- Email: [email protected]
Relapsing livedo reticularis in the setting of chronic pancreatitis
Journal of the American Academy of Dermatology Volume 39, Number 6
Brief communications 1035
Relapsing livedo reticularis in the setting of chronic pancreatitis Jennifer W. Gould, MD,a Stephen E. Helms, MD,a Susan M. Schulz, MD,b and Seth R. Stevens, MDa Cleveland, Ohio
Livedo reticularis (LR) is a characteristic netlike violaceous discoloration caused by a physiologic or pathologic cutaneous blood flow disturbance. Cutaneous vasculature consists of cones of arterioles whose margins have diminished arterial but prominent venous flow. Therefore, impeded blood flow through the skin can increase deoxyhemoglobin concentration, producing livid coloration in marginal zone venous areas.1 Benign causes include cutis marmorata and asymptomatic idiopathic LR. Diseases associated with LR include vessel wall disease, intravascular obstruction, and endocrine diseases.1-3 Rare clinical reports of LR associated with pancreatitis “Walzel’s Sign” exist.4 We describe a patient with asymmetric relapsing LR of the flank temporally associated with painful flares of her chronic pancreatitis. CASE REPORT A 45-year-old white woman had a 13-year history of chronic pancreatitis resulting from alcohol abuse. Her other medical history was notable for Crohn’s disease, peptic ulcer disease, and silicone breast implants. Starting in June 1996, she noticed a recurrent, reticulated, erythematous rash over the right flank, preceding painful flares in her pancreatitis by 1 to 2 days. She denied applying heat to the area. Physical examination revealed blanching, reticulated, violaceous patches on the trunk over the right flank (Fig 1). Evaluation of laboratory studies 3 weeks before presentation were notable for a white blood cell count of 11.8 × 109/L, hematocrit of 37%, platelets of 327 × 109/L, negative antinuclear antibody and panel, and negative lupus anticoagulant. Anticardiolipin antibodies, IgG and IgA, prothrombin and partial thromboplastin times, cold From the Departments of Dermatologya and Rheumatology,b University Hospitals of Cleveland/Case Western Reserve University. Reprint requests: Seth R. Stevens, MD, Chief, Inpatient Dermatology Service, University Hospitals of Cleveland, 11100 Euclid Ave, Cleveland, OH 44106-5028. J Am Acad Dermatol 1998;39:1035-6. Copyright © 1998 by the American Academy of Dermatology, Inc. 0190-9622/98/$5.00 + 0 16/54/93192
Fig 1. Recurrent livedo reticularis on flank during flare of pancreatitis.
agglutinins, cryoglobulins, and complement were normal. Ten days after presentation, blood amylase was 123 U/L (30 to 115) and lipase was 355 U/L (0 to 200). Three weeks after presentation, prothrombin and partial thromboplastin times were within normal limits, cold agglutinins were less than 1:4 (ref < 1:64), no cryoglobulins were detected, and complement levels were normal. An incisional skin biopsy specimen revealed a superficial perivascular mononuclear inflammatory cell infiltrate and hemorrhage. Direct immunofluorescence revealed IgM and C3 deposition in the upper dermal blood vessels. The patient subsequently underwent pancreaticojejunostomy, which has abated her pain and eruption.
1036 Brief communications DISCUSSION
In 1954, Sigmund and Shelley4 reported a patient with an acute flare of chronic pancreatitis associated with an asymptomatic, red, reticulated rash of the left flank that persisted post mortem.4 They hypothesized the LR was caused by escaped trypsin damaging cutaneous vessels, resulting in reduced blood flow. No histopathology was reported. They cited previous similar cases. In 1889, Hansemann reported 2 cases of acute pancreatitis associated with a morbilliform eruption persisting after death. Histopathology revealed subcutaneous fat necrosis. Walzel in 1927 described two cases of acute pancreatitis associated with a reticulated rash on the abdomen, chest, and thighs, persisting after death, which he believed corresponded to the subcutaneous venous network. No biopsy was reported. Sigmund and Shelley4 interpreted both Hansemann’s and Walzel’s findings as livedoid change associated with pancreatitis, and coined the term “Walzel’s Sign.” Our case of LR associated with pancreatitis is a rarely reported clinical finding. Like Sigmund and Shelley’s, our case is also unilateral. In our case, biopsy specimens were uniquely examined by hematoxylin-eosin stain and immunofluorescence. In contrast to Hansemann’s case, ours showed only superficial perivascular dermatitis and hemorrhage. Direct immunofluorescence revealed
Journal of the American Academy of Dermatology December 1998
immunoglobin and complement deposition. Although this finding can be seen in vasculitis, the lack of fibrin deposition on direct immunofluorescence and lack of vessel wall necrosis and fibrin thrombi on histopathology is indicative of immune complex deposition without vasculitis. However, it is also possible that disturbed vasculature in the flank region, perhaps brought on by the LR or for other reasons, allowed for the deposition of nonpathogenic immune complexes in this site, implying association without causation of LR and immune complex deposition. Our patient’s findings suggest an immune-mediated cause of livedo reticularis, possibly in concert with pancreatitis. This case is unique because of the relapsing nature of the LR, representing Walzel’s sign, with immune complex deposition and hemorrhage, but without fat necrosis or vasculitis. REFERENCES 1. Fleischer AB, Resnick SD. Livedo reticularis. Dermatol Clin 1990;8:347-54. 2. Champion RH. Livedo reticularis: a review. Br J Dermatol 1965;77:167-79. 3. Copeman PWM. Livedo reticularis: signs in the skin of disturbance of blood viscosity and of blood flow. Br J Dermatol 1975;93:519-29. 4. Sigmund WJ, Shelley WB. Cutaneous manifestations of acute pancreatitis, with special reference to livedo reticularis. N Engl J Med 1954;251:851-3.
