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Acquired extensive lipodystrophy in childhood
P3025
P3027
Acquired extensive lipodystrophy in childhood Rola Al Dhaybi, Division of Dermatology, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada; Julie Powell, MD, PhD, Division of Dermatology, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada; Victor Kokta, MD, Department of Pathology, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada
Congenital linear syringocystadenoma papilliferum mimicking nevus sebaceous Andrea Losch, MD, Indiana University School of Medicine, Granger, IN, United States; Anita Haggstrom, MD, Indiana University School of Medicine, Indianapolis, IN, United States
Lipodystrophy (LD) is a rare disease characterized by the depletion of subcutaneous adipose tissue. It can be divided into congenital and acquired types. Acquired LD may have its onset in childhood. We describe two patients seen in our clinic within the last 10 years. Case 1: A 6-year-old male presented with a 3-week history of painful and symmetrical subcutaneous nodules over posterior knees extending progressively to thighs, legs, and ankles. He had type 1 diabetes mellitus treated with insulin for 3 years. Subcutaneous nodules evolved within few months into LD of lower extremities. Blood tests showed elevated sedimentation rate with slightly uncontrolled diabetes. Case 2: A 3-year-old female presented with otitis followed by subcutaneous nodules over the legs extending to the ankles, thighs, upper extremities, and face within 1 month and evolving into generalized LD with a protuberant abdomen. A decreased appetite with 3 kg weight loss was noted in the first weeks followed by a voracious appetite. Blood tests showed leukocytosis, elevated sedimentation rate, and C-reactive protein with normal fasting glucose. Abdominal ultrasound revealed hepatosplenomegaly. The rest of blood tests including liver and renal function tests, lipid profile, thyroid-stimulating hormone, and autoimmune antibodies were within normal range in the two cases. Skin biopsy of subcutaneous nodules showed panniculitis in both cases. Acquired LD may present from the first to the third decades of life. Onset is insidious over months to years, but can be quite rapid after an episode of infectious disease. In children, acquired LD is strongly associated with panniculitis, juvenile dermatomyositis and systemic lupus erythematosus. Localized forms of LD have been noted after local trauma, particularly subcutaneous injections of various medications including insulin. LD has been associated with insulin resistance, hepatomegaly, hyperlipidemia, acanthosis nigricans, and diabetes mellitus. Diabetes mellitus usually presented subsequent to the onset of acquired generalized LD but can appear simultaneously or precede the onset of LD. Monitoring for complications should be performed with yearly investigations consisting of lipid profile, liver function tests, fasting glucose, and glucose tolerance testing for patients with abnormal fasting glucose. When patients are symptomatic, have acanthosis nigricans, or hepatomegaly, they may require closer monitoring for development of complications. Steroids may alleviate LD to a certain degree.
Background: A syringocystadenoma papilliferum (SP) is an uncommon benign skin tumor derived from apocrine sweat glands. Although SP often arises within a nevus sebaceous, it can also appear de novo. Approximately 75% of SP tumors affect the head or neck regions. Rarely, the lesions can manifest as linear plaques on the scalp. SP may clinically resemble verruca, organoid nevi, or other epidermal proliferations, but its histologic appearance is unique. Case report: A 6-week-old female infant presented with pink-yellow, crusted, verrucous papules in a linear distribution extending from the frontolateral scalp to the ipsilateral eyelid. Conclusions: SP in the absence of nevus sebaceous is a rare entity that clinically mimics organoid nevi such as nevus sebaceous. It is important to consider this diagnosis when evaluating a congenital linear plaque in order to guide appropriate management. Although rare, SP can develop various malignancies such as syringocystadenocarcinoma papilliferum. Typically, the definitive treatment is excision of the lesion. Commercial support: None identified.
Commercial support: None identified.
