Dermatology: Practical and Conceptual Letter to Editor | Dermatol Pract Concept. 2023;13(1):e2023044 1 Pigmented Macular Variant of Chronic Cutaneous Lupus Erythematosus: Clinico-dermoscopic Features of Three Patients With Skin of Color Biswanath Behera1, Rashmi Kumari2, Debasis Gochhait3, Pavithra Ayyanar4 1 Department of Dermatology, and Venereology, AIIMS, Bhubaneswar, India 2 Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India 3 Department of Pathology, JIPMER, Puducherry, India 4 Department of Pathology, AIIMS, Bhubaneswar, India Key words: macular pigment, lupus erythematosus, discoid, dermoscopy Citation: Behera B, Kumari R, Gochhait D, Ayyanar P. Pigmented macular variant of chronic cutaneous lupus erythematosus: Clinico-dermoscopic features of three patients with skin of color. Dermatol Pract Concept. 2023;13(1):e2023044. DOI: https://doi. org/10.5826/dpc.1301a44 Accepted: September 16, 2022; Published: January 2023 Copyright: ©2023 Behera et al. This is an open-access article distributed under the terms of the Creative Commons Attribution- NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited. Funding: None. Competing Interests: None. Authorship: All authors have contributed significantly to this publication. Corresponding Author: Rashmi Kumari, M.D., Associate Professor, Dept. of Dermatology, Venereology and Leprology, JIPMER, Puducherry, Pin-605006. Phone no- 09489692199, E-mail: rashmi.sreerag@gmail.com To the Editor, Chronic cutaneous lupus erythematosus (CCLE) is char- acterized by the presence of well-defined erythematous scaly plaques with associated atrophy, scarring, telangiectasia, and pigmentary alteration. The pigmented macular variant is a less recognized morphological variant [1]. All 3 cases in our series were female with Fitzpatrick skin phototype V. They presented with multiple, ill-defined, light-to-dark brown macules of variable size, predominantly on the face and head. Associated scarring was noticed only in Case #1. The dermoscopic feature was dominated by fol- licular plugging and pigment structures like brown dots, globules, homogenous areas, brown to blue-gray peppering, and brown pigment network. The brown to blue-gray pep- pering showed a peri-appendageal accentuation and resulted in target-like structures (central white clod/eccrine opening surrounded by brown structureless area) and perifollicu- lar gray ring. Case-1 showed also concentric structures as dark brown clod within a light brown clod (Figure 1 and 2). The clinico-dermoscopic details of the 3 cases are given in Table 1. Two 4 mm punch biopsies were done: one for histopathological examination and the other for direct im- munofluorescence from hyperpigmented macules. The histo- pathology of all the cases was diagnostic of CCLE. On direct immunofluorescence, linear deposition of immunoreactant IgG and, or IgM were noted along the basement membrane zone in all three cases. George et al in 1992 reported a hyperpigmented vari- ant of discoid lupus erythematosus and advocated that as- ymptomatic chronic hyperpigmented macules should alert the clinicians to suspect the diagnosis [1]. Boyd delineated 2 Letter to Editor | Dermatol Pract Concept. 2023;13(1):e2023044 a distinct profile of patients with solitary hyperpigmented macules over the face and neck without any scaling, at- rophy, scarring, or telangiectasia [2]. These lesions had a late- onset, short disease duration, lower incidence of photo- sensitivity, and overall better prognosis. They could clinically be mistaken for seborrheic keratosis, solar lentigo or lentigo maligna, but the histopathology was consistent with CCLE. The three cases in our series had multiple asymptomatic light-to-dark brown macules without any previous history of inflammation and were distributed primarily on the face and around the ear. The dermoscopic features, follicular plugging, brown to blue-gray dots, globules, or peppering in our series reflect the basic pathology of CCLE. The former corresponds to the dilated follicular infundibulum keratotic plugging, and the latter to the melanin in stratum corneum and to the der- mal pigment incontinence. Besides, we observed perifollic- ular gray ring and peri-eccrine target-like structures, which Figure 1. (A) Multiple ill-defined brown macules. (B) Dermoscopy under nonpolarized mode shows follicular plugging (blue arrow), brown dots, globules, and fine peppering (purple arrow), and concentric structures (red arrow), (C) Perifollicular gray ring (blue arrows), follicular plugging (red arrow), and perifollicular accentuated peppering (circle). (D) Brown globule (blue arrow), follicular plugging (red arrows), and perifollicular accentuated peppering (black arrow). (E) Peri-eccrine accentuation of peppering (blue ar- rows), and target-like structures (white clod surrounded by brown ring (red arrows). (F) Histology shows epidermal atrophy, melanin in stratum corneum, basal vacuolar degeneration, and pigment incontinence (H & E, x100). Letter to Editor | Dermatol Pract Concept. 