Brief communications 1035
Relapsing livedo reticularis in the setting of chronic pancreatitis Jennifer W. Gould, MD,a Stephen E. Helms, MD,a Susan M. Schulz, MD,b and Seth R. Stevens, MDa Cleveland, Ohio
Livedo reticularis (LR) is a characteristic netlike violaceous discoloration caused by a physiologic or pathologic cutaneous blood flow disturbance. Cutaneous vasculature consists of cones of arterioles whose margins have diminished arterial but prominent venous flow. Therefore, impeded blood flow through the skin can increase deoxyhemoglobin concentration, producing livid coloration in marginal zone venous areas.1 Benign causes include cutis marmorata and asymptomatic idiopathic LR. Diseases associated with LR include vessel wall disease, intravascular obstruction, and endocrine diseases.1-3 Rare clinical reports of LR associated with pancreatitis “Walzel’s Sign” exist.4 We describe a patient with asymmetric relapsing LR of the flank temporally associated with painful flares of her chronic pancreatitis. CASE REPORT A 45-year-old white woman had a 13-year history of chronic pancreatitis resulting from alcohol abuse. Her other medical history was notable for Crohn’s disease, peptic ulcer disease, and silicone breast implants. Starting in June 1996, she noticed a recurrent, reticulated, erythematous rash over the right flank, preceding painful flares in her pancreatitis by 1 to 2 days. She denied applying heat to the area. Physical examination revealed blanching, reticulated, violaceous patches on the trunk over the right flank (Fig 1). Evaluation of laboratory studies 3 weeks before presentation were notable for a white blood cell count of 11.8 × 109/L, hematocrit of 37%, platelets of 327 × 109/L, negative antinuclear antibody and panel, and negative lupus anticoagulant. Anticardiolipin antibodies, IgG and IgA, prothrombin and partial thromboplastin times, cold From the Departments of Dermatologya and Rheumatology,b University Hospitals of Cleveland/Case Western Reserve University. Reprint requests: Seth R. Stevens, MD, Chief, Inpatient Dermatology Service, University Hospitals of Cleveland, 11100 Euclid Ave, Cleveland, OH 44106-5028. J Am Acad Dermatol 1998;39:1035-6. Copyright © 1998 by the American Academy of Dermatology, Inc. 0190-9622/98/$5.00 + 0 16/54/93192
Fig 1. Recurrent livedo reticularis on flank during flare of pancreatitis.
agglutinins, cryoglobulins, and complement were normal. Ten days after presentation, blood amylase was 123 U/L (30 to 115) and lipase was 355 U/L (0 to 200). Three weeks after presentation, prothrombin and partial thromboplastin times were within normal limits, cold agglutinins were less than 1:4 (ref < 1:64), no cryoglobulins were detected, and complement levels were normal. An incisional skin biopsy specimen revealed a superficial perivascular mononuclear inflammatory cell infiltrate and hemorrhage. Direct immunofluorescence revealed IgM and C3 deposition in the upper dermal blood vessels. The patient subsequently underwent pancreaticojejunostomy, which has abated her pain and eruption.
1036 Brief communications DISCUSSION
In 1954, Sigmund and Shelley4 reported a patient with an acute flare of chronic pancreatitis associated with an asymptomatic, red, reticulated rash of the left flank that persisted post mortem.4 They hypothesized the LR was caused by escaped trypsin damaging cutaneous vessels, resulting in reduced blood flow. No histopathology was reported. They cited previous similar cases. In 1889, Hansemann reported 2 cases of acute pancreatitis associated with a morbilliform eruption persisting after death. Histopathology revealed subcutaneous fat necrosis. Walzel in 1927 described two cases of acute pancreatitis associated with a reticulated rash on the abdomen, chest, and thighs, persisting after death, which he believed corresponded to the subcutaneous venous network. No biopsy was reported. Sigmund and Shelley4 interpreted both Hansemann’s and Walzel’s findings as livedoid change associated with pancreatitis, and coined the term “Walzel’s Sign.” Our case of LR associated with pancreatitis is a rarely reported clinical finding. Like Sigmund and Shelley’s, our case is also unilateral. In our case, biopsy specimens were uniquely examined by hematoxylin-eosin stain and immunofluorescence. In contrast to Hansemann’s case, ours showed only superficial perivascular dermatitis and hemorrhage. Direct immunofluorescence revealed
Journal of the American Academy of Dermatology December 1998
immunoglobin and complement deposition. Although this finding can be seen in vasculitis, the lack of fibrin deposition on direct immunofluorescence and lack of vessel wall necrosis and fibrin thrombi on histopathology is indicative of immune complex deposition without vasculitis. However, it is also possible that disturbed vasculature in the flank region, perhaps brought on by the LR or for other reasons, allowed for the deposition of nonpathogenic immune complexes in this site, implying association without causation of LR and immune complex deposition. Our patient’s findings suggest an immune-mediated cause of livedo reticularis, possibly in concert with pancreatitis. This case is unique because of the relapsing nature of the LR, representing Walzel’s sign, with immune complex deposition and hemorrhage, but without fat necrosis or vasculitis. REFERENCES 1. Fleischer AB, Resnick SD. Livedo reticularis. Dermatol Clin 1990;8:347-54. 2. Champion RH. Livedo reticularis: a review. Br J Dermatol 1965;77:167-79. 3. Copeman PWM. Livedo reticularis: signs in the skin of disturbance of blood viscosity and of blood flow. Br J Dermatol 1975;93:519-29. 4. Sigmund WJ, Shelley WB. Cutaneous manifestations of acute pancreatitis, with special reference to livedo reticularis. N Engl J Med 1954;251:851-3.