A case of congenital neurocutaneous melanosis / 소아과

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi and benign pigment cell tumors of the leptomeninges. Neurocutaneous melanosis is thought to represent an error in the morphogenesis of embryonal neuroectoderm. We experienced a neonate who presented with giant, dark colored pigmented nevi covering chest, abdomen, neck and arms, with satellite lesions. Magnetic resonance image showed a nodular hyperintense lesion in the amygdala of the right temporal lobe, and T1-weighted images showed hyperintensities in the adjacent leptomeninges. We report a rare case of neurocutaneous melanosis with a brief review of related literature.

A Considered Case as Primary Cutaneous Mucormycosis / 대한의진균학회지

Mucormycosis is an invasive fungal infection, caused by members of the order Mucorales, that usually occurs in immunocompromised individuals. It manifests itself in a variety of ways and rarely may be confined to the cutaneous tissues. We report a case of primary cutaneous mucormycosis associated with longstanding diabetes mellitus and liver cirrhosis. A 53-year-old woman was presented because of cutaneous discoloration and bullae on the both lower extremities. Cutaneous lesions consisted of necrotic blackish discoloration with bullae and ill-defined margins with extending nature. A biopsy specimen from the cutaneous lesion revealed large, broad, nonseptate hyphae branched at right angles. Our patient was successfully treated with intravenous administration of amphotericin B and surgical debridement of necrotic tissue.

An Immunohistochemical Stain in Paget's Disease / 대한피부과학회지

BACKGROUND: Generally, there are few problems in the diagnosis of Paget's disease(PD) using the H&E stain. However, the differentiation of PD from the clonal type of Bowens disease and superficial spreading melanoma in situ that shows pagetoid spreading of tumor cells, may present diagnostic difficulties. In addition, the specia1 stains used for demonstrating the presenee of Pagets cells, such as PAS and mucicarmim, are non-specific and not always sensitive. So, inenunohistochemical stains with monoclonal antibodies against various antigens may be helpful for differentiating PD from ather morphologically similar skin lesions. OBJECTIVE: The purpose of this study was to investigate the diagnostic accuracy of immunohisto- chemical staining for diagnostic use in PD. METHODS: Immunohistochemical stains used in the biotin streptavidin amplificxl technique with monoclonal antibodies to several low rnolecular weight cytokeratin(CK)s, EMA and CEA, were performed on formalin-fixed, paraffin-embedded tissue. Twelve cases of PD(10 cases of extranmmmary PD and 2 cases of mammary PD), five cases of superficial spreading melanoma in situ and five cases of Bowens disease were investigated. RESULTS: The results were as follows. 1. Positive reactions with variable intensity using CK7, CKS, CK19 were seen in all cases(100%) of PD and the. staining intensity tor CK7 or CK19 was stronger than that of CKS. 2. Of the 12 cases of PD, both CK18 and CAM5.2 staining showed positivity in 11 cases(92%). 3. EMA and CEA staining showed positivity in 10(83%) and 9(75%) of 12 cases, respectively. 4. Some Pagets cells were negative for CK8, CK18 and EMA, although other positive cells were observed in the same sections. 5. All antigens were consistently negative in all cases of Bowens disease and superficial spreading melanoma in situ. CONCLUSION: The results show that moaoclonal antibodies to low molecular weight CKs are more sensitive than EMA or CEA in the demonstration of Pagets cells. Moreover, among the low molecular weight CK series, CK7 and CK19 are most useful for their high sensitivity and intensity.

A Case of Bednar Tumor / 대한피부과학회지

No abstract available.

Skin Manifestations of Patients with Liver Cirrhosis / 대한피부과학회지

BACKGROUND: The high level of susceptibility of patients with liver cirrhosis (LC) to septicemic infection is thought to be due to decreased reticuloendothelial function and irnpairment of several components of cell mediated and humoral immunity. It is well known that LC may cause many characteristic skin changes including jaundice, spider angioma, palmar erythema, xanthelasrna and nail changes such as clubbing and white nails. OBJECTIVE AND METHODS: During a 7 month period frorn April to October 1995, 100 patients who had been diagnosed as having LC were evaluated for the duration of their LC, the associated diseases they had apart from LC and various skin manifestations. Also, we performed the tuberculin test in 20 patients with LC and evaluated the correlation of the tuberculin test with severity of LC divided into 3 groups according to the Childs classification. RESULTS: The results are summarized as follows ; 1. The most frequent duration of LC was under 5 years (74%). 2. The most common associated disease of patients with LC was upper GI bleeding including esophageal varix (24 cases). Also, diabetes mellitus (13 cases) and hepatic coma (11 cases) were observed. 3. Jaundice (82 cases) was the most frequent skin rnanifestation of patients with LC. In addition, vascular changes (81 cases) and various nail changes (69 cases) were observed. Onychomychosis was the most common nail change of patients with LC. 4. 60% of the patients who were given the tuberculin test showed anergy, and the frequency of anergy increased in proport on to the severity of LC. However, it was not significant statistically (p> 0.05 ). CONCLUSIONS: Some of the skin manifestations help in the diagnosis of LC. Also, increased frequency of tuberculin test anergy in proportion to the severity of LC is thought to be related to decreased cell mediated immunity, although statistically it is not significant.

A Case of Infantile Myofibromatosis / 대한피부과학회지

Infantile myofibromatosis is an uncornmon, benign, self-limiting, localized or generalized process, probably of hamartomatous origin, which consists to a large degree of cells having the characteristics of myofibroblasts and sometimes of pericytes. Both solitary and multicentric forms occur. Most lesions are present at birth or in early infancy, and some are familial in origin. A female newborn presented with a firm, round, red colored, 3 x 3 cm sized tumor with central necrosis on the left chest. Histological examination revealed well-circumscribed nodules consisting of short bundles of plump, spindle shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. The tumor was immunoreactive for actin but did not stain for desmin. A Follow-up examination at the age of 3 months revealed a moderate degree of spontaneous regressior of the lesion.