October 2007

Fig.3

 Diagnosis: Subacute cutaneous lupus erythematosus (SCLE)

Histopathology: Interface dermatitis with a moderate superficial dermal chronic inflammatory infiltrate extending to the hair follicles and to a lesser extent, the eccrine ducts (Figure 3). There was extensive pigment incontinence and mild basal vacuolization. The overlying epidermis showed exocytosis. GMS stain was negative for organisms.

Clinical course: The patient underwent a successful living-related donor renal transplant one month later and was subsequently started on hydroxychloroquine 200 mg BID, betamethasone dipropionate ointment on the palms and tacrolimus 0.1% ointment on the axillae. Two months after starting treatment, her rash had resolved with residual post-inflammatory hyperpigmentation (Figure 4). At this time, she was also taking systemic immunosuppressants including mycophenolate mofetil 500 mg BID, tacrolimus 2 mg BID and prednisone 5 mg QD.
Discussion: This case represents a rare clinical presentation of SCLE. SCLE was first described in 1979 as a subset of cutaneous LE affecting young to middle-aged women (1). SCLE presents with hyperpigmented papulosquamous or annular lesions affecting sun-exposed areas (2). Coincident malar erythema, discoid lesions, non-scarring alopecia, and livedo reticularis have also been reported (3). Histologically, typical features include epidermal atrophy, vacuolar alteration of the basal layer, and a superficial, interface, perivascular and periadnexal lymphocytic infiltrate (4).
The pathogenesis of SCLE is multifactorial, likely involving genetic, environmental, and immune mechanisms. Approximately 70% of patients are reported to have circulating anti-Ro and anti-nuclear auto-antibodies (2). Other lab findings include anti-double stranded DNA antibodies and depressed C3 or C4 levels (3).
The clinical course of SCLE is variable. While most patients have a relatively mild disease course, others may develop SLE or other rheumatic disorders. Up to 50% of SCLE patients meet the criteria for systemic lupus erythematosus (SLE) with musculoskeletal involvement as the most common manifestation. Renal involvement has been reported in up to 20% of SCLE patients (3).
Hydroxychloroquine sulfate (200-400 mg per day) alone or in combination with quinacrine hydrochloride has been successfully used to treat SCLE (3). Other treatments include dapsone, retinoids, oral gold, thalidomide and immunosuppressants.
SCLE has not been reported on the palms and soles. However, palmoplantar involvement has been described in chronic cutaneous LE, affecting only 1.2% of patients (1). One case series described two African-American female patients in their 40s, both of whom had a history of SLE and extensive discoid LE of the face, presenting with tender discoid lesions of the palmar and plantar surfaces (3).

References:
1. Cardinali C, Caproni M, Bernacchi E, Amato L, Fabbri P. The spectrum of cutaneous manifestations in lupus erythematosus ­ the Italian experience. Lupus 2000;9:417-423.
2. Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin 2002;20:373-385.
3. Sontheimer RD. Subacute Cutaneous Lupus Erythematosus: A Decade's Perspective. Med Clin N Am 1989;73:1073-90.
4. David-Bejar KM, Davis BM. Pathology, immunopathology, and immunohistochemistry in cutaneous lupus erythematosus. Lupus 1997;6:145-157.
5. Goyal S, Nousari HC. Treatment of resistant discoid lupus erythematosus of the palms and soles with mycophenolate mofetil. J Am Acad Dermatol 2001;45:142-4.

This case is presented by Ilana Rosman and Drs. Grace Bandow and Michael Heffernan.