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Biomedical subjects

K C Moon

Publications and source records attributed to K C Moon.

At least 37 records · Page 2Linked to original sources

Waldenström's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis B virus infection.

Cutaneous manifestations of Waldenström's macroglobulinaemia (WM) include purpura, ulcers, urticaria, leukocytoclastic vasculitis, and immunobullous dermatoses. No association has been reported previously of WM and hepatitis B virus (HBV) infection. A 40-year-old female HBV carrier was admitted to hospital because of generalized oedema, oliguria, haematuria, hypertension, fever and blood-tinged sputum. Cutaneous manifestations included generalized petechiae, palpable purpura mainly on the legs, multiple necrotic ulcerations and gangrenous changes on the toes, and necrotic, giant confluent reticulate purpura on the trunk surmounted by several tense bullae. Laboratory investigations revealed monoclonal gammopathy of IgM kappa type (6.7 g/L), membranoproliferative glomerulonephritis associated with HBV infection, Bence Jones proteinuria, and an increased number of abnormal plasmacytoid cells in the bone marrow. Pathologic examination demonstrated immune complex-mediated vasculitis with deposits of IgM in the walls of dermal vessels and secondary subepidermal bulla formation. HBV infection may have caused WM or modified the clinical course in this fatal case.

Adult↗

Carcinoma erysipeloides associated with anaplastic thyroid carcinoma.

Cutaneous metastases from carcinoma of the thyroid gland are rare and carcinoma erysipeloides is even rarer. We present the clinical, histological, and immunohistochemical features of inflammatory erysipeloid metastases arising from an anaplastic carcinoma of the thyroid gland. In this case the anaplastic carcinoma probably transformed from a pre-existing, long-standing papillary carcinoma of the thyroid gland. Although visible inflammation is a hallmark of many benign skin disorders, it is not commonly present in cutaneous malignant metastases. As a result, the significance of a marked inflammatory changes in association with metastatic skin disease may not be recognized. Dermatologists need to be aware of the potential for inflammatory manifestations in cutaneous metastases from a thyroid carcinoma.

Aged↗

mRNA expression of urokinase and plasminogen activator inhibitor-1 in human crescentic glomerulonephritis.

AIMS: Weak staining for urokinase-plasminogen activator (uPA), tissue type plasminogen activator (tPA), or plasminogen activator inhibitor-1 (PAI-1) confined to crescents has been described in a few cases of severe crescentic glomerulonephritis. We evaluated the molecular mechanism by which these proteins are increased or induced within crescents. METHODS AND RESULTS: We examined uPA, tPA and PAI-1 mRNA expression in 12 renal biopsies with crescentic glomerulonephritis, and in six control renal biopsies with no detectable abnormalities by RNA in-situ hybridization. The expressions of uPA, tPA and PAI-1 proteins were also assessed by immunofluorescence. To better determine the cellular origin of uPA and PAI-1 transcripts, CD68 protein was studied by immunohistochemistry on the same sections on which in-situ hybridization had been performed. In controls, there were very low level signals of uPA and PAI-1 mRNAs in a few glomerular epithelial cells (GECs). Specific signals of uPA and PAI-1 mRNAs were detected in the cells forming crescents in all the cases with crescentic glomerulonephritis. However, weak expression of mRNA for tPA was detected in two cases only. Immunostaining for uPA and PAI-1 was positive in some but not all, cases of crescentic glomerulonephritis. A double-labelling study showed that the signal for PAI-1 and uPA mRNAs was mainly in CD68- cells. CONCLUSIONS: Local accumulation of uPA or PAI-1 in crescents is associated with enhanced mRNA expression of these proteins. The up-regulation of PAI-1 mRNA by GECs, in particular, could play a major role in the formation of persistent fibrin deposits and progression of the lesions in crescents. Whether up-regulation of uPA is an epiphenomenon or plays a pathogenic role in the formation of crescents remains to be clarified.

Adult↗

Wet pellagra.

