Alopecias in Lupus Erythematosus
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Review Lupus Sci Med: first published as 10.1136/lupus-2018-000291 on 25 October 2018. Downloaded from Alopecias in lupus erythematosus Josef Symon Salgado Concha,1,2 Victoria P Werth1,2 To cite: Concha JSS, ABSTRACT of the hair in the absence of other causes.1 Werth VP. Alopecias in Several patterns of hair loss can occur in lupus Several processes that result in non-scarring lupus erythematosus. erythematosus (LE). Alopecias which show histological alopecia must therefore be ruled out before Lupus Science & Medicine characteristics of LE are LE-specific, and include discoid 2018;5:e000291. doi:10.1136/ attributing hair loss to LE (boxes 1 and 2). LE (DLE), diffuse or patchy hair loss in acute LE, subacute lupus-2018-000291 In this paper, we discuss an approach to cutaneous LE, and rarely tumid LE. Lupus hair in SLE is a recognising the different causes of hair loss poorly characterised entity and may be a form of telogen that occur in LE and their differential diag- Received 4 July 2018 effluvium. Alopecia areata can coexist with LE and may Revised 15 August 2018 mimic DLE. Non-lupus alopecias such as telogen effluvium noses. The categorisation we employ is largely Accepted 2 September 2018 and anagen effluvium have a myriad of causes which based on how scalp biopsy features are consis- include disease flares, drugs and stress in the setting tent with the diagnosis of LE. We also expand of LE. The latest validated Systemic Lupus International on the alternate diagnoses of non-scarring Collaborating Clinics classification criteria for SLE includes alopecia in LE. Certain points in the history non-scarring alopecia as a criterion; therefore, recognising and physical exam (which may necessitate the the aetiology of hair loss in the setting of LE is crucial in use of dermoscopy) can, in the majority of classifying a patient to have systemic disease. cases, lead the physician to make a confident diagnosis. However, non-scarring alopecia in SLE has a wide range of differential diagnoses (boxes 1 and 2) which can challenge a physi- INTRODUCTION cian’s clinical acumen. In a patient suspected Lupus erythematosus (LE) is a chronic multi- to have SLE but with an unclear aetiology of organ autoimmune disease with a spectrum hair loss, working closely with dermatologists of clinical and serological presentations.1–3 and performance of ancillary tests such as The major target organs are the joints, skin, a scalp biopsy, direct immunofluorescence kidneys, lungs, and the nervous and serous (DIF) and/or serological markers may be systems, with ANA as the frequent hallmark warranted. http://lupus.bmj.com/ antibody.1 2 4 At any point during the disease course of SLE, dermatological findings may be found in over 80% of patients.4–7 Certain OVERVIEW OF ALOPECIAS IN LE presentations of LE on the skin and hair can LE-specific alopecia is classically exempli- aid in assessing, classifying and predicting fied by scalp discoid lupus erythematosus systemic involvement.4 8–10 (DLE), which is typically scarring.13–16 Several Hair loss is a frequent occurrence in SLE non-scarring forms of hair loss such as lupus on September 27, 2021 by guest. Protected copyright. and is present in more than half of the hair, alopecia areata and telogen/anagen patients at some point during the course of effluvium do not display biopsy features of LE the disease.8 11–14 Although several patterns of and are considered either LE non-specific or © Author(s) (or their hair loss can exist in the setting of SLE, the just coincidental with LE.14–16 Subacute cuta- employer(s)) 2018. Re-use aetiology is not always specific to LE (box 1). neous lupus erythematosus (SCLE), tumid permitted under CC BY-NC. No commercial re-use. See rights Determining whether alopecia is inherent LE and acute LE display histopathological and permissions. Published by to LE or just coincidental to LE is crucial features consistent with LE and are lupus-spe- BMJ. because it has been included in several clas- cific alopecias.17–24 However, attribution is not 1Corporal Michael J Crescenz sification systems for SLE (table 1), including always straightforward because LE-specific VAMC, Philadelphia, the latest Systemic Lupus International alopecias can coexist with the more common Pennsylvania, USA 2 Collaborating Clinics (SLICC) classification causes of hair loss and can possess similar clin- Department of Dermatology, 1 14 19 25–27 Perelman School of Medicine at criteria. Non-scarring alopecia, in particular, ical features. Moreover, it should be the University of Pennsylvania, has been incorporated in the SLICC criteria kept in mind that certain cutaneous findings Philadelphia, Pennsylvania, USA because its specificity to SLE is high (95.7) on the scalp (DLE, SCLE, diffuse non-scar- in the derivation sample, and it met the stan- ring hair loss due to acute LE) are distinct Correspondence to 1 2 Dr Josef Symon Salgado dards of clinical consensus among experts. items in the classification criteria for SLE and Concha; Josef. Concha@ uphs. Non-scarring alopecia is clinically defined by may count as separate items in fulfilling the upenn. edu the SLICC as diffuse thinning and fragility criteria (table 1). Concha JSS, Werth VP. Lupus Science & Medicine 2018;5:e000291. doi:10.1136/lupus-2018-000291 1 Lupus Science & Medicine Lupus Sci Med: first published as 10.1136/lupus-2018-000291 on 25 October 2018. Downloaded from Box 1 Alopecias in lupus erythematosus Box 2 Differential diagnoses of alopecias Lupus-specific alopecia. Scarring alopecias. ► Discoid lupus erythematosus.