P3026 Staphylococcus aureus suppression with sodium hypochlorite baths and intranasal mupirocin decreases clinical severity of atopic dermatitis Jennifer Huang, MD, MBBS, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Amy Paller, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Brook Tlougan, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Melissa Abrams, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States
P3028
Staphylococcus aureus infection is both a complication of atopic dermatitis (AD) and a trigger of exacerbations. A randomized investigator-blinded placebo-controlled study of children and adolescents (6 months to 17 years) with moderate to severe AD and clinical signs of secondary bacterial infection was performed to determine the incidence of community acquired methicillin-resistant S aureus (CAMRSA) colonization and whether suppression of S aureus growth with sodium hypochlorite (bleach) baths and intranasal mupirocin improved eczema severity (Eczema Area and Severity Index [EASI] score; primary objective). Subjects with superinfected AD were treated with oral cephalexin for 14 days and either concurrent intermittent intranasal mupirocin ointment and at least twice weekly sodium hypochlorite baths (1/4 cup of 6% solution in 1/2 bathtub ¼ 0.005% sodium hypochlorite solution; treatment group) or intranasal petrolatum ointment and water (placebo group) for 3 months. Thirty-one subjects were enrolled and 22 subjects completed the study. Subjects in the treatment group showed greater median reduction from baseline (1 SD) in EASI scores at both the 1 month visit (7.7 6 9.2) and the final 3-month visit (13.7 6 11.3) when compared to the placebo group at 1 month (2.5 6 6.1; P ¼.0086) and final (3.5 6 5.9; P ¼.0019) visits, respectively. Likewise, median percent change in EASI score from baseline to 3 months was significant (P ¼.0005) with a decrease in eczema severity of -57.8% 6 18.8% in the treatment group (vs 11.9% 6 39.5% in the placebo group). At baseline, 25 of 31 (81%) subjects grew S aureus from lesional skin and 27 of 31 (87%) subjects grew S aureus from the nares. One of 25 (4%) S aureus-positive nasal cultures was CA-MRSA, while 2 of 27 (7.4%) S aureus-positive lesional skin cultures were CA-MRSA. The incidence of CA-MRSA among S aureus nasal carriers in the general cultured population at Children’s Memorial Hospital during the study time period was 10%. These data suggest that chronic use of dilute bleach baths and intermittent application of intranasal mupirocin ointment decreases the clinical severity of AD in patients with clinical signs of secondary bacterial infection. In addition, there does not appear to be increased susceptibility to resistant strains of S aureus in patients with atopic dermatitis.
Eccrine angiomatous hamartoma Greg Jacobsen, MD, Geisinger Medical Center, Danville, PA, United States; Howard Pride, MD, Geisinger Medical Center, Danville, PA, United States We present a 21-year-old male with a slowly growing birthmark on the right ear. The patient reported only sweating and occasional trauma with bleeding. No other areas of the body were affected and the patient was otherwise healthy. On examination, the patient displayed diffuse, brownish red, pebbly thickening both on the anterior and posterior right ear. The lesion also extended to the pre- and postauricular areas and down the right side of the neck. A biopsy showed a vascular proliferation lined by plump endothelial cells and surrounded by areas of edematous and fibrous connective tissue. The vessels were tortuous. In addition, clusters of eccrine coils and ducts were also noted in the surrounding dermis. The overall clinical and pathologic picture was consistent with eccrine angiomatous hamartoma. Eccrine angiomatous hamartoma is a rare, benign malformation with both vascular and eccrine components, presenting most often in childhood, 50% being present at birth. They clinically present as a slowly growing papule, nodule, or plaque with a vascular hue. The extremities, especially the legs, are the classic location. Pain, tenderness, hyperhidrosis, and hypertrichosis are frequent complaints. Histology demonstrates circumscribed areas of benign-appearing eccrine gland and vascular proliferations. Areas may appear lobulated and many of the ducts and vessels are dilated and in close association. Thrombosis and hemorrhage can be found. Markers for both endothelial and glandular differentiation are present. Excision can be attempted for localized and otherwise small lesions and is curative. Because of the size and location in our patient, we attempted pulsed dye laser without success. An otherwise benign and persistent course with sweating and bleeding secondary to trauma occurs. Our case is unique as our search of the literature showed this is the first case presented on the ear. Only one other recent case was described on the face—the forehead of an adult. In conclusion, our patient with a large eccrine angiomatous hamartoma appears to be an uncommon presentation of a rare benign process.
Commercial support: 30% sponsored by Johnson & Johnson.
Commercial support: None identified.
