Papulosquamous Cutaneous Lesions on Chest and Arms - Clinical Advisor

Papulosquamous Cutaneous Lesions on Chest and Arms

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A 48-year-old white man presents to the dermatology clinic with papulosquamous cutaneous lesions that recently appeared on his chest, arms, and the dorsal aspects of his hands. The patient has a family history of autoimmune disorders, including systemic lupus erythematosus (SLE). Physical examination reveals that the lesions lack induration and do not appear to be leaving scars. Biopsy of the lesion is performed, revealing mild inflammatory infiltrate. Direct immunofluorescence shows a granular deposition at the dermal-epidermal junction. Serology indicates that the patient is positive for anti-Ro/SSA (Sjögren-syndrome-related antigen A).

Subacute cutaneous lupus erythematosus (SCLE) was first described in 1880 by Jonathan Hutchinson as a potential case of “lupus marginatus.”1 In a paper published in 1979, the condition was redefined as a subtype of cutaneous lupus erythematosus (CLE).2 SCLE often...

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Subacute cutaneous lupus erythematosus (SCLE) was first described in 1880 by Jonathan Hutchinson as a potential case of “lupus marginatus.”1 In a paper published in 1979, the condition was redefined as a subtype of cutaneous lupus erythematosus (CLE).2 SCLE often is diagnosed based on clinical, histologic, laboratory, and genetic findings. In classic cases, patients present with papulosquamous rash in photosensitive areas and have positive serologies for autoimmune markers such as anti-Ro/SSA.1-2

Like SLE, most cases of SCLE are idiopathic in origin. The average age of SCLE onset is during the fifth decade.1 Women are affected at a much higher rate than men, and a vast majority of SCLE patients are white.1,3 Ten percent to 20% of SCLE cases are drug induced,3-5 with the most common offenders including antihypertensive and antifungal medications, specifically hydrochlorothiazide, angiotensin-converting enzyme inhibitors, and terbinafine.3-6 Recently, the incidence of drug-induced SCLE has been increasing.6 However, it is unknown whether this rise is due to increased identification of SCLE or a true increase in the  number of drug-induced SCLE cases.

Genetic and environmental factors can predispose patients to develop SCLE. The human leukocyte antigen (HLA) A1, B8, and DR3 haplotypes are associated strongly with this condition.1,2,7 Genetic mutations that also can increase the risk for developing SCLE include single nucleotide polymorphisms in the tumor necrosis factor a gene promoter (-308A) or C1qA, a molecule involved in the classical complement pathway.1

Psychological stress has been linked with the development of SCLE, but this correlation has not yet been systematically investigated.1 Sun exposure and smoking exacerbate this condition, and smoking is associated with increased disease severity and decreased quality of life.1,8 

Clinically, SCLE presents as annular erythematous plaques or papulosquamous lesions.1,5,9,10  These lesions are distributed across sun-exposed areas of the upper body, including the “V” area of the neck, extensors of the upper extremities, and dorsum of the hands.1,9 The face and scalp often are spared.9 The lesions are nonindurated and nonscarring.1 Some patients may experience mild extracutaneous symptoms, including arthralgias and Raynaud phenomenon.10 Less than 10% of patients with SCLE experience severe systemic symptoms such as central nervous system lupus and nephritis.9

A number of key histologic, immunologic, and serologic findings indicate a diagnosis of SCLE. Histologically, a biopsied skin lesion can feature hydropic changes, mild superficial infiltration of inflammatory cells, hyperkeratosis, and follicular plugging.9 Direct immunofluorescence shows a granular or particulate deposition of immunoglobulin (Ig) M, IgG, and C3 at the dermal-epidermal junction.6,9,10

Approximately 70% to 99% of patients with SCLE are positive for anti-Ro/SSA antibodies, making this a useful, although nonspecific, marker in patients suspected of having this disease.1,3 In addition, antinuclear antibodies are found in 70% of SCLE patients, but these also are nonspecific.1 Other serologic markers, such as rheumatoid factor, citrullinated peptide, and anti-La/SSB antibody, are found in patients with SCLE at lower rates.3

