2Department of Medicine, Mackay Medical College, New Taipei City, Taiwan
3Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan
4Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
Keywords: lines, Linear; Lupus Erythematosus; Facial; Childhood;
The linear distribution of our patient is different from the typical DLE lesions. Linear Cutaneous Lupus Erythematosus (LCLE) was first described in 1978 [2]. It is a term proposed for DLE with a linear configuration [3]. This unusual presentation of lupus erythematosus is mostly seen in children and young adults. Till now, fewer than 30 cases in both adults and children have been published [4, 5]. Among them, only 15 cases of childhood diagnosed as facial LCLE have been reported (Table 1) [3, 6-16]. Nearly half of the cases presented with lesions before the age of 7 years. There seems to be a female predominance in children LCLE, although the female predominance is not as significant as in SLE patients. The typical clinical manifestations are single or multiple erythematous, atrophic, hyperkeratotic, dyschromic linear or annular plaques, patches, or even localized non-scarring alopecia distributed along the lines of the head and neck. The lesions are usually asymptomatic, with a low incidence of photosensitivity. This unique presentation can be considered as DLE following the lines of Blaschko, but other types of lupus with a linear distribution, such as Lupus Erythematosus Profundus (LEP), have also been described in some pediatric patients [15- 20]. Most reported findings in DIF are variable and DIF was not performed in nearly half of the cases. Serological analysis of these patients reveals that some autoantibodies (especially ANA) could be found in few cases, which suggests that the linear lesions might have the potential of triggering systemic autoimmune reaction. We noticed a low rate of progression to systemic disease in this group. However, the follow-up time of most cases in this group is not long enough. It has not been conclusively proven that weather these cases would eventually progress to SLE. A regular long-term follow up is still mandatory.
lines of the face and neck region form an unique arch shape from the oral angle to the orbital region on the face (Figure1c) [21, 22]. Blaschko-linear lesions probably reflect a mosaic condition from post-zygomatic somatic mutation or
References |
Age at onset, |
Types of LCLE |
Facial localization |
Laboratory exam |
DIF |
Systemic involvement |
Treatment |
Abe et al. [3] |
3/female |
CDLE |
Right cheek |
ANA: 1:80 homogeneous speckled |
IgM, C3 at the BMZ |
No |
Oral: dapsone |
Abe et al. [3] |
11/female |
CDLE |
Left side of chin and neck |
Unremarkable |
Negative |
No |
Oral: dapsone, |
Lee et al. [6] |
4/male |
CDLE |
Left upper cheek, perioral area |
Unremarkable |
IgM at DEJ |
No |
Oral: hydroxychloroquine |
Green and Baker [7] |
7/male |
CDLE |
Cheeks, NLFs, occiput, parietal scalp |
ANA: 1:80 (+) speckled |
NP |
No |
Topical: 0.05% aclometasone diproprinate, 0.2% hydrocortisone valerate |
Davies and Newman [8] |
12/female |
CDLE |
Left cheek |
Unremarkable |
NP |
No |
Oral: Dapsone |
Choi et al. [9] |
6/female |
CDLE |
Nose |
Unremarkable |
NP |
No |
Oral: hydroxychloroquine |
Requena et al.[10] |
3/male |
CDLE |
Right side of chin and neck |
Unremarkable |
NP |
No |
Topical: |
Imhof et al. [11] |
15/ female |
CDLE |
Right upper cheek, perioral area |
Unremarkable |
IgM, IgG, C3 at DEJ |
No |
Oral: hydroxychloroquine, isotretinoin |
Kawachi et al. [12] |
4/female |
CDLE |
Right side of cheek, auricle, neck |
Unremarkable |
IgM, C3 along DEJ |
No |
Topical: 0.03% tacrolimus ointment |
Jin et al. [13] |
9/male |
CDLE |
Forehead, left check, preauricular region |
Unremarkable |
IgG, IgA, IgM, C3 along DEJ |
No |
Oral: |
Jin et al. [13] |
12/female |
LCLE |
Right corner of mouth to right side of neck |
Unremarkable |
NP |
No |
Oral: |
Jin et al. [13] |
12/male |
LCLE |
Right side of forehead, nose, cheek, eye, right corner of mouth to neck |
ANA: 1:320 (+), C3: 0.70 g/L, C4: 0.14 g/L |
Negative |
No |
Oral: |
Ma et al. [14] |
6/male |
LCLE |
Left mandible, submandibular triangle, anterior auricle |
Unremarkable |
NP |
No |
Topical: |
Nagai et al. [15] |
10/female |
LEP |
Left side of forehead temporal area |
ANA: 1:320 (+) homogenous speckled |
NP |
No |
Topical: corticosteroids |
Tamiya et al. [16] |
6/female |
LEP |
Left forehead to the left glabella, nose |
Unremarkable |
IgM (+) |
No |
Oral: Prednisolone |
Present case |
4/female |
CDLE |
Right cheek and scalp |
anti-nDNA antibodies |
IgG, IgM, IgA, C3 at DEJ |
No |
Oral: |
The differential diagnosis of Blaschko-LCLE includes other acquired inflammatory diseases distributed along Blaschko's lines, such as lichen striatus, linear lichen planus, linear morphea, linear granuloma annulare, or linear psoriasis, etc. Skin biopsy is helpful but often not sufficient to make the correct diagnosis. It may be impossible to differentiate lichen striatus from LCLE [26]. Histologically, these two conditions share many common features. It is necessary to perform DIF to differentiate equivocal cases. Physicians should also take into account clinical presentation, histological evaluation and response to treatment before making a diagnosis of Blaschko-LCLE.
Previously reported therapies for facial LCLE of childhood include topical steroids [7, 8, 10, 14-16], pimecrolimus [11], tarcrolimus [12-14], oral steroids [16], dapsone [3, 8], hydroxychloroquine [6, 9, 11, 13], isotretinoin [11], and intense pulsed light [11]. However, patients show various responses to these therapies. Our patient improved after oral hydroxychloroquine and is still followed up regularly.
In conclusion, Blaschko-LCLE is a distinct subtype of lupus erythematosus, often occurring in children. Its typical clinical manifestation is Blaschko-linear distributed skin lesions with cutaneous manifestations of DLE or LEP. Photosensitivity and serological autoantibodies are often absent, with minimal possibility of progression to SLE. Blaschko-LCLE patients provide a promising model for studying underlying immunological and molecular mechanisms of cutaneous mosaicism.
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