A 70-year-old woman with an 8-year history of systemic sarcoidosis developed round, red-brown eruptions, with central atrophic lesions on her lower legs. The features of the biopsy specimen resembled those of necrobiosis lipoidica (NL), but although necrobiosis was present there were well-formed non-necrotizing granulomas in the dermis. The histological diagnosis was cutaneous sarcoidosis. Systemic sarcoidosis presenting with NL has rarely been reported. The histological features of cutaneous sarcoidosis sometimes mimic those of other granulomatous diseases, including NL and granuloma annulare, which are difficult to distinguish. We discuss the novel association between sarcoidosis and other granulomatous diseases.
Aged ; Biopsy ; Dermis ; Female ; Granuloma ; Granuloma Annulare ; Humans ; Hydrazines ; Leg ; Necrobiosis Lipoidica ; Necrobiotic Disorders ; Sarcoidosis

Necrobiosis lipoidica (NL) is a chronic granulomatous skin disease typified by indurated plaques on the shin. Although this condition is strongly associated with diabetes mellitus, its etiology and pathogenesis remains unknown. Localization of NL to the lower extremities suggests that local injury may be a contributing factor to the disease. A healthy 38-year-old man presented with localized erythematous to yellowish plaques on his right thigh that developed over several years. The lesion developed in the scar where suture had been done for laceration repair 25 years ago. A biopsy specimen showed degenerated collagen with surrounding palisading granulomas. Numerous lymphocytes and multinucleated giant cells were infiltrated throughout the reticular dermis. Plasma cells aggregation was found at the dermal subcutaneous junction.
Biopsy ; Cicatrix ; Collagen ; Dermis ; Diabetes Mellitus ; Giant Cells ; Granuloma ; Humans ; Lacerations ; Lower Extremity ; Lymphocytes ; Necrobiosis Lipoidica ; Necrobiotic Disorders ; Plasma Cells ; Skin Diseases ; Sutures ; Thigh

We report an unusual case of necrobiosis lipoidica with prominent cholesterol clefts in a 36-year-old man who had developed multiple, 0.5~5cm in diameter, round to oval-shaped erythematous plaques on both shins for over 3 years. Laboratory findings showed the following; cholesterol, 250mg/dl; triglyceride, 208mg/dl; blood sugar level fasting/2-h postprandial, 101/92mg/dl; and other findings were within normal limits or negative. There was no paraproteinemia. The biopsy specimen showed various lymphohistiocytic granuloma with many giants cell in the mid and deep dermis, and septal fibrosis in the subcutaneous tissue. There were also extensive areas of necrobiosis with prominent cholesterol clefts mimicking necrobiotic xanthogranuloma.
Adult ; Biopsy ; Blood Glucose ; Cholesterol* ; Dermis ; Fibrosis ; Granuloma ; Humans ; Necrobiosis Lipoidica* ; Necrobiotic Disorders* ; Necrobiotic Xanthogranuloma ; Paraproteinemias ; Subcutaneous Tissue ; Triglycerides

No abstract available.
Cyclosporine ; Necrobiosis Lipoidica ; Necrobiotic Disorders

Ehlers-Danlos Syndrome(EDS) is an inherited disorder of the connective tissue disease that is usually transmitted as autosomal dominant trait. The defect in the biogenesis of collagen results in varying degree of skin hyperextensibility, joint hypermobility, skin fragility and bruising. There have been several reports of cardiac or great vessel abnormalities in patients with the EDS. A 45-year-old Woman was admitted to Maryknoll hospital because of orthopnea, palpitation and epigastric discomfort. Physical examination reveals multiple variable sized bruises and increased hyperelasticity on skin, and hypermobile joint in knee and proximal interphalanges of both hands. Mitral valvular prolapse and atrial septal defect were detected by echocardiography. We report a case of EDS accompanied by mitral valvular prolapse and ostium secundum atral septal defect with brief review of literature.
Organelle Biogenesis ; Collagen ; Connective Tissue Diseases ; Contusions ; Echocardiography ; Ehlers-Danlos Syndrome* ; Female ; Hand ; Heart Septal Defects, Atrial* ; Humans ; Joint Instability ; Joints ; Knee ; Middle Aged ; Physical Examination ; Prolapse* ; Skin