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Alcoholism↗

Graves' disease presenting as elephantiasic pretibial myxedema and nodules of the hands.

A 67-year-old man presented with a 2-year history of asymptomatic, firm, multiple nodules and plaques and cerebriform hypertrophy of both lower legs and feet, and well-defined, skin-colored, firm nodules and tumors on both hands. He had been diagnosed as having Graves' disease 3 years previously, and had been treated with 10 mg of methimazole and 100 microg of thyroxin (T4) daily for 2 years. Physical examination revealed nonpitting edema, flesh-colored to erythematous, firm, confluent, polypoid nodules and fissured plaques extending from the shins to the dorsa of both feet (Fig. 1), and round to oval, firm, skin-colored, walnut-to-egg-sized tumors on all 10 fingers and the ulnar side of the dorsum of the right hand (Fig. 2). The thyroid gland was diffusely enlarged; however, there was no exophthalmos, and extraocular movements were normal. There was no weight loss, loss of appetite, tremor, heat intolerance, diarrhea, or fatigue. On laboratory evaluation, thyroid-stimulating hormone (TSH) had a markedly low titer of < 0.05 microU/mL (normal: 0.4-5.0), and the TSH receptor antibody was extremely high at 73.8% (normal: < 15%). Serum free triiodothyronine (T3), T4, antimicrosome, and antithyroglobulin antibodies were normal or negative. Skin biopsy samples from the shin and hand disclosed extensive mucin deposition throughout the dermis.

Aged↗

Treatment of "Cyrano" angioma with pulsed dye laser.

BACKGROUND: Hemangiomas of the nasal tip, the so-called Cyrano nose, are often deep, disfiguring, and persistent. OBJECTIVE: To evaluate the effect of treatment with pulsed dye laser on Cyrano nose. METHODS: A 3-month-old boy with hemangioma of the nasal tip of 1-months duration underwent six sessions of pulsed dye laser treatment with a 7 mm collimated beam at fluences of 5.75-6.5 J/cm2, 6 weeks apart. RESULTS: Initial improvement was noted after two treatments, and the lesion showed a marked reduction in size and improvement in color over a total treatment period of 9 months. CONCLUSION: Treatment with the 585 nm pulsed dye laser should be considered in the management of infants with mild to moderate degrees of nasal tip hemangiomas since it effectively reduces the lesions with minimal adverse effects.

Hemangioma↗

Idiopathic eruptive macular pigmentation: report of 10 cases.

Idiopathic eruptive macular pigmentation (IEMP) is a rare disease. IEMP is characterized by asymptomatic, pigmented macules involving the neck, trunk, and proximal extremities. This study describes 10 cases of idiopathic eruptive macular pigmentation seen during a 9-year period at the Asan Medical Center, Seoul, Korea. We present these characteristic consecutive cases to provide more insight into the clinical picture and course of IEMP. Skin lesions of 8 patients were multiple brown macules involving the trunk, face, neck, and extremities. In 2 patients, multiple dark brown macules and patches were noted. The age of onset varied from 1 to 20 years. Tentative diagnoses were usually ashy dermatosis (erythema dyschromicum perstans), fixed drug eruption, or mastocytosis. The history of any erythema and drug medication was absent. Darier's sign was absent. Skin biopsy specimens showed increased pigmentation of the basal layer in an otherwise normal epidermis. Pigmentary incontinence, melanophages, and mild perivascular lymphohistiocytic infiltrate in the papillary dermis were also revealed. Mast cells could not be found. The lesions gradually disappeared during a period of several months to years. The alleged rarity of IEMP may be partially caused by medical unfamiliarity with this entity, despite its clinical and histopathologic characteristic picture. Treatment of IEMP is unnecessary because spontaneous resolution of the lesions can be expected within several months to a few years.

Adolescent↗

Advanced glycosylation end products stimulate collagen mRNA synthesis in mesangial cells mediated by protein kinase C and transforming growth factor-beta.

