A Clinical Observation of the Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Jeju Island / 대한피부과학회지

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, acute life-threatening, hypersensitivity reaction usually to certain medications. Although SJS and TEN occur at an estimated incidence of 0.4 and 1.2 cases per million per year, these conditions have occurred, not infrequently, on Jeju island. OBJECTIVE: We performed this study to evaluate the clinical characteristics of the 15 patients diagnosed as SJS and TEN in Jeju island. METHODS: The retrospective clinico-epidemiologic analysis of 10 months' data of 15 inpatients, including 5 SJS patients, 8 SJS/TEN overlap patients and 2 TEN patients, was conducted through the available medical records. RESULTS: 1. Mean time of onset of clinical disease following the institution of a new drug was 10.1 days. Mean duration of drug exposure was 9.3 days. There were 4 cases showing positive response to skin test. The most common culprit drugs were anti-glaucoma agents (26.7%) such as methazolamide or acetazolamide, and anti-convulsants (26.7%) including valproic acid. 2. The clinical outcome was as follows; recovery in 13 patients, transfer in one patient, and expire in one. Time from appearance of first skin lesions to the initiation of therapy (time to treat) in 15 patients was 3.5 days; time from start of hospital treatment to interruption of further progression (time to arrest), 4.1 days; time to heal, 14.1 days; length of hospital stay, 20.6 days. The four patients with delayed withdrawal of drugs represented a longer time to treat (4.8 days), time to arrest (4.5 days), time to heal(15 days), and length of hospital stay (26.5 days) than those in the 11 patients with early withdrawal. 3. The fraction of patients corresponding to risk factors of SJS or TEN was as follows; elder (>60 years old) (46.6%), widespread lesions (>10%) (66.6%), lymphopenia (66.6%), leukopenia (40%), neutropenia (26.7%), thrombocytopenia (20%), abnormal BUN/Cr (20%), abnormal liver function (46.7%), symptoms of respiratory tract (53.3%), sepsis (13.3%). There were conjunctivitis (66.6%), keratitis (60%), gastroenterologic complications (40%), anxiety disorder (13.3%), and urologic complications (13.3%) in the accompanied systemic complications at first visit. 4. The analysis of a medication history in 15 patients showed 10 cases of prescribed medication and 5 female cases of non-prescribed medication. The patients taking prescribed medication were younger (47.3 years old) than the cases of non-prescribed medication (68 years old). Four cases among the patients with non-prescribed medication at the pharmacy corresponded to the prescription drugs. CONCLUSION: In Jeju island, the incidence of SJS and TEN seems to be higher than that in the published literatures. Along with the habitual pattern of too frequent, prolonged medication by elder patients, the territorial trait of seeing a pharmacist for the disease care at first, and the pharmacists practicing medicine like a physician instead of dispensing a prescription, might contribute to this higher incidence. Anti-glaucoma ophthalmics and anti-convulsants might be the principal causative drugs leading to SJS or TEN in Jeju island.

A Case of Angioma Serpiginosum Accompanied with Ipsilateral Nevus Flammeus on the Hand / 대한피부과학회지

Angioma serpiginosum is an acquired benign vascular neoplasm characterized by minute purple-colored, grouped nonpalpable punta on the extremities. However, nevus flammeus is a congenital vascular malformation that shows unilateral red macules. Although the evidence for association between vascular tumor and malformation have been posed, angioma serpiginosum is known to be rarely associated with nevus flammeus. We herein report a rare case of angioma serpiginosum accompanied with nevus flammeus on the ipsilateral hand.

A Case of Metastatic Small Cell Neuroendocrine Carcinoma of the Lung Mimicking a Merkel Cell Carcinoma / 대한피부과학회지

Metastatic small cell neuroendocrine carcinomas(MSCNC) from distant sites, such as the lung, are high grade tumors with poor prognosis. Histopathologically, it is difficult to distinguish between MSCNC and Merkel cell carcinoma solely on the basis of histologic morphology, electron microscopy, and immunohistochemistry. In recent years, differential expression of cytokeratin(CK) 20 and thyroid transcription factor(TTF)-1 has been proposed as useful distinguishing features. It must be emphasized that clinical correlation(e.g. symptomatology, chest X-ray) and a combination of CK 20 and TTF-1 markers, are essential to differentiate these two disease entities. We herein present a case of MSCNC of the lung that might be misdiagnosed as Merkel cell carcinoma.

