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Necrobiotic xanthogranuloma in an 18-year-old female
P2310
P2312
Benefits of mild, moisturizing body cleanser for pediatric patients with atopic eczema Jamie Regan, MS, Unilever R&D, Trumbull, CT, United States; Kumar Subramanyan, PhD, Unilever R&D, Trumbull, CT, United States
The cutaneous spectrum of nutritional deficiencies Temitope Soares, PharmD, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States; Xuan Nguyen, MD, Phoenix Children’s Hospital, Phoenix, AZ, United States; Ronald Hansen, MD, Phoenix Children’s Hospital, Phoenix, AZ, United States
Atopic eczema is an intensely pruritic cutaneous inflammatory condition that usually occurs in children and affects more than 15% of the population. Common clinical features of this disease include erythema and pruritus. Among other factors, it is known that this condition can be exacerbated by bathing, especially with harsh cleansers. A single-center, institutional review boardeapproved, double-blinded, randomized, monadic design clinical study in children (6 months to 15 years) diagnosed with mild to moderate atopic eczema was conducted to evaluate potential benefits of daily bathing with a mild, emollient-containing, moisturizing cleanser. Forty-one subjects were enrolled into the test cell (emollient liquid body cleanser) while 22 subjects were enrolled in a control cell (continued normal bathing product) for a period of 4 weeks. Eczema severity was evaluated at baseline, week 2 and week 4 by a Board-certified pediatrician using the standardized Eczema Area Severity Index (EASI) score. In addition, patient or parent evaluation scores were collected on eczema severity, itching, tightness, and dryness at the same time points. Results indicate that while patients in both cells showed significant improvements in the EASI score at weeks 2 and 4, the subjects using the mild emollient containing cleanser showed a directionally better improvement over the test period. This is further corroborated by the patient self-assessment data that shows a significantly better improvement in itching, tightness and directionally better improvement in dryness and roughness for patients using the test cleanser. The data from this study clearly suggests that a mild, emollient containing cleanser is an effective adjuvant in managing atopic eczema in pediatric patients. Fully funded by Unilever R&D.
Many cases of nutritional deficiencies are diagnosed only after evident skin changes. We present a few cases to illustrate the vital role of cutaneous symptoms in the diagnosis and treatment of nutritional deficiencies. Case 1: a 17-year-old male presents with a 1-year history of scaly rash worsening over the previous few weeks. The rash consisted of confluent pink plaques with flaky scale distributed on the scalp, face, neck, trunk, and some crusted blisters on both feet. Further evaluation revealed decreased zinc levels, hypoalbuminemia, and decreased alkaline phosphatase. The patient admitted to a 2-year history of a special zero-protein diet in response to a history of lupus to avoid nephropathy. The rash cleared significantly within 24 hours of treatment with zinc. A regular diet was reinstituted. Case 2: a 2month-old male born with meconium ileus was admitted to the hospital for failure to thrive and diarrhea. He also had been hitherto diagnosed with atopic dermatitis unresponsive to topical steroid therapy. His rash appeared as sharply demarcated pink-red plaques with adherent scale with some desquamation on arms, legs, and in the groin region. The skin lesions in addition to the history led to a suspicion of cystic fibrosis, which was eventually confirmed by further studies including gene mutation studies. Case 3: a 4-month-old male was admitted to the hospital for presumed recalcitrant cutaneous infection and/or cellulitis. He had been exclusively breast-fed. He had sharply demarcated bright-red weeping plaques with some overlying crust scales distributed on the neck, arms, inguinal and perianal regions. Zinc deficiency was suspected, and confirmed by laboratory studies. Prompt resolution was observed with oral zinc supplementation. Case 4: a 9-month-old male was admitted to the hospital for failure to thrive and a desquamative rash. His mother had abandoned him after many weeks of neglect and malnutrition. He had mostly confluent mildly erythematous scaly plaques with the typical ‘‘peeling paint’’ appearance of kwashiorkor. Multiple laboratory abnormalities were found. Appropriate nutritional support resulted in resolution of the rash. The presented cases have different etiologies; a fad diet, undiagnosed cystic fibrosis, zinc deficiency from breastfeeding, and malnutrition from neglect. They all, however, have a common thread: definitive diagnosis of nutritional deficiency after skin manifestations. Commercial support: None identified.
