We observed the relationship between horn cysts which are one of the most important findings in seborrheic keratosis, and the intra-epidermal adnexal epithelium in 481 cases of seborrheic keratosis. In 280 out of 481 cases (58.2%), the horn cysts had continuity to normal hair follicles or hairs were seen with in the horn cysts. In 33 out of 481 (6.9%), the horn cysts had continuity to normal eccrine ducts. In 13 out of 481 cases (2.7%), the horn cysts had relationships with both hair follicles and eccrine ducts. From those findings, we speculate that the horn cyst of SK is not formed by invagination of the epidermis, but rather by proliferation of neoplastic cells in the adnexal epithelium.
Netherton syndrome is a congenital ichthyosis associated with variable erythroderma, hair shaft defects, and atopic features. Mutations in the SPINK5 gene encoding the serine protease inhibitor LEKIT (lymphoepithelial Kazal-type related inhibitor) have been found in Netherton syndrome. These mutations cause over-desquamation of corneocytes, high skin permeability, and barrier dysfunction. A 6-year old girl diagnosed with atopic dermatitis under went a clinical trial of 0.1% tacrolims ointment, which was applied twice daily. However, her blood tacrolims concentration increased significantly. Because the girl was suspected to have Netherton syndrome, her corneum trypsin-like hydrolytic activity was examined and found to be approximately twice as high as those of controls. A mutation of the SPINK5 gene was identified. Based on these results, the girl was diagnosed as Netherton syndrome.
We report a case of 37-year-old man with a solitary, skin colored and smooth surfaced nodule on the back. The nodule was 10 mm in diameter without symptoms. Histopathologically, the lesion was a well-circumscribed nodule in the dermis and composed of two different components. One component was a proliferation of spindle cells with an interlacing pattern, and the other was an aggregation of glandular structures. The spindle cells had variations in their nuclear sizes, but no mitotic figures. Neither pallisading nor Verocay bodies were found in the spindle cells component. There were mucin deposits positive for colloid iron staining in the lumens of the glands and in the stroma around the glands. Immunohistchemically, the spindle cells were positive for S-100, but negative for AE-1/AE-3. The glandular cells were positive for AE-1/AE-3 and synaptophysin, but negative for S-100. According to these histopathological and immunohistochemical examination, we diagnosed this case as glandular neurofibroma.
We report a case of erythema elevatum diutinum (EED) associated with autoimmune neutropenia (AIN) and Sjögren syndrome. The patient was a 71-year-old man who had had Sjögren syndrome for 15 years. He almost simultaneously developed EED and AIN. He was presented with symmetrical erythematous plaques with elevations on the bilateral elbows and knees. The skin biopsy revealed leukocytoclastic vasculitis throughout the dermis. The administration of diaminodiphenyl sulfone was an effective treatment for the skin lesions. To our knowledge, this is the first case of EED associated with AIN and Sjögren syndrome. Although the causes of these three diseases are unknown, this case seems to support the hypothesis of immunological disturbances underlying these diseases.
We have previously reported that narrow-band UVB (NBUVB) monotherapy and a combination therapy of NBUVB plus topical calcipotriol are effective modalities for recalcitrant generalized vitiligo vulgaris. Additionally, low dose NBUVB therapy without incremental increases is also an effective modality for vitiliginous patients, especially those presenting a high threshold nMED. This report included a total of four vitiliginous patients. Two vitiliginous patients presenting a low threshold nMED, one vitiligo vulgaris (segmental type) presenting a high threshold nMED, and one vitiligo vulgaris (localized type), were irradiated with low dose NBUVB at 0.7nMED, to the facial or cervical vitiliginous region. Subsequent to the treatment, pigmented macules formed in the vitiliginous patches on the face and/or the cervix. Our results indicate that low dose NBUVB therapy, regardless of the type of vitiligo vulgaris or the extent of nMED threshold, is an effective and safe modality that brought about an improvement in cosmetic color match for pigmentation to facial or cervical vitiligo at an early stage.