tacrolimus and Lipodystrophy

Topics: Anti-Bacterial Agents; Biopsy; Child; Child, Preschool; Female; Follow-Up Studies; Glucocorticoids; Humans; Hydroxychloroquine; Infant; Injection Site Reaction; Injections, Intramuscular; Injections, Subcutaneous; Lipodystrophy; Male; Retrospective Studies; Subcutaneous Fat; Tacrolimus; Transplantation, Autologous; Treatment Outcome

Topics: Abdomen; Administration, Topical; Child, Preschool; Humans; Immunosuppressive Agents; Lipodystrophy; Male; Tacrolimus

Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a rare, self-limiting disease typically affecting Asian children around the age of three. In classic cases, patients present with a hypopigmented patch with central atrophy and an erythematous border in the groin area. We present a case of LCAI affecting a female Caucasian toddler that shows striking perieccrine inflammation, a finding not previously reported in this entity. LCAI has been rarely reported in Caucasian children; as a result, there is sparse literature on the histopathological findings in this subset. This case illustrates how significant deep dermal and perieccrine inflammation can be seen in this condition, thus broadening the histopathological spectrum of this disorder.

Topics: Child, Preschool; Dermatologic Agents; Eccrine Glands; Female; Groin; Humans; Immunohistochemistry; Inflammation; Lipodystrophy; Skin; Tacrolimus; White People

We describe the generation and characterization of the first inducible 'fatless' model system, the FAT-ATTAC mouse (fat apoptosis through targeted activation of caspase 8). This transgenic mouse develops identically to wild-type littermates. Apoptosis of adipocytes can be induced at any developmental stage by administration of a FK1012 analog leading to the dimerization of a membrane-bound, adipocyte-specific caspase 8-FKBP fusion protein. Within 2 weeks of dimerizer administration, FAT-ATTAC mice show near-knockout levels of circulating adipokines and markedly reduced levels of adipose tissue. FAT-ATTAC mice are glucose intolerant, have diminished basal and endotoxin-stimulated systemic inflammation, are less responsive to glucose-stimulated insulin secretion and show increased food intake independent of the effects of leptin. Most importantly, we show that functional adipocytes can be recovered upon cessation of treatment, allowing the study of adipogenesis in vivo, as well as a detailed examination of the importance of the adipocyte in the regulation of multiple physiological functions and pathological states.

Topics: Adipocytes; Adipose Tissue; Animals; Apoptosis; Caspase 8; Caspases; Dimerization; Eating; Enzyme Activation; Glucose Intolerance; Inflammation; Insulin; Insulin Secretion; Leptin; Lipodystrophy; Lipopolysaccharides; Mice; Mice, Transgenic; Recombinant Fusion Proteins; Tacrolimus; Tacrolimus Binding Proteins