ABSTRACT
Eruptive pseudoandiomatosis (EPA) is a rare, self-limiting exanthem, which is characterized by numerous erythematous angioma-like papules surrounded by a pale halo. We report two cases of EPA, which developed in adulthood. The first patient, a 50-year-old female, had erythematous angioma-like papules surrounded by a pale halo on the face and arms. The second patient, a 34-year-old female, had lesions on the upper and lower extremities. The histological features included dilated blood vessels with plump endothelial cells, but there was no evidence of frank vascular proliferation or vasculitis.
Subject(s)
Adult , Female , Humans , Middle Aged , Arm , Blood Vessels , Endothelial Cells , Exanthema , Lower Extremity , VasculitisABSTRACT
BACKGROUND: Pemphigus is a chronic blistering disease of the skin and mucous membranes, with severe morbidity and occasional mortality. It is necessary to investigate the clinical features, treatment and long-term outcome of Korean patients with pemphigus as it is the most common and severe autoimmune bullous disease in Korea. OBJECTIVE: The purpose of this study was to evaluate the clinical features, treatment and prognosis of Korean patients with pemphigus. METHOD: A retrospective analysis was conducted on 124 pemphigus patients seen between 1987 and 2003 at Youngdong Severance Hospital. RESULTS: Out of 124 patients diagnosed with pemphigus during the study period, 62 patients had pemphigus vulgaris (PV), and 62 patients had pemphigus foliaceus (PF). For the 62 PV patients, the mean age of onset was 48.3 years, and the male to female ratio was 1: 1.7. For the 62 PF patients, the mean age of onset was 44.7 years, and the male to female ratio was 1.4: 1. Average initial dose of oral prednisolone was 30.2mg in PV patients and 19.9mg in PF. Fifty six (90%) patients with PV and 38 (61%) patients with PF received immunosuppressive agents. Partial remission was induced in 9.7%, 23.0%, 41.7%, and 75% of PV patients and in 12.9%, 19.0%, 13.6% and 50% of PF patients after 1, 3, 5, and 10 years of diagnosis, respectively. Five (8%) of each PV and PF patients died during the treatment period. CONCLUSION: The clinical manifestations and results of immunofluorescent studies on 124 Korean pemphigus patients were similar to those of previous studies. Contrast to previous studies, the PV to PF ratio was 1: 1, and a male preference was observed on PF. More studies are needed to improve the prognosis and reduce the complications in the treatment of pemphigus patients.
Subject(s)
Female , Humans , Male , Age of Onset , Blister , Diagnosis , Immunosuppressive Agents , Korea , Mortality , Mucous Membrane , Pemphigus , Prednisolone , Prognosis , Retrospective Studies , SkinABSTRACT
Calcinosis cutis results from deposits of calcium and phosphorus in the skin. Calcinosis cutis may be divided into four major categories: dystrophic, metastatic, idiopathic and iatrogenic. A 19-year-old female presented with multiple brown or skin-colored, stony hard plaques on the face, trunk, arm and thigh for 18 months. She did not have any familial and medical history. None of the laboratory findings, including serum calcium, phosphate and autoantibodies for connective tissue disease, was significant. X-ray examination of bones evidenced multiple reticulated heterotrophic calcifications and whole body bone scan evidenced increased radiotracer uptake according to cutaneous or subcutaneous tissue. This case is compatible with idiopathic calcinosis universalis. Treatment with diltiazem was started.
Subject(s)
Female , Humans , Young Adult , Arm , Autoantibodies , Calcinosis , Calcium , Connective Tissue Diseases , Diltiazem , Phosphorus , Skin , Subcutaneous Tissue , ThighABSTRACT
Lichen myxedematosus (synonym, papular mucinosis) is a disorder characterized by lichenoid papules, nodules and plaques due to dermal mucin deposition, and a variable degree of fibrosis without thyroid dysfunction. Discrete lichen myxedematosus is a subtype of the localized lichen myxedematosus. Clinically, firm, smooth, waxy or flesh-colored papules measuring 2 to 5 mm in diameter, numbering just a few to hundreds, and involving limbs and trunk. The lesions progress slowly without systemic symptoms and rarely resolve spontaneously. Histologically, the upper and mid dermis shows edema and diffuse or focal mucinous deposit under normal epidermis. Fibroblast proliferation is variable, but there is neither collagen deposition nor sclerosis. On experiencing a case of discrete grouped papules on the arm and thigh of a sixty three year-old women, we present it as discrete lichen myxedematosus.
Subject(s)
Female , Humans , Arm , Collagen , Dermis , Edema , Epidermis , Extremities , Fibroblasts , Fibrosis , Lichens , Mucins , Scleromyxedema , Sclerosis , Thigh , Thyroid GlandABSTRACT
Progressive macular hypomelanosis is an idiopathic acquired hypopigmentary disorder first described in 1988. The disorder is characterized by ill-defined, non-scaly, round to oval, hypopigmented patches symmetrically localized on the trunk. No clinical symptoms have been observed. Currently, diagnosis is made on clinical grounds and any histological, laboratory investigations are not diagnostic. This disorder does not respond to conventional treatment and its clinical course is variable. We experienced a case of progressive macular hypomelanosis that was successfully treated with narrow band UVB phototherapy.
Subject(s)
Diagnosis , Hypopigmentation , PhototherapyABSTRACT
Repeated nasal flare is a condition that one repeatedly dilates one's nostrils unconsciously. It is not pathogenic, however, may be a cosmetic problem. This is caused by excessive activities of the alar part of nasalis. We, herein, report a successfully treated case with the botulinum toxin A.
Subject(s)
Botulinum ToxinsABSTRACT
Rothmund-Thomson syndrome is a rare autosomal recessive genodermatosis, which is characterized by poikiloderma and photosensitivity with variable features including alopecia, sparse hair, short stature, skeletal abnormalities, juvenile cataracts, and an increased risk of developing skin and bone malignancies. A 19-year-old man presented with poikilodermatous skin change on the whole body since three months old, and accompanied by photosensitivity, alopecia, and brachydactyly. As far as we know, this is the first case report of Rothmund-Thomson syndrome in Korea.
Subject(s)
Humans , Young Adult , Alopecia , Brachydactyly , Cataract , Hair , Korea , Rothmund-Thomson Syndrome , SkinABSTRACT
Dyskeratosis congenita is a rare multisystemic genodermatosis of ectodermal dysplasia, and is characterized by the diagnostic triad consisting of reticulated hyperpigmentation, dystrophic nails, and leukoplakia. Complications such a malignancy and bone marrow involvement will be predisposition. A 44-year-old male was presented with reticulated hyperpigmentation, nail dystrophy, oral leukoplakia, mild hyperkeratosis of the palms and soles, and short stature. His brothers were presented with reticulated hyperpigmentation and nail dystrophy, and underwent a surgical operation due to oral cavity cancer. The histopathological findings taken from the reticulated lesion showed epidermal thinning and a mild inflammatory cell infiltration with melanophages in the dermis. We report a typical case of dyskeratosis congenita in a male with family history.