Incontinentia pigmenti is an uncommon neurocutaneous genodermatosis characterized by three stages; vesicular lesions, verrucous lesions and hyperpigmentation. A two-week-old female infant showed grouped erythema-based vesiculopustules on the whole body since birth. One month later, vesiculopustular lesions began to disappear gradually. At this time, she developed linear verrucous plaques on the dorsum of the feet and hands and reticulated hyperpigmented patches on the lower extremities. At 2 months of age, vesicular lesions completely disappeared and the pigmented patches spread to the abdomen which became darker with time. The verrucous lesions diminished at 3 months of age. There were no neurological or ocular defects. We describe a case of incontinentia pigmenti with typical clinical and pathological features at each stage.
Abdomen ; Female ; Foot ; Hand ; Humans ; Hyperpigmentation ; Incontinentia Pigmenti* ; Infant ; Lower Extremity ; Parturition

We rep a case of vulvar syringoma in a 32-year-old woman who had pruritus vulvae for 2 years. An examination of both the labia major revealed slightly thickened hyperpigmented or hypopigmented patches without yellow papules. A skin punch biopsy revealed characteristic histological findings of syringoma. Syringoma of the vulva is a rare disorder and is usually asymptomatic. Five cases of vulvar syringoma preriously reported in the Korean literature all had asymptomatic yellow or brown colored papules, but this case presented here was clinically considered as pruritus vulvae.
Adult ; Biopsy ; Female ; Humans ; Pruritus Vulvae* ; Pruritus* ; Skin ; Syringoma* ; Vulva

BACKGROUND: There is a long-standing controversy whether melanocytes in vitiligo of more than 1 year duration are actually lost or still present. Resolving this matter is essential in understanding the underlying pathology and for the development of the treatment. On previous immunohistochemical and ultrastructural studies of vitiligo lesions, damage of melanocyte and keratinocyte in early lesions were reported and complete absence of melanocyte in long standing lesions were known. OBJECTIVE: This study aimed to determine the existence of the differences in pathologic changes in melanocytes according to the duration of the lesion. METHODS: We investigated the vitiliginous skin samples from 31 patients with early(less than 1 year duration) vitiligo and 30 patients with long standing(l to 5 years duration) vitiligo under the electron microscopy. RESULTS: Multiple degenerative changes in melanocytes were observed in the early and long standing lesions. In long standing lesions, degeneration of melanocytes including pyknotic, in-dented nuclei, vacuolated cytoplasms and blunted dendrites were more pronounced than early lesions. Even in long standing lesions, definite or presumptive melanocytes were observed in 16(53.3%) of 30 cases. CONCLUSION: Our results suggest that the melanocytes of vitiligo lesions were damaged and that the percentage of degenerative changes increase in accordance with the duration of the lesion. However, in long standing lesions as well as in early lesions, some residual melanocytes can be observed ultrastructurally.
Cytoplasm ; Dendrites ; Humans ; Keratinocytes ; Melanocytes ; Microscopy, Electron ; Pathology ; Skin ; Vitiligo*

Dermatofibroma with granular cells is composed mainly of spindle shaped cells mixed with in-conspicuous foamy cells that contain granular cytoplasm. We present a case of dermatofibroma with granular cells in a 36-year-old male who visited our clinic to evaluate a slow-growing nodule on the medial side of the left lawer leg. He have had the nodule for five years and sometimes felt an itching sensation. Biopsy of the nodule identified a dermal nodular tumor showing cellular spindle cell proliferation admixed with peripheral foamy histiocytes. With the immunohistochemical staining, the granular cell was positive for alpha-1-antitrypsin and CD68, and negative for S-100. A few electron-dense granules were seen in the cytoplasm on the electronmicroscopic study. These results suggested that the granular cells originated from histiocytes.
Adult ; Biopsy ; Cell Proliferation ; Cytoplasm ; Histiocytes ; Histiocytoma, Benign Fibrous* ; Humans ; Leg ; Male ; Pruritus ; Sensation

