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Biomedical subjects

H-S Hong

Publications and source records attributed to H-S Hong.

11 recordsLinked to original sources

Livedoid vasculopathy: long-term follow-up results following hyperbaric oxygen therapy.

BACKGROUND: Livedoid vasculopathy, also known as atrophie blanche, is a recurrent painful vasculopathy appearing mostly on the lower limbs. Treatment is challenging and relapses are frequent. OBJECTIVES: To analyse the long-term effect and safety of hyperbaric oxygen (HBO) therapy in treating livedoid vasculopathy. METHODS: Twelve patients with active livedoid vasculopathy were included in this study. All patients underwent HBO therapy five times a week. Each week photographs were taken and the total dose of analgesics was recorded. Side-effects were documented and assessed. Recurrence was defined as the presence of skin ulceration. RESULTS: Of the eight patients who completed the treatment, resumption of ambulation and reduction of analgesics were achieved at an average of 4.9 HBO therapy sessions. Leg ulcers in all eight patients healed completely at a mean of 3.4 weeks (range 2-5 weeks). Six patients suffered relapses of ulceration and responded to additional HBO therapy. No significant side-effects were found. CONCLUSIONS: HBO is a relatively safe, fast and effective method to treat patients with livedoid vasculopathy.

Adolescent↗

Genital porokeratosis: a series of 10 patients and review of the literature.

BACKGROUND: Porokeratosis (PK) is an uncommon disorder of epidermal keratinization with a unique clinical appearance, unknown aetiology, and an unpredictable outcome. Genital PK (defined as localized PK confined to the genital area in this study) is extremely rare and is not well documented. OBJECTIVES: To evaluate the clinical manifestations, histopathology, clinical course and treatment response for genital PK. METHODS: We reviewed the clinicohistological data from 10 patients with genital PK seen at Chang Gung Memorial Hospital from 1990 to 2005. RESULTS: Seven patients had lesions only in the genital area, and three patients had genital and adjacent areas involved. All patients were male and the mean age at initial diagnosis was 46.0 years (range 36-59). All but one patient presented with pruritic lesions. Three patients (30%) had diabetes mellitus. No malignant transformation was observed. Surgical excision was the most effective treatment if it could be performed. CONCLUSIONS: Genital PK appeared more frequently in the Asian population than in reports from western countries. Genital PK presented mostly as pruritic lesions in Taiwan, with a wide age distribution. Long-term follow-up might be needed.

Adult↗

Folliculosebaceous cystic hamartoma arising within a port-wine stain.

A 34-year-old woman presented with 2-year history of a dome-shaped papule on a well-circumscribed, thickened, port-wine stain on the left side of the chin. Squeezing on the port-wine-stain plaque revealed many comedos within dilated follicular orifices. The papule was excised and submitted for histological examination. Histopathological study showed a lobular neoplasm, comprising dilated, cystic pilosebaceous structures surrounded by fibrous stroma, bearing the characteristics of folliculosebaceous cystic hamartoma. The reported case shows that, in addition to the vascular nature, both ectodermal and mesenchymal abnormalities may be involved in port-wine stains.

Adult↗

Carcinoma erysipelatoides from squamous cell carcinoma of unknown origin.

Carcinoma erysipelatoides, also known as inflammatory metastatic carcinoma, is a rare form of cutaneous metastasis from a malignancy. The characteristic histopathological finding is metastatic tumour cells inside the dermal lymphatic ducts. It is frequently observed in patients with breast carcinoma as well as adenocarcinoma of pancreas, rectum, lung, ovary and parotid gland. We present a 66-year-old man diagnosed to have metastatic squamous cell carcinoma by aspiration cytology from an enlarged neck lymph node and a core biopsy of a left axillary mass. He subsequently received radiotherapy; however, due to intolerance to erythema and swelling on local irradiated skin, radiotherapy was deferred. Skin lesions on upper chest and neck area, consisting of erythematous induration with telangiectasia and tenderness, progressed slowly and were treated as cellulitis. The erythema remained stationary with antibiotic treatment. Skin biopsy shows poorly differentiated squamous carcinoma cells within dermis and dilated dermal vessels.

Aged↗

Seeing the gene therapy: application of gene gun technique to transfect and decolour pigmented rat skin with human agouti signalling protein cDNA.

