Nevus lipomatosus cutaneous superficialis is a very rare skin disease which is characterized by the ectopic presence of rnature adipose tissue in the dermis. There are two clinical variants. The first type is the multiple lesions of zonal distribution usually on the buttocks or the lower back area. Sometimes coalescence of the papules tend to form cerebriform plaques. The second type is the solitary papule or nodule with less restricted tendency in its distribution. We describe a 24-year-old female patient who had a soft yellowish, well defined, fetal head-sized cerebriform tumor with comedo-like plugs on the right buttock and the perineal area. The skin lesions began about 10 years ago and gradually increased in size. Routine laboratory findings were within the normal limit. Histopathological findings are hyperkeratosis, acanthosis, deep crypt-like indenation in the epidermis, and ectopic collection of mature adipose tissue in the derrnis. Diagnosis was confirrned by clinical and histopathological findings. Literature was briefly reviewed for discussion.
Adipose Tissue ; Buttocks ; Dermis ; Diagnosis ; Epidermis ; Female ; Fluconazole ; Humans ; Nevus* ; Skin ; Skin Diseases ; Young Adult

Acromelanosis Progressiva which was described by Mishirna et al. In 1962 is a very rare pigmentary disorder and it seems that this is the first case reporting in Korean literature. The patient we observed was 11 years old girl having dark brown macules on the dorsa of her hands and feet, forearms, lower legs and face. These pigmented macules at first appeared on the dorsal surfaces of her fingers and toes when her age of 7. Thereafter these macules spread somewhat progressively to the proximal portions of the extremities and recently those have been spread upon her forearms and lower legs. The same pigmented macules appeared meanwhile on her face when her age of 9 and also have been spread diffusely on the cheeks and eyelids at visiting. Sh was otherwise normal on physically and had no familial history of such pigmentary disorder. But she had mental deficiency (I.Q. 60).
Cheek ; Child ; Extremities ; Eyelids ; Female ; Fingers ; Foot ; Forearm ; Hand ; Humans ; Intellectual Disability ; Leg ; Toes

Sweet's syndrome is an important cutaneous sign of underlying myeloproliferative disorder. The majority of cases have occurred with acute leukemia, primarily of the myelogenous type. We described a case of Sweet's syndrome in a patient with myelodysplastic syndrome that preceded acute myelogenous leukemia by 9 months.
Humans ; Leukemia ; Leukemia, Myeloid, Acute* ; Myelodysplastic Syndromes* ; Myeloproliferative Disorders ; Sweet Syndrome*

Erythema multiforme(EM) is an acute, self-limited eruption of the skin and mucous membrane, characterized by distinctive target lesions. Although a wide range of factors have been implicated as precipitating EM, the pathogenesis is unknown. Recently, several studies on EM have been reportecl, showing the presence of immune complexes in patient with EM, and these authors suggested that such complexes may be cf immunopathogenic significance. The atudy was undertaken to irivestigate the pathogenesis of EM. In ten patients with EM, we performed serological study and direct immunofluorescence study. The reaults obtained were as follows. 1) There was no signifir.ant abnormality in CBC, urinalysis, chest PA, stool, and serology including ASO, CH, Ig quantitation, VDRL, HBsAg, ANA, RF, and cryoglobul in. 2 In direct immunofluorescence study, 4 biopsies showed IgM deposits in the su!erficial blood vessels, 4 demonstrated C, 2 showed IgG deposition and 2 biopsies showed fibrin deposition. All biopsies were negative for lgA. Additionally 1 biopsy demonstrated IgM depnsition along the dermoepidermal junction, and 1 showed both IgG and fihrin deposition. This study supports the view that deposition of immune complexes may play a role in the pathogenesis of EM.
Biopsy

No abstract available.
Poroma*

A 25-year-old woman developed generalized urticaria and an anaphylactic syndrome of sudden onset while she was being treated for her decubitus ulcer with chlorhexidine antiseptic solution. Prick test with 0.5% chlorhexidine produced a wheal in a few minutes. A passive intradermal transfer test (PK test) to her mother was positive. These enabled us diagnose her as having an immunologic contact urticaria to chlorhexidine.
Adult ; Chlorhexidine* ; Female ; Humans ; Mothers ; Pressure Ulcer ; Urticaria*