P2313
Discussion: Tumors with benign smooth muscle differentiation include congenital and/or acquired smooth muscle hamartomas, leiomyoma, and myofibroma. There is controversy whether congenital smooth muscle hamartoma is a distinct entity or a form of Becker’s nevus, in spite of similar histologic findings. Others consider congenital smooth muscle hamartoma and Becker’s nevus at opposite ends of the same spectrum. Congenital smooth muscle hamartomas are not always associated with hypertrichosis or hyperpigmentation whereas Becker’s nevi typically have these features. Acquired smooth muscle hamartomas may or may not be associated with Becker’s nevus. Smooth muscle hamartomas may also be the underlying lesions in Michelin tire baby syndrome in boys. Reported locations include intra-oral, trunk, extremities, and neck. Approximately, a total of five facial Becker’s nevi have been reported. Only one clinical case of linear presentation has been reported as a congenital smooth muscle hamartoma on the hip. The combination of blaschkoid presentation on the face makes this case unique.
Necrobiotic xanthogranuloma in an 18-year-old female Phil Ecker, MD, Mayo Clinic, Rochester, MN, United States; Dawn Davis, MD, Mayo Clinic, Rochester, MN, United States An 18-year-old female presented with a 3-month history of asymptomatic lesions on her lower extremities. She had come in seeking treatment for warts and brought up her leg lesions in passing. The lesions did not seem to change in size, shape, or color, and they came on gradually in the early summer. When she first noticed them, she had a cough and rhinorrhea, but had been feeling well for several months. She had a recent travel history of being in Hawaii but denied any insect bites. She denied recent camping or hiking. She also denied fevers, chills, diarrhea or constipation. On examination, she had multiple erythematous subcutaneous nodules and a few ecchymoses on the bilateral lower extremities, especially prominent on the anterior surfaces. There was no pain on palpating the lesions. There was no yellow quality to the lesions and no ulcerations were appreciated. Although the clinical impression was erythema nodosum, a biopsy was obtained due to the persistent nature of the lesions. A portion of the specimen was also sent for culture, including mycobacteria and bacteria. Histologically, dermal and pannicular palisading granulomatous inflammation was appreciated. Collections of multinucleated giant cells were appreciated, some of which were very large, foamy, and bizarre. Focal areas of cholesterol clefts were also observed. An ESR, streptococcal antibody, and stool microbiology were within normal limits. Serum electrophoresis (SPEP) with immunofixation was normal. An ECG and chest radiograph were negative. A complete blood count was within normal limits with the exception of a slight elevation in eosinophils of 0.55 (normal range, 0.05-0.5). A glucose was 84, ACE level was slightly elevated at 50 (normal range, 7-46), and cholesterol panel was within normal limits. She had negative cryoglobulin and cryofibrinogen studies. Total compliment, C1 esterase inhibitor, C1Q, C3, and C4 both quantitative and qualitative were within normal limits. Urinalysis was negative. A skin TB test showed 0 mm induration and no erythema. Despite extensive workup for underlying disease, and a clinical picture more consistent with persistent erythema nodosum, a histologic diagnosis of necrobiotic xanthogranuloma was favored. We monitor her every 6 months, and her SPEP has remained normal. She reported complete resolution of her lesions, but then developed new lesions in different areas on her legs. They continue to be asymptomatic.
Commercial support: None identified.
Commercial support: None identified.
P2311 Blaschkoid smooth muscle hamartoma of the face Marjan Yousefi, Geisinger Medical Center, Danville, PA, United States; Howard Pride, Geisinger Medical Center, Danville, PA, United States; William Tyler, Geisinger Medical Center, Danville, PA, United States Introduction: Cutaneous smooth muscle hamartoma is a benign proliferation of smooth muscles typically of arrector pili muscle origin most commonly located on trunk and proximal extremities. It is more frequently seen in males. Herein, we report a case of smooth muscle hamartoma with two unusual features: location of the face and Blaschkoid presentation. Case presentation: An 8-year-old male presented with an asymptomatic lesion on the left lateral face present since the age of 3. It had been unresponsive to topical lotions and had been slowly enlarging. Physical examination showed subtle slightly brownish agminated oval papules in a Blaschkoid linear fashion distally curved inwards on the left lateral face parallel to the left mandible with no epidermal changes or hypertrichosis. A three millimeter punch biopsy of the lesion showed fascicles of smooth muscles not directly associated with hair follicles within the dermis consistent with a smooth muscle hamartoma. Elastic stain was normal.
