Expression of Pro-inflammatory Protein S100A12 (EN-RAGE) in Behcet's Disease and Its Association with Disease Activity: A Pilot Study

BACKGROUND: S100A12 is a member of the S100 family of calcium-binding proteins and is secreted either in inflamed tissues or in the bloodstream by activated neutrophils. Expression of S100A12 has been reported in various diseases, especially non-infectious inflammatory diseases, such as Kawasaki disease, giant cell arteritis and inflammatory bowel disease. OBJECTIVE: This study was conducted to determine both the tissue expression and the serum levels of S100A12 in Behcet's disease (BD) patients and the correlation of the S100A12 serum level with disease activity of BD. METHODS: We included in this study ten BD patients who fulfilled the criteria for diagnosis, according to the International Study Group for BD. The activity of BD was calculated using the BD Current Activity Form. The serum concentrations of both S100A12 and interleukin-8 were measured by the enzyme-linked immunosorbent assay, before and after treatment. Immunohistochemical studies were also performed to detect S100A12 expression in the skin. RESULTS: The serum S100A12 level was significantly increased in the active BD period (p<0.001), in the inactive BD period (p=0.041) and in patients with active Kawasaki disease (p=0.028), compared with the serum level in the healthy controls. The serum S100A12 level decreased significantly from baseline, compared to post-treatment (p=0.017). The activity score of BD was significantly correlated with the serum level of S100A12 (Spearman's coefficient=0.464, p=0.039). Immunohistochemical studies showed that S100A12 was strongly expressed in the erythema nodosum-like skin lesions of patients. CONCLUSION: S100A12 contributes to the pathogenesis of BD related to neutrophil hyperactivity and reflects the disease activity in BD patients.

Evaluation of the Articular Involvement of Behcet's Disease Via Bone Scintigraphy / 대한피부과학회지

BACKGROUND: Behcet's disease (BD) is a chronic relapsing inflammatory disease that involves various organ systems. Articular involvement was reported to be present in approximately 50% of Korean BD patients. The joint symptoms of BD patients have usually been described as intermittent, self-limiting and non-erosive, and they are mostly monoarticular and oligoarticular arthritis. OBJECTIVE: The purpose of our investigations was to evaluate the usefulness of bone scintigraphy for detecting the articular involvement of BD. METHODS: We reviewed the medical records, laboratory findings and bone scintigraphy findings of 89 patients who were diagnosed with BD from January 2005 to June 2007. RESULTS: Of the 89 BD patients, 14 patients were male and 75 patients were female with a mean age of 43.92+/-8.49 yr. The most frequently involved site on bone scintigraphy was the wrist (44.9%) with the decreasing order of frequency as follows: the feet (39.3%), the hands (25.8%), the knee (24.7%), the sacroiliac joint (22.4%), the shoulder (18%), the ankle (16.9%), the hip (12.6%), the spine (10%) and the elbow (3%). The pattern of involvement, which was defined as the number of joints showing hot uptake on the bone scintigraphy at one episode of arthropathy, was monoarticular in 5.6%, oligoarticular in 44.9%, polyarticular in 38.2% and there was no uptake in 11.2%. Among 130 joints, 63.1% of the joints showed close correlation between the clinical symptoms and the bone scintigraphy uptake. CONCLUSION: We suggest that bone scintigraphy can be a useful tool to determine the presence and site of articular involvement. However, more studies are needed to exclude non-specific bone scintigraphy uptake and to determine the correlation between clinical symptoms and the bone scintigraphy findings.

A Case of Osteoma Cutis, a Diagnostic Clue for Albright's Hereditary Osteodystrophy / 대한피부과학회지

Albright's hereditary osteodystrophy (AHO) has a broad spectrum of physical findings, including short stature, flattened nasal bridge, round facies, obesity, skeletal anomalies of the hands with brachydactyly, osteoma cutis, mental retardation, dental defects, cataracts, and calcification in the basal ganglia. It also includes endocrinologic abnormalities such as pseudohypoparathyroidism and pseudopseudohypoparathyroidism. Primary osteoma cutis, which can precede other physical findings of AHO, might be a diagnostic clue for AHO. Therefore, close follow-up and regular laboratory tests should be done to detect the early development of AHO in young children with primary osteoma cutis. Early diagnosis and treatment of AHO can prevent irreversible mental retardation and developmental delay.

