Prednisolone

Distinctive lupus panniculitis of scalp with linear alopecia along Blaschko’s lines: a review of the literature

Suparuj Lueangarun1, MD, MSc, Urairack Subpayasarn1, MD, MSc, and Therdpong Tempark2, MD

Abstract

Lupus panniculitis of the scalp (LPS) is a rare and distinctive clinical feature of lupus erythematosus panniculitis (LEP) with linear alopecia along Blaschko’s lines. In this study, we investigated clinical features and treatments of LPS by literature review of articles in the English language from PubMed and SCOPUS databases up to April 2018. The following key words, “lupus panniculitis, “lupus erythematosus panniculitis”, “lupus profundus”, “head”, and “scalp”, were used. Twenty cases of LPS were identified
(mean age = 26.4 [10–53] years, female: male ratio = 1:1, mean disease duration = 86.89 [8–336] weeks). The most commonly affected areas of scalp included parietal (70%), frontal (45%), temporal (40%), occipital (30%), and vertex (10%), along 70% of Blaschko’s lines with morphologic lesions linear, annular, arch-shaped, and ulcer. Besides, ANA (60%) was in particular noted. Hydroxychloroquine, oral prednisolone, intralesional corticosteroid, and methotrexate were the most common treatments, with complete response after an average period of 8.08 (2–12) weeks. Systemic lupus erythematosus (SLE) was developed in four cases (20%) during follow-up, with high recurrence of 35%. We reported distinctive and rare cases of LPS.The continuing follow-up was highly recommended to avoid probable recurrence and SLE development.

Introduction

Lupus erythematosus panniculitis (LEP) is a clinical disorder of inflamed subcutaneous fat rarely found in only 1–3% of patients with lupus erythematosus (LE) at the most frequent areas of upper arms, shoulders, face, and buttocks.1,2 Lupus panniculitis of the scalp (LPS) is clinically a distinctive manifestation of LEP along the lines of Blaschko and beyond3 of linear, arch, or annular alopecia (Fig. 1). Thus, a review of the literature of 20 cases with distinctively presented LPS was performed to investi- gate its clinical features and potential treatments.

Materials and methods

We conducted a literature review of the related full-text English articles from PubMed and SCOPUS databases up to April 2018, using the key words, “lupus panniculitis, “lupus erythematosus panniculitis”, “lupus profundus”, “head”, and
“scalp”, which were particularly screened and selected in both Figure 1 A case with lupus panniculitis of the scalp presenting with
linear non-scarring alopecia along the Blaschko’s line 1

Discussion

In 1976, LEP of varying patterns on the scalp was firstly reported, with subsequent 19 described cases, by Mark et al. in different ethnic groups of genetic LEP predisposition. 11 Commonly, the most morphologic presenting lesions include linear (55%), arch-shaped (35%), annular (30%), and ulcer (10%). The LPS affected areas along the Blaschko’s lines 12 was depicted in Figure 2, especially at the mean onset age of
26.1 years compared to 27 years of the non-Blaschko’s lines, with shorter duration of diseases prior to diagnosis (60 vs. 125 weeks), lower percentage of ANA positivity (50% vs. 83.3%), and less SLE development (7.1% vs. 50%). Nonetheless, there was no apparent pathogenesis of LEP along Blaschko’s lines, with the cellular mosaicism from the mutation during embryogen- esis in the genetically abnormal clone of cells before migration into deeper component structure such as lipocytes or fibroblasts.2 These present as new antigens that trigger local inflammatory responses following the exposure to stimulations,13 with immune tolerance depletion due to local LE inflammation.12
The clinical presentation of hair loss in LPS varies with the extent of inflammation. In the purely subcutaneous form, the fol- licular bulbs are targeted by lymphocytes, while the permanent part of the follicle is spared. In this instance, the process resulted in non-scarring alopecia, which could have the over- lapped clinical presentation with alopecia areata and may poten- tially produce short exclamation and dystrophic hairs as a clinical and dermoscopic sign in addition to yellow plugs. Once the dermis is also involved, the follicles are often consequently destroyed with a scarring alopecia.

Despite the appearance of LEP surface with DLE features,1 other scalp abnormalities could possibly be presented, including scarring alopecia (five cases, 25%),8,11,14,15 erythema (14 cases, 70%),3,7–10,14–20 induration (five cases, 25%),8,14,16,17,18 tenderness (six cases, 30%),7–9,16,18,19 mass (two cases, 10%),19,21 skin atrophy (two cases, 10%),7,20 and follicular papule (5%).15 Although, there were 20% of the alopecia cases without other abnormal physical findings on the scalp. 4,20,22,23 Consequently, the extent of inflammation in LPS could be involved from the subcutaneous that caused lipoatrophy and to the dermis that resulted in skin atrophy and scar.
No skin atrophy or sclerotic changes were observed at the alopecia areas of sparse hair,4 faint interfollicular erythema, and follicular plugging. The dermoscopic examination showed hair loss patch with miniaturization hair and empty follicle, as well as follicular opening with yellow dots, perifollicular white scale, and some of patchy erythematous areas but no broken hairs (Fig. 3). In addition, the perifollicular white dot associated with honeycomb pigment pattern was also seen at some alopecia areas.3 With noninvasive dermoscopy, the clinical diagnosis of LPS could be facilitated for the differentiation from other alope- cia types. Numerous vellus hairs with or without diffuse telang- iectasia and follicular plugging were presented with epidermal changes. However, there were no typical broken hairs in alope- cia areata or trichotillomania and the loss of follicular opening (fibrotic white dots) in primary scarring alopecia.11
The scalp lupus panniculitis could be a solitary presentation, while multiple sites were also involved with other associated clinical presentations, including LEP of parotid gland and eye- lid,19 axilla,15 bilateral cheeks and upper arms,8 subcutaneous nodule on the elbow,11 left periorbital erythema, and swelling of the face with progression to facial hemiatrophy, facial nerve paralysis, and enophthalmos.8

