- Email: [email protected]
Woman with multiple tender plaques with subsequent atrophy and scarring
J AM ACAD DERMATOL VOLUME 53, NUMBER 5
Self-Assessment examination 929
Millard T, Harris A, MacDonald D. Acute infantile hemorrhagic edema. J Am Acad Dermatol 1999;41:873-9. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AIHE)—a variant of Henoch-Scho¨nlein purpura or a distinct clinical entity? J Allergy Clin Immunol 1990;86:473-83.
Woman with multiple tender plaques with subsequent atrophy and scarring Karima Khechmoune, BS,a Shahbaz A. Janjua, MD,b and Amor Khachemoune, MD, CWSc Annaba, Algeria;a Lalmusa, Pakistan;b and Boston, Massachusettsc A 45-year-old woman presented with a several-year history of multiple painful and tender nodular swellings involving her face, breasts, and upper aspects of her arms followed by atrophic scarring of the affected areas (Fig 7). She also had chronic arthralgia, myalgia, and fatigue.
symptoms include nontender acral edema and low-grade fever. Occasionally diarrhea, melena, arthralgias, transient microscopic hematuria, and mild proteinuria are present; however, these complications are rare and transient. Laboratory studies may reveal leukocytosis, eosinophilia, thrombocytosis, an elevated erythrocyte sedimentation rate, or circulating immune complexes; however, laboratory results frequently produce normal findings. Histology will show a leukocytoclastic vasculitis with C3 and fibrinogen deposits. Perivascular IgA deposits are seen in roughly one third of the cases of AIHE. The prognosis for AIHE is good. Complete spontaneous resolution typically occurs within 3 weeks of onset with treatment being supportive only. Of interest is the striking similarity and frequent overlap of findings in AIHE and Henoch-Scho¨nlein purpura; however Henoch-Scho¨nlein purpura portents a much poorer prognosis because of a higher risk of internal organ involvement, a longer course (# 6 weeks), and a nearly 50% rate of relapse. For this series, the recommended choices are as follows: 14, b; 15, b; 16, c; 17, a; 18, e. BIBLIOGRAPHY Baselga E, Drolet BA, Esterly NB. Purpura in infants and children. J Am Acad Dermatol 1997;37:673-705. Crowe MA, Jonas PP. Acute hemorrhagic edema of infancy. Pediatr Dermatol 1998;62:65-6. Legrain V, Lejean S, Taieb A, Guillard J-M, Battin J, Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol 1991;24:17-22.
19. The most likely diagnosis is (Choose single best response.) a. discoid lupus erythematosus b. lupus profundus c. lupus tumidus d. lupus pernio e. erythema nodosum 20. A deep excisional biopsy specimen revealed a lobular panniculitis with a dense infiltrate of lymphocytes. The differential diagnosis includes the following except (Choose single best response.) a. traumatic panniculitis b. lupus panniculitis c. erythema nodosum d. pancreatic panniculitis e. erythema induratum of Bazin 21. Lupus profundus occurs in what percentage of patients with cutaneous lupus erythematosus? (Choose single best response.) a. 1% to 3% b. 5% to 10% c. 20% to 40% d. 40% to 60% e. 60% to 80% 22. Successful treatment of lupus profundus include the following except (Choose single best response.) a. dapsone b. hydroxychloroquine c. systemic glucocorticoids d. azathioprine e. systemic retinoids
930 Self-Assessment examination
Discussion Lupus profundus, also known as lupus panniculitis, was first described by Kaposi in 1869. It is a chronic relapsing panniculitis that occurs in 1% to 3% of cutaneous lupus erythematosus cases, and affects mostly women (male:female ratio is 1:2) between the ages of 20 and 60 years. It is characterized by the development of deep-seated, tender, subcutaneous nodules or plaques with a predilection for the upper aspects of arms, shoulders, face, and buttocks. Less commonly involved areas include the subcutaneous fat of the breast, and orbital and parotid/periparotid areas. The surface of the lesions may appear as normal skin or may show the classic features of discoid lupus erythematosus. The progressive regression of lesions is usually followed by atrophy and scarring. Subsequent, preceding, or concomitant lesions of discoid lupus erythematosus have also been described in association with lupus profundus. Histopathologic examination of a biopsy specimen in more than half of the cases may reveal atrophy of the epidermis, vacuolar changes at the dermoepidermal junction, interstitial mucin deposition between collagen bundles of the dermis, and
J AM ACAD DERMATOL NOVEMBER 2005
