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Medial calcific arteriosclerosis mimicking giant cell arteritis
2025
718
Hypercarotenemia: An etiology of xanthoderma Parth Shah, Mayo Clinic, Phoenix, AZ, United States; Krishnaswamy Chandrasekaran, MD, Mayo Clinic, phoenix, AZ, United States Case presentation: A 61-year-old male with moderate to severe aortic stenosis underwent transesophageal echocardiography to assess aortic root dilatation. Observation of the patient’s skin revealed yellow-orange discoloration. The patient admitted that he is an athletic male who drinks two gallons of carrot juice per day in addition to a daily turmeric supplement. Hypercarotenemia was thus suspected. Laboratory results confirmed our suspicion as the carotene level was elevated at 622 mcg/dL (the normal range is 48-200 mcg/dL). Jaundice was excluded as no signs or history of liver disease was present, the sclera was clear without any icterus, and laboratory results showed that the total bilirubin was normal at 1.1 mg/dL (the normal range is 0.1-1.1 g/dL). Discussion: Yellow-orange skin discoloration, or ‘‘xanthoderma,’’ is a physical finding that can complicate a differential diagnosis. Hypercarotenemia can cause this phenomenon and it results from a high level of carotenoids in the blood which leads to carotene deposition in the stratum corneum layer of the epidermis. Carotenoids are found conjugated with proteins or in crystalline complexes in fruits and vegetables such as carrots, and in other sources, such as palm oil. Excessive intake of carotenoids via diet can lead to hypercarotenemia, as seen in our patient. Hypercarotenemia can also be caused by hypothyroidism and diabetes mellitus (both absent in our patient) as these diseases can impair the conversion of betacarotene to retinol and can increase carotenoid bound serum lipoproteins. The distinction between hypercarotenemia and jaundice is crucial as hypercarotenemia is largely known to be harmless, whereas jaundice is a sign of a potentially threatening underlying disease. In jaundice, xanthoderma is caused by the build-up of bilirubin in the elastic tissues which leads to yellow-discoloration of the epithelium. Several diseases can cause jaundice and they are prehepatic, hepatic, or posthepatic in nature. Jaundice also causes yellowing of the sclera, which is affected before the skin. The presence of yellow sclera and hyperbilirubinemia, both absent in our patient, distinguish jaundice from hypercarotenemia. Thus, xanthoderma should alert for the possibility of hypercarotenemia, and requires additional consideration of the aforementioned diseases.
Necrolytic acral erythema Jeanyoung Kim, MD, Drexel Dermatology, Philadelphia, PA, United States; Dorothy Wilson, MD, Drexel Dermatology, Philadelphia, PA, United States; Carrie Ann Cusack, MD, Drexel Dermatology, Philadelphia, PA, United States Necrolytic acral erythema (NAE) is a rare condition that was first described in seven patients in 1996 in Egypt. It is strongly associated with hepatitis C (HCV) and may be considered an early marker of infection or correlate with degree of liver disease. It is typically described as a papulosquamous vesiculobullous eruption localized to acral surfaces; however, extensive disease can progress to upper thighs and upper arms. A 59-year-old African-American male with a history of HCV and HIV presented with several months of rough, dry skin on his hands. He reported progressive itchiness, scaling and dark discoloration. He denied any new exposures or work with chemicals. He denied involvement of his feet. Treatment with multiple topical therapies provided mild improvement. He recently started antiretroviral therapy for HIV; however, he has never been treated for HCV. Cutaneous examination revealed scaly hyperpigmented lichenified erythematous plaques on bilateral dorsal fifth metacarpals. On the lower back extending to the gluteal cleft, there was a hyperpigmented lichenified plaque. Laboratory evaluation was significant for hepatitis C genotype 1a and viral load 5,762,760 IU/mL. Serum zinc was 82 ug/dL (normal 56-134 ug/dL). Skin biopsy from the right dorsal hand showed parakeratosis, hyperkeratosis, irregular epidermal acanthosis and collections of lymphocytes with rare neutrophils in the upper epidermis. Slight pallor to the upper epidermis was noted. Rare necrotic keratinocytes and exocytosis of lymphocytes were appreciated. Within the dermis there was a perivascular lymphohistiocytic infiltrate along with extravasated erythrocytes. The patient was started on zinc supplementation for NAE. He is currently awaiting treatment for HCV. The pathogenesis of NAE is unknown, but it is thought to be related to dysregulation of zinc metabolism. Because of its rarity, there are no definitive treatment recommendations for NAE. Topical corticosteroids are largely ineffective. There has been some success with hyperalimentation and protein supplements. Zinc supplementation has been shown to be effective, even in the setting of normal zinc levels. Treatment of HCV with interferon-a and ribavirin has shown the most benefit.
