Subacute cutaneous lupus erythematosus

A large part of James N. Gilliam's abbreviated investigative career was devoted to testing a hypothesis that strong relationships do exist between the cutaneous and systemic manifestations of lupus erythematosus (LE). As a result of clinical observations made during his early studies designed to test this hypothesis, he introduced the term “subacute cutaneous lupus erythematosus” (SCLE) to designate a clinically distinctive nonscarring type of histologically confirmed cutaneous LE that he felt might represent a cutaneous marker for a discrete subset of LE patients.¹ A series of studies carried out in our and other laboratories have since confirmed that patients who develop SCLE skin lesions do indeed share other clinical, pathologic, serologic, and immunogenetic features. Dr. Gilliam died on June 6, 1984, before the full impact of his initial clinical observations had been fully recognized. I would, therefore, like to dedicate the following discussion of the clinical and laboratory features of patients with SCLE skin lesions to his memory.     

Verrucous cutaneous lupus erythematosus

An unusual case of cutaneous lupus erythematous with verrucous lesions is reported for its rarity and clinical interest.     

Thalidomide in cutaneous lupus erythematosus

For nearly 50 years, thalidomide has struggled between success and controversy. After causing an epidemic of phocomelia and other birth defects during the 1960s, affecting thousands of neonates, thalidomide was used as a sedative in selective disorders including leprosy. The potent anti-inflammatory properties of thalidomide were serendipitously discovered while treating patients with erythema nodosum leprosum, and the drug is now approved by the US FDA for the treatment of this disease. Subsequently, the immunosuppressant effects of thalidomide, including the complex modulation of many cytokines, have been recognized. One promising application of thalidomide has been the treatment of cutaneous lupus erythematosus. Among the largest series reviewed, the drug has been found to ameliorate cutaneous lupus erythematosus in 90% of patients, on average. Remission is achieved in approximately 15-20% of patients with cutaneous lupus erythematosus at doses between 50-400 mg daily. Contraceptive concerns and the recognized neuropathic effects of thalidomide limit the use of the drug in patients with cutaneous lupus. Physicians who prescribe thalidomide in the US must be registered with the drug manufacturer. With appropriate control of drug access and close physician monitoring, thalidomide provides a needed therapeutic option for the treatment of refractory cases of cutaneous lupus erythematosus.     

Histopathology of cutaneous lupus erythematosus

The basic histopathologic feature of all cutaneous lesions of lupus erythematosus involves a perivascular mononuclear cell infiltrate with subsequent involvement of the epidermis and appendages. The various histologic alterations affecting the epidermis, dermis, and adnexal structures reflect the particular type of lesion biopsied and the duration of the lesion at the time of biopsy. The different clinical and serologic forms of lupus erythematosus cannot reliably be distinguished histologically, which supports the premise that lupus erythematosus is a disease with a wide spectrum of clinical manifestations but with a common underlying pathogenesis.     

Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset

We have characterized the clinical and laboratory features of 27 patients who had in common a recurring, superficial, nonscarring type of cutaneous lupus erythematosus (LE) that occurred in a characteristic distribution (subacute cutaneous lupus erythematosus [SCLE]). This clinically distinct form of cutaneous LE has not previously been analyzed as a separate entity and thus, its clinical importance has not been fully appreciated. We found that these patients frequently had a mild systemic illness marked by musculoskeletal complaints and serologic abnormalities. Forty-eight percent had systemic LE by American Rheumatism Association criteria; however, none had serious CNS or renal disease. Thus, those with SCLE are a subset of patients with LE who generally have an illness intermediate in severity between discoid LE and severe systemic LE.     

Extracoporeal photochemotherapy in cutaneous lupus erythematosus

We report two female subjects with therapy-resistant cutaneous lupus erythematosus (LE), one with subacute cutaneous and the other chronic discoid LE, both treated with extracorporeal photochemotherapy (ECP). The responses after six and eight cycles of ECP led to a prolonged remission of 18 and 11 months, respectively. ECP seems to be a treatment option for patients not responding to or showing unwanted side-effects during conventional standard therapy.     

Subacute cutaneous lupus erythematosus in childhood

We report a 7-year-old girl who presented with erythematous-infiltrated, figurate, well-defined lesions over sun-exposed skin and antinuclear and Ro/SSA antibodies. Lupus band test revealed granular IgM and microgranular C3 deposits at the dermoepidermal junction. Histopathologic examination of lesional skin showed orthohyperkeratosis, epidermal atrophy, widespread hydropic degeneration of the epidermal basal cell layer, and a dermal perivascular lymphohistiocytic infiltrate. The clinical, immunopathologic, and histopathologic findings were consistent with the diagnosis of annular-polycyclic variant of subacute cutaneous lupus erythematosus (SCLE). Treatment with hydroxychloroquine cleared the cutaneous lesions. We report this patient because SCLE is extremely rare in childhood, and discuss the two previous cases reported in the literature.     

Bupropion-induced subacute cutaneous lupus erythematosus

Subacute cutaneous lupus erythematosus is a subset of cutaneous lupus erythematosus with unique immunological and clinical features. The first series of patients found to have drug-induced subacute cutaneous lupus erythemotosus were secondary to hydrochlorothiazide. Since that time, several other drugs have been implicated in the induction of subacute cutaneous lupus erythemotosus. A 44-year-old woman presented with a 9-week history of a mildly pruritic, photosensitive rash that started on her chest. One month prior to her skin outbreak she was started on bupropion for mild depression. She was noted to have multiple annular erythematous plaques on her anterior chest, shoulders, back, arms and face. The patient was advised to stop the bupropion and to start topical tacrolimus, and was encouraged to apply a broad-spectrum sunscreen. Her skin completely cleared within 1 month of initiating this treatment regimen. This case is a unique example of bupropion-induced subacute cutaneous lupus erythemotosus. Our patient exemplifies the necessity of a complete medical history, including current medications, especially when subacute cutaneous lupus erythemotosus is suspected.     

Terbinafine-induced subacute cutaneous lupus erythematosus

BACKGROUND: Recently, the induction of subacute cutaneous lupus erythematosus (SCLE) and exacerbation of systemic lupus erythematosus by terbinafine have been reported. OBJECTIVE: We describe 4 cases of SCLE, one associated with chilblain lupus, which occurred during therapy with oral terbinafine for onychomycosis. METHODS: Of 21 consecutive patients with SCLE attending the outpatient dermatology department at Muenster University clinic during a 1-year period, 4 patients with terbinafine-induced SCLE were seen. Patients were examined fully and photographed; histologic findings as well as serologic and follow-up data were evaluated. RESULTS: In addition to high titers of antinuclear antibodies (ANA) with a homogeneous pattern, anti-Ro(SS-A) antibodies were present; in 3 of 4 women, anti-La(SS-B) antibodies were also found. All patients had anti-histone antibodies as in drug-induced lupus and showed the characteristic genetic association of SCLE with the HLA-B8,DR3 haplotype; moreover, in 2 cases, HLA-DR2 was also present. After discontinuation of terbinafine, ANA titers decreased; anti-histone antibodies also became undetectable within 4(1/2) months in 3 patients concomitant with subsidence of the SCLE eruption in all patients. CONCLUSION: Terbinafine is a drug that appears to infrequently induce SCLE with high titers of ANAs and anti-histone antibodies in genetically susceptible persons.