CLINICAL IMAGES
manujam M, Zhang W, et al. Cross-species transcriptional network analysis defines shared inflammatory responses in murine and human lupus nephritis. J Immunol 2012;189:988–1001. 54. Triantafyllopoulou A, Franzke CW, Seshan SV, Perino G, Kallio-
1607
lias GD, Ramanujam M, et al. Proliferative lesions and metalloproteinase activity in murine lupus nephritis mediated by type I interferons and macrophages. Proc Natl Acad Sci U S A 2010;107: 3012–7.
DOI 10.1002/art.38426
Clinical Images: Toxic epidermal necrolysis–like lesions as a manifestation of lupus erythematosus— the clinical picture of two cases
The patients, women ages 43 and 29 years, respectively, presented with photodistributed macular exanthema of 4 weeks’ duration on the face, trunk, and upper and lower limbs with cephalocaudal progression. Both women had skin phototype IV–V. Examination revealed target lesions, bullae, erosions, and sheet-like detachment covering 60% of total body surface area in the first patient (left) and 80% of the total body surface area in the second patient (right). No involvement of mucous membranes was noted and no new drugs were administered in the months preceding presentation; however, asthenia, fatigue, and arthritis were noted. Laboratory tests revealed anemia, leukopenia, lymphopenia, antinuclear antibody and anti-Sm positivity, and anti–double-stranded DNA in both patients. In addition, the second patient was positive for anti-Ro, anti-La, and anti-RNP antibodies, as well as pericarditis. Proteinuria was measured; urinary protein was 1.5 gm in the first patient and 3.16 gm in the second patient in a 24-hour urine collection, and cutaneous biopsy showed histologic changes similar to lupus erythematosus, with numerous necrotic keratinocytes throughout the thickness of the epidermis and dermal–epidermal junction. The patients were diagnosed as having systemic lupus erythematosus (SLE) associated with toxic epidermal necrolysis–like lesions, and treatment with prednisone, hydroxychloroquine, and mycophenolate mofetil was initiated. Renal biopsy was not performed, since renal function recovered within 2 weeks and cutaneous wounds healed in 2 months. The diagnosis is supported by a clinical history of active lupus erythematosus in the first patient and by the clinical presentation of initial photodistribution, absence of mucosa involvement, prolonged clinical course, histologic changes of lupus erythematosus, and autoimmune markers in both patients. Erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis–like lesions may be a cutaneous sign of SLE (during the evolution as in the first patient or the initial presentation as in the second patient). However, unlike classic erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis, such lesions do not follow any preferential localization, infrequently involve mucous membrane, and triggering factors are rare (e.g., herpes simplex virus or drug-induced forms) (1,2). Moreover, the prognosis is better than that of toxic epidermal necrolysis, for which mortality is high (1,2). Toxic epidermal necrolysis–like lesions are included as diagnostic criteria and a variant of acute cutaneous lupus according to the Systemic Lupus International Collaborating Clinics group (3). 1. Torchia D, Romanelli P, Kerdel FA. Erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis associated with lupus erythematosus. J Am Acad Dermatol 2012;67:417–21. 2. Antiga E, Caproni M, Bonciani D, Bonciolini V, Fabbri P. The last word on the so-called “Rowell’s syndrome”? Lupus 2012;21:577–85. 3. Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677–86.
Bele´n Rubio-Gonza´lez, MD Lara Angulo-Martı´nez, MD Francisco Vanaclocha-Sebastia´n, MD Concepcio ´n Postigo-Llorente, MD Rafael Llamas-Martı´n, MD Hospital Universitario 12 de Octubre Madrid, Spain
manujam M, Zhang W, et al. Cross-species transcriptional network analysis defines shared inflammatory responses in murine and human lupus nephritis. J Immunol 2012;189:988–1001. 54. Triantafyllopoulou A, Franzke CW, Seshan SV, Perino G, Kallio-
1607
lias GD, Ramanujam M, et al. Proliferative lesions and metalloproteinase activity in murine lupus nephritis mediated by type I interferons and macrophages. Proc Natl Acad Sci U S A 2010;107: 3012–7.
DOI 10.1002/art.38426
Clinical Images: Toxic epidermal necrolysis–like lesions as a manifestation of lupus erythematosus— the clinical picture of two cases
The patients, women ages 43 and 29 years, respectively, presented with photodistributed macular exanthema of 4 weeks’ duration on the face, trunk, and upper and lower limbs with cephalocaudal progression. Both women had skin phototype IV–V. Examination revealed target lesions, bullae, erosions, and sheet-like detachment covering 60% of total body surface area in the first patient (left) and 80% of the total body surface area in the second patient (right). No involvement of mucous membranes was noted and no new drugs were administered in the months preceding presentation; however, asthenia, fatigue, and arthritis were noted. Laboratory tests revealed anemia, leukopenia, lymphopenia, antinuclear antibody and anti-Sm positivity, and anti–double-stranded DNA in both patients. In addition, the second patient was positive for anti-Ro, anti-La, and anti-RNP antibodies, as well as pericarditis. Proteinuria was measured; urinary protein was 1.5 gm in the first patient and 3.16 gm in the second patient in a 24-hour urine collection, and cutaneous biopsy showed histologic changes similar to lupus erythematosus, with numerous necrotic keratinocytes throughout the thickness of the epidermis and dermal–epidermal junction. The patients were diagnosed as having systemic lupus erythematosus (SLE) associated with toxic epidermal necrolysis–like lesions, and treatment with prednisone, hydroxychloroquine, and mycophenolate mofetil was initiated. Renal biopsy was not performed, since renal function recovered within 2 weeks and cutaneous wounds healed in 2 months. The diagnosis is supported by a clinical history of active lupus erythematosus in the first patient and by the clinical presentation of initial photodistribution, absence of mucosa involvement, prolonged clinical course, histologic changes of lupus erythematosus, and autoimmune markers in both patients. Erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis–like lesions may be a cutaneous sign of SLE (during the evolution as in the first patient or the initial presentation as in the second patient). However, unlike classic erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis, such lesions do not follow any preferential localization, infrequently involve mucous membrane, and triggering factors are rare (e.g., herpes simplex virus or drug-induced forms) (1,2). Moreover, the prognosis is better than that of toxic epidermal necrolysis, for which mortality is high (1,2). Toxic epidermal necrolysis–like lesions are included as diagnostic criteria and a variant of acute cutaneous lupus according to the Systemic Lupus International Collaborating Clinics group (3). 1. Torchia D, Romanelli P, Kerdel FA. Erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis associated with lupus erythematosus. J Am Acad Dermatol 2012;67:417–21. 2. Antiga E, Caproni M, Bonciani D, Bonciolini V, Fabbri P. The last word on the so-called “Rowell’s syndrome”? Lupus 2012;21:577–85. 3. Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677–86.
Bele´n Rubio-Gonza´lez, MD Lara Angulo-Martı´nez, MD Francisco Vanaclocha-Sebastia´n, MD Concepcio ´n Postigo-Llorente, MD Rafael Llamas-Martı´n, MD Hospital Universitario 12 de Octubre Madrid, Spain