The differential diagnoses with clinical and dermoscopic examination of the linear non-scarring alopecia, including alope- cia areata, trichotillomania, and traction alopecia, could importantly yield explicit data for better diagnosis. Hair loss could be presented in alopecia areata, with a band-like distribu- tion along the hairline,24 mostly shown as dermoscopic yellow dots, black dots, and exclamation mark hair.25 Although the bizarre-shaped patches of hair loss was usually found in tri- chotillomania, with the multiple dermoscopic broken hair shaft abnormalities such as flame-shaped and tulip hair sign without significant inflammation or changes in the perifollicular area.25,26 Meanwhile, the history of a traction hairstyle, the traction corre- lated frontotemporal pattern of hair loss, the fringe sign, and the dermoscopic findings of cylindrical white hair casts,27 broken hairs, and black dot should be comprised in diagnosis of trac- tion alopecia.28 Moreover, the differential diagnosis of linear alopecia should also cover linear morphea of paramedian face and scalp or en coup de sabre following the linear presentation and the reported alopecia along Blaschko’s lines.29 In particular, the linear sclerotic atrophic plaques may cause cicatricial alope- cia while extending onto the scalp.30,31 The laboratory findings showed antinuclear antibody (ANA) of 60%, which was less than the typical LEP (95.2%).5 There were three cases (15%) of LEP with positive ANA and SLE develop- ment during the follow-up.8,14 In addition, Anti-Ro/SSA antibody was positive in 10%16,18 and compatible with the detection rate in chronic cutaneous LE (22%).30 .The histopathologic examination revealed a lobular lymphocytic panniculitis (100%) with mucin deposition (65%),3,4,9,10,15–17,20–23 hyaline fat degeneration (60%),3,4,7,9–11,15,16,18,20,22 basal vac- uolization or thickening of basement membrane (20%),7,11,15,18 and epidermal changes of DLE (30%).3,7,11,15,19,20 The infiltration by plasma cell (15%)4,18,22 and nodular lymphoid aggregation (15%)9,15,18 was also illustrated in Figure 4, while direct immunofluorescences (DIF) was positive in 20%, comprising of IgM, IgG, and C3 at basement membrane,16,18,19 and follicular epithelium15 as typical LEP.1 Besides, the immunohistochemical studies of LPS revealed the presence of CD3+, CD4+, CD8+, CD56-T cell with normal CD4: CD8 ratio and no abnormal poly- clonality on T-cell receptor gene rearrangement21 similar to the typical LEP.6

However, the clinical presentations and histology of LEP needed to be differentiated from subcutaneous panniculitis-like T cell lymphoma (SPTCL) of typical painless subcutaneous nod- ules or poorly-circumscribed indurated plaques on the extremi- ties, trunk, and face with weight loss, fever, and fatigue (B symptoms).31 The histopathologic examination of SPTCL revealed atypical lymphocyte infiltration within the subcutaneous tissue, cytophagocytosis, and adipocytes rimmed by atypical lymphocytes. In addition, the immunohistochemistry showed the features of cytotoxic T-cell and polyclonal T-cell receptor gene rearrangements.32 Other differential diagnoses of histology find- ing lobular panniculitis in LEP included erythema induratum of Bazin, pancreatic panniculitis, cold panniculitis, and post-steroid panniculitis.1 prednisolone (55%), intralesional corticosteroid (30%), and methotrexate (10%), while other treatments included mycophe- nolate mofetil (MMF), dapsone, pulse methylprednisolone, oral cyclophosphamide, gold therapy, intravenous pulse cyclophos- phamide, topical steroid, and topical minoxidil. The mean dura- tions of response to treatment were 8.08 (2–12) weeks, with complete response (30%) and improvement (60%). The scarring alopecia and skin atrophy were present in five cases (25%) and three cases (15%), respectively, after treatment. Hence, the early combination treatment is suggested due to deep scalp involvement, possibility of scarring alopecia development of LPS, and good response to treatment in most frequent reported cases. However, the recurrence rate was high in 35%. Considerably, four cases (20%) developed SLE (two cases with lupus nephri- tis14,19) during the median duration of 9 (2–15)8,14,19 years of follow-up, and one case had Parry-Romberg syndrome,8 although three cases with SLE development during follow-up were noted with positive ANA8,14 and two cases had previous DLE.8In conclusion, the distinctive features of lupus panniculitis of the scalp are reviewed with clinical, dermoscopic
findings, histopathology, immunohistochemistry, and treatments. Combi- nation therapy yields significant treatment outcome and improvement. However, the continuing follow-up is truly sug- gested to avoid ANA positivity, high possibilities of recurrence, and risk of SLE development.

Acknowledgments

The authors would like to extend special thanks to Dr. Worapop Suthiwartnarueput for his histopathological photo and analysis, as well as Ms. Sunattee Kessung for her assistance in editing and revising this manuscript.

Authors’ Contribution

Dr. Suparuj Lueanagrun was responsible for the initiation, exe- cution of the project, data collection, and analysis and drafting of the paper. Dr. Urairack Subpayasarn was responsible for the review of the literature and drawing the graphic. Dr. Suparuj Lueanagrun and Dr. Therdpong Tempark were responsible for the critical revision of manuscript and approved the final paper.

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