Commercial support: None identified.
Commercial support: None identified.
741 Medial calcific arteriosclerosis mimicking giant cell arteritis Liza Gill, Henry Ford Hospital, Detroit, MI, United States; Pranita Rambhatla, MD, Henry Ford Hospital, Detroit, MI, United States; Laurie Kohen, MD, Henry Ford Hospital, Detroit, MI, United States Introduction: Medial calcific arteriosclerosis is characterized by diffuse mineral deposition in the tunica media of arterial walls. First described in patients with diabetes mellitus, medial calcific arteriosclerosis has been increasingly recognized in patients with chronic kidney disease. Giant cell arteritis typically presents in people over 50 years old and shows granulomatous inflammatory infiltrate in all layers of the arterial wall. We report a case of medial calcific arteriosclerosis mimicking giant cell arteritis seen in the HFHS Dermatology Clinic.
1129
Case report: A 63-year-old African-American female was seen in the HFHS dermatology day hospital for treatment of pruritis from reactive perforating collagenosis secondary to end-stage renal disease. On day six of her treatment, the patient expressed concern of firm, cord-like areas on her bilateral temporal scalp present for one week. She endorsed a 3-month history of chronic headaches, jaw pain and intermittent vision changes. Pulsatile, thickened cord-like vessels were palpated on physical examination. Clinical presentation was suggestive of giant cell arteritis and she was referred for same day consultation with ophthalmology. Her symptoms, elevated erythrocyte sedimentation rate and C-reactive protein were suggestive of giant cell arteritis, and she was immediately started on prednisone. A temporal artery biopsy was performed. Pathology demonstrated M€ onckeberg medial calcific arteriosclerosis without any evidence of active or healed arteritis. Despite her negative biopsy, prednisone alleviated her scalp tenderness, jaw claudication, and visual disturbances. She has been continued on 10 mg prednisone daily and is followed by ophthalmology. Discussion: This patient’s symptoms and clinical examination closely mimicked findings commonly associated with giant cell arteritis. However, temporal artery biopsy was consistent with medial calcific arteriosclerosis. M€ onckeberg medial calcific arteriosclerosis is a calcification process that affects the media of large and medium-sized arteries and is associated with calcification limited to the arterial media. While medial calcific arteriosclerosis is not an entity commonly seen in dermatology, it should be included in the differential diagnosis of end-stage renal disease patients presenting with clinical manifestations of vascular diseases.
