11/164. adult-onset Still's disease with persistent plaques. adult-onset Still's disease (AOSD) is a systemic disorder characterized by intermittent fever, evanescent rash, arthralgias or arthritis and predominantly neutrophilic leucocytosis. We report on a 16-year-old woman with Still's disease who developed, in addition to the typical rash, persistent papular lesions on her face, neck and upper and lower back. Although the presence of fixed skin lesions is not a characteristic feature of AOSD, their appearance at the onset of the disease and their evolution suggest that they represent a specific manifestation of the disease. ( info) |
12/164. Papular elastolytic giant cell granuloma: a clinical variant of annular elastolytic giant cell granuloma or generalized granuloma annulare? A 71-year-old man with asymptomatic red papules on the trunk and upper arms was reported as a case of papular elastolytic giant cell granuloma. A skin biopsy specimen from a papule on the back showed similar findings to those of annular elastolytic giant cell granuloma. However, centrifugal annular lesions were not clinically observed. He was successfully treated with tranilast and topical steroids. ( info) |
13/164. Prominent pruritic periumbilical papules: A diagnostic sign in pediatric atopic dermatitis. Establishment of diagnostic criteria for atopic dermatitis has been a subject of controversy and frequent reevaluation. The diagnostic criteria of Hanifin and Rajka are those most frequently cited. In order to fit the diagnosis, a patient must demonstrate three major criteria plus four or more minor criteria. Although individually the minor criteria are not diagnostic, their presence suggests the possibility of atopic dermatitis. Recently we evaluated several children who developed prominent periumbilical papules as a major component of their atopic dermatitis. This finding, while not present in all children with atopic dermatitis, can provide a specific clue to diagnosis and should be considered as a new minor criterion for atopic dermatitis in children. ( info) |
14/164. Papulonecrotic tuberculide and stenosis of the abdominal aorta. Papulonecrotic tuberculide (PNT) is a rare form of skin tuberculosis affecting predominantly young adults, with a history of immunity to mycobacterium tuberculosis. We report a case of a young Caucasian female with PNT who was also documented to have a stenotic segment in the abdominal aorta. The difficulty in clarifying and treating the primary disease and the association between a tuberculous infection and Takayasu's arteritis are discussed. ( info) |
15/164. Generalized eruptive keratoacanthoma (Grzybowski variant). We describe a patient with generalized eruptive keratoacanthoma (KA) of Grzybowski showing the characteristic features of this extremely rare condition. Since the first description by Grzybowski in 1950, only 28 additional cases (including the present one) have been reported. This variety of KA most commonly affects patients during the fifth to seventh decade of life and appears as a generalized eruption of hundreds to thousands of follicular papules. The small pruriginous papules often have a keratotic centre and show microscopic features of KA. Marked facial involvement is characteristic and can lead to masked facies with ectropion, as in our patient. The course of the disease is chronic and the response to therapy is poor. ( info) |
| A 30-year-old man presented with numerous papules, nodules and inflamed cysts. The lesions were located all over the body, including the scalp, except the palms and soles. His mother and one sister had had similar but less extensive lesions. Histopathology of the biopsy specimens obtained from the anterior chest wall, axillae and the back region was consistent with steatocystoma multiplex (SM). A diagnosis of steatocystoma multiplex suppurativum was made. The inflamed lesions were treated with oral isotretinoin (1 mg/kg per daily) for 6 months. At the same time, cryotherapy was used for non-suppurating lesions smaller than 2 cm. When the patient was evaluated 6 months later, cosmetic results were good. No new lesions have appeared in the subsequent 12-month follow up. ( info) |
17/164. A figurate papulosquamous variant of inflammatory vitiligo. The classic description of inflammatory vitiligo is an erythematous rim at the periphery of a patch of hypopigmented or depigmented skin. The histological correlate is a superficial perivascular infiltrate of mononuclear cells. However, we observed a 61-year-old patient with inflammatory vitiligo who had thin solid pink scaly plaques as well as serpiginous lesions with fine scale. Histologically, a lichenoid infiltrate was seen as was a thickened stratum corneum with parakeratosis. In a review of the literature, scattered case reports of similar findings were identified, either in the Japanese and French literature or from over 25 years ago. The clinical and histological spectrum of inflammatory vitiligo should be expanded to include solid and annular papulosquamous plaques as well as lichenoid infiltrates with exocytosis. ( info) |
18/164. Degos' disease. A 47-year-old woman presented with slightly pruritic, burning lesions on her submammary area and legs of 3.5 years' duration. Similar lesions had appeared on her arms and feet. She was hospitalized at the Pulmonary Diseases Hospital, Izmir, with a complaint of thoracic pain, and was diagnosed with nonspecific pleuritis by pulmonary X-ray, thoracic computed tomography (CT) scanning, bacterial cultures of phlegm, and pleural biopsy examination. Fifteen days after leaving hospital, she was admitted to the Surgical Clinic of Ataturk Training Hospital, Izmir, with a complaint of severe abdominal pain, and diagnosed with nonspecific peritonitis. On dermatologic examination, she had multiple, scattered papules (2-3 mm in diameter), with a typical, central, white, porcelain-like zone of atrophy, on the trunk (Fig. 1), legs (Fig. 2), and arms. The lesions were encircled by a slightly raised erythematous border. Some of the lesions had coalesced giving polycyclic atrophic areas and ulcerations. Histopathologic examination of the skin biopsy specimen showed hyperkeratosis, epidermal atrophy, dermo-epidermal separation, edema, and necrosis in the papillary dermis (Fig. 3). Fibrinoid necrosis and thrombosis were seen in the papillary dermis and in the vessels below the lesions. The patient was diagnosed with Degos' disease from these clinical and histopathologic findings. She was treated with anti-inflammatory drugs. One year later, the patient was hospitalized at the Department of internal medicine of Ataturk Training Hospital, Izmir, with a diagnosis of pericarditis and pleuritis. ( info) |
19/164. Haemorrhagic papular rash associated to Flavimonas oryzihabitans bacteraemia in a child. Flavimonas oryzihabitans is a gram-negative rod that has rarely been implicated in human infections. The involvement of this organism has been documented in serious infections, the majority of which were cases of bacteraemia or peritonitis. We report the first isolation of the organism in greece, from a case of bacteraemia, associated with haemorrhagic papular rash, in a paediatric patient and describe the phenotypic characteristics of the strain. ( info) |
20/164. Degos' disease with constrictive pericarditis: a case report. A 47-year-old man with Degos' disease was examined by echocardiography, which showed hypokinesis of the apical left ventricular wall with pericardial effusion. To evaluate the myocardial perfusion and coronary flow reserve, 201Tl scintigraphy and intracoronary Doppler flowmetry were performed. The coronary flow reserve was not decreased nor was there angiographical coronary stenosis, although a pressure study revealed constrictive dysfunction of both ventricles. The constrictive pericarditis might have been induced by pericardial vasculitis, thereby causing the left ventricular wall motion abnormality. ( info) |