Ehlers-Danlos syndrome (EDS) is an inherited disorder of collagen biosynthesis and structure, characterized by skin hyperextensibility, joint hypermobility, aberrant scars, and tissue friability. Besides the skin, skeleton (joint) and vessels, other organs such as the eyes and the intestine can be affected in this syndrome. Accordingly, interdisciplinary cooperation is necessary for a successful treatment. Three basic surgical problems are arising due to an EDS: decreased the strength of the tissue causes making the wound dehiscence, increased bleeding tendency due to the blood vessel fragility, and delayed wound healing period. Surgery patients with an EDS require an experienced surgeon in treating EDS patients; the treatment process requires careful tissue handling and a long postoperative care. A surgeon should also recognize whether the patient shows a resistance to local anesthetics and a high risk of hematoma formation. This report describes a patient with a wide open wound on the foot dorsum and delayed wound healing after the primary approximation of the wound margins.
Anesthetics, Local ; Blood Vessels ; Cicatrix ; Collagen ; Connective Tissue Diseases ; Ehlers-Danlos Syndrome ; Foot ; Hematoma ; Hemorrhage ; Humans ; Intestines ; Joint Instability ; Postoperative Care ; Skeleton ; Skin ; Wound Healing ; Wounds and Injuries

Granuloma, annulare is a chronic dermatosis characterized by skin colored, firm intradermal papules or subutaneous nodules, which may be discrete or arranged in an annular configuration. Histologically the dermis shows well define8 areas of necrobiosis of collagen tissue surrounded. by palisading histiocytes and lymphocytes. There may be lobules of epithelioid cells ani multinucleated giant ce.1s surrounding the necrobiotic areas. Many factors have been implicated in the genesis of this reaction. Recent studies have indicated that delayed hypersensitivity reactions rnay be involved in the pathogenesis of granuloma annulare. We present a typical granulorna annulare on a 16 year-old boy who had flesh colored, hyperkeratotic small nodules arranged in ring fashion on the dorsum of hands and right ear auricle.
Adolescent ; Collagen ; Dermis ; Ear Auricle ; Epithelioid Cells ; Granuloma Annulare* ; Granuloma* ; Hand ; Histiocytes ; Humans ; Hypersensitivity, Delayed ; Lymphocytes ; Male ; Necrobiotic Disorders ; Skin ; Skin Diseases

Osteogenesis imperfecta is one of the groups of hereditary disorders of connective tissue which includes the Ehlers-Danlos syndrome, the Marfan syndrome, pseudoxanthoma elasticum, and Hurler syndrome. While cardiovascular involvement is associated with each of these disorders, it is least common in osteogenesis imperfecta and is overshadowed by the bony, ocular, otologic, cutaneous, and dental manifestations that are characteristic of the disorder. In evaluating patients with osteogenesis imperfecta, careful attention should be paid to cardiovascular findings and if valvular lesions are noted, patients should be instructed regarding the need for antibiotic prophylaxis for dental and surgical procedures. We report a case of osteogenesis imperfecta associated with aortic regurgitation.
Antibiotic Prophylaxis ; Aortic Valve Insufficiency* ; Connective Tissue ; Ehlers-Danlos Syndrome ; Humans ; Marfan Syndrome ; Mucopolysaccharidosis I ; Osteogenesis Imperfecta* ; Osteogenesis* ; Pseudoxanthoma Elasticum

Transepidermal elimination is a mechanism whereby foreign or altered constituents can be removed from the dermis. The phenomenon of transepidermal elimination may occur as a primary process characterizing disorders such as elastosis perforans serpiginosa, reactive perforating collagenosis and chondrodermatitis nodularis chronica helicis; as well as a secondary process characterizing disorders such as granuloma annulare, necrobiosis lipoidica diabeticorum, calcinosis cutis, pseudoxanthoma elasticum, spitz nevi, pimented nevi, porokeratosis plantaris discreta and metastatic tumors. A 17-year-old man presented with a 2X2 cm sized well-circumscribed slightly erythematous plaque with some black colored papules on the left dorsum of his hand. The histopathology of the lesion indicated angiokeratoma circumscriptum with transepidermal elimination.
Adolescent ; Angiokeratoma* ; Calcinosis ; Dermis ; Granuloma Annulare ; Hand ; Humans ; Necrobiosis Lipoidica ; Nevus ; Porokeratosis ; Pseudoxanthoma Elasticum

Necrobiosis Lipoidica is a skin disorder of unknown cause, which shows characteristic clinical and histological findings. With histochemical studies, we report of a case which oecurred on the both pretibial surfaces in a 5]-year-old female. Having had treatment with aspirin and dipyridarnole for 9 months. The results were successful.
Aspirin ; Female ; Humans ; Necrobiosis Lipoidica* ; Necrobiotic Disorders* ; Skin