Erythematoviolaceous Papules and Plaques on Sun-Exposed Areas

Abstract

A 71-year-old Hispanic female patient presented to the dermatology department with a 6-year history of cutaneous lesions. Personal medical history was notable for hypertension, diabetes mellitus, and previous breast cancer. Her chronic treatment consisted of metformin, insulin, irbesartan and omeprazole. She reported lesions appearing first on her scalp and face with significant pruritus and then extending to the back of her upper extremities, with worsening after sun-exposure. Previous treatment with topical and oral steroids had a poor response.

On physical examination, erythematoviolaceous papules and plaques, some with annular configuration and diffuse white scaling were seen (Figure 1). Dermoscopy revealed shiny white structures in some areas. In other parts, a pinkish–red background with whitish scales, orange–yellow structureless areas, and a mixed vascular pattern (dotted, linear and branched vessels) was evidenced (Figure 2).

Laboratory testing revealed anaemia (haemoglobin: 11.1 mg/dL) with normal leucocyte and platelet count. Kidney and liver function tests were normal. Positive antinuclear antibodies (ANA) and extractable nuclear antigen antibodies (ENA) were found, with detection of Anti-Ro and Anti-La antibodies. Anti-ds-DNA antibodies were negative. No other lab anomalies were detected. Skin biopsies from her back and left arm were done (Figure 3).

CLE/LP overlap syndrome is very rare, with few cases published so far. Diagnostic criteria define classic CLE/LP overlap syndrome as patients with mixed clinical characteristics of both CLE and LP, histopathological characteristics consistent with LP (with or without CLE features), and positive serologic markers of CLE (positive ANA with 1:80 or higher titers, ENA antibodies, double-stranded DNA antibodies, or antiphospholipid antibodies) [1, 2]. Our patient fulfilled all the criteria. Direct immunofluorescence (DIF) can be helpful, but is not necessary for diagnosis, and can present features of either CLE or LP [2].

According to literature, cutaneous lesions in this entity most often affect the extremities, face, and trunk. They have been described as centrally atrophic, hypopigmented, with scaling, sometimes painful or pruritic [3, 4]. However, a wide variety of different morphologies have been reported, with oral compromise also being present in many cases [2]. Our patient presented with lesions in photo-exposed areas, some of them morphologically consistent with lichen planus and some more suggestive of psoriasiform subacute cutaneous lupus. She also presented autoantibodies more commonly found in the subacute form of CLE. The coexistence of these findings has been reported before [5, 6].

In addition to presenting clinical overlapping features of both CLE and LP, dermoscopic findings in our patient were also consistent with both conditions. Classic dermoscopic elements described in subacute cutaneous lupus such as white scales, pink–red background, orange–yellow structureless areas and mixed vascular pattern [7] were all present in our patient. No follicular keratotic plugs, typical of chronic cutaneous lupus [8], were found. Dermoscopy in other areas also revealed white structures resembling Wickham striae, suggestive of lichen planus [9].

Due to its infrequency, there is no standardised treatment for this condition. Case reports have evidenced efficacy to oral steroids, antimalarials, thalidomide and acitretin [2]. Our patient had an excellent response to treatment with hydroxychloroquine.

All authors equally contributed to the elaboration of the manuscript. Dr. Martín-Zamora, Dr. Gamboa-Flores, Dr. Pozuelo-Díaz and Dr. Campos-Hidalgo contributed with the elaboration and edition of the manuscript. Dr. Martín-Zamora, Dr. Gamboa-Flores, Dr. Pozuelo-Díaz took the clinical and dermoscopic pictures of the patient. Dr. Campos-Hidalgo took the histologic pictures of the biopsies and their descriptions.

All patients in this manuscript have given written informed consent for participation in the study and the use of their deidentified, anonymized, aggregated data and their case details (including photographs) for publication. Ethical Approval: not applicable.

The authors declare no conflicts of interest.