Dermatology Online Journal

Discoid lupus erythematosus in an infant
Talel Badri, Rym Kort Khaddar, Saadia Bouraoui, Mourad Mokni, FaïKa Cherif And Amel Ben Osman Dhahri MD
Dermatology Online Journal 11 (3): 38

Department of Dermatology, Rabta Hospital, 1007 Tunis, Tunisia. talel_badri@yahoo.fr

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Abstract

Discoid lupus erythematosus (DLE) is rare in childhood. We report the case of a 15-month-old female infant who presented with erythematous telangectatic lesions and photosensitivity involving the sun-exposed areas. Histological examination confirmed the diagnosis of DLE. Direct immunofluorescence (DIF) on lesional skin showed granular IgM deposits along basement membrane zone. Laboratory tests were normal. External photo-protection and topical corticosteroids lead to complete healing. Summer recurrences that responded to topical corticosteroids were noted but there was no progression to systemic lupus erythematosus. Several authors note the absence of female predominance in children with DLE; prevalence of photosensitivity is controversial. Histological confirmation of DLE is easy and important for diagnosis. DIF is not specific but can be helpful in establishing the diagnosis of DLE. Laboratory tests rarely show low titers of antinuclear antibodies. Treatment is based on sun avoidance and photoprotection. Topical corticosteroids are indicated for active lesions. For resistant cases antimalarials are the treatment of choice. Progression to SLE is probably more frequent in children than in adults.

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Introduction

Discoid lupus erythematosus (DLE) is rare in childhood and exceptional in infancy. Until now, about seventy cases were reported in literature. Similar to the presentation in adults, papules or discoid plaques occur on sun-exposed areas. Telangiectasia, follicular plugging, atrophy, and dyschromia are usually present. Childhood DLE is a separate entity from neonatal systemic lupus erythematosus.

Clinical synopsis

A 15-month-old Tunisian female infant presented with a 3-month history of erythematous and telangiectatic plaques with slightly atrophic centers located on the cheeks, the nose, and the ears. These lesions did not respond to topical antibiotic and antifungal treatments and they worsened in summer. There was no familial or maternal history of SLE or inflammatory arthritis and no associated fever was noted. The patient was otherwise in good general health. There was no osteo-articular or pulmonary involvement.

Figure 1

Histological examination of a skin biopsy showed follicular plugging and atrophy alternating with acanthosis. There was also vacuolization of the basal cell layer and a perivascular and periadnexal inflammatory infiltrate containing mainly lymphocytes and histiocytes. The findings were consistent with a diagnosis of DLE. Direct immunofluorescence on lesional skin showed granular IgM deposits along basement membrane zone (BMZ). Direct immunofluorescence on normal skin was negative. Laboratory tests including complete blood cell count, antinuclear antibodies, complement, and renal investigations were normal.

Treatment with photo-protection and midpotency topical corticosteroids lead to complete healing of the lesions leaving the dyschromic sequels. After a 2-year followup we noted summer recurrences that responded within a few days to topical corticosteroids.

Figure 2

The mother also reported mild articular pain involving knees and elbows. There has been no other indication of progression of DLE to SLE. The patient remains under supervision.

Discussion

DLE is a benign dermatitis of unknown pathogenesis. It is uncommon in childhood and exceptional in infancy [1, 2]. Less than 3 percent of patients with DLE develop the disease before age 10 years [2]. However, in our patient DLE began before age 2. Several authors note the absence of female predominance [1, 2]. Prevalence of photosensitivity in children with DLE is thought by some authors to be lower than in adults [1]. However, Mokhtar et al. [3] and Cherif et al. [2] in two Tunisian studies reported a prevalence of photosensitivity as high as 81 percent.