Eosinophilic leukemia is a rare variant of granulocytic leukemia. It has distinct clinical features to involve central nervous system, heart, lung and skin more frequently than other leukemia and laboratory findings to reveal persistent eosinophilia with immature tendency, leukocytosis, anernia, thrombocytopenia and usually negative philadelphia chromosome in cytogenetic study. We describe a 33 year-old male patient who has suffered from generalizid weakness exertional dyspnea and has had well defined, hen eggs ized, oval shaped and non-in-durated ulcerative lesions on the right thigh since about 4 months prior to admission. The laboratory findings were persistent leukocytosis, eosinophilia and immature tendency in peripheral blood, nearly 100% cellularity and eosinophilic tendency (70%) in bone marrow aspiration and biopsy, and negative philadelphia chromosome in cytogenetic study. A biopsy specimen from ulcer margin showed relatively intact epidermis and heavy cellular infiltration composed of eosinophilic cytoplasm, dark and indented nucleus in entire dermis. Normal appearing skin also revealed same cellular infiltration in dermis with perivascular and periadnexal pattern.
Adult ; Biopsy ; Bone Marrow ; Central Nervous System ; Cytogenetics ; Cytoplasm ; Dermis ; Dyspnea ; Eggs ; Eosinophilia ; Eosinophils* ; Epidermis ; Heart ; Humans ; Hypereosinophilic Syndrome* ; Leukemia ; Leukemia, Myeloid ; Leukocytosis ; Lung ; Male ; Ovum ; Philadelphia Chromosome ; Skin ; Thigh ; Thrombocytopenia ; Ulcer

We presented two cases of bullous dermatoses in childhood. The first case was a 13-year-old boy who had numerous tense bullae on the scalp, both forearms and dorsa of hands, trunk and both thighs in symmetrical distribution, but he had not any oral lesion. On histologic examination, subepidermal bullae containing many neutrophils and only a few eosinophils were demonstrated. The lesions began to subside prornptly since 2 days after ornl administration of prednisolone and there was no evidence of recurrence during the follow-up studies of 4 months. The second case was a 14-year-old boy who had erythematous macules, vesicles, bullae and crusts scattered irregularly on almost entire body surface and had some oral lesions. Histologically erythematous macular lesions revealed many microabscesses composed almost entirely oi neutrophils on the tip of dermal papillae but vesicular lesions showed intraepidermal bulla containing numerous eosinophils exclusively. His skin lesions were well suppressed by DDS but reappeared one or two days after cessation of DDS. We thought these two cases were not the typical cases of bullous pemphigoid or dermatitis herpetiforrnis. We did not perform immunologic studies, so we cannot assure that these two cases were belong to either bullous pemphigoid or dermatitis herpetiformis and the so-called benign chronic bullous dermatosis of childhood.
Adolescent ; Dermatitis ; Dermatitis Herpetiformis ; Eosinophils ; Follow-Up Studies ; Forearm ; Hand ; Humans ; Male ; Neutrophils ; Pemphigoid, Bullous ; Prednisolone ; Recurrence ; Scalp ; Skin ; Skin Diseases ; Skin Diseases, Vesiculobullous* ; Thigh ; Transcutaneous Electric Nerve Stimulation

Cutis marmirata telangiectatica congenita, first described by van Lohuizen in 1922, is a rare congenital vascular anomaly. This name is still generally used in Europe, but in American literature the most comrnon designation for this disease is generalized congenital phlebectasia. We represent the first reported case of cutis marmorata telangiectatica, congenita in Korea. The patient, 8 months old boy, has generalized cutis marmorata-like reticulated mottlings on his entire skin except the rnucous membranes since birth. He also has complete cleft palate but no other detectable congenital vascular malformation. The skin lesion shows spontaneous gradual improvement from three months of age.
Cleft Palate ; Europe ; Humans ; Infant ; Korea ; Male ; Membranes ; Parturition ; Skin ; Vascular Malformations

Generalized lipodyst,rophy is characterized by generalized loss of body fat, and is asociated vith metabolic ahnormalities, including insulin resistance, hyperglycemia., and hypertriglyceridemia. like acanthosis nigricans, generalized lipodystrophy is a cutaneous marker of insulin re.istant diabetes. We report. herein a twenty year old female witti both classic generalized lipodystripin and acanthosis nigricans, in association with insulin resistant diabetes.
Acanthosis Nigricans ; Adipose Tissue ; Female ; Humans ; Hyperglycemia ; Hypertriglyceridemia ; Insulin ; Insulin Resistance ; Lipodystrophy, Congenital Generalized*