Linear lupus erythematosus profundus on the scalp following the lines of Blaschko

ARTICLE

Auteur(s) : Yayoi NAGAI¹, Osamu ISHIKAWA², Tomoyasu HATTORI², Tetsushi OGAWA³

¹ Division of Dermatology, Tone Central Hospital, 1855-1, Higashiharashinmachi, Numata city, 378-0053 Gunma, Japan
² Department of Dermatology, Gunma University School of Medicine
³ Division of Pediatrics, Tone Central Hospital

Article accepted on 30/01/2003

Linear configuration of cutaneous lupus erythematosus or discoid lupus erythematosus (DLE) in childhood has rarely been described. We report a girl with lupus erythematosus profundus (LEP) displaying a blaschko linear pattern.

Case report

A 10-year-old Japanese girl presented with a 2 month history of hair loss of the scalp on April 9, 2001. She had complained of general fatigue and slight fever for a month, but not of clinical photosensitivity. She suffered from congenital bilateral hearing loss. There was no family history of any autoimmune rheumatic diseases.
Physical examination revealed linear alopecia on the left temporal area. On the parietal area, arch-shaped alopecia was also noted around the right side of a hair weal (Fig. 1). These two lines of alopecia were not continuous. The overlying skin showed normal color without atrophic or sclerotic change. Furthermore, band-like erythema existed on the left side of the forehead, which seemed to extend to the alopecia (Fig. 2).
Histological examination of erythema of the forehead revealed edema in the dermis with slight perivascular and periappendageal infiltrate of lymphocytic cells. The epidermis was normal with no liquefaction degeneration. There were no vacuolar alterations at the basement membrane zone of the skin appendages. In the subcutaneous tissue, fat degeneration was observed along with slight lymphocytic infiltration (Fig. 3). Characteristically, abundant deposition of a mucoid material throughout the dermis and the fat tissue was demonstrated by Alcian blue stain. Histological examination of alopecia of the scalp also showed fat degeneration with less lymphocytic infiltration. Hair roots were atrophic, and slight lymphocytic infiltration and abundant mucin deposits were noted around them. Immunofluorescence microscopy was not performed.
Laboratory investigations revealed normal values for complete blood cell count, erythrocyte sedimentation rate, liver function test, immunoglobulin level and urinalysis. Anti-nuclear antibody (ANA) was positive at 1:320 with homogenous speckled type. Negative or normal laboratory data included anti SS-A/SS-B, RNP and Sm antibodies, anti-double-stranded DNA antibody and CH50. Since these clinical and laboratory findings do not fulfil the classification criteria for systemic lupus erythematosus, we diagnosed her as having LEP, mainly based on the histological findings.
With the application of a topical corticosteroid, erythema was improved but alopecia has shown no change during a one and a half year follow up period.

Discussion

LEP is a rare variant of LE. The lesion is usually noted as a subcutaneous nodule or induration that preferentially develops on the face, buttock, upper arm or thigh. The overlying skin is often unchanged, but occasionally coexists with discoid lesions. The diagnosis of LEP is clinically difficult when it develops in the absence of other typical cutaneous or systemic manifestations.
LEP is so extremely rare in children that only a few cases have been reported [1-6]. A review of the pediatric cases [1] pointed out female preponderance and preferentially involved sites such as the face, shoulder and upper arm. The epidermis may look normal or resemble DLE. The patients often have systemic lupus erythematosus (SLE), with positive ANA (27%) or anti DNA antibody (15%).
In our case, the clinical diagnosis of LEP was difficult because of the linear distribution without surface change. But with respect to the histological change, LEP shows an image of lobular panniculitis with hyalinization of adipose tissue, as well as perivascular, periadnexal or dermo-epidermal lymphoid infiltration. Mucinous deposition through the dermis may be helpful to confirm a diagnosis of LEP.
Including the present case, seventeen cases with cutaneous LE have been reported to show a linear lesion [2, 7-18]. Seven patients had the lesion on the face, eight on the extremities, and three on the trunk. In three patients, the lesions developed on two different areas. Three cases of LEP with linear distribution have been reported [2, 16, 17], but our patient is the only one who had a linear lesion on the scalp. The patients’ age of onset ranged from 4 to 55 years and in nine cases, the lesions developed in childhood. Ten cases noted the relationship with Blaschko’s lines, and other cases also seem to be associated with these lines. Neither photosensitivity nor progression to SLE was documented in these patients. Overall, several important characteristics of patients with linear LE are as follows; childhood onset, blaschkolinear distribution and a low rate of progression to systemic disease. The eruption of LEP as well as other symptoms seems to respond promptly to oral predonisolone. Some cases of linear cutaneous lesions were improved considerably with oral dapsone[7, 8, 14, 18] or anti malarial agents [11, 13].
Inflammatory blaschkolinear lesions include a variety of clinical and histological appearances, such as linear scleroderma, linear cutaneous LE, linear psoriasis, linear Darier’s disease and linear lichen nitidus [19, 20]. So Blaschko’s lines have been suggested for this group of disorders [10, 21] and sometimes it is not possible to differentiate fully between them. In our patient, the most probable differential diagnosis is linear scleroderma en coup de sabre that often shows blaschkolinear distribution [20, 22]. The histological findings enabled us exclude the diagnosis of linear scleroderma. We differentiated our case from it by the histological findings. The coexistence of DLE and linear scleroderma has been reported [23-25], in which the authors emphasized the definite but rare relationship between the cutaneous forms of scleroderma and LE.

Blashcko’s lines are considered to be the expression of epidermal rather than dermal mosaicism. The nature of the lines, however, still remains a matter of debate. On the trunk and limbs, the linear arrangement is distinguishable without difficulty. In contrast, recognizing whether or not a small lesion on the face follows Blaschko’s lines is not always easy in certain patients. Originally, Blaschko himself left the scalp as a blank area because of the paucity of information, and drew the lines of the face and the ventral aspect of the neck in a rather cursory way. Later, some authors proposed the system of Blaschko’s lines on the head and neck [19, 26, 27]. A more precise system of Blaschko’s lines on the head and neck was elaborated by Happle R et al [28]. The definite lines are presented in a frontal, lateral and dorsal view, respectively. In the lateral view, two lines were drawn, one running horizontally from the upper margin of the ear to the external canthus, and the other running to the vertex of head. In the dorsal view, the lines show a spiral configuration. The direction of the spiral apparently does not concur with the direction of the hair whorl. They reported that they could not determine whether or not the direction of the spiral was constant because of the limited number of pertinent cases [28]. Nevertheless, the lesion in our patient seemed to follow these lines (Fig. 4).

It is suggested, however, that monoclonality on Blaschko’s lines is limited to epidermal cells and does not reach dermal cells [29]. In fact, most of the diseases following Blaschko’s lines have the main locus in the epidermis or adnexa. There may be a possibility that LEP as well as scleroderma en coup de sabre are caused by a hidden anomaly of the epidermis or adnexa. Alternatively, cellular mosaicism may also involve dermal cells. To verify this hypothesis, further study is required. n

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