Uremic frost in a critically ill patient
2008; 73 (6): 790-790
Peripheral T-cell lymphoma with erysipelaslike spread
2008; 81 (1): 33-36
Soft papules and nodules on the buttock.
Archives of dermatology
2007; 143 (12): 1583-1588
Persistent erythema multiforme and CMV infection.
Journal of drugs in dermatology
2007; 6 (3): 333-336
An 88-year-old man from the Dominican Republic with a history of gastric adenocarcinoma was admitted with one month of fatigue, anorexia, weight loss, and abdominal pain. The dermatology department was consulted to evaluate an asymptomatic, shiny, firm, red nodule on the lower left chest, with an expanding rim of erythema. Skin biopsies were performed from the nodule and surrounding rim of erythema, which were both diagnostic of peripheral T-cell lymphoma (PTCL). This case is a unique example of PTCL with erysipelaslike spread.
View details for Web of Science ID 000252763300005
View details for PubMedID 18306845
Acquired ichthyosis as a manifestation of acute cutaneous graft-versus-host disease
2007; 24 (1): 49-52
Persistent erythema multiforme is a rare disorder with only 23 known cases reported in the literature. Persistent erythema multiforme has been reported in association with Epstein-Barr virus, herpes virus, hepatitis C virus, influenza, inflammatory bowel disease, and a variety of neoplasms. To our knowledge, this case is the first case of persistent erythema multiforme reported in association with cytomegalovirus infection.
View details for PubMedID 17373197
Extranodal nasal-type natural killer T-cell lymphoma in an adolescent from Bangladesh.
Journal of the American Academy of Dermatology
2006; 54 (5): S192-7
Acquired ichythosis has been described in adult hematopoietic stem cell transplant recipients. While some authors have suggested the association of acquired ichythosis with cutaneous graft-versus-host disease, the evidence to support this association is rare. We describe a patient who developed enteritis secondary to acute graft-versus-host disease and had concomitant ichthyosiform lesions. Several months later, he was diagnosed with cutaneous graft-versus-host disease. This patient is presented to suggest that acquired ichthyosis is an underrecognized manifestation of acute cutaneous graft-versus-host disease. Given the important prognostic implications of this diagnosis, we recommend a low threshold for performing a skin biopsy to rule out GVHD in the appropriate clinical setting.
View details for Web of Science ID 000243941400011
View details for PubMedID 17300650
Netherton syndrome with extensive skin peeling and failure to thrive due to a homozygous frameshift mutation in SPINK5
2005; 210 (4): 308-314
Natural killer (NK)-cell neoplasms are relatively uncommon tumors. Classification schemes prior to that of the World Health Organization (2001) did not account clearly for these neoplasms. Advances in immunohematology over the past 10 years have aided in the recognition and categorization of NK-cell tumors. One type of tumor that belongs to this class is extranodal, nasal-type NK/T-cell lymphoma. These lymphoma cells express a cytotoxic T-cell or NK-cell phenotype, including CD56 and TIA-1. Nasal-type NK/T-cell lymphomas arise from extranasal sites such as the skin and are often associated with Epstein-Barr virus infection. Asian adults are affected most commonly. Very few pediatric cases are reported in the literature, of which only 5 presented with cutaneous involvement. We report a case of an adolescent Bangladeshi boy with extranodal, nasal-type NK/T-cell lymphoma who had cutaneous nodules on the extremities.
View details for PubMedID 16631938
ARCHIVES OF DERMATOLOGY
2003; 139 (6): 803-?
Netherton syndrome (NTS) is a rare autosomal recessive multisystem disorder characterized by congenital erythroderma and ichthyosis, hair shaft abnormalities and immune dysregulation. The disorder is caused by deleterious mutations in the SPINK5 gene, encoding the serine protease inhibitor LEKTI.Our objective was to investigate if the erythrodermic variant of peeling skin syndrome is also caused by SPINK5 mutations and to study the consequences of the disease on infantile brain development.In an infant with extensive erythroderma, peeling skin and failure to thrive, we analyzed the SPINK5 gene for pathogenic mutations by direct DNA sequencing and performed repeated brain MRI studies with diffusion-weighted imaging.We identified a homozygous 4-base-pair insertion in exon 5 of SPINK5, which introduces a premature termination codon and appears to be a common mutation among West Indies islanders. MRI analyses revealed a persistent diffuse volume loss.Our results confirm that early truncation mutations of the coding sequence of SPINK5 produce a severe phenotype and that generalized peeling skin is one of the manifestations of NTS. We further demonstrate for the first time that NTS may be associated with MRI abnormalities indicative of a permanent tissue injury of the brain.
View details for Web of Science ID 000229432600010
View details for PubMedID 15942217