a lymphocytic perivascular inflammatory infiltrate. In the rest of the cases, epidermis and dermis are spared; the histopathologic changes are confined to the subcutaneous fat and consist of mostly lobular panniculitis with a lymphocytic infiltrate. The characteristic histology features of lupus profundus include lipoatrophy, hyaline necrosis, and abundant lymphoid follicles. Serologic examination may show positive antinuclear antibodies and, less commonly, antidoublestranded DNA antibodies may also be detected. The diagnosis is, however, based on the clinical and histopathologic findings. In some cases where histopathology may not be suggestive, a positive lupus band test along the dermoepidermal junction supports the diagnosis of lupus profundus. Other types of panniculitis may need to be differentiated from lupus profundus both clinically and histopathologically. Erythema nodosum often involves the shins, and erythema induratum of Bazin characteristically involves the calves of lower extremities. On histology, morphea panniculitis, traumatic panniculitis, or localized lipoatrophy may closely resemble lupus profundus. Topical glucocorticoids under occlusion may greatly help alleviate the symptoms, but often systemic glucocorticoids and hydroxychloroquine are required. Intralesional glucocorticoids are usually ineffective and may exacerbate the associated atrophy. Isolated case reports have described success with dapsone, azathioprine, and thalidomide. Supportive measures include avoidance of trauma and protection from exposure to UV light. For this series, the recommended choices are as follows: 19, b; 20, c; 21, a; 22, e. BIBLIOGRAPHY Janjua SA, McColl I, Thomas J. Lupus panniculitis involving breast and parotid/periparotid areas; a rare presentation. J Ayub Med Coll 2004;16:86-8. Martens PB, Moder KG, Ahmed I. Lupus panniculitis: clinical perspectives from a case series. J Rheumatol 1999;26:68-72. Requena L, Sanchez Yus E. Panniculitis, part II: mostly lobular panniculitis. J Am Acad Dermatol 2001;45:325-61. White WL, Sherertz EF, Berg D, Clark RE. Periparotid lupus erythematosus panniculitis: clinicopathologic correlation of two cases presenting as primary parotid disease. Arch Pathol Lab Med 1993;117:535-9.
Self-Assessment examination 929
Millard T, Harris A, MacDonald D. Acute infantile hemorrhagic edema. J Am Acad Dermatol 1999;41:873-9. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AIHE)—a variant of Henoch-Scho¨nlein purpura or a distinct clinical entity? J Allergy Clin Immunol 1990;86:473-83.
Woman with multiple tender plaques with subsequent atrophy and scarring Karima Khechmoune, BS,a Shahbaz A. Janjua, MD,b and Amor Khachemoune, MD, CWSc Annaba, Algeria;a Lalmusa, Pakistan;b and Boston, Massachusettsc A 45-year-old woman presented with a several-year history of multiple painful and tender nodular swellings involving her face, breasts, and upper aspects of her arms followed by atrophic scarring of the affected areas (Fig 7). She also had chronic arthralgia, myalgia, and fatigue.
symptoms include nontender acral edema and low-grade fever. Occasionally diarrhea, melena, arthralgias, transient microscopic hematuria, and mild proteinuria are present; however, these complications are rare and transient. Laboratory studies may reveal leukocytosis, eosinophilia, thrombocytosis, an elevated erythrocyte sedimentation rate, or circulating immune complexes; however, laboratory results frequently produce normal findings. Histology will show a leukocytoclastic vasculitis with C3 and fibrinogen deposits. Perivascular IgA deposits are seen in roughly one third of the cases of AIHE. The prognosis for AIHE is good. Complete spontaneous resolution typically occurs within 3 weeks of onset with treatment being supportive only. Of interest is the striking similarity and frequent overlap of findings in AIHE and Henoch-Scho¨nlein purpura; however Henoch-Scho¨nlein purpura portents a much poorer prognosis because of a higher risk of internal organ involvement, a longer course (# 6 weeks), and a nearly 50% rate of relapse. For this series, the recommended choices are as follows: 14, b; 15, b; 16, c; 17, a; 18, e. BIBLIOGRAPHY Baselga E, Drolet BA, Esterly NB. Purpura in infants and children. J Am Acad Dermatol 1997;37:673-705. Crowe MA, Jonas PP. Acute hemorrhagic edema of infancy. Pediatr Dermatol 1998;62:65-6. Legrain V, Lejean S, Taieb A, Guillard J-M, Battin J, Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol 1991;24:17-22.