* ► Lichen planopilaris. ► Acute lupus erythematosus.† ► Frontal fibrosing alopecia. ► Subacute cutaneous lupus erythematosus.† ► Central centrifugal cicatricial alopecia. ► Tumid lupus erythematosus.† ► Pseudopelade of Brocq. Tinea capitis (late stage). Lupus non-specific alopecia. ► ► Lupus hair.† Non-scarring alopecias. ► Alopecia areata/ophiasis.† ► Patterned hair loss. Acute diffuse and total alopecia areata. Non-lupus alopecia. ► ► Trichotillomania. ► Telogen effluvium.† ► Syphilitic alopecia. ► Anagen effluvium.† ► Tinea capitis (early stage). *Non-scarring in its early stage. †Typically non-scarring. Epidemiology Scalp is the most common site affected by DLE, occurring LUPUS-SPECIFIC ALOPECIA in 30%–50% of reported cases.29–31 In its early stages, DLE DLE of the scalp lesions are only slightly inflammatory and non-scarring.25 The scarring alopecia of DLE is an LE-specific cutaneous However, as much as 60% of patients with scalp DLE lesion according to Gilliam’s classification of cutaneous continue to progress towards permanent and irrevers- 32 LE.28 DLE is a cutaneous item in the SLICC criteria and is ible hair loss. It accounts for 30%–40% of all scarring 33–35 counted separately from non-scarring alopecia.1 alopecias combined and about 60% of all primary lymphocytic alopecias, making it the most common scarring alopecia in this subgroup.14 33 In a prospective cross-sectional study, the proportion of patients with scar- Table 1 SLE criteria throughout the years with cutaneous 1 2 ring alopecia did not differ across LE subgroups (gener- features alised DLE, localised DLE, systemic LE).31 Although scalp Alopecia as a DLE is more frequent in women,31 32 35 there is an equal Criteria Criteria items criterion proportion of both sexes in terms of the tendency to 31 1971 ACR 6 cutaneous items Rapid loss of a large develop scarring alopecia from DLE. The age of onset 14 30 33 35 (malar rash, discoid amount of scalp is usually between 20 and 40 years of age, and it http://lupus.bmj.com/ rash*, Raynaud’s hair, by patient’s is reported to be most prevalent among African–Ameri- phenomenon, alopecia, history or physician’s cans.30 32 The presence of scarring alopecia in a patient photosensitivity, oral/ observation.† with DLE portends a prolonged disease course.14 31 nasopharyngeal ulcers). 1982 ACR 4 cutaneous items Criteria do not include (malar rash, discoid alopecia as an item. Pyatholog rash*, photosensitivity, The hallmark finding in cicatricial alopecias is fibrosis on September 27, 2021 by guest. Protected copyright. oral ulcers). of the follicular structure, which is appreciated clinically 36 1997 ACR 4 cutaneous items Criteria do not include as obscured follicular openings on the scalp. The scar- (malar rash, discoid alopecia as an item. ring process usually begins with the inflammatory cells rash*, photosensitivity, infiltrating the follicular bulge, a contiguous part of oral ulcers). the outer root sheath located at the insertion point for 2012 4 cutaneous items Diffuse thinning or the arrector pili muscle. This marks the bottom of the SLICC (acute cutaneous hair fragility with permanent portion of hair follicles and is also where the lupus erythematosus, visible broken hairs epidermal hair stem cells are located.14 Once replace- subacute cutaneous in the absence of ment by fibrous tissue is established, hair loss is perma- lupus erythematosus*, other causes such nent and irreversible.36 37 Scarring alopecias are usually oral ulcers, non- as alopecia areata, either (1) primary, in which the hair follicle is the main scarring alopecia). drugs, iron deficiency and androgenetic target of inflammation; or (2) secondary, if a separate alopecia.† disease process causes destruction of the nearby follicular structure.34 In 2001, the North American Hair Research *Can also present clinically as alopecia. Society proposed a classification scheme for the primary †Definition does not require histopathological/immunopathological confirmation. scarring alopecias based on the predominant inflamma- ACR, American College of Rheumatology; SLICC, Systemic Lupus tory cell type on histology (lymphocytic, neutrophilic, 36 International Collaborating Clinics. mixed, non-specific). Scalp DLE serves as a model for 2 Concha JSS, Werth VP. Lupus Science & Medicine 2018;5:e000291. doi:10.1136/lupus-2018-000291 Review Lupus Sci Med: first published as 10.1136/lupus-2018-000291 on 25 October 2018.