We treated a case of extramammary Paget's disease that affected the scalp of a 45-year-old female. It is extremely rare that the disease arises in areas other than the anogenital region and the axillae. The lesion was a round erythematous oozing crusted hairless patch. Histopathologically, many pagetoid cells were found within the epidermis and dermis. The cytoplasms of these cells stained with alcian blue at pH 2.5, CEA, EMA and low-molecular-weight-cytokeratin. The patient underwent a wide local excision. We used a mapping technique to reveal the distribution of microscopically involved lesions.
Alcian Blue ; Axilla ; Cytoplasm ; Dermis ; Epidermis ; Female ; Humans ; Hydrogen-Ion Concentration ; Middle Aged ; Paget Disease, Extramammary* ; Scalp*

A 19 year old female patient presented with diffuse alopecia as her chief medical complaint. A clinical examination revealed hirsutism limited only to the on midline lower abdomen with elevated DHEA-S(dehydroepiandrosterone sulfate) and total testosterone levels. Polycystic ovarian disease (PCOD) was diagnosed during the treatment with dexamethasone and spironolactone, which was effective to improve her alopecia. We believe that, with increasing, concerns about hair conditions of teen-age girls there should be increasing chances for dermatologists to care for patients of PCOD first before other specialities in medicine.
Abdomen ; Alopecia* ; Dexamethasone ; Female* ; Hair ; Hirsutism ; Humans ; Ovarian Diseases* ; Spironolactone ; Testosterone ; Young Adult

Systemic sclerosis is an autoimmune rheumatic disease of unknown etiology, often manifested with excessive, fibrosis of the skin. The viscous properties of the skin are impaired, and the main histologic changes include a thicker dermis, absence of pilosebaceous units, and a decreased space between collagen bundles. We present a case of 20-year-old Korean woman with micrognathia and microchilia due to systemic sclerosis who came for improvement of her doll-like appearance. We saw an improvement in her micrognathia and microchilia by using Restylane(R) and Perlane(R).
Collagen ; Dermis ; Female ; Fibrosis ; Humans ; Rheumatic Diseases ; Scleroderma, Systemic* ; Skin ; Young Adult

Heterotopia refers to the finding of normal tissue in foreign sites, entirely separate from the main organ. Heterotopic gastric mucosa has been observed throughout the alimentary tract, everywhere from the oral cavity to the rectum. However, occurrences in the umbilicus are an extremely rare and peculiar phenomena. We report the case of heterotopic gastric mucosa in the umbilicus.
Gastric Mucosa ; Mouth ; Rectum ; Umbilicus

Dermatofibrosarcoma protuberance is a relatively rare malignant soft tissue tumor which is locally invasive and looks like a protruded hardened plaque composed of multiple nodules. It seldom occurs on the scalp and the occurrence rate is less than 5%. Nowadays there is a tendency to choose Mohs surgery as a primary treatment instead of a wide excision, because Mohs surgery can reduce cases of recurrence. We report a case of multiple dermatofibrosarcoma protuberans on the scalp of a 32-year-old man. We planned a delayed operation using tissue expanders. We inserted two skin expanders and successfully expanded scalp tissue more than 150% after 7 months. Mohs surgery was performed with a 4 cm distance from borderline of lesions and then the large scalp skin defect was covered by the expanded skin which had been prepared by tissue expanders. It has been 9 months since the operation was performed and there have been no signs of recurrence.
Adult ; Dermatofibrosarcoma* ; Humans ; Mohs Surgery* ; Recurrence ; Scalp* ; Skin ; Tissue Expansion Devices ; Tissue Expansion*

Leukocytoclastic vasculitis is a small vessel inflammatory disease, mediated mostly by deposition of immune complexes. Medications cause 10~24% of cases of leukocytoclastic vasculitic skin lesions, but warfarin has rarely been implicated. We report a case of warfarin-induced leukocytoclastic vasculitis in a 72-year-old woman which developed 45 days after warfarin medication. Palpable purpura, hemorrhagic vesicles, and ulcers developed on both lower legs. A skin biopsy showed characteristic features of leukocytoclastic vasculitis. Warfarin was replaced by clopidogrel, and the skin lesions gradually disappeared after 3 weeks.
Aged ; Antigen-Antibody Complex ; Biopsy ; Female ; Glycosaminoglycans ; Humans ; Leg ; Purpura ; Skin ; Ticlopidine ; Ulcer ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous ; Warfarin