76 (https://doi.org/10.12688/f1000research.3267.1)
NOTE: If applicable, it is important to ensure the information in square brackets after the title is included in all citations of this article.
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Open Peer Review

Current Reviewer Status: ?
Key to Reviewer Statuses VIEW
ApprovedThe paper is scientifically sound in its current form and only minor, if any, improvements are suggested
Approved with reservations A number of small changes, sometimes more significant revisions are required to address specific details and improve the papers academic merit.
Not approvedFundamental flaws in the paper seriously undermine the findings and conclusions
Version 1
VERSION 1
PUBLISHED 21 Mar 2014
Views
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Reviewer Report 29 Apr 2014
Theresa T. Lu, Autoimmunity and Inflammation Program and Pediatric Rheumatology, Hospital for Special Surgery, New York, NY, USA 
Approved with Reservations
VIEWS 29
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 ... Continue reading
CITE
CITE
HOW TO CITE THIS REPORT
Lu TT. Reviewer Report For: A case of hypertrophic lupus erythematosus with negative CD123 staining and transepidermal elimination of elastin [version 1; peer review: 2 approved with reservations]. F1000Research 2014, 3:76 (https://doi.org/10.5256/f1000research.3507.r4445)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
  • Reader Comment 28 May 2014
    Jerad Gardner, UAMS, USA
    28 May 2014
    Reader Comment
    Thank you for your commentary. The second version of this report will contain a histological photo of the positive control and negative CD 123 staining of the biopsy specimen.
    Competing Interests: No competing interests were disclosed.
COMMENTS ON THIS REPORT
  • Reader Comment 28 May 2014
    Jerad Gardner, UAMS, USA
    28 May 2014
    Reader Comment
    Thank you for your commentary. The second version of this report will contain a histological photo of the positive control and negative CD 123 staining of the biopsy specimen.
    Competing Interests: No competing interests were disclosed.
Views
25
Cite
Reviewer Report 14 Apr 2014
Victoria P. Werth, Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA 
Approved with Reservations
VIEWS 25
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 ... Continue reading
CITE
CITE
HOW TO CITE THIS REPORT
Werth VP. Reviewer Report For: A case of hypertrophic lupus erythematosus with negative CD123 staining and transepidermal elimination of elastin [version 1; peer review: 2 approved with reservations]. F1000Research 2014, 3:76 (https://doi.org/10.5256/f1000research.3507.r4229)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
  • Reader Comment 28 May 2014
    Jerad Gardner, UAMS, USA
    28 May 2014
    Reader Comment
    Thank you for your commentary. The title has been changed to more clearly reflect the absence of transepidermal elimination in this case. The treatments listed in the report were a ... Continue reading
COMMENTS ON THIS REPORT
  • Reader Comment 28 May 2014
    Jerad Gardner, UAMS, USA
    28 May 2014
    Reader Comment
    Thank you for your commentary. The title has been changed to more clearly reflect the absence of transepidermal elimination in this case. The treatments listed in the report were a ... Continue reading

Comments on this article Comments (0)

Version 2
VERSION 2 PUBLISHED 21 Mar 2014
Comment
Alongside their report, reviewers assign a status to the article:
Approved - the paper is scientifically sound in its current form and only minor, if any, improvements are suggested
Approved with reservations - A number of small changes, sometimes more significant revisions are required to address specific details and improve the papers academic merit.
Not approved - fundamental flaws in the paper seriously undermine the findings and conclusions
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