Correspondence
scopically visible brown pigmentation and an increased number of melanocytes and melanin at the light-microscopic level.1,3 As far as we are aware, there have heretofore been only six reported cases of pigmented CCA.1,2 Including the present case, four men and three women have been reported, and the mean age was 42 (range 25– 58) years. Seven cases involved three thighs, back, shin, toe, and finger. Interestingly, four of six cases had been clinically diagnosed as pigmented nevus, and three of seven patients were of non-European descent (Mexican, Filipino, and Japanese), including our case. A relationship to skin phototype or sun exposure seems possible, as has been indicated by Pierard.4 Langer et al.1 reported that melanocytes and their dendrites contained large amounts of melanin, and many stage III and IV melanosomes were seen by electron microscopy, whereas keratinocytes contained relatively few single melanosomes, indicating an impaired melanin transfer. We suspected pigmented nevus or lentigo simplex from the clinical and dermoscopic features; however, this was ruled out by histological findings. Although the relationship between the dermoscopic features and histological findings in our case was not clear, we speculated that the pigment network found on dermoscopy reflected the presence of many interspersed melanocytes with long dendrites, indicating a disturbed melanin transfer. It is necessary to keep in mind a pigmented CCA as one of the potential diagnoses when we see a patient with a pigmented skin lesion. Hidehisa Saeki, MD Hiroyuki Matsuzaki, MD Keigo Ito, MD Yoshimasa Nobeyama, MD Hidemi Nakagawa, MD Department of Dermatology The Jikei University School of Medicine Tokyo Japan E-mail: [email protected] Conflicts of interest: None.
References 1 Langer K, Wuketich S, Konrad K. Pigmented clear cell acanthoma. Am J Dermatopathol 1994; 16: 134–139. 2 Scheinfeld N. A glistening brown nodule. Pigmented clear cell acanthoma. Arch Dermatol 2007; 143: 255–260. 3 Fanti PA, Passarini B, Varotti C. Melanocytes in clear cell acanthoma. Am J Dermatopathol 1990; 12: 373–376. 4 Pierard GE. Melanocytes in clear cell acanthoma. Am J Dermatopathol 1991; 13: 430.
ª 2014 The International Society of Dermatology
Localized vasculitis in newly formed striae: a unique manifestation of systemic lupus erythematosus
Editor, Patients with lupus vasculitis (LV) display a variety of cutaneous lesions, although hemorrhagic bullae are rarely observed. We report a case of LV presenting as hemorrhagic bullae overlying striae in a patient with systemic lupus erythematosus (SLE) and lupus nephritis. A 23-year-old woman with a history of SLE, lupus nephritis (class IV glomerulonephritis), nephrotic-range proteinuria, volume overload, dyslipidemia, hypertension, and anemia presented to the emergency department with a 4-day history of progressive anasarca. Two months earlier, the patient had been diagnosed with SLE (malar rash, and positivity for antinuclear antibodies [ANAs], anti-Smith antibodies, and anti-dsDNA antibodies) and lupus nephritis and had been started on high-dose steroids and mycophenolate mofetil (500 mg twice per day by mouth). Within two months of the initiation of this regimen, she began to develop striae overlying the genital area, flanks, and lower extremities (Fig. 1a). Four days prior to the present admission, the patient experienced the worsening of lower extremity edema, which spread to her abdomen and genital area. The patient’s medications included clonidine, plaquenil, mycophenolate, nifedipine, prednisone, and ranitidine. On admission, laboratory findings showed leukocytosis (23 9 103/ll [normal range: 3.8–10.8 9 103/ll]), thrombocytosis (535 000/mmol [normal range: 150 000– 450 000/mmol]), hyperkalemia (4.9 mM [normal range: 3.5–5.0 mM]) and an elevated creatinine level (5.1 mg/dl [normal range: 0.6–1.3 mg/dl]). Urinalysis showed proteinuria (4+), hematuria, and neutrophils. Soon after admission, the patient developed pseudomonal septicemia. Simultaneously, hemorrhagic bullae developed overlying the pre-existing striae on the bilateral lower flanks and thighs (Fig. 1b,c). The bullae were decompressed with a sterile needle. Bullae fluid when cultured was negative. Biopsies of the bullae showed features consistent with LV bullae. Direct immunofluorescence (DIF) showed findings consistent with vasculitis, including 2+ linear staining of vessels with immunoglobulin A (IgA), IgM, C3, and fibrinogen. The patient was subsequently started on clobetasol paste and silvadene. Ten days later, superficial erosions and deeper ulceration developed in the areas of the decompressed bullae, which eventually healed with minimal scarring. The patient remained in the hospital for an additional two weeks for management and control of lupus nephritis and was discharged in a stable condition with proper wound care instruction.