We developed a gene gun method for the transfer of human agouti signalling protein (ASP) cDNA to alter rat skin colour in vivo. Human ASP cDNA was cloned into a modified cytomegalovirus plasmid and delivered to the skin of Long-Evans rats by gene gun bombardment. Skin pigmentation, body weight and blood sugar of ASP cDNA-transfected rats were recorded against the control group, which were injected with plasmids encoding for green fluorescent protein. The treated skin showed lighter skin colour after 3 days of ASP gene transfection. This depigmentation effect was most prominent on day 14 and the skin gradually returned to its original pigmentation by day 28. Successful transfection of ASP gene in skin and hair follicles, as well as downregulation of melanocortin-1 receptor (MC1R) and tyrosinase expression upon treatment, was confirmed using immunohistochemistry and Western blot analysis. Body weight and blood sugar in the treated rats did not show statistically significant differences as compared to control groups. These observations demonstrate that gene transfer using the gene gun method can induce high cutaneous ASP production and facilitate a switch from dark to fair colour without systemic pleiotropic effects. Such a colour switch may be that ASP is acting in a paracrine fashion. In addition, this study verifies that ASP exerts its functions by acting as an independent ligand that downregulates the melanocyte MC1R and tyrosinase protein in an in vivo system. Our result offers new, interesting insights about the effect of ASP on pigmentation, providing a novel approach to study the molecular mechanisms underlying skin melanogenesis.

Agouti Signaling Protein↗

Clinical association of anti-golgi autoantibodies and their autoantigens.

Anti-Golgi autoantibodies (AGAs) and their targets have been reported from several diseases. However, the association of AGAs, selective autoantigens and related clinical diseases is still obscure. In this study, the presence of AGAs in the sera of 5983 patients was screened to explore the association of AGAs and clinical diseases. By means of indirect immunofluorescence using HEp-2 cells, sera of 12 patients bearing AGAs were identified. The location of recognized Golgi autoantigen(s) was confirmed by the treatment of monensin and double immunostaining using beta-COP. Using the immunoelectron microscopy, AGA immunoreactivity was clearly demonstrated at a stack structure, characteristic of the Golgi complex. Furthermore, analysis of the 12 AGA-positive sera by Western blot revealed at least 15 components of Golgi antigens with relative molecular weights ranging from 54 to 350 kDa, and several Golgi autoantigens identified may be novel. Notably, over half of the AGA-positive cases found belong to non-autoimmune diseases, particularly hepatic disorder. This study presents the association of AGAs, components of the Golgi complex and clinical diseases.

Adult↗

Somatosensory organization and behavior in naked mole-rats: II. Peripheral structures, innervation, and selective lack of neuropeptides associated with thermoregulation and pain.

African naked mole-rats are subterranean rodents that have a robust orienting response to stimulation of unique vibrissa-like body hairs that are widely spaced over an otherwise hairless skin. To determine whether these large body hairs have a specialized organization similar to facial vibrissae, the structure and innervation of facial vibrissa follicles, body hair follicles, and intervening skin in naked mole-rats was compared with that in rats and a furred African mole-rat species (the common mole-rat). Immunofluorescence and lectin-binding analyses revealed that the body hair follicles in naked mole-rats were exceptionally large and well innervated, similar to guard hairs of furred species. However, these body vibrissae lacked the anatomic specializations and unique types of innervation affiliated with follicle sinus complexes of facial vibrissae. In contrast to the furred species, naked mole-rats had a paucity of Abeta-fiber Merkel endings at all peripheral locations. Naked mole-rats also were completely lacking in cutaneous C-fibers immunoreactive for substance P and calcitonin gene-related peptide. In contrast, the hairless skin of the naked mole-rats had an exceptional abundance of presumptive Adelta-fibers. The unusual features of the cutaneous innervation in naked mole-rats are presumably adaptations to their subterranean environment and that they are the only known poikilothermic mammal. The features of this mammalian model system provide unique opportunities to discriminate mechanisms related to tactile spatial orientation, vascular regulation, and nociception.

Afferent Pathways↗

Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen.

We describe a new method for treating livedoid vasculopathy. The typical presentation of livedoid vasculopathy includes chronic, recurrent painful ulcers, satellite scar-like atrophy and telangiectasia involving the lower extremities. Histologically, these lesions show areas of ulceration and dermal vessel occlusion without frank inflammatory cell infiltration. There is currently no satisfactory therapy available for this disease. Hyperbaric oxygen (HBO) has recently established itself as one of the most effective methods of treating ischaemic wounds, including diabetic ulcers. We used this therapy in two patients whose lesions were resistant to multiple therapeutic modalities. Not only did their ulcers respond rapidly to the HBO therapy, but the disturbing wound pain also resolved at the same time. To our knowledge, this is the first successful trial of HBO therapy in livedoid vasculopathy. We believe this to be a very promising new therapy for livedoid vasculopathy and to be worth further investigation.

Adolescent↗

Abdominal cavernous Iymphangiomas: CT findings.

Two adult patients with histopathologically proved cavernous lymphangiomas and one adult patient with lymphangiomas of strongly presumed cavernous type by cytologic and computed tomographic findings are reported. On computed tomograms, multiple, aggregated, small, and tiny cysts without a solid portion, along the lymphatic channels are characteristic computed tomographic findings for cavernous lymphangiomas.

Abdominal Neoplasms↗