Linear lupus erythematosus profundus in a child

Tada, Jôji; Arata, Jirô; Katayama, Haruko

doi:10.1016/0190-9622(91)70136-p

Cited by 59 publications

(56 citation statements)

References 11 publications

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“…Several cases of linear lupus profundus have been cur-rently reported, but most of them were on the extremities and less frequently, on the face [8][9][10][11] . In the field of dermatology, it is known that many naevoid skin lesions and dermatoses display an arrangement following the Blaschko lines 12 .…”

Section: Discussionmentioning

confidence: 99%

Two Cases of Linear Alopecia on the Occipital Scalp

Rhee

Kim

et al. 2009

Ann Dermatol

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Section: Discussionmentioning

confidence: 99%

Two Cases of Linear Alopecia on the Occipital Scalp

Rhee

Kim

et al. 2009

Ann Dermatol

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“…A linear configuration of LEP has very rarely been described [1,2,3,4]. On the other hand, rare descriptions of the coexistence of cutaneous LE and localized scleroderma or morphea have been reported [5, 6].…”

Section: Introductionmentioning

confidence: 99%

Sclerodermic Linear Lupus Panniculitis: Report of Two Cases

Marzano¹,

Tanzi²,

Caputo³

et al. 2005

Dermatology

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Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or ‘overlap’ forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, ‘sclerodermic’ subset of lupus erythematosus panniculitis.

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“…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][16][17][18][19][20]. Most reported findings in DIF are variable and DIF was not performed in nearly half of the cases.…”

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confidence: 99%

Functional Regulation Triad of Mitosis, Apoptosis and Thigmotropism of Survivin in Cutaneous Malignant Melanoma

Chen¹

2016

CRDOA

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A 5-year-old girl went to our clinic presented with a more than 1 year history of linear and annular erythematous patches and telangiectasia from right cheek to right forehead (Figure1a), which was consistent with the implemented lines of Blaschko on the head (Figure1b). Some mild atrophic center over right forehead was noted (Figure1c). She was treated with topical steroids by other dermatologists for more than 1 year but in vain. There were no oral ulcer, alopecia, muscular weakness, or arthralgia, but photosensitivity was noted. The clinical impression was Discoid Lupus Erythematosus (DLE). The initial screening laboratory examination results revealed negative Antinuclear Antibodies (ANA), and positive anti-ENA antibodies. The complement C3, C4, and complete blood count were all within normal limits. The further laboratory examination confirmed positive anti-nDNA antibodies (45.3 IU/ml, >10 IU/ml), and negative anti-Sm/RNP, anti-SSA or anti-SSB antibodies. A skin biopsy was performed on the right forehead and the sample was sent for histological and Direct Immuno fluorescent Examination (DIF). Microscopic features showed papillary dermal edema and perivascular and periappendageal infiltration in both superficial and deep dermis. There were increased dermal mucin deposition and perivascular lymphocytic infiltration (Figure 2). The higher magnification showed interface dermatitis in the epidermis characterized by presence of basal cell vacuolar change, lymphocyte exocytosis, and dyskeratotic cells (Figure 3). Pigment incontinence was present in the papillary dermis. The DIF of the skin biopsy demonstrated a thick linear-granular IgG deposition in the basement membrane zone (Figure 4). There were also granular IgA, IgM, and C3 deposition at the dermo-epidermal junction. These findings were consistent with cutaneous lupus erythematosus. The systemic workup revealed normal chest x ray, and urianalysis. The repeated laboratory workup showed complete blood count, renal profile, total protein and albumin were within normal limits. The ANA, anti-dsDNA antibody, anticardiolipin IgM antibody, C3, C4, and direct Coombs test were all negative. Oral hydroxychloroquine 100mg a day was then prescribed. The skin lesions improved after treatment. DiscussionThe clinical presentation of childhood DLE is similar to that of the adult form. Over 50% of patients presented with lesions before the age of 10 years. The remainder presented between the ages of 10 and 16 years [1]. Unlike adults with a female predominance, it seems that there is no sex association in children. Some authors AbstractWe report on a 5-year-old girl with a pruritic, erythematous cutaneous eruption on the right side of the face for more than 1 year. The lesion had a linear distribution following the lines of Blaschko. Histopathological findings and direct immunofluorescence were compatible with cutaneous lupus erythematosus. We consider this unusual clinical presentation of childhood facial "Blaschko-linear cutaneous lupus erythematosus" to be a distinct subtyp...

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Linear lupus erythematosus profundus in a child

Cited by 59 publications

References 11 publications

Two Cases of Linear Alopecia on the Occipital Scalp

Two Cases of Linear Alopecia on the Occipital Scalp

Sclerodermic Linear Lupus Panniculitis: Report of Two Cases

Functional Regulation Triad of Mitosis, Apoptosis and Thigmotropism of Survivin in Cutaneous Malignant Melanoma

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