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.
Adolescent ; Blindness ; Dupuytren Contracture ; Extremities ; Fathers ; Fingers ; Hand ; Humans ; Male ; Muscular Atrophy ; Neck ; Neurilemmoma* ; Neurofibroma ; Neurofibromatoses* ; Nevus ; Nevus, Pigmented

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.
Bony Callus ; Buttocks ; Child ; Elbow ; Fingers ; Hair ; Hoarseness ; Humans ; Hyperhidrosis ; Knee ; Leg ; Leukoplakia ; Male ; Mothers ; Nails, Malformed* ; Pachyonychia Congenita* ; Scalp ; Thigh ; Toes ; Transcutaneous Electric Nerve Stimulation

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.
Anthozoa ; Basophils ; Biopsy ; Dermis ; Fluorescence ; Porphyrias ; Skin ; Sodium Bicarbonate ; Urinalysis

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.
Adolescent ; Epilepsy ; Fathers ; Female ; Fibroma ; Humans ; Ichthyosis ; Ichthyosis Vulgaris ; Intellectual Disability ; Korea* ; Mothers ; Neurologic Manifestations ; Siblings ; Tuberous Sclerosis* ; Young Adult

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.
Biopsy ; Buttocks ; Dermatitis Herpetiformis* ; Dermatitis* ; Eosinophils ; Erythema* ; Extremities ; Humans ; Male ; Neutrophils ; Pigmentation ; Pruritus ; Sensation ; Shoulder ; Skin ; Young Adult

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.
Humans ; Staphylococcal Scalded Skin Syndrome* ; Staphylococcus

The statistical study of new outpatients of skin problems seen in dermatologic department of Seoul Red Cross Hospital from l976 to 1980 was done and the 10 most common dermatoses were analysed in detail, The results were summarized as follows: 1) Of 19,679 outpatients, males were 10,530(53.5%) and females were 9,149(46.5%). The sex ratio of male to female was 1. 15: l. 2) The most common dermatoses were atopic dermatitis/eczema(16. 9%), fungal infection(16. 2%), contact dermatitis(15. 3%), scabies(10. 7%), urticaria(10. 6%), acne(9. 5%), pyoderma(8. 3%), insect bite(5. 6%), syphilis(4. 1%) and psoriasia (2. 8%). 3) Of 10 most common dermatoses, contact dermatitis, acne, scabies and urticaria were revealed to have a tendency to increase year by year, syphilis and psoriasis were constant pattern and other common dermatoses showed irregular pattern in their incidence. 4) The sex ratio of male to female of l0 most common dermatoses was l. 23: l. Fungal infection, scabies, pyoderma, insect bite, syphilis and psoriasis wereoutnumbered in male and atopic dermatitis/eczema, contact dermatitis, urticaria and acne were outnumbered in female. 5) Atopic dermatitis/eczema & scabies were prevalent in winter, acne, fungal infection, urticaria and pyoderma in summer, and contact dermatitis in spring and summer. 6) The most frequent age groups of atopic dermatitis/eczema were 1 to 10 years, acne and fungal infection, 11 to 30 years, and contact dermatitis and fungal infection, 31 to 50 years.
Acne Vulgaris ; Dermatitis, Contact ; Female ; Humans ; Incidence ; Insect Bites and Stings ; Insects ; Male ; Outpatients* ; Psoriasis ; Pyoderma ; Red Cross ; Scabies ; Seoul ; Sex Ratio ; Skin ; Skin Diseases ; Statistics as Topic* ; Syphilis ; Urticaria

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.
Adrenal Cortex Hormones ; Behcet Syndrome* ; Busan ; Dermatology ; Erythema Multiforme ; Erythema Nodosum ; Female ; Humans ; Incidence ; Iridocyclitis ; Male ; Pyoderma ; Stomatitis, Aphthous ; Ulcer

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.
Adenocarcinoma ; Adolescent ; Brain ; Female ; Humans ; Lower Extremity ; Male ; Porokeratosis*

Tow patients with severe glomerulonephitis and their serum creatinine clearance of 6.0ml/min/1.73m2 in case 2 were treated with high dosage intravenous methylprednisolone, 30mg/kg given over one hour period on each of six slternate days. Follwing the ?ulse therapy, both patients with rapidly deteriorathin renal funtion began to inprove and theit serum creatinine clearance levels showed over douvble by sixdays In both patients treated early in the curse of their disease, creatinine clearance increased to 80ml/min/1.73m2 in case 2 after one year follow-up and in recent study, clinical and laboratory findings are within normal limit.
Creatinine ; Follow-Up Studies ; Humans ; Methylprednisolone*