International Journal of Dermatology 2014, 53, e578–e596
e581
e582
Correspondence
(a)
(b)
(c)
Figure 1 Clinical examination of a 23-year-old woman with a history of systemic lupus erythematosus shows (a) the formation of abdominal striae, and (b, c) the eruption of hemorrhagic bullae overlying pre-existing striae on the lower flanks and thighs
Bullous lesions in SLE are infrequently reported.1 Etiological factors in patients with SLE include worsening disease, photosensitivity, drug eruption, and steroid withdrawal.2 Distinct diseases including bullous pemphigoid3 and dermatitis herpetiformis4 may occur concurrently with SLE. Bullous lesions occur through two separate mechanisms. The first of these involves vesicle formation caused by a subepidermal blistering disease with a neutrophil-predominant inflammatory infiltrate in the upper dermis and is termed bullous SLE.5 Alternatively, hydropic degeneration of the epidermal basal layer with substantial edema can occur in the upper dermis, a manifestation referred to as SLE with blisters.5 This patient’s histopathology demonstrates epidermal atrophy with marked basal hydropic degeneration (Fig. 2a), accompanied by a lymphocytic infiltrate and fibrinoid change of superficial blood vessels (Fig. 2b). Bullous pemphigoid and dermatitis herpetiformis were ruled out with DIF. Hemorrhagic bullae are rarer in SLE than non-hemorrhagic bullae.6 Cutaneous vasculitis occurs in 19– 28% of patients with SLE.7,8 Vessel damage is thought to result from complement fixation and other mechanisms of immune-mediated damage,9 which include increased vessel wall susceptibility to inflammatory cell deposition from endothelial cell activation10 and adhesion molecule activation.11 This patient’s histology demonstrates fibrinoid necrosis with fibrin deposition in vessels, consistent with vasculitis. Furthermore, dermal mucinosis was present, which supports an SLE-mediated process. Given the clinical and histological findings, we postulate that the development of hemorrhagic bullae occurred secondary to lupus-associated vasculitic changes which permitted the collection of hemorrhagic fluid within bullae. When bullous lesions occur in conjunction with SLE, they are often generalized. The finding that bullae formed only directly over striae is unique to this case. International Journal of Dermatology 2014, 53, e578–e596
The development of striae distensae is a common adverse effect of systemic corticosteroid administration. Mechanical forces and alterations in the dermal extracellular matrix caused by intrinsic or exogenous factors are believed to underlie their development in prone anatomic areas such as the abdomen, lower back, buttocks, and thighs. Histological changes within striae demonstrate ultrastructural remodeling and the reduction of vertical fibrillin fibers subjacent to the dermo–epidermal junction and elastin fibers throughout the papillary dermis.12 This net reduction in extracellular matrix density and structural alteration of the elastic fiber network may have contributed to the focal bullae formation in the setting of this patient's generalized edema by predisposing her striae to both the greatest relative tension and a diminished capacity to resist expansile forces, respectively. Vasculitis, as demonstrated in this patient’s histology, most commonly presents in stasis-prone anatomic locations. This observation is consistent with the pathogenic hypothesis of immune complex and complement deposition in the vascular endothelium, which is believed to increase within areas of reduced fluid velocity. The focal and relatively greatest areas of tissue expansion within the dermis of the striae may have distended these vascular networks, resulting in narrower and more elongated vessels, and consequently reduced fluid velocity, compromised endothelial function, and enhanced complex– endothelial interactions, thereby effectively designating these striae as the locus minoris resistentiae for the development of vasculitis. Alternatively or in conjunction, these forces may have precipitated vessel fragility and rupture as a foci of hemorrhage into the bullae, which may not have been observed within biopsied samples. A previous case of urticarial vasculitis localized to striae distensae has been described.13 However, to the present authors’ knowledge, this is the first report of LV ª 2014 The International Society of Dermatology
Correspondence
(a)
(b)
(c)
Figure 2 (a) Histopathology shows epidermal atrophy with marked basal hydropic degeneration accompanied by a lymphocytic infiltrate. (b) The blood vessel wall shows a fibrinoid change and a surrounding neutrophilic infiltrate. (c) The eccrine gland shows necrotic epithelial cells. [Hematoxylin and eosin stain; original magnification (a) 9400, (b) 9200, (c) 9400]
sequelae presenting as non-infectious, hemorrhagic bullae developing only over striae in a patient with recently diagnosed SLE and lupus nephritis.