19. The most likely diagnosis is (Choose single best response.) a. discoid lupus erythematosus b. lupus profundus c. lupus tumidus d. lupus pernio e. erythema nodosum 20. A deep excisional biopsy specimen revealed a lobular panniculitis with a dense infiltrate of lymphocytes. The differential diagnosis includes the following except (Choose single best response.) a. traumatic panniculitis b. lupus panniculitis c. erythema nodosum d. pancreatic panniculitis e. erythema induratum of Bazin 21. Lupus profundus occurs in what percentage of patients with cutaneous lupus erythematosus? (Choose single best response.) a. 1% to 3% b. 5% to 10% c. 20% to 40% d. 40% to 60% e. 60% to 80% 22. Successful treatment of lupus profundus include the following except (Choose single best response.) a. dapsone b. hydroxychloroquine c. systemic glucocorticoids d. azathioprine e. systemic retinoids
930 Self-Assessment examination
Discussion Lupus profundus, also known as lupus panniculitis, was first described by Kaposi in 1869. It is a chronic relapsing panniculitis that occurs in 1% to 3% of cutaneous lupus erythematosus cases, and affects mostly women (male:female ratio is 1:2) between the ages of 20 and 60 years. It is characterized by the development of deep-seated, tender, subcutaneous nodules or plaques with a predilection for the upper aspects of arms, shoulders, face, and buttocks. Less commonly involved areas include the subcutaneous fat of the breast, and orbital and parotid/periparotid areas. The surface of the lesions may appear as normal skin or may show the classic features of discoid lupus erythematosus. The progressive regression of lesions is usually followed by atrophy and scarring. Subsequent, preceding, or concomitant lesions of discoid lupus erythematosus have also been described in association with lupus profundus. Histopathologic examination of a biopsy specimen in more than half of the cases may reveal atrophy of the epidermis, vacuolar changes at the dermoepidermal junction, interstitial mucin deposition between collagen bundles of the dermis, and
J AM ACAD DERMATOL NOVEMBER 2005
a lymphocytic perivascular inflammatory infiltrate. In the rest of the cases, epidermis and dermis are spared; the histopathologic changes are confined to the subcutaneous fat and consist of mostly lobular panniculitis with a lymphocytic infiltrate. The characteristic histology features of lupus profundus include lipoatrophy, hyaline necrosis, and abundant lymphoid follicles. Serologic examination may show positive antinuclear antibodies and, less commonly, antidoublestranded DNA antibodies may also be detected. The diagnosis is, however, based on the clinical and histopathologic findings. In some cases where histopathology may not be suggestive, a positive lupus band test along the dermoepidermal junction supports the diagnosis of lupus profundus. Other types of panniculitis may need to be differentiated from lupus profundus both clinically and histopathologically. Erythema nodosum often involves the shins, and erythema induratum of Bazin characteristically involves the calves of lower extremities. On histology, morphea panniculitis, traumatic panniculitis, or localized lipoatrophy may closely resemble lupus profundus. Topical glucocorticoids under occlusion may greatly help alleviate the symptoms, but often systemic glucocorticoids and hydroxychloroquine are required. Intralesional glucocorticoids are usually ineffective and may exacerbate the associated atrophy. Isolated case reports have described success with dapsone, azathioprine, and thalidomide. Supportive measures include avoidance of trauma and protection from exposure to UV light. For this series, the recommended choices are as follows: 19, b; 20, c; 21, a; 22, e. BIBLIOGRAPHY Janjua SA, McColl I, Thomas J. Lupus panniculitis involving breast and parotid/periparotid areas; a rare presentation. J Ayub Med Coll 2004;16:86-8. Martens PB, Moder KG, Ahmed I. Lupus panniculitis: clinical perspectives from a case series. J Rheumatol 1999;26:68-72. Requena L, Sanchez Yus E. Panniculitis, part II: mostly lobular panniculitis. J Am Acad Dermatol 2001;45:325-61. White WL, Sherertz EF, Berg D, Clark RE. Periparotid lupus erythematosus panniculitis: clinicopathologic correlation of two cases presenting as primary parotid disease. Arch Pathol Lab Med 1993;117:535-9.