Diagnosis of DLE is often suspected clinically in the presence of discoid erythemato-squamous sometimes telangectatic lesions, usually localized to the face and the scalp [1, 4]. Less commonly, DLE can present in vespertilio or as melasma-like skin lesions [2]. Histological confirmation of DLE is easy. According to George et al., vacuolization of the basal cell layer, and perivascular and periadnexal lymphocytic infiltrate, as seen in our patient, have an important diagnostic significance in childhood, whereas epidermal lesions can be absent [5]. Direct immunofluorescence (DIF) in lesional skin is not specific and its absence does not eliminate the diagnosis of DLE. DIF can be helpful in establishing the diagnosis of DLE; in 80 percent of the cases granular deposits of IgG, IgM, or complement are present along the BMZ [5]. In rare instances, laboratory tests show inflammation associated with low titers of antinuclear antibodies. In our patient, diagnoses of hydroa vacciniforme, photosensitive eczema, erythropoietic protoporphyria [4], and actinic lichen planus were excluded on the base of clinical and histological data. According to various publications, DLE in childhood has features that are different from the adult form (Table) [1-7]. Treatment of DLE in childhood is based on sun avoidance associated with photoprotection [1, 2, 4]. Topical corticosteroids are indicated as initial therapy for active lesions; they are given for a short period to avoid atrophy [1, 2, 4]. In case of resistance, antimalarials (AM) (hydroxychloroquine 4-6 mg/ kg daily) are the choice of treatment. Patients on antimalarial therapy need regular ophthalmologic supervision [1, 2, 6]. Systemic corticosteroids (1-2 mg/kg daily), with or without antimalarials, are indicated for inadequate response [1]. Our patient responded to topical corticosteroids alone. Outcome is marked essentially by post-inflammatory pigmentary alteration as seen in our patient. Progression to SLE is probably more frequent in children than in adults (Table). George and Tunnessen reported a progression of DLE to SLE in 5 of the 16 children included in their study [1]. More recently, Moises-Alfaro et al. estimate the risk of SLE in children with DLE to be as high as 26 percent over a 3-year followup period [7]. These authors also contend that the confined or disseminated character of the lesions, the early onset of the disease (age less than 10 years), duration, the presence of antinuclear antibodies, and poor response to antimalarials, are not predictive factors for progression to SLE [7]. On the other hand, inflammatory arthritis may herald progression to SLE [7]. Our patient does not present signs of progression to SLE after a 2-year followup but she is still under supervision.

Conclusion

Discoid lupus erythematosus in childhood and especially in infancy is a rare disease. It has some particularities making it different from DLE in adults. Prognosis depends mainly on the risk of progression to SLE.

References

1. George PM, Tunnessen WW. Childhood discoid lupus erythematosus. Arch Dermatol 1993;129(5):613-7. PubMed

2. Cherif F, Mebazaa A, Mokni M, El Euch D, Azaiz MI, Ben Osman Dhahri A. Childhood discoid lupus erythematosus: a Tunisian retrospective study of 16 cases. Pediatr Dermatol 2003;20(4):295-8. PubMed

3. Mokhtar I, Ezzine N, Fazaa B, Zeglaoui F, Zghal M, Kamoun MR. Le lupus discoïde chez lÕenfant. Les Nouvelles Dermatologiques 1999;18(1):30-3.

4. McMullen EA, Armstrong KDB, Bingham EA, Walsh MY. Childhood discoid lupus erythematosus: a report of two cases. Pediatr Dermatol 1998;15(6):439-42. PubMed

5. George PM, Hood AF, Rest EB. Histopathology and immunofluorescence of discoid lupus erythematosus in children. Pediatr Dermatol 1996;13(4):269-73. PubMed

6. Magaña-García M. Antimalarials for children. J Am Acad Dermatol 1994;30(3):510. PubMed

7. Moises-Alfaro C, Berron-Perez R, Carrasco-Daza D, Gutierrez-Castellon P, Ruiz-Maldonado R. Discoid lupus erythematosus in children: clinical, histopathologic and follow up features in 27 cases. Pediatr Dermatol 2003;20(2):103-7. PubMed

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