Natalie C. Yin, BS Sonal Choudhary, MD Joshua R. Freedman, MD, MS Francisco A. Kerdel, MD Department of Dermatology and Cutaneous Surgery University of Miami Miller School of Medicine Miami FL, USA E-mail: [email protected] Clara Milikowski, MD Department of Pathology University of Miami Miller School of Medicine Miami FL USA
References 1 Tuffanelli DL, Dubois EL. Cutaneous manifestations of systemic lupus erythematosus. Arch Dermatol 1964; 90: 377–386. 2 Callen JP. Cutaneous bullae following acute steroid withdrawal in systemic lupus erythematosus. Br J Dermatol 1981; 105: 603–606. 3 Kumar V, Binder WL, Schotland E, et al. Coexistence of bullous pemphigoid and systemic lupus erythematosus. Arch Dermatol 1978; 114: 1187–1190. 4 Penneys NS, Wiley HE III. Herpetiform blisters in systemic lupus erythematosus. Arch Dermatol 1979; 115: 1427–1428. 5 Yell JA, Wojnarowska F. Bullous skin disease in lupus erythematosus. Lupus 1997; 6: 112–121. ª 2014 The International Society of Dermatology
6 Scholtz M. Lupus erythematosus acutus disseminatus hemorrhagicus. Arch Derm Syphilol 1922; 6: 466–475. 7 Vitali C, Bencivelli W, Isenberg DA, et al. Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. II. Identification of the variables indicative of disease activity and their use in the development of an activity score. The European Consensus Study Group for Disease Activity in SLE. Clin Exp Rheumatol 1992; 10: 541–547. 8 Laman SD, Provost TT. Cutaneous manifestations of lupus erythematosus. Rheum Dis Clin North Am 1994; 20: 195–212. 9 Calamia KT, Balabanova M. Vasculitis in systemic lupus erythematosus. Clin Dermatol 2004; 22: 148–156. 10 Belmont HM, Abramson SB, Lie JT. Pathology and pathogenesis of vascular injury in systemic lupus erythematosus. Interactions of inflammatory cells and activated endothelium. Arthritis Rheum 1996; 39: 9–22. 11 Cid MC. Endothelial cell biology, perivascular inflammation, and vasculitis. Cleve Clin J Med 2002; 69 (Suppl. 2): 45–49. 12 Watson RE, Parry EJ, Humphries JD, et al. Fibrillin microfibrils are reduced in skin exhibiting striae distensae. Br J Dermatol 1998; 138: 931–937. 13 Kwon CW, Lee CW, Kim YT, et al. Urticarial vasculitis developed on the striae distensae during pregnancy. Int J Dermatol 1993; 32: 751–752.