SCLE may occur in the context of autoimmune connective tissue disorders. The most common conditions associated with SCLE include SLE, Sjögren syndrome, and rheumatoid arthritis.3 Approximately 50% of patients with SCLE meet at least 4 classification criteria from the American Rheumatism Association for SLE.1 This disorder also can present in association with other CLE manifestations, such as discoid lesions, butterfly rash on the face, or a generalized SLE rash.1

There are numerous conditions to consider when formulating a differential diagnosis for SCLE, including other forms of CLE such as discoid lupus erythematosus,10 as well as dermatomyositis, tinea corporis, granuloma annulare, pemphigus foliaceus, photolichenoid drug eruptions, and cutaneous T-cell lymphoma.9 Histologic and immunologic examination of a biopsied lesion is useful in differentiating these diseases.9

Recommendations for the initial treatment of SCLE include topical corticosteroids or other topical anti-inflammatory agents because they are associated with fewer side effects1; however, most patients will require systemic treatment to resolve cutaneous lesions.  Systemic therapy with the antimalarial agent hydroxychloroquine has been found to be effective in treating SCLE, with approximately 75% of patients responding to this treatment.1,3 If treatment with hydroxychloroquine is not effective or tolerated, systemic steroid therapy and immunosuppressive agents such as methotrexate may be considered.1,3

Most cases of SCLE resolve within 1 year of treatment.1 In drug-induced SCLE, cessation of the offending drug alone can lead to resolution of SCLE.3 Clothing to cover sun-exposed areas and broad-spectrum sunscreen can reduce the severity of disease and prevent exacerbations.1

For the patient in our case, clinical, serologic, and immunohistologic findings were consistent with SCLE. The patient was treated with hydroxychloroquine and topical steroids, and the cutaneous lesions resolved.

Kadon Caskey, BS, and Eleanor Johnson, BA, are medical students at Baylor College of Medicine, and Christopher Rizk, MD, is a dermatologist at Elite Dermatology in Houston, Texas.

References

1. Sontheimer RD. Subacute cutaneous lupus erythematosus: 25-year evolution of a prototypic subset (subphenotype) of lupus erythematosus defined by characteristic cutaneous, pathological, immunological, and genetic findingsAutoimmun Rev. 2005;4(5):253-263.

2. Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115(12):1409-1415.

3 Alniemi DT, Gutierrez A Jr, Drage LA, Wetter DA. Subacute cutaneous lupus erythematosus: clinical characteristics, disease associations, treatments, and outcomes in a series of 90 patients at Mayo Clinic, 1996-2011. Mayo Clin Proc. 2017;92(3):406-414.

4. Wilkerson E, Hazey MA, Bahrami S, Callen JP. Golimumab-exacerbated subacute cutaneous lupus erythematosusArch Dermatol. 2012;148(10):1186-1190.

5. Hazey M, Callen J. Subacute cutaneous lupus erythematosus exacerbated by golimumab. J Am Acad Dermatol. 2012;66(4 suppl 1):AB67.

6. Lowe GC, Henderson CL, Grau RH, Hansen CB, Sontheimer RD. A systematic review of drug-induced subacute cutaneous lupus erythematosusBr J Dermatol. 2011;164(3):465-472.

7. Sontheimer RD, Stastny P, Gilliam JN. Human histocompatibility antigen associations in subacute cutaneous lupus erythematosus. J Clin Invest. 1981;67(1):312-316.

8. Piette EW, Foering KP, Chang AY, et al. Impact of smoking in cutaneous lupus erythematosusArch Dermatol. 2012;148(3):317-322.

9. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013;27(3):391-404.

10. Vera-Recabarren MA, García-Carrasco M, Ramos-Casals M, Herrero C. Comparative analysis of subacute cutaneous lupus erythematosus and chronic cutaneous lupus erythematosus: clinical and immunological study of 270 patientsBr J Dermatol. 2010;162(1):91-101.

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