Discoid lupus erythematosus
Thomas Casey Gallagher and Helen T Shin
Dermatology Online Journal 7(2): 17

History

This 11-month-old-boy presented with a nine month history of a rash involving the face, antecubital fossae, hands, trunk, and knees. The patient was born full-term by normal spontaneous vaginal delivery without complications. At two months of age, he suddenly developed an erythematous, pruritic patch that involved his cheeks and chin. Subsequently, a pruritic eruption appeared on his trunk and extremities. The eruption has shown only a minimal improvement with trials of multiple topical glucocorticoids. The patient originally presented to another institution where a biopsy specimen showed a lichenoid dermatitis. There is no family history of connective-tissue diseases.

Physical Examination

Figure 1	Figure 2

Erythematous, crescent-shaped patches were noted on the cheeks and chin. There were scaly, verrucous, erythematous papules on the dorsal aspects of the hands and erythematous papules involving the trunk, antecubital fossae, and knees. There was no involvement of the buccal mucosa, fingernails, or flexor aspects of the wrists.

Laboratory Data

A complete blood count and a urinalysis were normal. An anti-nuclear antibody test was negative.

Histopathology

A superficial and mid-dermal perivascular infiltrate of lymphocytes and scattered macrophages containing melanin was present. There was focal vacuolar alteration of the basal-cell layer, and a periodic acid-Schiff stain highlighted focal smudging of the basement membrane. A colloidal iron stain revealed deposits of connective tissue mucin.

A direct immunofluorescence test showed no abnormal epidermal or vascular deposits of IgG, C3, IgM, IgA, or fibrin. There were thin-to-broad intermittent deposits of IgM (1+) at the basement-membrane zone.

Diagnosis

Discoid lupus erythematosus

Comment

Discoid lupus erythematosus is a rare disease in children. There are fewer than 20 cases reported in the English literature. A study by Burch and Rowell found that only 2% of discoid lupus erythematosus patients have onset of their disease in childhood, with the youngest patient reported presenting at one week of age. A survey by Hough of 204 patients found that only three developed discoid lupus erythematosus before age ten.

As in the adult form of discoid lupus erythematosus, there are multiple clinical manifestations of childhood discoid lupus erythematosus. The classic lesion is the discoid plaque with atrophy, follicular plugs, scale, and scarring alopecia. In childhood discoid lupus erythematosus, a family history of discoid or systemic lupus erythematosus occurs in 25% of reported cases. Unlike the adult form, childhood discoid lupus erythematosus does not demonstrate a female preponderance. Photoexacerbation occurs in 31% of children with discoid lupus erythematosus, whereas sun exposure aggravates the disease in 60% of adults with discoid lupus erythematosus. Over 30% of patients with childhood discoid lupus erythematosus develop systemic symptoms. There are no reliable ways to predict which children will develop systemic disease: equal numbers of ANA-positive and ANA-negative patients progress to systemic disease; also, equal numbers of patients with localized versus disseminated lesions may develop systemic disease.

Lupus-like skin lesions have been reported in association with X-linked chronic granulomatous disease, which is characterized by recurrent infections with catalase-positive bacteria and fungi. While rare, it is important to be aware of this association for early diagnosis and treatment of affected children.

Treatment of childhood discoid lupus erythematosus consists primarily of controlling disease activity and preventing scars. Sun protection and sun avoidance are the mainstays of prevention, while potent topical glucocorticoids are used to treat active lesions. In refractory cases, hydroxychloroquine, 4 to 6 mg/kg/day, has been helpful. A two-week taper of prednisolone, starting with doses of 1 to 3 mg/kg/day can be used to bring a severe flare of disease under control. Close follow-up is mandatory to watch for signs of progression to systemic disease.

References

© 2001 Dermatology Online Journal