A Case of SAPHO Syndrome / 대한피부과학회지

The term, SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is an acronym to designate a clinico-radiologic entity with combination of skin, bone, and joint manifestations. We, herein, present a case of SAPHO syndrome in a 27-year-old man with pruritic, localized, crops of deep-seated pustules on both palms and soles for 2 years, and multiple joints pain including anterior upper chest pain for 3 years. Histopathologically, the palm showed a large intraepidermal cavity filled with many neutrophils, and many typical spongiform pustules at the wall of it. Radiographically, the left sternoclavicular joint showed synovitis, osteitis and hyperostosis.

Bowen's Disease Treated by Rhombic Transposition Flap / 대한피부과학회지

Bowen's disease is a form of intraepidermal (in situ) squamous cell carcinoma, originally discovered in 1912. Bowen's disease is treated because of the risk of progression to invasive squamous cell carcinoma. The treatment options for Bowen's disease include curettage and cautery, excision, cryotherapy, laser ablation, photodynamic therapy and topical 5-fluorouracil. Surgical excision is generally regarded as the treatment of choice for most Bowen's disease lesions. Such a treatment requires lesions that are small, well-defined, and located in an area which permits such a procedure. However larger lesions of Bowen's disease are difficult to close primarily, and they have typically been treated with large skin grafts and required hospitalization of up to two or three weeks. We report two cases of Bowen's disease treated by rhombic transposition flap. In our cases, there were no significant complications and the results were cosmetically acceptable. The post-operative hospitalization time was reduced to one week. Skin flap is a simple and satisfactory alternative for the reconstruction of large-sized lesions of Bowen's disease.

A Case of Plaque-Type Blue Nevus / 대한피부과학회지

Plaque-type blue nevus is a rare type of blue nevus, which shows numerous macules and papules within a circumscribed area. Histopathologically, it is reported to be common or cellular. A 24-year-old female presented with a 10.5x8.6 cm blue-black plaque on the right shoulder, which had existed since birth, and was characterized by multiple confluent or grouped blue-black macules and papules with intervening areas of blue discoloration. Histopathologic examination showed irregular bundles of numerous, greatly elongated, grouped, heavily melanin-laden melanocytes with long, branching dendritic processes in the reticular dermis. Numerous melanophages were intermingled with melanocytes. We, herein, report a case of plaque-type blue nevus with histopathologic finding of common blue nevus.

A Case of Nasal Glioma / 대한피부과학회지

Nasal glioma is a rare, benign, congenital tumor, which is ectopic sequested brain tissue that has lost its intracranial connection. It is thought to be the result of an abnormality in embryonic development. A 2-month-old female patient presented with firm, noncompressible, pinkish, 0.6 cm-diameter, protruding mass on the glabella from birth. No intracranial connection was found by MRI. Histopathological finding showed an unencapsulated pale-staining fibrillary glial tissue, which contains astrocytes and some neurons. Immunohistochemically, the glial tissue and astrocytes were positive for GFAP(glial fibrillary acidic protein), and neurons were positive for NSE(neuron-specific enolase).

A Case of Bowen's Disease on the Vulva / 대한피부과학회지

Bowen's disease was originally described by Bowen in 1912 and is believed to be a squamous cell carcinoma in situ. It develops on any part of the skin surfaces or mucous membranes. But the lesions on the vulvar region are relatively rare. The gross appearance of vulvar lesion, which shows erosion, ulceration or pigmentation, is different from that of typical Bowen's disease. We, herein, report a case of Bowen's disease arising on a vulvar region with a review of the literature.

A Case of Bilateral Segmental Neurofibromatosis Associated with Partial Unilateral Lentiginosis and Nevus of Ota / 대한피부과학회지

The term, bilateral segmental neurofibromatosis had been used to refer to patients who had unilateral neurofibromas with contralateral pigmented lesions or bilateral neurofibromas. Partial unilateral lentiginosis is characterized by numerous lentigines localized to a body segment. The coexistence of bilateral neurofibromas and partial unilateral lentiginosis raises the possibility that partial unilateral lentiginosis could be a variant of segmental neurofibromatosis. Nevus of Ota arise from dermal melanocytes and it can be associated with neurofibromatosis. A 60-year-old man presented with bilateral segmental neurofibromatosis with partial unilateral lentiginosis on the right arm and right leg, nevus of Ota on right forehead, and one cafe-au-lait spot on the abdomen.