Advanced glycosylation end products (AGE) seem to be implicated in the pathogenesis of diabetic nephropathy. The present study has examined the effects of AGE on protein kinase C (PKC) activity and transforming growth factor-beta1 (TGF-beta1) in relation to collagen gene regulation in cultured human mesangial cells (HMCs). Quiescent HMCs were exposed to serum-free media containing bovine serum albumin (BSA), AGE-modified BSA (AGE-BSA), or glycated BSA in which AGE formation was prevented by the use of aminoguanidine (BSA-AM). AGE-BSA (200 microg/mL) induced a peak membrane-associated PKC activity, particularly PKC-a, at 4 hours. AGE-BSA stimulated alpha1(I) and alpha1(IV) collagen mRNA expression after 24-hour incubation with HMCs, which remained elevated until hour 60. HMCs incubated with AGE-BSA induced a significant inhibition of cell proliferation compared with cells incubated with BSA. AGE-BSA stimulated TGF-beta mRNA and protein expression in HMCs. The TGF-beta secreted by HMCs was shown by CCL-64 mink lung cell assay to be bioactive. In contrast, BSA-AM did not affect either collagen or TGF-beta mRNA or protein expression in HMCs. The stimulatory effects of AGE-BSA on collagen gene regulation in HMCs could be negated by the pretreatment of HMCs with GF 109203X for 30 minutes or with phorbol myristate acetate for 24 hours before AGE-BSA administration. Neutralizing antibody to TGF-beta inhibited increased collagen mRNA expression by HMCs exposed to AGE-BSA. These results suggest that AGE-BSA stimulates collagen mRNA expression by activating PKC and the transcriptional upregulation of TGF-beta1 in HMCs. Thus, PKC and TGF-beta may function as key signaling intermediaries in the AGE-up-regulated collagen gene expression pathway in HMCs.

Adult↗

A case of juvenile xanthogranuloma arising on a nevus sebaceus.

Various epithelial and mesenchymal neoplasms can arise in nevus sebaceus (NS). Juvenile xanthogranuloma arising on an NS has not been reported previously. Juvenile xanthogranuloma, a disease characterized by reactive histiocytic proliferation, could be included in the list of secondary alterations arising on an NS.

Adolescent↗

Clinicohistopathologic comparison of adult type and juvenile type xanthogranulomas in Korea.

Xanthogranuloma (XG) is an uncommon benign disorder characterized by solitary or multiple yellow-red papulonodules on the skin, and occasionally, in other organs. It is predominantly a disease of infancy or early childhood, although adults may rarely be affected. To compare the clinicohistopathological featues of juvenile-type xanthogranulomas UXGs) and adult-type xanthogranulomas (AXGs) (>14 years) in Korea, 30 cases of JXGs and 15 cases of AXGs were compared clinically and histopathologically. Except for the fact AXGs were more often solitary and larger and showed neither other associated systemic diseases nor spontaneous regression, the clinical features such as color, mean number, or site of the lesions in AXGs were not statistically different from JXGs. Histologically, AXCs were not significantly different in amounts of foamy cells, giant cells including Touton cells, and inflammatory cells, although subcutaneous involvement was seen only in the two infant cases. In conclusion, in contrast to AXGs, JXGs need special attention to accompanying systemic diseases and do not need excisional procedures, considering their frequent spontaneous regression.

Adolescent↗

Chronic tophaceous gout presenting as hyperpigmented nodules in the limbs of a patient with coexisting psoriasis.

We describe a 53-year-old male renal transplant recipient with hypertension and triglyceridemia, who showed rare manifestations of gout presenting as brownish nodules on the arms and legs as well as chronic tophaceous gouty arthritis of the hands and feet mimicking rheumatoid arthritis, in association with subsequently developed psoriasis of the palms. In elderly Asian men, hypertension and renal insufficiency may be risk factors predisposing to the development of multiple hyperpigmented nodules of tophi in the more proximal extremities.

Arm↗