Necrolytic migratory erythema as the first presentation of pancreatic glucagonoma Pai-Shan Cheng, MD, Chi Mei Medical Center, Tainan, Taiwan; Chi-Hsuan Chiang, MD, Chi Mei Medical Center, Tainan, Taiwan; Min-Xian Lin, MD, Chi Mei Medical Center, Tainan, Taiwan; Chao-Chin Wang, MD, Chi Mei Medical Center, Tainan, Taiwan; Feng-Jie Lai, MD, PhD, Chi Mei Medical Center, Tainan, Taiwan A previously healthy 49-year-old female presented with some itchy crusted papules over back, dorsal feet and knees for about 1.5 months. Skin biopsy was done, and the pathology showed focal pallor and vacuolated keratinocytes at bilateral edges of the specimen which suggested a diagnosis of deficiency diseases such as zinc deficiency, pellagra or necrolytic migratory erythema. However, she lost of follow-up until one month later with progression of the lesions to entire bilateral lower legs, inguinal area, back and face. Besides, atrophic glossitis, generalized malaise and acute body weight loss (8 kg in 2 months) were also noted. The laboratory data revealed hyperglycemia (glucose AC 147 mg/dL), anemia (Hb: 10g/dL), mild elevated liver enzyme (AST/ALT: 57/65 IU/L) and hypoalbuminemia (2.9 g/dL). Under the impression of necrolytic migratory erythema, survey for the pancreatic tumor was done. The abdominal computerized tomography showed a pancreatic head tumor with multiple liver metastases. Serum glucagon level was markedly elevated (4255 pg/mL, normal range: 50-100 pg/mL). The biopsies of the pancreatic and hepatic tumors both showed neuroendocrine tumors which stained positive for glucagon. Thus, she was diagnosed with pancreatic glucagonoma with multiple liver metastases and glucagonoma associated necrolytic migratory erythema. Somatostatin and sunitinib were given, and the skin lesions showed dramatic improvement after the medication 1 month later. However, she sought for conventional treatment and was lost of follow-up again. The glucagonoma syndrome is a rare disease in which a typical skin disorder, necrolytic migratory erythema, is usually one of the first presenting symptoms. Weight loss, diabetes mellitus, normochromic normocytic anemia, elevated liver enzymes, low albumin level are other prevalent characteristics of this syndrome. Recognizing the early presentation of the characteristic skin manifestations may lead to early diagnosis of this pancreatic cancer; however, tumors are frequently large and metastatic by the time of diagnosis for most patients.
Commercial support: None identified.
Commercial support: None identified.
MAY 2015
J AM ACAD DERMATOL
AB149
718
Hypercarotenemia: An etiology of xanthoderma Parth Shah, Mayo Clinic, Phoenix, AZ, United States; Krishnaswamy Chandrasekaran, MD, Mayo Clinic, phoenix, AZ, United States Case presentation: A 61-year-old male with moderate to severe aortic stenosis underwent transesophageal echocardiography to assess aortic root dilatation. Observation of the patient’s skin revealed yellow-orange discoloration. The patient admitted that he is an athletic male who drinks two gallons of carrot juice per day in addition to a daily turmeric supplement. Hypercarotenemia was thus suspected. Laboratory results confirmed our suspicion as the carotene level was elevated at 622 mcg/dL (the normal range is 48-200 mcg/dL). Jaundice was excluded as no signs or history of liver disease was present, the sclera was clear without any icterus, and laboratory results showed that the total bilirubin was normal at 1.1 mg/dL (the normal range is 0.1-1.1 g/dL). Discussion: Yellow-orange skin discoloration, or ‘‘xanthoderma,’’ is a physical finding that can complicate a differential diagnosis. Hypercarotenemia can cause this phenomenon and it results from a high level of carotenoids in the blood which leads to carotene deposition in the stratum corneum layer of the epidermis. Carotenoids are found conjugated with proteins or in crystalline complexes in fruits and vegetables such as carrots, and in other sources, such as palm oil. Excessive intake of carotenoids via diet can lead to hypercarotenemia, as seen in our patient. Hypercarotenemia can also be caused by hypothyroidism and diabetes mellitus (both absent in our patient) as these diseases can impair the conversion of betacarotene to retinol and can increase carotenoid bound serum lipoproteins. The distinction between hypercarotenemia and jaundice is crucial as hypercarotenemia is largely known to be harmless, whereas jaundice is a sign of a potentially threatening underlying disease. In jaundice, xanthoderma is caused by the build-up of bilirubin in the elastic tissues which leads to yellow-discoloration of the epithelium. Several diseases can cause jaundice and they are prehepatic, hepatic, or posthepatic in nature. Jaundice also causes yellowing of the sclera, which is affected before the skin. The presence of yellow sclera and hyperbilirubinemia, both absent in our patient, distinguish jaundice from hypercarotenemia. Thus, xanthoderma should alert for the possibility of hypercarotenemia, and requires additional consideration of the aforementioned diseases.
