Congenital zinc deficiency from mutations of the SLC39A4 gene as the genetic background of acrodermatitis enteropathica.

Acrodermatitis enteropathica (AE) is an autosomal recessive disorder with the clinical triad of acral dermatitis, diarrhea and alopecia. AE is known to be caused by mutations of the SLC39A4 gene on the chromosome band 8q24.3, encoding the zinc transporter in human.

A Case of Gardner's Syndrome Associated with Desmoid Tumor.

Desmoid tumors are uncommon benign neoplasm of the fibroblasts. They occur rarely in the general population, but they are comparatively common in patients with familial polyposis coli with or without other elements of Gardner's syndrome. Herein, we report a 16-year-old woman with Gardner's syndrome complicated by desmoid tumors on the right subscapular area.

A case of eccrine spiradenoma in a patient with neurofibromatosis.

Neurofibromatosis is a systemic hereditary disorder with varied manifestations in bone, soft tissue, the nervous system and skin. Cutaneous manifestations of neurofibromatosis are characterized by café-au-lait macules, multiple neurofibromas, Lisch nodules and intertriginous freckling. Some benign or malignant tumors such as juvenile xanthogranuloma, pheochromocytoma, and malignant melanoma can accompany neurofibromatosis.

Two cases of linear alopecia on the occipital scalp.

Alopecia of a scalp shows various shapes and extents of hair loss, from a small round patch to polymorphous patches or total global alopecia. But alopecia of a linear shape is very rare. Only a few such cases have currently been reported in the medical literature.

Fungal melanonychia.

Melanonychia is characterized by tan, brown, or black pigmentation within the nail plate. Fungal melanonychia is rare and may simulate longitudinal melanonychia caused by melanocytic lesions. We report six cases of fungal melanonychia which were confirmed histopathologically or mycologically.

Recalcitrant oral pyoderma gangrenosum in a child responsive to cyclosporine.

We report a case of recalcitrant pyoderma gangrenosum which developed on the tongue of an 8-year-old, previously healthy boy without any systemic associated disease. The lesion was confined to the tongue and was not responsive to conventional treatment with prednisolone and sulfa drug, but it showed dramatic improvement with oral cyclosporine therapy. In addition, the histopathology of the lesion included many eosinophils, which caused difficulties in diagnosis to differentiate this condition from other oral ulcerative diseases showing tissue eosinophilia including eosinophilic ulcer of the tongue.

Becker's nevus with ipsilateral breast hypoplasia: improvement with spironolactone.

Ipsilateral breast hypoplasia is a rare abnormality in Becker's nevus. The pathogenesis of the breast hypoplasia is not understood, but an increased level of androgenic receptor in the affected area may play a role. We report a case of Becker's nevus with ipsilateral breast hypoplasia.