FEBRUARY 2008
J AM ACAD DERMATOL
AB109
P2312
Benefits of mild, moisturizing body cleanser for pediatric patients with atopic eczema Jamie Regan, MS, Unilever R&D, Trumbull, CT, United States; Kumar Subramanyan, PhD, Unilever R&D, Trumbull, CT, United States
The cutaneous spectrum of nutritional deficiencies Temitope Soares, PharmD, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States; Xuan Nguyen, MD, Phoenix Children’s Hospital, Phoenix, AZ, United States; Ronald Hansen, MD, Phoenix Children’s Hospital, Phoenix, AZ, United States
Atopic eczema is an intensely pruritic cutaneous inflammatory condition that usually occurs in children and affects more than 15% of the population. Common clinical features of this disease include erythema and pruritus. Among other factors, it is known that this condition can be exacerbated by bathing, especially with harsh cleansers. A single-center, institutional review boardeapproved, double-blinded, randomized, monadic design clinical study in children (6 months to 15 years) diagnosed with mild to moderate atopic eczema was conducted to evaluate potential benefits of daily bathing with a mild, emollient-containing, moisturizing cleanser. Forty-one subjects were enrolled into the test cell (emollient liquid body cleanser) while 22 subjects were enrolled in a control cell (continued normal bathing product) for a period of 4 weeks. Eczema severity was evaluated at baseline, week 2 and week 4 by a Board-certified pediatrician using the standardized Eczema Area Severity Index (EASI) score. In addition, patient or parent evaluation scores were collected on eczema severity, itching, tightness, and dryness at the same time points. Results indicate that while patients in both cells showed significant improvements in the EASI score at weeks 2 and 4, the subjects using the mild emollient containing cleanser showed a directionally better improvement over the test period. This is further corroborated by the patient self-assessment data that shows a significantly better improvement in itching, tightness and directionally better improvement in dryness and roughness for patients using the test cleanser. The data from this study clearly suggests that a mild, emollient containing cleanser is an effective adjuvant in managing atopic eczema in pediatric patients. Fully funded by Unilever R&D.
Many cases of nutritional deficiencies are diagnosed only after evident skin changes. We present a few cases to illustrate the vital role of cutaneous symptoms in the diagnosis and treatment of nutritional deficiencies. Case 1: a 17-year-old male presents with a 1-year history of scaly rash worsening over the previous few weeks. The rash consisted of confluent pink plaques with flaky scale distributed on the scalp, face, neck, trunk, and some crusted blisters on both feet. Further evaluation revealed decreased zinc levels, hypoalbuminemia, and decreased alkaline phosphatase. The patient admitted to a 2-year history of a special zero-protein diet in response to a history of lupus to avoid nephropathy. The rash cleared significantly within 24 hours of treatment with zinc. A regular diet was reinstituted. Case 2: a 2month-old male born with meconium ileus was admitted to the hospital for failure to thrive and diarrhea. He also had been hitherto diagnosed with atopic dermatitis unresponsive to topical steroid therapy. His rash appeared as sharply demarcated pink-red plaques with adherent scale with some desquamation on arms, legs, and in the groin region. The skin lesions in addition to the history led to a suspicion of cystic fibrosis, which was eventually confirmed by further studies including gene mutation studies. Case 3: a 4-month-old male was admitted to the hospital for presumed recalcitrant cutaneous infection and/or cellulitis. He had been exclusively breast-fed. He had sharply demarcated bright-red weeping plaques with some overlying crust scales distributed on the neck, arms, inguinal and perianal regions. Zinc deficiency was suspected, and confirmed by laboratory studies. Prompt resolution was observed with oral zinc supplementation. Case 4: a 9-month-old male was admitted to the hospital for failure to thrive and a desquamative rash. His mother had abandoned him after many weeks of neglect and malnutrition. He had mostly confluent mildly erythematous scaly plaques with the typical ‘‘peeling paint’’ appearance of kwashiorkor. Multiple laboratory abnormalities were found. Appropriate nutritional support resulted in resolution of the rash. The presented cases have different etiologies; a fad diet, undiagnosed cystic fibrosis, zinc deficiency from breastfeeding, and malnutrition from neglect. They all, however, have a common thread: definitive diagnosis of nutritional deficiency after skin manifestations. Commercial support: None identified.