Necrolytic acral erythema Jeanyoung Kim, MD, Drexel Dermatology, Philadelphia, PA, United States; Dorothy Wilson, MD, Drexel Dermatology, Philadelphia, PA, United States; Carrie Ann Cusack, MD, Drexel Dermatology, Philadelphia, PA, United States Necrolytic acral erythema (NAE) is a rare condition that was first described in seven patients in 1996 in Egypt. It is strongly associated with hepatitis C (HCV) and may be considered an early marker of infection or correlate with degree of liver disease. It is typically described as a papulosquamous vesiculobullous eruption localized to acral surfaces; however, extensive disease can progress to upper thighs and upper arms. A 59-year-old African-American male with a history of HCV and HIV presented with several months of rough, dry skin on his hands. He reported progressive itchiness, scaling and dark discoloration. He denied any new exposures or work with chemicals. He denied involvement of his feet. Treatment with multiple topical therapies provided mild improvement. He recently started antiretroviral therapy for HIV; however, he has never been treated for HCV. Cutaneous examination revealed scaly hyperpigmented lichenified erythematous plaques on bilateral dorsal fifth metacarpals. On the lower back extending to the gluteal cleft, there was a hyperpigmented lichenified plaque. Laboratory evaluation was significant for hepatitis C genotype 1a and viral load 5,762,760 IU/mL. Serum zinc was 82 ug/dL (normal 56-134 ug/dL). Skin biopsy from the right dorsal hand showed parakeratosis, hyperkeratosis, irregular epidermal acanthosis and collections of lymphocytes with rare neutrophils in the upper epidermis. Slight pallor to the upper epidermis was noted. Rare necrotic keratinocytes and exocytosis of lymphocytes were appreciated. Within the dermis there was a perivascular lymphohistiocytic infiltrate along with extravasated erythrocytes. The patient was started on zinc supplementation for NAE. He is currently awaiting treatment for HCV. The pathogenesis of NAE is unknown, but it is thought to be related to dysregulation of zinc metabolism. Because of its rarity, there are no definitive treatment recommendations for NAE. Topical corticosteroids are largely ineffective. There has been some success with hyperalimentation and protein supplements. Zinc supplementation has been shown to be effective, even in the setting of normal zinc levels. Treatment of HCV with interferon-a and ribavirin has shown the most benefit.
Commercial support: None identified.
Commercial support: None identified.
741 Medial calcific arteriosclerosis mimicking giant cell arteritis Liza Gill, Henry Ford Hospital, Detroit, MI, United States; Pranita Rambhatla, MD, Henry Ford Hospital, Detroit, MI, United States; Laurie Kohen, MD, Henry Ford Hospital, Detroit, MI, United States Introduction: Medial calcific arteriosclerosis is characterized by diffuse mineral deposition in the tunica media of arterial walls. First described in patients with diabetes mellitus, medial calcific arteriosclerosis has been increasingly recognized in patients with chronic kidney disease. Giant cell arteritis typically presents in people over 50 years old and shows granulomatous inflammatory infiltrate in all layers of the arterial wall. We report a case of medial calcific arteriosclerosis mimicking giant cell arteritis seen in the HFHS Dermatology Clinic.
