Nesidioblastosis, also known as persistent hyperinsulinemic hypoglycemia of infancy(PHHI) or familial hyperinsulinsm, is the most common cause of recurrent severe hypoglycemia in infancy. It is an autosomal recessive disorder characterized by irregular insulin secretion leading to inappropriately raised plasma insulin concentration compared to blood glucose levels. Recently, mutations in the sulfonylurea receptor(SUR) have been described in association with PHHI. The mainstay of medical treatment is glucose infusion and diazoxide or long acting somatostatin. If medical treatment fails in preventing hypoglycemia, near total pancreatectomy is recommended. We report one case of nesidioblastosis cured by near total pancreatectomy with brief review of literatures.
Blood Glucose ; Congenital Hyperinsulinism* ; Diazoxide ; Glucose ; Humans ; Hypoglycemia ; Infant, Newborn* ; Insulin ; Nesidioblastosis* ; Pancreatectomy ; Plasma ; Somatostatin

5 alpha-reductase deficiency resulting in male pseudohermaphroditism is a rare disease characterized by clitoral-like phallus, bifid scrotum, urogenital sinus, testis cited in labioscrotal folds. Evaluation of plasma T/DHT ratios in infancy, particularly after hCG stimulation of the testes and elevated urinary tetrahydrocortisol (THF) to 5 alpha-tetrahydrocortisol(5 alpha-THF) ratios provide a valuable dianostic test for 5 alpha-reductase deficiency. We report one case of 5 alpha-reductase deficiency who were presented with ambiguous genitalia and elevated T/DHT ratio before and after hCG stimulation.
46, XY Disorders of Sex Development ; Cholestenone 5 alpha-Reductase* ; Disorders of Sex Development ; Plasma ; Rare Diseases ; Scrotum ; Testis ; Tetrahydrocortisol

Twenty-nail dystrophy is a rare entity in which all 20 nail are uniformly and simultaneously affected with excess longitudinal ridging and loss of luster. This condition is thought to be idiopathic but, many cases are associated with alopecia areata, lichen planus, psoriasis, and ichthyosis. In such cases, it has been hypothesized that immunplogical disorders could play a role in pathogenesis. A 29-year-old male patient presented with dystrophic nail chatge on his all finger and toe nails for 3 years. On phisical examination, all nails were uniformly affected with longitudinal ridging and loss of luster and all body hairs were lost. Thyroid scan showed a finding of chronic thyroiditis. Herein we report a case of twenty-nail dystrophy associated with chronic thyroiditis and alopecia areata.
Adult ; Alopecia Areata ; Fingers ; Hair ; Humans ; Ichthyosis ; Lichen Planus ; Male ; Psoriasis ; Thyroid Gland* ; Thyroiditis* ; Toes

A case of acne conglobata associated with hidradenitis suppurativa, dissecting cellulitis and disseminated superficial actinic porokeratosis in 44 year-old man is presented with brief review of literature. The patient had acne vulgaris for a few years during his highschool days, about 5 years prior to the development of such a typical lesions of acne conglobata as multiple comedones, papules, pustules, abscesses, cysts and draining sinnses and subsquent hypertrophic scars and keloids. We are ps,rticularly interested in association of hidradenitis suppurativa and dissecting cellulitis with ance conglobata that comprise follicular occlusion triad in tbis teint. The pstient was treztei with systemic antibiotics and corticosteroids injections and surgical interventions with considerable improvement.
Abscess ; Acne Vulgaris* ; Adrenal Cortex Hormones ; Adult ; Anti-Bacterial Agents ; Cellulitis ; Cicatrix, Hypertrophic ; Hidradenitis Suppurativa ; Humans ; Keloid ; Porokeratosis

A 50-year-old female, who had been suffered from diabetes rielitus, showed typical cutaneous findinhs of dermatomyositis with only transient and mild proximal muscle weakness at sometime during the course of disease. She had Gottrons papules, Gottrons sign, heliotrope rash, macular violsceous erythema involving neck, anterior chest, back, extensor site of forearms and arms and dorsa of hands. However, he serum CPK, LDH and sGOP levels were within normal limits. Her fasting and 2 hours potprandial blood glucose levels were elevated. There were no histological abnormality in muscle biopsy. Histopathologic findigs of the skin lesion on her back were that of poikiloderma atrophicans vasculare. We diagnosed our patient as Rebeccas type 2 amyopathic dematomyositis which is chracterized by skin disease with subjective myalgias and musle weakness, but without laboratory evidence of muscle disease.
Arm ; Biopsy ; Blood Glucose ; Dermatomyositis* ; Erythema ; Exanthema ; Fasting ; Female ; Forearm ; Hand ; Humans ; Middle Aged ; Muscle Weakness ; Myalgia ; Neck ; Skin ; Skin Diseases ; Thorax

A case of circular eczematous dermatitis around seborrheic keratosis was presented. A 54-year-old man presented with two weeks history of a round pruritic eruption around a preexisting lesion of seborrheic keratosis. Microscopic findings showed central tumor nest composed mainly of basaloid cells with occasional spongtosis and exocytosis. A diffuse, dense, mononuclear cell infiltrate with increased contents of melanophages was seen beneath the tumor. The surrounding lesion of halo dermatitis showed mild dilatation of the capillaries and perivascular infiltration of mononuclear cells in the upper and mid-dermis. The lesion of halo dermatitis disappeared gradually over a three week period following excision of the central lesion. This peculiar phenomenon seemed to be different from that of Suttons halo nevus, and might be a manifestation of an eczematous condition which might have a dermatitis provoking factor.
Capillaries ; Dermatitis* ; Dilatation ; Eczema ; Exocytosis ; Humans ; Keratosis, Seborrheic* ; Middle Aged ; Nevus, Halo

Erythema multiforme can be induced by various causes, including infections like virus or fungus, drugs like sulfonamides and penicillins, internal malignences, connective tissue diseases, pregnancy, menstruation, and exoessive sun exposure ramoxone, one of the paraquat dichloride compounds, is the most commonly used weed-kiIler in Korea. It is sometimes used in suicides due to its fatal toxicity, and even weak transutaneous absorption can lead to death. Herein we report a case of erythema multiform in a 46-year-old man induced by accidental contact with the Gramoxone
Absorption ; Connective Tissue Diseases ; Erythema Multiforme* ; Erythema* ; Female ; Fungi ; Humans ; Korea ; Menstruation ; Middle Aged ; Paraquat ; Penicillins ; Pregnancy ; Solar System ; Suicide ; Sulfonamides

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

A 34-year-old man visited our hospital with alopecia areata on the occipital scalp, which began to develop two months prior to his visit. He was sensitized with 0.2% diphenylcyclopropenone(DPCP) in acetone that was applied to the inner side of his right arm. Two weeks after sensitization, we applied DPCP on his bald lesion once weekly for skin challenge. Following the third application of DPCP, polycyclic erythematous target-like lesions developed around the sensitized area. A clinical diagnosis of erythema multiforme was made. Histologically, the target-like lesion showed few eosinophilic dyskeratosis, exocytosis, and hydropic de-generation of basal layer in the epidermis, and mononuclear infiltration around superficial blood vessels in the dermis. We report herein a rare case of erythema multiforme following topical application of DPCP in the treatment of alopecia areata. This complication must be noted because of the wide-spread and frequent use of DPCP in dermatotherapeutic fields.
Acetone ; Adult ; Alopecia Areata ; Arm ; Blood Vessels ; Dermis ; Diagnosis ; Eosinophils ; Epidermis ; Erythema Multiforme* ; Erythema* ; Exocytosis ; Humans ; Scalp ; Skin