Clinical and Serological Study in Xanthoma with Hyperlipoproteinemia / 대한피부과학회지

BACKGROUND: Xanthoma is an interesting disease entity that often indicates underlying lipid abnormalities. but, clinical studies about hyperlipopropeinemia in xanthoma were rarely present. OBJECTIVE: The purpose of this study is to observe the clinical and histopathologic features in xanthoma and to evaluate relationship between hyperlipoproteinemia and underlying xanthoma. METHOD: 57 cases of xanthoma were analyzed. In 35 cases, blood lipids including triglyceride, cholesterol and high density lipoprotein were analyzed. And clinical characterics of xanthoma and hyperlipoproteinemia were evaluated in 10 cases of our hyperlipoproteinemic patients confirmed by electrophoresis. RESULTS: The ratio between male and female was 1: 1.45 in xanthoma excluding xanthelasma and was 1: 1.86 in xanthelasma only. The mean age of each group was 28 years and 52 years. The incidence of each type, in descending order, was xanthelasma(35%), tuberous xanthoma(20%), eruptive xanthoma(14%), plane xanthoma(7%), tendinous xanthoma(2%), and unclassified(17.5%). The associated diseases, in descending order, were diabetes mellitus(9/15), fatty liver(2/15) etc. Among these, diabetes mellitus was the most common associated disease in hyperlipoproteinemia group(6/10). Histopathological features in 26 cases revealed lipid-laden foamy cells in all cases, and foreign body giant cells, fibrosis, cholesterol clefts in some cases. Among 35 cases, the rate of abnormal lipid profiles, in descending order, was xanthelasma(63.3%), eruptive xanthoma(62.5%), and tuberous xanthoma(38%). The frequent types of hyperlipoproteinemia in our cases, in descending order, are V(5/10), type IIa(3/10), IV(2/10). In previously reported hyperlipoproteinemia with xanthoma patients, the frequent types of xanthoma, are tuberous xanthoma(12/30), plane xanthoma(8/30), eruptive xanthoma(6/30), mixed type (2/30), and xanthelasma(1/30), and the frequent types of hyperlipoproteinemia in descending order are IIa (9/30), IV(8/30), V(4/30), IIb(3/30), III(3/30), unclassified(2/30), and I(1/30). CONCLUSION: The most common type of hyperlipoproteinemia in our cases and literatures is type IIa. but, recently type IV and type V are increasing and are associated with increased incidence of diabetes. The incidence of abnormal lipid profiles in xanthelasma is 63.3% and routine lipid profile study will be needed in the evaluation of xanthoma including xanthelasma.

Two Cases of Lichen Amyloidosis Associated With Atopic Dermatitis / 대한피부과학회지

Lichen amyloidosis is a chronic intensely pruritic skin disorder characterized histologi-cally by the deposition of amyloid in the papillary dermis. Lichen amyloidosis has been reported in association with several pruritic skin disorders including atopic dermatitis, lichen planus, and mycosis fungoides. It is hypothesized that the scratching in pruritic skin diseases is the most important step in forming amyloid deposit. We report two cases of lichen amyloidosis occuring in two patients with atopic dermatitis.

Two Cases of Multiple Lichen Striatus / 대한피부과학회지

Lichen striatus is a self-limited, linear dermatosis of unknown etiology seen mainly in children. Clinically, it is usually seen as unilateral, flesh to red brown, flat-topped papules that follow Blaschko's lines. Many authors have theorized that viral or environmental agents acting on genetically predisposed individual might be involved in its etiology. We present two cases of multiple lichen striatus. The first case is 9 year-old boy, who has a past history of measles. The second case is 6 year-old girl, who was diagnosed of type I neurofibromatosis.

A Case of Granular Cell Basal Cell Carcinoma / 대한피부과학회지

Granular cell basal cell carcinoma is a rare histopathologic variant of basal cell carcinoma in which some or all of the neoplastic cells show cytologic features of granular cells. These distinctive cells contain abundant eosinophilic cytoplasm housing numerous fine and coarse refractile granules. The 62-year-old man presented with a 5-year history of asymptomatic, solitary 1 cm-diameter, hemispherical, brown to black, smooth-surfaced, hard nodule on the left chest. The histopathological features showed a tumor with the general configuration of a solid basal cell carcinoma, in which there were masses of granular cells with eosinophilic and granular cytoplasm. The tumor was positive for cytokeratin and negative for lysozyme and PAS. Surgical excision was performed.

A Case of Segmental Neurofibromatosis / 대한피부과학회지

A 30-year-old woman had localized several cutaneous neurofibromas on the scalp in a dermatomal distribution(C2). Family history was negative, and evaluation for other signs such as cafe-au-lait spots, axillary frecklings, or Lisch nodules were not revealed. Biopsy specimen showed a well-circumscribed encapsulated tumor which consists of spindle cells with elongated, wavy nuclei and thin wavy collagenous strands loosely spaced in a clear matrix as seen in the neurofibroma. The diagnosis of bilateral segmental neurofibromatosis was made on the basis of the clinical features including the distribution of the neurofibromas and the absence of the family history and the histology consistent with a neurofibroma.

A Case of Linear Lichen Planus / 대한피부과학회지

Linear lichen planus, a rare variant of lichen planus, accounts for 0.24-0.62% of all patients with lichen planus. Clinically, it consists of itchy linear-distributed violaceous papules, usually last for more than 1 year and the mucous membranes may also be affected. This disorder shows the same histologic findings of lichen planus, which is characterized by a band-like dermal inflammatory infiltration with hyperkeratosis, focal hypergranulosis, acanthosis, exocytosis and the presence of Civatte body. We report a 46-year-old woman showing typical clinical and histological features of linear lichen planus without pruritus.

A Case of Generalized Plane Xanthoma Associated with Multiple Myeloma and Hyperlipoproteinemia / 대한피부과학회지

Generalized plane xanthoma is a group of plane xanthomas that appear as yellow to yellow-brown flat patches or slightly elevated plaques with a wide-spread distribution. The disease is frequently associated with multiple myeloma or other reticulo-endothelial malignancies and monoclonal gammopathy with unknown significance, and when accompanied by these hematologic malignancies, normolipoproteinemia is invariably associated. We present a case of generalized plane xanthoma associated with multiple myeloma and type III hyperlipoproteinemia in a 48 year-old woman, and this is the first report in Korean literature.