A Case of Precursor B-cell Lymphoblastic Lymphoma Misdiagnosed as Rosacea / 대한피부과학회지

Precursor B-cell lymphoblastic lymphoma is a rare type of non-Hodgkin's lymphoma seen exclusively in children and young adults. The neoplasm is rare in old age. We report a case of a 58-year-old male, who presented with variable-sized, erythematous to brownish papules and plaques on the scalp and face. There were no other symptoms. Pathological examination showed non-specific, sparse superficial and deep perivascular lymphocyte infiltration. We diagnosed the condition as rosacea and prescribed 100 mg of minocycline per day for 10 days. When he was seen 10 days later, his skin lesions were aggravated and re-biopsy was completed. Histopathology revealed diffuse infiltrates of small to medium-sized lymphoid cells with blastic nuclear chromatin and a high mitotic rate. Immunohistochemical study showed that infiltrated lymphoid cells were precursor B-cell type. Physical examination and staging work-up revealed extensive involvement of lymphoma in bilateral kidney, heart, pancreas, axial and proximal appendicular bones, scalp and cervical lymph node. The patient is being treated with combination chemotherapy.

Two Cases of Primary Cutaneous Marginal Zone B-cell Lymphoma / 대한피부과학회지

Primary cutaneous marginal zone B-cell lymphoma, known as the cutaneous counterpart of mucosa-associated lymphatic tissue lymphoma (MALT lymphoma), is the most frequent of the primary cutaneous B-cell lymphoma. It is one of the low-grade lymphomas of B-cell type, which has an excellent prognosis with indolent behavior, despite frequent cutaneous recurrences. Histologically, cutaneous marginal zone B-cell lymphoma is composed of polymorphous infiltrate that includes centrocyte-like, centroblast-like, monocytoid, and lymphoplasmacytoid lymphocytes. We present two cases of primary cutaneous marginal zone B-cell lymphoma. A 48-year-old woman visited the dermatologic clinic with 1.5x1.5 cm sized, skin colored, palpable nodule on the left temporal area and a 26-year-old man visited the dermatologic clinic with 1x1 cm sized erythematous nodule on the nose. Both of them showed compatible histologic findings of marginal zone B-cell lymphoma and had no past medical history and no evidence of metastasis on PET-CT and bone marrow biopsy. The woman was treated with radiation treatment after excision and the man was treated with radiation treatment alone. Follow-up indicates that 1 year after treatment there is no evidence of recurrence.

A Case of Papular Xanthoma Developed in an Adult / 대한피부과학회지

Papular xanthoma is a normolipemic xanthomatosis, characterized by nonconfluent papular to papulonodular eruptions on the face, trunk, extremities and occasionally mucous membranes. Histologically, there was an infiltration of foamy histiocytes and Touton type giant cells in the dermis without inflammatory cells or a pure histiocytic component. But, occasional lymphocytes were interspersed between the foamy macrophage. No systemic involvement could be found and the blood lipid profiles were normal. We report a case of papular xanthoma in a 30-year-old man with typical clinical, histopathologic findings.

A Case of Disseminated Epidermolytic Acanthoma / 대한피부과학회지

Epidermolytic acanthoma is an uncommon benign tumor characterized histologically by epidermolytic hyperkeratosis. Clinically, the tumor resembles a verruca or seborrheic keratosis and may occur in either isolated or disseminated forms. Herein, we present a case of a 46-year-old male who developed multiple seborrheic keratosis or verruca like papules on his trunk which showed epidermolytic hyperkeratosis upon microscopic examination. Following clinical and histological findings, the patient was diagnosed as having disseminated epidermolytic acanthoma.

A Case of Idiopathic Neutrophilic Eccrine Hidradenitis / 대한피부과학회지

Neutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis which clinically presents with various cutaneous manifestations with or without tenderness and pruritus. NEH has a characteristic histologic pattern, with necrosis of the eccrine glands and local neutrophilic infiltrations. It occurs mostly in patients receiving chemotherapy for acute myeloblastic leukemia or, less frequently, other malignancies. More recently, NEH has been observed in other conditions, such as infections, drugs or even in generally healthy individuals. We report a case of a 29- year-old female with no remarkable medical history who has suffered from a solitary skin-colored indurated macule with tenderness on the left sole for 2 weeks. The skin biopsy showed typical features of neutrophilic eccrine hidradenitis. The patient was successfully treated with oral prednisolone for 2 weeks and an intralesional injection of triamcinolone.