A Retrospective Clinical Epidemiological Study of the Health Care Utilization of the Outpatients in the Dermatologic Field after 1 year's Separation of Dispensary from Medical Practice / 대한피부과학회지

BACKGROUND: Despite 2 years' separation of dispensary from medical practice since year of 2000, many outpatients in Korea have a lack of understanding this separation. In addition, the illegal practices of pharmacists such as medical examination have contributed to the recent problematic situation. OBJECTIVE: We performed this study to evaluate the actual situation of the separation of dispensary from medical practice in the dermatologic field. METHODS: The clinico-epidemiologic analysis of 17 months' data of 18, 230 outpatients was conducted through the available medical records and serial questionnaires. RESULTS: The medical or non-medical institutions that 18, 230 outpatients with skin diseases had chosen at first, were as follows by the order of frequency; pharmacy(78.5%), folk remedies or self-medication(9.8%), dermatologic institutions(5.5%), non-dermatologic medical clinics(3.4%), herb clinics(2.8%). Accordingly, most(94.5%, 17, 223) of the new patients did not select a dermatologic institution for the care of their skin diseases. The patterns of health care utilization of the patients mostly(72.9%) showed a fixed tendency to visit the one particular institution or formula continuously prior to final visit to the research hospitals. Most of the patients(62.8%) firstly visited a pharmacy for their disease care and did not revisit another institution. Since the first visit to a pharmacy, 9.6% of the patients repetitively utilized one or more herb clinic(s) or folk remedies in addition to one or more medical institution(s). The patients utilizing non-dermatologic measures for skin disease care at first, were mostly in their fifties(25.3%). The patients seeking herb medicine or non-dermatologic medical clinics, were in their teens(27.3% and 24.3%, respectively). Of the cases misdiagnosed as another disease or aggravated in the patients choosing non-dermatologic care, fungal infections are most common(24.0%). In front of 97.1% of the patients seeking pharmacy at first, the pharmacists practiced medicine like a physician in a wrong way instead of dispensing a prescription. CONCLUSION: In Korea, the majority of dermatologic clinics has been deprived of a position as an institution for primary care of skin diseases. It is imperative that dermatologists should be granted independent and unconstrained authority in the medical profession for the benefit of their patients.

Pachyonychia Congenita / 대한피부과학회지

A case of pachyonychia congcnita occurring in a 8 year-old boy is described. A few weeks after delivery, his mother noticed thickening of all the finger and toe nails of her baby. Family history was denied. In addition to characteristic features of pachyonychia congenita of all the nails, follicular keratotic papules were noted on the knees, elbows, buttocks and legs, and also tender thick calluses, on the soles with palnioplantar hyperhidrosis. There were three cornu cutaneurn on the popliteal and left antecubital fossae. Other associated fea.tures include a painful tense bulla at the left fourth finger, irnpetiginous patches on the anterior thighs, hoarseness and leukokeratosis linguae, sparseness of the scalp hairs, and granulosis rubra nasi accompanied by facial hyperhidrosis.

Multiple Neurilemomas Associated with Neurofibromatosis / 대한피부과학회지

Multiple neurilerv,omas occurred i.n a 14-year-old boy with neurofibromatosis and right eye blindness. His father succumbed to neurofibromatosis at the age of 42. Histologically-proved neurilemornas and neurofibromas were noted on the face, neck, trunk and extremities, but no cafe au-Iait spots ohserved. There were nevus anemicus, hairy nevus and pigmented moles. The associated features in this patient includc muscular atrophies, weakness of the left hand, parest,hesia of the medial side of left forcarm and Dupuytrens contracture of left fourth finger.

A Clinical Study on Myltiple Neurofibromatosis / 대한피부과학회지

No abstract available.

