Bilateral absence of the patella as an isolated congenital abnormality is an extremly rare condition. Three members in a family were found to be afflicted with the condition. Clinical examination failed to reveal any stigmata of hereditary osteo-onychodysplasia.
Christianity ; Congenital Abnormalities ; Humans ; Nail-Patella Syndrome ; Patella

Melorheostosis is a rare from of hyperostosis characterized by candle greasing down along the major axis of long bones. A case of melorheostosis combined with exostosis in the fifth toe was presented here because of its rarity.
Exostoses ; Hyperostosis ; Melorheostosis ; Toes

The term muscle herniation represents focal muscular protrusions through an acquired or congenital fascial defect. The anterior tibialis muscle is the most common site of herniation. Dynamic ultrasonography has become an important tool in dermatology for diagnosing suspected muscle herniation because it is a non-invasive, highly accurate, readily available and cost-effective imaging technique. We present here the case of a 21-year-old male Korean soldier who underwent dynamic ultrasonography to confirm the diagnosis of anterior tibialis muscle herniation.
Dermatology ; Humans ; Leg ; Male ; Military Personnel ; Muscles ; Young Adult

Hodgkin's disease (HD) is a lymphoproliferative disorder that presents as a clinically asymptomatic mass or painless lymph node swelling. Cutaneous HD is a very rare manifestation in patients with HD. A 66-year-old man complained of a maculopapular rash waxing and waning for one year. A painful mass had developed on his left axilla 7 weeks prior to presenting. The biopsy specimens of both skin and lymph node revealed Hodgkin's lymphoma with atypical lymphoid cells and Reed-Sternberg cells. We report a case of cutaneous involvement of HD, which had been presented as a maculopapular rash for one year.
Aged ; Axilla ; Biopsy ; Exanthema ; Hodgkin Disease ; Humans ; Lymph Nodes ; Lymphocytes ; Lymphoproliferative Disorders ; Reed-Sternberg Cells ; Skin

Common blue nevi are solitary, asymptomatic blue-gray, or blue-black papules, usually less than 10 mm in diameter. Rarely, lesions may be multiple and agminated or arranged in large plaques. Common blue nevi may occur anywhere, but about half the reported cases present on the dorsa of hands and feet, whereas the plaque-type blue nevi occur mainly on the trunk and extremities. Histopathologically, the plaque-type blue nevi are reported to be common or cellular. We report a rare case of plaque-type blue nevus on the right temporal area in a 32-year-old female.
Adult ; Extremities ; Female ; Foot ; Hand ; Humans ; Nevus, Blue ; Scalp

The skin lesion of herpes zoster is classically limited to a single dermatome, and most cases of multi-dermatomal herpes zoster have contiguous skin lesions. Noncontigous multi-dermatomal herpes zoster is very rare in both immunocompetent and immunosuppressed persons. The phenomenon of zoster occurring in two non-contiguous dermatomes has been referred to as zoster duplex unilateralis or bilateralis. We report here on a case of herpes zoster duplex bilateralis in a 49-year-old woman who had previously received chemotherapy for breast cancer treatment.
Breast ; Breast Neoplasms ; Female ; Herpes Zoster ; Humans ; Middle Aged ; Skin

Syphilitic aortitis, passing out of our mind, is the most common systemic manifestation of late syphilis and is more typically manifestated 10 to 30 years afterward. This diagnosis has been made less frequently in recent decades than in the past, because of public awareness of syphilis and screening program. Treponema pallidum lodge within vasa vasorum, especially ascending aorta cause the histologic changes, which are responsible for the three major forms of symptomatic cardiovascular syphilis, including aortic insufficiency, coronary ostial stenosis, and aortic aneurysm. We experienced a case of syphilitic aortitis with aortic insufficiency and aortic aneurysm in a 48-year-old man presented with progressive dyspnea. Echocardiography, chest CT, and later surgical correction were performed and surgical specimen revealed the histologic finding consistent with syphilitic aortitis. We report this case with a review of the literature.
Aorta ; Aortic Aneurysm* ; Aortitis ; Constriction, Pathologic ; Diagnosis ; Dyspnea ; Echocardiography ; Humans ; Mass Screening ; Middle Aged ; Syphilis ; Syphilis, Cardiovascular* ; Tomography, X-Ray Computed ; Treponema pallidum ; Vasa Vasorum

