Kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme p... more Kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme poverty in developing countries and with chronic malabsorptive conditions such as cystic fibrosis in developed countries. Rare cases of kwashiorkor in affluent countries unrelated to chronic illness have been reported. We present 12 cases of kwashiorkor unrelated to chronic illness seen over 9 years by pediatric dermatologists throughout the United States, and discuss common causative themes in this easily preventable condition. Twelve children were diagnosed as having kwashiorkor in 7 tertiary referral centers throughout the United States. The diagnoses were based on the characteristic rash and the overall clinical presentation. The rash consisted of an erosive, crusting, desquamating dermatitis sometimes with classic "pasted-on" scale-the so-called flaky paint sign. Most cases were due to nutritional ignorance, perceived milk intolerance, or food faddism. Half of the cases wer...
Trichotillomania is a relatively common cause of childhood alopecia. We report our observations o... more Trichotillomania is a relatively common cause of childhood alopecia. We report our observations of 10 children with trichotillomania seen over a 2-year period at Texas Children's Hospital. Patient ages ranged from 9 to 14 years (mean: 11.3 years) with an equal gender ratio. The duration of hair-pulling ranged from 1 month to 10 years (median: 4.6 months). The scalp alone was affected in 8 cases, the scalp and eyelashes in 1 case, and the eyelashes alone in 1 case. The frontal scalp and vertex were the most common sites affected. Associated findings included nail-biting in 2 cases, “picking” of the skin in 1 case, and headaches in another case. Noted precipitating factors in 3 patients included “stress” at home and school. Associated psychopathology included major depression in 1 case, attention-deficit/hyperactivity disorder in 1 case, and an “anxious and nervous personality” in 1 case. The most important differential diagnosis to exclude from trichotillomania is alopecia areata...
Journal of the American Academy of Dermatology, 2006
Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis that is typified by distin... more Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis that is typified by distinctive cutaneous findings and often by abnormalities of teeth, hair, nails, eyes, musculoskeletal system, and central nervous system. The gene that is mutated in patients with IP has been mapped to Xq28 and encodes the NF-kappaB essential modulator, NEMO. Female patients with IP show functional mosaicism and cutaneous manifestations follow Blaschko's lines of ectodermal embryologic development. The condition is generally considered to be lethal in utero in male fetuses, suggesting that having some normal gene expression is critical for survival. We observed 9 boys with IP. All had normal karotypes and no apparent family history of IP. In 8 of these 9 patients, lesions were localized to one extremity at presentation. The diagnosis was confirmed by histopathologic examination that showed eosinophils within intraepidermal, multiloculated vesicles. One of the boys later developed dental and neurologic abnormalities. The case series was small and the workup for these patients from different sites was not uniform. Male individuals may show cutaneous and noncutaneous features of IP in a limited distribution that allows survival. Postzygotic mutation/somatic mosaicism is the likely mechanism. Given the potential sequelae associated with this condition, continuing follow-up of these patients is recommended.
Kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme p... more Kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme poverty in developing countries and with chronic malabsorptive conditions such as cystic fibrosis in developed countries. Rare cases of kwashiorkor in affluent countries unrelated to chronic illness have been reported. We present 12 cases of kwashiorkor unrelated to chronic illness seen over 9 years by pediatric dermatologists throughout the United States, and discuss common causative themes in this easily preventable condition. Twelve children were diagnosed as having kwashiorkor in 7 tertiary referral centers throughout the United States. The diagnoses were based on the characteristic rash and the overall clinical presentation. The rash consisted of an erosive, crusting, desquamating dermatitis sometimes with classic "pasted-on" scale-the so-called flaky paint sign. Most cases were due to nutritional ignorance, perceived milk intolerance, or food faddism. Half of the cases wer...
Trichotillomania is a relatively common cause of childhood alopecia. We report our observations o... more Trichotillomania is a relatively common cause of childhood alopecia. We report our observations of 10 children with trichotillomania seen over a 2-year period at Texas Children's Hospital. Patient ages ranged from 9 to 14 years (mean: 11.3 years) with an equal gender ratio. The duration of hair-pulling ranged from 1 month to 10 years (median: 4.6 months). The scalp alone was affected in 8 cases, the scalp and eyelashes in 1 case, and the eyelashes alone in 1 case. The frontal scalp and vertex were the most common sites affected. Associated findings included nail-biting in 2 cases, “picking” of the skin in 1 case, and headaches in another case. Noted precipitating factors in 3 patients included “stress” at home and school. Associated psychopathology included major depression in 1 case, attention-deficit/hyperactivity disorder in 1 case, and an “anxious and nervous personality” in 1 case. The most important differential diagnosis to exclude from trichotillomania is alopecia areata...
Journal of the American Academy of Dermatology, 2006
Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis that is typified by distin... more Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis that is typified by distinctive cutaneous findings and often by abnormalities of teeth, hair, nails, eyes, musculoskeletal system, and central nervous system. The gene that is mutated in patients with IP has been mapped to Xq28 and encodes the NF-kappaB essential modulator, NEMO. Female patients with IP show functional mosaicism and cutaneous manifestations follow Blaschko's lines of ectodermal embryologic development. The condition is generally considered to be lethal in utero in male fetuses, suggesting that having some normal gene expression is critical for survival. We observed 9 boys with IP. All had normal karotypes and no apparent family history of IP. In 8 of these 9 patients, lesions were localized to one extremity at presentation. The diagnosis was confirmed by histopathologic examination that showed eosinophils within intraepidermal, multiloculated vesicles. One of the boys later developed dental and neurologic abnormalities. The case series was small and the workup for these patients from different sites was not uniform. Male individuals may show cutaneous and noncutaneous features of IP in a limited distribution that allows survival. Postzygotic mutation/somatic mosaicism is the likely mechanism. Given the potential sequelae associated with this condition, continuing follow-up of these patients is recommended.
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Papers by Moise Levy