Flagellate erythema in parvovirus B19 infection
Editor, Human parvovirus B19 (PVB19) is a single-stranded DNA virus of the family Parvoviridae and the etiologic agent of erythema infectiosum or fifth disease. Other clinical manifestations of this virus are papular purpuric gloves and socks syndrome, purpuric exanthemas, arthropathy, hydrops fetalis, and aplastic crisis.1–3 We report an International Journal of Dermatology 2014, 53, e578–e596
e583
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
scopically visible brown pigmentation and an increased number of melanocytes and melanin at the light-microscopic level.1,3 As far as we are aware, there have heretofore been only six reported cases of pigmented CCA.1,2 Including the present case, four men and three women have been reported, and the mean age was 42 (range 25– 58) years. Seven cases involved three thighs, back, shin, toe, and finger. Interestingly, four of six cases had been clinically diagnosed as pigmented nevus, and three of seven patients were of non-European descent (Mexican, Filipino, and Japanese), including our case. A relationship to skin phototype or sun exposure seems possible, as has been indicated by Pierard.4 Langer et al.1 reported that melanocytes and their dendrites contained large amounts of melanin, and many stage III and IV melanosomes were seen by electron microscopy, whereas keratinocytes contained relatively few single melanosomes, indicating an impaired melanin transfer. We suspected pigmented nevus or lentigo simplex from the clinical and dermoscopic features; however, this was ruled out by histological findings. Although the relationship between the dermoscopic features and histological findings in our case was not clear, we speculated that the pigment network found on dermoscopy reflected the presence of many interspersed melanocytes with long dendrites, indicating a disturbed melanin transfer. It is necessary to keep in mind a pigmented CCA as one of the potential diagnoses when we see a patient with a pigmented skin lesion. Hidehisa Saeki, MD Hiroyuki Matsuzaki, MD Keigo Ito, MD Yoshimasa Nobeyama, MD Hidemi Nakagawa, MD Department of Dermatology The Jikei University School of Medicine Tokyo Japan E-mail: [email protected] Conflicts of interest: None.
References 1 Langer K, Wuketich S, Konrad K. Pigmented clear cell acanthoma. Am J Dermatopathol 1994; 16: 134–139. 2 Scheinfeld N. A glistening brown nodule. Pigmented clear cell acanthoma. Arch Dermatol 2007; 143: 255–260. 3 Fanti PA, Passarini B, Varotti C. Melanocytes in clear cell acanthoma. Am J Dermatopathol 1990; 12: 373–376. 4 Pierard GE. Melanocytes in clear cell acanthoma. Am J Dermatopathol 1991; 13: 430.
ª 2014 The International Society of Dermatology
Localized vasculitis in newly formed striae: a unique manifestation of systemic lupus erythematosus
Editor, Patients with lupus vasculitis (LV) display a variety of cutaneous lesions, although hemorrhagic bullae are rarely observed. We report a case of LV presenting as hemorrhagic bullae overlying striae in a patient with systemic lupus erythematosus (SLE) and lupus nephritis. A 23-year-old woman with a history of SLE, lupus nephritis (class IV glomerulonephritis), nephrotic-range proteinuria, volume overload, dyslipidemia, hypertension, and anemia presented to the emergency department with a 4-day history of progressive anasarca. Two months earlier, the patient had been diagnosed with SLE (malar rash, and positivity for antinuclear antibodies [ANAs], anti-Smith antibodies, and anti-dsDNA antibodies) and lupus nephritis and had been started on high-dose steroids and mycophenolate mofetil (500 mg twice per day by mouth). Within two months of the initiation of this regimen, she began to develop striae overlying the genital area, flanks, and lower extremities (Fig. 1a). Four days prior to the present admission, the patient experienced the worsening of lower extremity edema, which spread to her abdomen and genital area. The patient’s medications included clonidine, plaquenil, mycophenolate, nifedipine, prednisone, and ranitidine. On admission, laboratory findings showed leukocytosis (23 9 103/ll [normal range: 3.8–10.8 9 103/ll]), thrombocytosis (535 000/mmol [normal range: 150 000– 450 000/mmol]), hyperkalemia (4.9 mM [normal range: 3.5–5.0 mM]) and an elevated creatinine level (5.1 mg/dl [normal range: 0.6–1.3 mg/dl]). Urinalysis showed proteinuria (4+), hematuria, and neutrophils. Soon after admission, the patient developed pseudomonal septicemia. Simultaneously, hemorrhagic bullae developed overlying the pre-existing striae on the bilateral lower flanks and thighs (Fig. 1b,c). The bullae were decompressed with a sterile needle. Bullae fluid when cultured was negative. Biopsies of the bullae showed features consistent with LV bullae. Direct immunofluorescence (DIF) showed findings consistent with vasculitis, including 2+ linear staining of vessels with immunoglobulin A (IgA), IgM, C3, and fibrinogen. The patient was subsequently started on clobetasol paste and silvadene. Ten days later, superficial erosions and deeper ulceration developed in the areas of the decompressed bullae, which eventually healed with minimal scarring. The patient remained in the hospital for an additional two weeks for management and control of lupus nephritis and was discharged in a stable condition with proper wound care instruction.
