INTRODUCTION
Discoid lupus erythematosus (DLE) is an autoimmune disease that represents one of the subtypes of chronic cutaneous lupus erythematosus (CCLE). Apart from DLE, CCLE comprises chilblain lupus erythematosus (CHLE), a deep variant of lupus erythematosus (LE profundus — LEP), lupus erythematosus tumidus (LE tumidus — LET) and Blaschko’s linear lupus erythematosus (BLLE) [1]. DLE lesions that develop on the scalp may lead to permanent hair loss. A patient is presented with scarring alopecia, in whom dermoscopic examination allowed a selection of a suitable site for surgical biopsy (trichoscopy-guided biopsy) and make a diagnosis of underlying DLE.
CASE REPORT
A 60-year-old woman, with a history of hyperthyroidism, was admitted to the Department of Dermatology in Rzeszow for evaluation of gradually progressing irregular alopecic patches in the parietal-occipital scalp. The first patch of loss of hair, accompanied by pruritus, started to develop 4 years before hospitalization. Her previous treatment included numerous topical corticosteroid preparations of varying potency, minoxidil and an irritant capsaicin mixture, with no clinical improvement.
Dermatological examination showed several slightly depressed confluent foci of alopecia on the vertex arranged in the pattern of “footprints in the snow”. Under dermoscopy, white structureless areas on a predominantly pinkish-whitish background, and focally present honeycomb pigment pattern were observed in the parietal region. The follicular openings were absent. Dermoscopic findings differed in the occipital area, where red-brown dots, large yellow dots and pronounced honeycomb pigment pattern on a pink-white background were observed (Fig. 1). Ultraviolet (UV) enhanced-dermoscopy allowed for better visualisation of perifollicular scaling. In addition, under UV-dermoscopy dark round and oval structures, corresponding to hair follicles, were observed in place of red dots (Fig. 2).
The clinical presentation did not allow for a definite diagnosis. It was most consistent with the pseudopelade of Brocq, a controversial condition considered to be rather an end stage of various scarring alopecias than a separate entity [2–4].
Therefore, the decision to take a biopsy from the most representative region was made. Considering the presence of reddish dots under trichoscopy in the occipital region, this site was selected for biopsy and direct immunofluorescence. The histopathological examination showed dermal perivascular and peri adnexal infiltrate composed of lymphocytes and histiocytes; atrophic epidermis with vacuolar degeneration of the basal layer and some apoptotic keratinocytes; flattening of the rete ridges; lymphocytic infiltrate around hair follicles and vessels; as well as hyperkeratosis with condensed follicular keratotic plugs (Fig. 3). Fibrosis and scarring of the dermis were also present. Histopathology favoured the diagnosis of DLE. In extended laboratory work-up, low titters (1:160) of anti-nuclear (dsDNA) and anti-mitochondrial (AMA) antibodies were detected. Direct immunofluorescence was negative. Complement components C3 and C4 were within reference values.
magnification 400×) (B); perifollicular lymphocyte infiltration (HE staining, magnification 400×) (C); apoptosis and vacuolization (HE staining magni-
fication 400×) (D); hyperkeratosis at the hair follicle ostium (HE staining, magnification 400×) (E)
DISCUSSION
The differential diagnosis of scaring alopecia can be challenging and requires invasive biopsy. Dermoscopy is a non-invasive imaging method that is increasingly used for the evaluation of inflammatory skin conditions (inflammoscopy) and scalp diseases (trichoscopy) [5, 6].
When used to examine the scalp, this method can provide valuable assistance in the clinical differential diagnosis of different types of hair loss, both scarring alopecias (e.g. lichen planopilaris, DLE, frontal fibrosing alopecia), and non-scarring alopecias (e.g. alopecia areata, androgenetic alopecia). DLE affects the hairy scalp in up to 60% of patients [7, 8]. It accounts for about 30–40% of all scarring alopecias and about 60% of primary lymphocytic alopecias [2]. Despite such a high prevalence of conditions with permanent hair loss, one of the most common mistakes clinicians still make is taking biopsies from areas with completed scarring, as these areas are the easiest to identify. A scalp biopsy should be taken from an area where the hair follicles are affected by the inflammatory process, but still present [7].