BACKGROUND: In perinatal hypoxic-ischemic encephalopathy (HIE), cerebral blood flow is impaired and the activity of nitric oxide systhase (NOS) is markedly increased. For the association with the development of a stroke, the endothelial NOS (eNOS) polymorphisms are well-known. METHODS: Three clinically relevant polymorphisms of the eNOS gene were determined in 37 term/near-term infants with perinatal HIE (HIE group) and 54 normal term newborn infants without any perinatal problems (control group) using a polymerase chain reaction with or without restriction fragment enzyme digestion. The differences in the genotype, allele, and haplotype frequencies were evaluated between the groups. RESULTS: The analysis of the allele frequencies showed that the G allele of Glu298Asp was more frequent in the HIE group than in the controls. The comparisons between the controls and each subgroups with complications that occurred with HIE showed that the TC genotype and C allele of T(-786)C were more common in patients with persistent pulmonary hypertension of the newborn (PPHN) than in the controls. The frequency of the A b T haplotype was lower in the HIE patients than in the controls. CONCLUSIONS: The G allele of Glu298Asp was associated with perinatal HIE, while the TC genotype and C allele of T(-786)C were associated with PPHN.
Alleles ; Digestion ; Gene Frequency ; Genotype ; Haplotypes ; Humans ; Hypertension, Pulmonary ; Hypoxia-Ischemia, Brain ; Infant ; Infant, Newborn ; Nitric Oxide ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Stroke

BACKGROUND: Atopic dermatitis (AD) is a chronic, relapsing inflammatory skin disease that's influenced by many genetic and environmental factors. Various treatment modalities are being applied for AD, including phototherapy, topical applicants and systemic agents. However, there has been no mass survey in Korea concerning the compliance of patients' to use their prescribed medication, which might influence the clinical efficacy of the physicians' treatment. OBJECTIVE: The purpose of the present study is to evaluate the efficacy, safety and patient compliance with using topical pimecrolimus to treat AD. METHODS: We reviewed the medical recordings, laboratory profiles, clinical severity scoring and photographs of AD patients at 9 general hospitals in Seoul and the local area of Korea. Interviews with the patients and a telephone survey were also done. Those patients who weren't perscribed topical applicants or those who did not use topical pimecrolimus or steroid within 6 months were excluded from this study. RESULTS: Topical pimecrolimus cream effectively controlled AD with a reduction of the EASI score from baseline 13.75 to 11.39 at 2 weeks and 4.46 at 10 weeks of topical pimecrolimus application. When topical pimecrolimus cream was applied for more than 12 months it significantly suppressed the recurrence and reactivation of AD (p<0.05). Although 22.6% of the patients complained of adverse effects, these were all transient and they did not evoke significant medical problems. Using topical pimecrolimus cream did not show significant adverse effects or complications. CONCLUSION: Topical pimecrolimus might well be an effective treatment modality for treating AD when patients show good compliance for applying the cream.
Compliance ; Dermatitis, Atopic ; Hospitals, General ; Humans ; Korea ; Medical Records ; Patient Compliance ; Phototherapy ; Recurrence ; Skin Diseases ; Tacrolimus ; Telephone

BACKGROUND: Atopic dermatitis (AD) is a chronic, relapsing inflammatory skin disease that's influenced by many genetic and environmental factors. Various treatment modalities are being applied for AD, including phototherapy, topical applicants and systemic agents. However, there has been no mass survey in Korea concerning the compliance of patients' to use their prescribed medication, which might influence the clinical efficacy of the physicians' treatment. OBJECTIVE: The purpose of the present study is to evaluate the efficacy, safety and patient compliance with using topical pimecrolimus to treat AD. METHODS: We reviewed the medical recordings, laboratory profiles, clinical severity scoring and photographs of AD patients at 9 general hospitals in Seoul and the local area of Korea. Interviews with the patients and a telephone survey were also done. Those patients who weren't perscribed topical applicants or those who did not use topical pimecrolimus or steroid within 6 months were excluded from this study. RESULTS: Topical pimecrolimus cream effectively controlled AD with a reduction of the EASI score from baseline 13.75 to 11.39 at 2 weeks and 4.46 at 10 weeks of topical pimecrolimus application. When topical pimecrolimus cream was applied for more than 12 months it significantly suppressed the recurrence and reactivation of AD (p<0.05). Although 22.6% of the patients complained of adverse effects, these were all transient and they did not evoke significant medical problems. Using topical pimecrolimus cream did not show significant adverse effects or complications. CONCLUSION: Topical pimecrolimus might well be an effective treatment modality for treating AD when patients show good compliance for applying the cream.
Compliance ; Dermatitis, Atopic ; Hospitals, General ; Humans ; Korea ; Medical Records ; Patient Compliance ; Phototherapy ; Recurrence ; Skin Diseases ; Tacrolimus ; Telephone