MARCH 2009
J AM ACAD DERMATOL
AB149
P3027
Acquired extensive lipodystrophy in childhood Rola Al Dhaybi, Division of Dermatology, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada; Julie Powell, MD, PhD, Division of Dermatology, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada; Victor Kokta, MD, Department of Pathology, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada
Congenital linear syringocystadenoma papilliferum mimicking nevus sebaceous Andrea Losch, MD, Indiana University School of Medicine, Granger, IN, United States; Anita Haggstrom, MD, Indiana University School of Medicine, Indianapolis, IN, United States
Lipodystrophy (LD) is a rare disease characterized by the depletion of subcutaneous adipose tissue. It can be divided into congenital and acquired types. Acquired LD may have its onset in childhood. We describe two patients seen in our clinic within the last 10 years. Case 1: A 6-year-old male presented with a 3-week history of painful and symmetrical subcutaneous nodules over posterior knees extending progressively to thighs, legs, and ankles. He had type 1 diabetes mellitus treated with insulin for 3 years. Subcutaneous nodules evolved within few months into LD of lower extremities. Blood tests showed elevated sedimentation rate with slightly uncontrolled diabetes. Case 2: A 3-year-old female presented with otitis followed by subcutaneous nodules over the legs extending to the ankles, thighs, upper extremities, and face within 1 month and evolving into generalized LD with a protuberant abdomen. A decreased appetite with 3 kg weight loss was noted in the first weeks followed by a voracious appetite. Blood tests showed leukocytosis, elevated sedimentation rate, and C-reactive protein with normal fasting glucose. Abdominal ultrasound revealed hepatosplenomegaly. The rest of blood tests including liver and renal function tests, lipid profile, thyroid-stimulating hormone, and autoimmune antibodies were within normal range in the two cases. Skin biopsy of subcutaneous nodules showed panniculitis in both cases. Acquired LD may present from the first to the third decades of life. Onset is insidious over months to years, but can be quite rapid after an episode of infectious disease. In children, acquired LD is strongly associated with panniculitis, juvenile dermatomyositis and systemic lupus erythematosus. Localized forms of LD have been noted after local trauma, particularly subcutaneous injections of various medications including insulin. LD has been associated with insulin resistance, hepatomegaly, hyperlipidemia, acanthosis nigricans, and diabetes mellitus. Diabetes mellitus usually presented subsequent to the onset of acquired generalized LD but can appear simultaneously or precede the onset of LD. Monitoring for complications should be performed with yearly investigations consisting of lipid profile, liver function tests, fasting glucose, and glucose tolerance testing for patients with abnormal fasting glucose. When patients are symptomatic, have acanthosis nigricans, or hepatomegaly, they may require closer monitoring for development of complications. Steroids may alleviate LD to a certain degree.
Background: A syringocystadenoma papilliferum (SP) is an uncommon benign skin tumor derived from apocrine sweat glands. Although SP often arises within a nevus sebaceous, it can also appear de novo. Approximately 75% of SP tumors affect the head or neck regions. Rarely, the lesions can manifest as linear plaques on the scalp. SP may clinically resemble verruca, organoid nevi, or other epidermal proliferations, but its histologic appearance is unique. Case report: A 6-week-old female infant presented with pink-yellow, crusted, verrucous papules in a linear distribution extending from the frontolateral scalp to the ipsilateral eyelid. Conclusions: SP in the absence of nevus sebaceous is a rare entity that clinically mimics organoid nevi such as nevus sebaceous. It is important to consider this diagnosis when evaluating a congenital linear plaque in order to guide appropriate management. Although rare, SP can develop various malignancies such as syringocystadenocarcinoma papilliferum. Typically, the definitive treatment is excision of the lesion. Commercial support: None identified.
Commercial support: None identified.