2023;13(1):e2023044 3 possibly reflected the predominance of peri-appendageal interface dermatitis and the resulting pigment incontinence [3,4]. The  pigmented macular variant of CCLE will have more prominent pigment incontinence in skin of color. This feature may not be striking in Fitzpatrick skin phototype I-II. Diagnosing the pigmented macular variant of CCLE in skin of color, as in our patients, is challenging. It can mimic various acquired hyperpigmentation disorders such as li- chen planus pigmentosus (LPP), pigment contact derma- titis (PCD), and melasma. Besides, it can also be confused Figure 2. (A) Multiple ill-defined light brown macules on the ear. (B) Dermoscopy under nonpolarized mode shows blue-gray peppering (arrow) over a light brown background. (C) Histology shows epidermal atrophy, follicular plugging, interface dermatitis, and perifollicu- lar lymphocytes and macrophages infiltration (H & E, x100). (D) Multiple ill-defined dark-brown macules over the retro auricular area. (E)  Dermoscopy under nonpolarized mode shows follicular plugging (purple arrows), pigment network (red arrow), and peppering (blue arrows). (F) Brown pigment network, follicular plugging (red arrows), and periappendageal accentuated peppering (blue arrows). (G) Focal white homogenous area with overlying peppering (arrow). (H) Histology shows atrophic epidermis along with interface dermatitis (H & E, x100). Inset shows Periodic acid–Schiff positive thick basement membrane (PAS, x400). 4 Letter to Editor | Dermatol Pract Concept. 2023;13(1):e2023044 similarity to various facial pigmentary lesions. We describe the clinico-dermoscopic features in three patients with skin of color. Future studies will determine the role of dermos- copy in diagnosing and differentiating this rare entity from its clinical mimics. References 1. George R, Mathai R, Kurian S. Cutaneous lupus erythema- tosus in India: immunofluorescence profile. Int J Derma- tol. 1992;31(4):265-269. DOI: 10.1111/j.1365-4362.1992. tb03568.x. PMID: 1634292. 2. Boyd AS. Localized chronic cutaneous lupus erythematosus mas- querading as pigmented lesions: a new clinical subset?. Lupus. 2006;15(5):292-295. DOI: 10.1191/0961203306lu2304xx. PMID: 16761504. 3. Krueger L, Saizan A, Stein JA, Elbuluk N. Dermoscopy of ac- quired pigmentary disorders: a comprehensive review. Int J Dermatol. 2022;61(1):7-19.DOI: 10.1111/ijd.15741. PMID: 34235719. 4. Chatterjee M, Neema S. Dermoscopy of Pigmentary Disorders in Brown Skin. Dermatol Clin. 2018;36(4):473-485. DOI: 10.1016/j.det.2018.05.014. PMID: 30201156. with both benign and malignant pigment tumors such as flat seborrheic keratosis, large cell acanthoma, solar lentigo and lentigo maligna. Clinically, it may not always be possi- ble to diagnose and differentiate pigmented macular CCLE from other causes of acquired hyperpigmentation, includ- ing post-inflammatory hyperpigmentation. Dermoscopy may help in solving this problem. Dermoscopic features described for various acquired facial pigmentary conditions in skin of color are as follows: melasma and PCD show a brown pigmentation, pseudo-pigment network, brown to gray dots and globules, and characteristically spare the peri-appendageal areas; LPP is found to have diffuse brown pigmentation, pseudo-pigment network, slate-gray to blue dots and globules, hem-like pigment pattern, and perifollic- ular and peri-eccrine brown to blue-gray dots. In the future, immunohistochemistry stain for melanin and /or melano- cytes over a larger number of patients, as well as electron mi- croscopy, may help to support dermatoscopy over the naked eye in the diagnosis of CCLE. In conclusion, the clinical diagnosis of the pigmented macular variant of CCLE can be challenging due to its Table 1. Clinical and dermoscopic details of the pigmented macular variant of chronic cutaneous lupus erythematosus. Cases Gender/Age in years Location Duration in months Associated features Dermoscopic features Case-1 Female/35 Face, upper trunk Two Nil Brown dots, globules, and peppering Blue-gray peppering Follicular plugging Peri-appendageal accentuation of peppering Perifollicular gray ring Target-like structures Pseudo-pigment (brown) network Concentric structures Case-2 Female/30 Bilateral ear and infra-auricular area Three SLE Non-scarring alopecia ISM Brown homogenous area Blue-gray peppering Follicular plugging Perifollicular gray ring Case-3 Female/30 Bilateral ear, pre- and-post-auricular area Two SLE Lupus nephritis ISM Brown and white homogenous area Brown and blue-gray peppering Peri-appendageal accentuation of peppering Follicular plugging Perifollicular gray ring Brown pigment network SLE = Systemic lupus erythematosus.