1129
Case report: A 63-year-old African-American female was seen in the HFHS dermatology day hospital for treatment of pruritis from reactive perforating collagenosis secondary to end-stage renal disease. On day six of her treatment, the patient expressed concern of firm, cord-like areas on her bilateral temporal scalp present for one week. She endorsed a 3-month history of chronic headaches, jaw pain and intermittent vision changes. Pulsatile, thickened cord-like vessels were palpated on physical examination. Clinical presentation was suggestive of giant cell arteritis and she was referred for same day consultation with ophthalmology. Her symptoms, elevated erythrocyte sedimentation rate and C-reactive protein were suggestive of giant cell arteritis, and she was immediately started on prednisone. A temporal artery biopsy was performed. Pathology demonstrated M€ onckeberg medial calcific arteriosclerosis without any evidence of active or healed arteritis. Despite her negative biopsy, prednisone alleviated her scalp tenderness, jaw claudication, and visual disturbances. She has been continued on 10 mg prednisone daily and is followed by ophthalmology. Discussion: This patient’s symptoms and clinical examination closely mimicked findings commonly associated with giant cell arteritis. However, temporal artery biopsy was consistent with medial calcific arteriosclerosis. M€ onckeberg medial calcific arteriosclerosis is a calcification process that affects the media of large and medium-sized arteries and is associated with calcification limited to the arterial media. While medial calcific arteriosclerosis is not an entity commonly seen in dermatology, it should be included in the differential diagnosis of end-stage renal disease patients presenting with clinical manifestations of vascular diseases.
Necrolytic migratory erythema as the first presentation of pancreatic glucagonoma Pai-Shan Cheng, MD, Chi Mei Medical Center, Tainan, Taiwan; Chi-Hsuan Chiang, MD, Chi Mei Medical Center, Tainan, Taiwan; Min-Xian Lin, MD, Chi Mei Medical Center, Tainan, Taiwan; Chao-Chin Wang, MD, Chi Mei Medical Center, Tainan, Taiwan; Feng-Jie Lai, MD, PhD, Chi Mei Medical Center, Tainan, Taiwan A previously healthy 49-year-old female presented with some itchy crusted papules over back, dorsal feet and knees for about 1.5 months. Skin biopsy was done, and the pathology showed focal pallor and vacuolated keratinocytes at bilateral edges of the specimen which suggested a diagnosis of deficiency diseases such as zinc deficiency, pellagra or necrolytic migratory erythema. However, she lost of follow-up until one month later with progression of the lesions to entire bilateral lower legs, inguinal area, back and face. Besides, atrophic glossitis, generalized malaise and acute body weight loss (8 kg in 2 months) were also noted. The laboratory data revealed hyperglycemia (glucose AC 147 mg/dL), anemia (Hb: 10g/dL), mild elevated liver enzyme (AST/ALT: 57/65 IU/L) and hypoalbuminemia (2.9 g/dL). Under the impression of necrolytic migratory erythema, survey for the pancreatic tumor was done. The abdominal computerized tomography showed a pancreatic head tumor with multiple liver metastases. Serum glucagon level was markedly elevated (4255 pg/mL, normal range: 50-100 pg/mL). The biopsies of the pancreatic and hepatic tumors both showed neuroendocrine tumors which stained positive for glucagon. Thus, she was diagnosed with pancreatic glucagonoma with multiple liver metastases and glucagonoma associated necrolytic migratory erythema. Somatostatin and sunitinib were given, and the skin lesions showed dramatic improvement after the medication 1 month later. However, she sought for conventional treatment and was lost of follow-up again. The glucagonoma syndrome is a rare disease in which a typical skin disorder, necrolytic migratory erythema, is usually one of the first presenting symptoms. Weight loss, diabetes mellitus, normochromic normocytic anemia, elevated liver enzymes, low albumin level are other prevalent characteristics of this syndrome. Recognizing the early presentation of the characteristic skin manifestations may lead to early diagnosis of this pancreatic cancer; however, tumors are frequently large and metastatic by the time of diagnosis for most patients.
Commercial support: None identified.
Commercial support: None identified.
MAY 2015
J AM ACAD DERMATOL
AB149