International Journal of Dermatology 2014, 53, e578–e596
e581
e582
Correspondence
(a)
(b)
(c)
Figure 1 Clinical examination of a 23-year-old woman with a history of systemic lupus erythematosus shows (a) the formation of abdominal striae, and (b, c) the eruption of hemorrhagic bullae overlying pre-existing striae on the lower flanks and thighs
Bullous lesions in SLE are infrequently reported.1 Etiological factors in patients with SLE include worsening disease, photosensitivity, drug eruption, and steroid withdrawal.2 Distinct diseases including bullous pemphigoid3 and dermatitis herpetiformis4 may occur concurrently with SLE. Bullous lesions occur through two separate mechanisms. The first of these involves vesicle formation caused by a subepidermal blistering disease with a neutrophil-predominant inflammatory infiltrate in the upper dermis and is termed bullous SLE.5 Alternatively, hydropic degeneration of the epidermal basal layer with substantial edema can occur in the upper dermis, a manifestation referred to as SLE with blisters.5 This patient’s histopathology demonstrates epidermal atrophy with marked basal hydropic degeneration (Fig. 2a), accompanied by a lymphocytic infiltrate and fibrinoid change of superficial blood vessels (Fig. 2b). Bullous pemphigoid and dermatitis herpetiformis were ruled out with DIF. Hemorrhagic bullae are rarer in SLE than non-hemorrhagic bullae.6 Cutaneous vasculitis occurs in 19– 28% of patients with SLE.7,8 Vessel damage is thought to result from complement fixation and other mechanisms of immune-mediated damage,9 which include increased vessel wall susceptibility to inflammatory cell deposition from endothelial cell activation10 and adhesion molecule activation.11 This patient’s histology demonstrates fibrinoid necrosis with fibrin deposition in vessels, consistent with vasculitis. Furthermore, dermal mucinosis was present, which supports an SLE-mediated process. Given the clinical and histological findings, we postulate that the development of hemorrhagic bullae occurred secondary to lupus-associated vasculitic changes which permitted the collection of hemorrhagic fluid within bullae. When bullous lesions occur in conjunction with SLE, they are often generalized. The finding that bullae formed only directly over striae is unique to this case. International Journal of Dermatology 2014, 53, e578–e596
The development of striae distensae is a common adverse effect of systemic corticosteroid administration. Mechanical forces and alterations in the dermal extracellular matrix caused by intrinsic or exogenous factors are believed to underlie their development in prone anatomic areas such as the abdomen, lower back, buttocks, and thighs. Histological changes within striae demonstrate ultrastructural remodeling and the reduction of vertical fibrillin fibers subjacent to the dermo–epidermal junction and elastin fibers throughout the papillary dermis.12 This net reduction in extracellular matrix density and structural alteration of the elastic fiber network may have contributed to the focal bullae formation in the setting of this patient's generalized edema by predisposing her striae to both the greatest relative tension and a diminished capacity to resist expansile forces, respectively. Vasculitis, as demonstrated in this patient’s histology, most commonly presents in stasis-prone anatomic locations. This observation is consistent with the pathogenic hypothesis of immune complex and complement deposition in the vascular endothelium, which is believed to increase within areas of reduced fluid velocity. The focal and relatively greatest areas of tissue expansion within the dermis of the striae may have distended these vascular networks, resulting in narrower and more elongated vessels, and consequently reduced fluid velocity, compromised endothelial function, and enhanced complex– endothelial interactions, thereby effectively designating these striae as the locus minoris resistentiae for the development of vasculitis. Alternatively or in conjunction, these forces may have precipitated vessel fragility and rupture as a foci of hemorrhage into the bullae, which may not have been observed within biopsied samples. A previous case of urticarial vasculitis localized to striae distensae has been described.13 However, to the present authors’ knowledge, this is the first report of LV ª 2014 The International Society of Dermatology
Correspondence
(a)
(b)
(c)
Figure 2 (a) Histopathology shows epidermal atrophy with marked basal hydropic degeneration accompanied by a lymphocytic infiltrate. (b) The blood vessel wall shows a fibrinoid change and a surrounding neutrophilic infiltrate. (c) The eccrine gland shows necrotic epithelial cells. [Hematoxylin and eosin stain; original magnification (a) 9400, (b) 9200, (c) 9400]
sequelae presenting as non-infectious, hemorrhagic bullae developing only over striae in a patient with recently diagnosed SLE and lupus nephritis.