P3026 Staphylococcus aureus suppression with sodium hypochlorite baths and intranasal mupirocin decreases clinical severity of atopic dermatitis Jennifer Huang, MD, MBBS, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Amy Paller, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Brook Tlougan, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Melissa Abrams, Departments of Dermatology and Pediatrics, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States
P3028
Staphylococcus aureus infection is both a complication of atopic dermatitis (AD) and a trigger of exacerbations. A randomized investigator-blinded placebo-controlled study of children and adolescents (6 months to 17 years) with moderate to severe AD and clinical signs of secondary bacterial infection was performed to determine the incidence of community acquired methicillin-resistant S aureus (CAMRSA) colonization and whether suppression of S aureus growth with sodium hypochlorite (bleach) baths and intranasal mupirocin improved eczema severity (Eczema Area and Severity Index [EASI] score; primary objective). Subjects with superinfected AD were treated with oral cephalexin for 14 days and either concurrent intermittent intranasal mupirocin ointment and at least twice weekly sodium hypochlorite baths (1/4 cup of 6% solution in 1/2 bathtub ¼ 0.005% sodium hypochlorite solution; treatment group) or intranasal petrolatum ointment and water (placebo group) for 3 months. Thirty-one subjects were enrolled and 22 subjects completed the study. Subjects in the treatment group showed greater median reduction from baseline (1 SD) in EASI scores at both the 1 month visit (7.7 6 9.2) and the final 3-month visit (13.7 6 11.3) when compared to the placebo group at 1 month (2.5 6 6.1; P ¼.0086) and final (3.5 6 5.9; P ¼.0019) visits, respectively. Likewise, median percent change in EASI score from baseline to 3 months was significant (P ¼.0005) with a decrease in eczema severity of -57.8% 6 18.8% in the treatment group (vs 11.9% 6 39.5% in the placebo group). At baseline, 25 of 31 (81%) subjects grew S aureus from lesional skin and 27 of 31 (87%) subjects grew S aureus from the nares. One of 25 (4%) S aureus-positive nasal cultures was CA-MRSA, while 2 of 27 (7.4%) S aureus-positive lesional skin cultures were CA-MRSA. The incidence of CA-MRSA among S aureus nasal carriers in the general cultured population at Children’s Memorial Hospital during the study time period was 10%. These data suggest that chronic use of dilute bleach baths and intermittent application of intranasal mupirocin ointment decreases the clinical severity of AD in patients with clinical signs of secondary bacterial infection. In addition, there does not appear to be increased susceptibility to resistant strains of S aureus in patients with atopic dermatitis.
Eccrine angiomatous hamartoma Greg Jacobsen, MD, Geisinger Medical Center, Danville, PA, United States; Howard Pride, MD, Geisinger Medical Center, Danville, PA, United States We present a 21-year-old male with a slowly growing birthmark on the right ear. The patient reported only sweating and occasional trauma with bleeding. No other areas of the body were affected and the patient was otherwise healthy. On examination, the patient displayed diffuse, brownish red, pebbly thickening both on the anterior and posterior right ear. The lesion also extended to the pre- and postauricular areas and down the right side of the neck. A biopsy showed a vascular proliferation lined by plump endothelial cells and surrounded by areas of edematous and fibrous connective tissue. The vessels were tortuous. In addition, clusters of eccrine coils and ducts were also noted in the surrounding dermis. The overall clinical and pathologic picture was consistent with eccrine angiomatous hamartoma. Eccrine angiomatous hamartoma is a rare, benign malformation with both vascular and eccrine components, presenting most often in childhood, 50% being present at birth. They clinically present as a slowly growing papule, nodule, or plaque with a vascular hue. The extremities, especially the legs, are the classic location. Pain, tenderness, hyperhidrosis, and hypertrichosis are frequent complaints. Histology demonstrates circumscribed areas of benign-appearing eccrine gland and vascular proliferations. Areas may appear lobulated and many of the ducts and vessels are dilated and in close association. Thrombosis and hemorrhage can be found. Markers for both endothelial and glandular differentiation are present. Excision can be attempted for localized and otherwise small lesions and is curative. Because of the size and location in our patient, we attempted pulsed dye laser without success. An otherwise benign and persistent course with sweating and bleeding secondary to trauma occurs. Our case is unique as our search of the literature showed this is the first case presented on the ear. Only one other recent case was described on the face—the forehead of an adult. In conclusion, our patient with a large eccrine angiomatous hamartoma appears to be an uncommon presentation of a rare benign process.
Commercial support: 30% sponsored by Johnson & Johnson.
Commercial support: None identified.
MARCH 2009
J AM ACAD DERMATOL
AB149