Behcet's Syndrome: Clinical Studies of Thirteen Cases / 대한피부과학회지

Clinical studies on thirteen patients with Behcet's syndrome who had visited during the period from Jan. 1970 to Sep. 1973, to the Department of Dermatology & Ophthahnology, Busan National University Hospital, were made and the following results obtained. 1) The ratio of male vs. female was l. 0: l. 6 with no definite sexual differences. The ages ranged from 17 to 56 years with the peak incidence in third decade of life. 2) The "complete" form consisting of oculo- oro-genital and cutaneous lesions appeared in . 4 patients (30. 8%) and the "incomplet" in 9 patients (69. 2%). 3) Aphthous stomatitis was most predominant, being observed in 12 out of all 13 cases (92.3%), the next was cutaneous manifestations such as erythema nodosum, pyodermas, acneiform & pustular lesions and erythema multiforme in 10 cases (76. 1%) and genital ulcers in 9 cascs (69.2%). Ocular lesions and articular involvements came fourth, each being found in 8 cases (61. 5%). 4) The commonest feature of ocular involvement was relapsing iridocyclitis, being observed in 57. 7% and the disease led to a bilateral loss of vision in 38. 4% 5) Single or combined therapy including corticosteroids and antimicrobials gave transient conservative effects and was of no value to prevent recurrent attacks.

Porokeratosis: Clinical Studies of 14 Cases / 대한피부과학회지

Fourteen cases of porokeratosis were studied clinically. There were four clinical types consisting of classical plaque type(Mibelli), superficial disseminated eruptive form of Respighi (SDE type) and linear type, each in 3 cases, and disseminated superficial actinic porokeratosis (DSAP) in 5 cases. The Mibelli and linear type of the disease appeared in the early teens but other two showed late onset of second to third decade. All cases of SDE type occurred in males and most cases of Mibelli and DSAP type in females. Five cases of DSAP were apparently associated with sun-exposure. The disease process of case 6 (SDE type) seemed to be related to metastatic adenocarcinoma of the brain. The family occurrence of porokeratosis was found in 2 cases of SDE typc and 3 of five cases of DSAP type. They were transrnitted as an autosomal dominant trait. The linear type oppears to be distinct variety from the early appearance of lesions in linear fashion over the lower extremity. The histological features were essentially the same including the typical cornoid lamella, but the features in types other than classical plaque were often minimal, No effective mode of therapy has been found.

Porphytia Cutanea Tarda: Report of a Case / 대한피부과학회지

A case of porphyria cutanca tarda is reported, in which the photosensitive cutaneous symptoms developed in association with impaired hepatic function possibly related to chronic consumption of alcohol. Wood's light examination of the urine revealed coral red fluorescence, and qualitative chemical urinalysis was strong positive for uroporphyrin and coproporphyrin. Biopsy of the skin showed subepidermal bulla with festooning arrangement of the naked papillae and basophilic degeneration of the dermis. The patient's favorable response to alkalinization therapy by sodium bicarbonate per os is encouraging, even for a short period's observation. Clinical, histopathological, and biochemical features of this disease are also discussed.

A Case of Dermatitis Herpetiformis Associated with Erythema Annulare Centrifugum / 대한피부과학회지

A case of dermatitis herpetiformis assaciated with erythema annulare centrifugum is reported. The patient was a 21 year-old male, who had papulovesieles, erythematous lesions, pigmentation, and erythema annulare centrifugum lesions on the trunk, shoulders, buttocks and extensor surfaces of the extremities, and severe itching sensation of 9 months' duration. The skin biopsy from the vesicular and erythema annulare centrifugum lesion showed subepidermal bulla and vesicles with neutrophil and eosinophil infiltrations.

Tuberous Sclerosis Report of Two Cases and Review of Literatures in Korea / 대한피부과학회지

Two cases of tuberous sclerosis are reported. Case 1: A 13 years-old girl had a triad of adenoma sebaceum, mental deficiency and epileptic seizures associated with ichthyosis vulgaris, but family history revealed no evidences of the disease except ichthyosis in her mother. Case 2 : A 21 year-old woman had a adenoma sebaceum, shagreen patch, periungual fibroma and fihromatous plaque, showing intracranial calcificatins on roentgenographic examination, but has not demonstrated any neurological manifestations as yet. Family history also revealed adenoma sebaceum in her father and two younger sisters. We feel this case is a "forme fruste" of tuberous sclerosis.

Ritter's Disease: Report of Three Cases / 대한피부과학회지

We have presented three cases of Ritter's disease appearing on 11, 14, and 14 days old boys respectively. Staphyloccccus albus was cultured from bullar contents of case 1 and 2, and coaguIase positive staphylococcus from case 3. These patients were successfully treated with systemic and topical antimicrobials.