Natalie C. Yin, BS Sonal Choudhary, MD Joshua R. Freedman, MD, MS Francisco A. Kerdel, MD Department of Dermatology and Cutaneous Surgery University of Miami Miller School of Medicine Miami FL, USA E-mail: [email protected] Clara Milikowski, MD Department of Pathology University of Miami Miller School of Medicine Miami FL USA
References 1 Tuffanelli DL, Dubois EL. Cutaneous manifestations of systemic lupus erythematosus. Arch Dermatol 1964; 90: 377–386. 2 Callen JP. Cutaneous bullae following acute steroid withdrawal in systemic lupus erythematosus. Br J Dermatol 1981; 105: 603–606. 3 Kumar V, Binder WL, Schotland E, et al. Coexistence of bullous pemphigoid and systemic lupus erythematosus. Arch Dermatol 1978; 114: 1187–1190. 4 Penneys NS, Wiley HE III. Herpetiform blisters in systemic lupus erythematosus. Arch Dermatol 1979; 115: 1427–1428. 5 Yell JA, Wojnarowska F. Bullous skin disease in lupus erythematosus. Lupus 1997; 6: 112–121. ª 2014 The International Society of Dermatology
6 Scholtz M. Lupus erythematosus acutus disseminatus hemorrhagicus. Arch Derm Syphilol 1922; 6: 466–475. 7 Vitali C, Bencivelli W, Isenberg DA, et al. Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. II. Identification of the variables indicative of disease activity and their use in the development of an activity score. The European Consensus Study Group for Disease Activity in SLE. Clin Exp Rheumatol 1992; 10: 541–547. 8 Laman SD, Provost TT. Cutaneous manifestations of lupus erythematosus. Rheum Dis Clin North Am 1994; 20: 195–212. 9 Calamia KT, Balabanova M. Vasculitis in systemic lupus erythematosus. Clin Dermatol 2004; 22: 148–156. 10 Belmont HM, Abramson SB, Lie JT. Pathology and pathogenesis of vascular injury in systemic lupus erythematosus. Interactions of inflammatory cells and activated endothelium. Arthritis Rheum 1996; 39: 9–22. 11 Cid MC. Endothelial cell biology, perivascular inflammation, and vasculitis. Cleve Clin J Med 2002; 69 (Suppl. 2): 45–49. 12 Watson RE, Parry EJ, Humphries JD, et al. Fibrillin microfibrils are reduced in skin exhibiting striae distensae. Br J Dermatol 1998; 138: 931–937. 13 Kwon CW, Lee CW, Kim YT, et al. Urticarial vasculitis developed on the striae distensae during pregnancy. Int J Dermatol 1993; 32: 751–752.
Flagellate erythema in parvovirus B19 infection
Editor, Human parvovirus B19 (PVB19) is a single-stranded DNA virus of the family Parvoviridae and the etiologic agent of erythema infectiosum or fifth disease. Other clinical manifestations of this virus are papular purpuric gloves and socks syndrome, purpuric exanthemas, arthropathy, hydrops fetalis, and aplastic crisis.1–3 We report an International Journal of Dermatology 2014, 53, e578–e596
e583
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
![[Acute initial manifestation of systemic lupus erythematosus in the puerperium].](/assets/img/hold.png)