The issue of dermoscopy-guided biopsies in scarring alopecias was initially raised by Miteva and Tosti [9]. The authors analysed 80 patients with cicatricial hair loss treated over 2 years at the Department of Dermatology, University of Miami. Patients with central centrifugal alopecia (CCCA), frontal fibrosing alopecia (FFA), lichen planopilaris (LPP), DLE and folliculitis decalvans (FD) were included in the study. Dermoscopy was used to visualize features indicative of disease activity at the margins of hairless patches. Identification of these features allowed for a definitive diagnosis in 95% of cases. When DLE is suspected, the authors strongly recommend taking a biopsy from a site showing follicular red dots under trichoscopy. On histopathological examination, follicular red dots correspond to dilated follicular ostia obstructed by keratin deposits and surrounded by dilated vessels and extravasated erythrocytes [9]. In DLE, these features are indicative of perifollicular infiltration with/without perifollicular fibrosis.
In recent years, the dermoscopic features of DLE have gained interest and have been reported by many other authors. These include red dots, large yellow dots, reduced numbers of follicles, whitish patchy scales, arborising vessels, follicular plugs and speckled pigmentation [10–13]. A summary of these features, including a division into the active and end-stage phases of DLE, is presented in Table 1.
|
Early (active) stages of DLE [15] |
Late stages of DLE [15] |
|
Thick arborizing vessels |
Thick arborizing vessels |
|
Big yellow dots |
Yellow dots with thin spide-like vessels |
|
Gentle scaling |
Milky red areas |
|
Scattered brown discoloration |
White patches |
|
Follicular red dots |
Loss of sebaceous lobules |
|
Reduced numbers of follicles |
Absence of follicular orifices |
|
Follicular keratotic plug |
Chrysalides and rossetes [16, 17] |
|
Peripilar sign [16] (brown hue around the hair follicle) |
Knowing the dermoscopic features of the active phase of the disease, it is possible to select the most diagnostic site for taking a skin specimen. This is particularly important in cases of atypical clinical presentation, in which clinical-histopathological correlation is extremely important.
Recently, there has been growing interest in UV-enhanced dermoscopy. UV light emitted by a Wood’s lamp (340–450 nm) may be useful in the differential diagnosis of conditions such as tinea capitis due to Microsporum canis or erythrasma, a bacterial infection caused by Corynebacterium minutissimum [14, 15]. The increasing availability of dermoscopy/trichoscopy combined with UV light with a wavelength of 365 nm potentially opens up new possibilities in the differential diagnosis of skin diseases, and thus a new direction for research. Recently, Pietkiewicz and Navarrete-Dechent described a bright-blue fluorescence of the burrow and a bright-green fluorescence of scabies mites under UV-dermoscopy [18].
In the presented patient, the use of UV-enhanced trichoscopy enabled better visualization of the follicular ostia and confirmed the initial selection of the biopsy site. It should be emphasized that the early phase of DLE, without clearly marked scarring, requires early diagnosis, as prompt implementation of appropriate treatment may prevent progression to the chronic phase and the development of permanent hair loss [19].
CONCLUSION
In scarring alopecias, early diagnosis and management are crucial to prevent permanent hair loss. Dermoscopy (trichoscopy) is a non-invasive imaging method of use for the evaluation of scalp conditions. It is postulated, that in all cases of alopecia, picking the spot for an invasive procedure of biopsy should be guided by dermoscopy (trichoscopy).
Article information and declarations
Acknowledgements
The authors would like to thank the patient for her involvement.
Author contributions
All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this article, take responsibility for the integrality of the work as a whole, and have given their approval for this version to be published.
Conflict of interest
Kinga Kołcz, Magdalena Żychowska, and Ewa Kaznowska declare that they have no conflict of interest. Adam Reich has worked as a consultant or speaker for AbbVie, Bioderma, Celgene, Chema Elektromet, Eli Lilly, Galderma, Janssen, Leo Pharma, Medac, Menlo Therapeutics, Novartis, Pierre-Fabre, Sandoz, and Trevi, and participated as Principal Investigator or Subinvestigator in clinical trials sponsored by AbbVie, Drug Delivery Solutions Ltd, Galderma, Genentech, Janssen, Kymab Limited, Leo Pharma, Menlo Therapeutics, MetrioPharm, MSD, Novartis, Pfizer, and Trevi. Adam Reich is a member of the journal’s Editorial Board.
Ethics statement
Informed consent was obtained from the patient for participation in the study and publication of the article, including publication of clinical photographs.
Funding
No funding was received for the publication of this article.
Supplementary material
None.