P2313
Discussion: Tumors with benign smooth muscle differentiation include congenital and/or acquired smooth muscle hamartomas, leiomyoma, and myofibroma. There is controversy whether congenital smooth muscle hamartoma is a distinct entity or a form of Becker’s nevus, in spite of similar histologic findings. Others consider congenital smooth muscle hamartoma and Becker’s nevus at opposite ends of the same spectrum. Congenital smooth muscle hamartomas are not always associated with hypertrichosis or hyperpigmentation whereas Becker’s nevi typically have these features. Acquired smooth muscle hamartomas may or may not be associated with Becker’s nevus. Smooth muscle hamartomas may also be the underlying lesions in Michelin tire baby syndrome in boys. Reported locations include intra-oral, trunk, extremities, and neck. Approximately, a total of five facial Becker’s nevi have been reported. Only one clinical case of linear presentation has been reported as a congenital smooth muscle hamartoma on the hip. The combination of blaschkoid presentation on the face makes this case unique.
Necrobiotic xanthogranuloma in an 18-year-old female Phil Ecker, MD, Mayo Clinic, Rochester, MN, United States; Dawn Davis, MD, Mayo Clinic, Rochester, MN, United States An 18-year-old female presented with a 3-month history of asymptomatic lesions on her lower extremities. She had come in seeking treatment for warts and brought up her leg lesions in passing. The lesions did not seem to change in size, shape, or color, and they came on gradually in the early summer. When she first noticed them, she had a cough and rhinorrhea, but had been feeling well for several months. She had a recent travel history of being in Hawaii but denied any insect bites. She denied recent camping or hiking. She also denied fevers, chills, diarrhea or constipation. On examination, she had multiple erythematous subcutaneous nodules and a few ecchymoses on the bilateral lower extremities, especially prominent on the anterior surfaces. There was no pain on palpating the lesions. There was no yellow quality to the lesions and no ulcerations were appreciated. Although the clinical impression was erythema nodosum, a biopsy was obtained due to the persistent nature of the lesions. A portion of the specimen was also sent for culture, including mycobacteria and bacteria. Histologically, dermal and pannicular palisading granulomatous inflammation was appreciated. Collections of multinucleated giant cells were appreciated, some of which were very large, foamy, and bizarre. Focal areas of cholesterol clefts were also observed. An ESR, streptococcal antibody, and stool microbiology were within normal limits. Serum electrophoresis (SPEP) with immunofixation was normal. An ECG and chest radiograph were negative. A complete blood count was within normal limits with the exception of a slight elevation in eosinophils of 0.55 (normal range, 0.05-0.5). A glucose was 84, ACE level was slightly elevated at 50 (normal range, 7-46), and cholesterol panel was within normal limits. She had negative cryoglobulin and cryofibrinogen studies. Total compliment, C1 esterase inhibitor, C1Q, C3, and C4 both quantitative and qualitative were within normal limits. Urinalysis was negative. A skin TB test showed 0 mm induration and no erythema. Despite extensive workup for underlying disease, and a clinical picture more consistent with persistent erythema nodosum, a histologic diagnosis of necrobiotic xanthogranuloma was favored. We monitor her every 6 months, and her SPEP has remained normal. She reported complete resolution of her lesions, but then developed new lesions in different areas on her legs. They continue to be asymptomatic.
Commercial support: None identified.
Commercial support: None identified.
P2311 Blaschkoid smooth muscle hamartoma of the face Marjan Yousefi, Geisinger Medical Center, Danville, PA, United States; Howard Pride, Geisinger Medical Center, Danville, PA, United States; William Tyler, Geisinger Medical Center, Danville, PA, United States Introduction: Cutaneous smooth muscle hamartoma is a benign proliferation of smooth muscles typically of arrector pili muscle origin most commonly located on trunk and proximal extremities. It is more frequently seen in males. Herein, we report a case of smooth muscle hamartoma with two unusual features: location of the face and Blaschkoid presentation. Case presentation: An 8-year-old male presented with an asymptomatic lesion on the left lateral face present since the age of 3. It had been unresponsive to topical lotions and had been slowly enlarging. Physical examination showed subtle slightly brownish agminated oval papules in a Blaschkoid linear fashion distally curved inwards on the left lateral face parallel to the left mandible with no epidermal changes or hypertrichosis. A three millimeter punch biopsy of the lesion showed fascicles of smooth muscles not directly associated with hair follicles within the dermis consistent with a smooth muscle hamartoma. Elastic stain was normal.
FEBRUARY 2008
J AM ACAD DERMATOL
AB109