Search PubMed⌕ Search

Biomedical subjects

K A Jang

Publications and source records attributed to K A Jang.

30 records · Page 2Linked to original sources

Methotrexate nodulosis.

Methotrexate (MTX) nodulosis in patients with rheumatoid arthritis treated with MTX has become a well recognized phenomenon. It has not been described in patients receiving MTX for treatment of other diseases, e.g., dermatological or malignant diseases. Recently, MTX nodulosis was described in a patient with psoriasis and arthritis. The pathophysiology and treatment of MTX nodulosis are yet unsettled. We experienced a case with dermatomyositis who developed multiple subcutaneous nodules after treatment with MTX. In our patient, numerous, small, symmetrically distributed, grouped subcutaneous nodules were seen on a V-shaped area of the anterior chest, both axillary areas, and the medial sides of both upper arms and thighs. We histopathologically examined a nodule and started treatment with hydroxychloroquine after discontinuation of MTX. The histopathology revealed septal panniculitis, and the nodules have been under regression. MTX nodulosis may be a drug-specific phenomenon. Discontinuation of MTX and replacement with hydroxychloroquine are recommended in severe cases. Awareness of this entity is important for diagnosing and treating cases of multiple nodules developing after the administration of MTX, which is widely used for many dermatological diseases.

Arm↗

Juvenile chronic myelogenous leukemia, neurofibromatosis 1, and xanthoma.

The triple association of leukemia, xanthomatous skin lesions, and neurofibromatosis 1 (NF) was first described by Royer et al. in 1958. Most of the leukemias were of the juvenile chronic myelogenous type (JCML). We describe a 7-year-old male child with xanthoma, neurofibromatosis 1, and juvenile chronic myelogenous leukemia. His mother also had NF1. We suggest that the presence of xanthomas and NF1 in a young child should raise awareness of the possible development of JCML, especially in patients with a family history of NF1.

Bone Marrow↗

Subcutaneous emphysema with spontaneous pneumomediastinum and pneumothorax in adult dermatomyositis.

We describe a 32-year-old patient with adult dermatomyositis who developed dyspnea and worsening of pre-existing infarcted skin lesions of the fingers. Chest radiographs showed diffuse hazy reticulonodular infiltration in both lungs, subcutaneous emphysema, pneumomediastinum, and pneumothorax. The pulmonary symptoms and cutaneous lesions gradually improved with a high dose of prednisolone. Although subcutaneous emphysema and pneumomediastinum occur frequently in association with traumatic disruption of cutaneous and mucosal barriers and assisted ventilation, it has rarely been observed in patients with interstitial pneumonitis in connective tissue diseases. Although dermatomyositis and subcutaneous emphysema are all relatively well-known diseases to dermatologists, the occurrence of spontaneous pneumomediastinum and pneumothorax and subsequent subcutaneous emphysema in connective tissue diseases such as dermatomyositis is unfamiliar. We discuss the possible mechanisms of this condition.

Adult↗

Four cases of pustulotic arthro-osteitis.

We describe four cases of pustulotic arthro-osteitis. All of them suffered from anterior chest wall symptoms, such as swelling and pain on the sternoclavicular joint, sternal angle area, sternocostal joint, and costochondral junction. Although chest X-ray findings were all normal, 99mTc-scintigraphy revealed increased uptake in the affected areas in all cases. Laboratory examinations including rheumatoid factor were negative or normal except for elevation of the erythrocyte sedimentation rate and C-reactive protein in 2 cases. After treatment with non-steroidal anti-inflammatory drugs and etretinate or topical PUVA, the skin lesions on the palms and soles and the arthro-osteitis were moderately improved. Pustulotic arthro-osteitis should be considered when a patient with pustulosis palmoplantaris complains of anterior chest wall pain and other joint pain.

Adult↗

Cutaneous mesenchymal hamartoma on the foot.

We report a 16-year-old man with a cutaneous mesenchymal hamartoma on the left foot since infancy. Clinically, the lesion was 10x6 cm in size, relatively ill-defined, skin colored, and comprised of confluent nodules. Histopathologic examination revealed histology compatible with cutaneous mesenchymal hamartoma.

Adipocytes↗

Eosinophilic pustular folliculitis (Ofuji's disease) in myelodysplastic syndrome.

We describe a case of eosinophilic pustular folliculitis (EPF, Ofuji's disease) in a 12-year-old male who suffered from myelodysplastic syndrome. Bone marrow study revealed an increase in the eosinophil cell line without peripheral blood eosinophilia in our case. We suggest that the immunologic abberations ascribed to myelodysplastic syndrome and the increase in the eosinophil cell line in the bone marrow might play roles in the development of EPF in our case.

Adrenal Cortex Hormones↗

Apoptosis is induced by anti-Fas antibody alone in cultured human keratinocytes.

Fas is a well-known cell surface receptor whose main function is the induction of apoptosis in many cell types including human keratinocytes. Several reports indicate that anti-Fas antibody can induce apoptosis in cultured keratinocytes after interferon gamma (IFN gamma) pretreatment. Because IFN gamma is synthesized by activated T cells, but not by keratinocytes, these results suggest that Fas may only be effective in apoptosis occurring in T-cell mediated inflammatory skin diseases. We hypothesized that Fas alone might mediate apoptosis in normal human keratinocytes without any other help and thus play a role in normal epidermal homeostasis. By using Cell Death Detection ELISA, we observed keratinocyte apoptosis 24 hours after anti-Fas antibody stimulation not only in IFN gamma-pretreated conditions but also in non-pretreated conditions. Even though the percentage of cultured keratinocytes stained by anti-Fas antibody increased from 7.8 to 25.8% 24 hours after IFN gamma stimulation, the apoptotic rate of the anti-Fas only group was the same as that of the anti-Fas plus IFN gamma treated group. In both conditions, we have verified apoptotic phenomena in cultured keratinocytes in situ by TUNEL staining. Some apoptotic bodies were phagocytosed by neighboring keratinocytes. Fas-mediated apoptosis was not inhibited by the protein synthesis inhibitor cycloheximide and was enhanced by inhibitors of several protein kinases, including PKC and staurosporine. These results suggest that Fas-mediated apoptosis may play a role in both T cell-mediated skin diseases and normal epidermal homeostasis.

Antibodies↗

Histiocytic disorders with spontaneous regression in infancy.

The histiocytic disorders are uncommon, have a wide spectrum, and are poorly understood. We describe seven cases developing in infancy, seen during a period of 9 years at Asan Medical Center, Seoul, Korea. Clinically the patients had multiple papules over the face, trunk, and extremities that developed at birth or during infancy. Histopathologic examinations revealed an infiltrate of many histiocytic cells in the upper dermis with or without epidermotropism. Four cases were classified as congenital self-healing reticulohistiocytosis in that the histiocytes were identified as Langerhans cells by positive immunohistochemical staining for S-100 protein, ultrastructural studies showing many Birbeck granules, and spontaneous regression of the lesions within 1-4 months. One infant with a solitary lesion on the forehead was diagnosed as solitary, congenital, indeterminate cell histiocytoma because the histiocytic cells were S-100 protein positive, but meticulous ultrastructural studies did not detect Birbeck granules. The lesion was removed by shave excision. Two cases were classified as generalized eruptive histiocytoma. The histiocytic cells were S-100 protein negative and ultrastructurally Birbeck granules were absent. In one patient, eyeball- or popcornlike lysosomal structures were seen. The lesions regressed completely.

Diagnosis, Differential↗