1/16. Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis).We report a case of a 16-yr-old white female patient with acute abdominal pain due to visceral involvement of Degos' disease that required extensive small bowel resection. skin manifestations of her disease had been present for 2 yr before the correct diagnosis. She died as a result of central nervous system involvement from Degos' disease.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/16. 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.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
3/16. Depigmented genital extramammary Paget's disease: a possible histogenetic link to Toker's clear cells and clear cell papulosis.BACKGROUND: The histogenesis of extramammary Paget's disease (EMPD) is still controversial. Benign pagetoid cells of the nipple first described by Toker and the similar clear cells found in white maculopapules of clear cell papulosis (CCP) have been proposed to be potential precursor cells giving rise to EMPD and primary intraepidermal Paget's disease in the nipple. The observation of a rare case of depigmented EMPD provided us with a chance to examine further the interesting Toker's clear cell/CCP hypothesis. methods: We performed pathologic studies, including Fontana-Masson stain and immunostaining for AE1/AE3 and S100P, on a new case of depigmented EMPD manifesting a 4 x 3 cm hypopigmented-depigmented patch on the root of the penis. RESULTS: The lesion showed extensive intraepithelial proliferation of atypical pagetoid cells with markedly reduced epidermal melaninization but nearly normal numbers of melanocytes. The tumor cells were strongly positive for AE1/AE3 by immunostaining. Some tumor cells displayed tadpole-like morphology resembling the pagetoid cells of CCP. Such morphology was not observed in two random examples of non-depigmented genital EMPD. CONCLUSIONS: The findings of tadpole-shaped pagetoid cells and depigmentation in the present case suggest that depigmented EMPD may be histogenetically related to CCP. Depigmented EMPD should be considered in the differential diagnosis of vitiligo, depigmented mycosis fungoides and lichen sclerosus located along the milk line.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
4/16. Benign cutaneous Degos' disease: a case report with emphasis on histopathology as papules chronologically evolve.The following case report details a 53-year-old man with a 6-year history of the benign cutaneous or skin-limited form of Degos' disease. Clinically, the patient demonstrated a diffuse eruption of papules on the upper trunk and arms. Many papules demonstrated the classic porcelain-white centers characteristic of Degos' disease, but others exhibited different clinical morphologies that corresponded to the evolutionary stages of papules originally described by Degos. Over the course of several clinic visits, the patient underwent a total of 5 punch biopsies, the histologies of which were correlated with their clinical morphologies. Early papules were skin-colored and demonstrated a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition. The overlying epidermis showed a mild vacuolar interface reaction and the histologic appearances at this early stage resembled tumid lupus erythematosus. Fully developed papules were raised with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-epidermal junction, melanin incontinence, epidermal atrophy, and a developing zone of papillary dermal sclerosis that resembled the early stages of lichen sclerosus et atrophicus in miniature. These interface reactions were invariably confined to the central portion of the punch biopsy specimen, corresponding to the central porcelain-white area seen clinically. Additional features of fully developed papules included a prominent lymphocytic vasculitis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial mucin deposition. In late-stage papules, the porcelain-white areas were better developed and the lesion flattened. Histologically, the degree of inflammation was generally sparse and the overall picture mirrored the classic histologic description of Degos' disease with a central roughly wedge-shaped zone of sclerosis surmounted by an atrophic epidermis and hyperkeratotic compact stratum corneum. These late-stage papules closely resembled a miniaturized version of fully developed lichen sclerosus et atrophicus confined to the center of the punch biopsy specimen.- - - - - - - - - - ranking = 4keywords = white (Clic here for more details about this article) |
5/16. Aquagenic syringeal acrokeratoderma (transient reactive papulotranslucent acrokeratoderma).In 1996, English and McCollough described an unusual entity in 2 sisters characterized by a transient and recurrent keratoderma exclusively on the palms after water exposure. The condition developed 3-5 min after exposure to water and resolved within a short time after drying. This finding was associated with a tightening sensation. Yan et al. coined the term 'aquagenic palmoplantar keratoderma', and the designation 'aquagenic syringeal acrokeratoderma' was suggested by MacCormack et al. Until now, a total of 8 cases have been reported. We documented 2 new cases with acquired aquagenic syringeal acrokeratoderma. A 25-year-old female had observed within the last 3 months a burning sensation on the palms after some minutes of water contact. physical examination revealed a perfectly normal skin on the palms. Three minutes after water immersion of 20 degrees C, a whitish discoloration appeared on the palms and a thickening of the palmar skin was visible. In addition, the eccrine pores were much more prominent. Few minutes after drying the skin, the situation returned to a normal state. The second patient, a 33-year-old female noticed a painful whitish discoloration of the skin on the palms after a short period of water immersion. Sometimes the white skin could be peeled off. In the last year, hyperhidrosis developed, and a more reddish aspect of the palms appeared. In our office after rinsing the hands with water at room temperature, a whitish discoloration in the center of the palms appeared which was associated with a painful sensation. After drying, the whitish lesions disappeared almost completely within 30 min. Aquagenic palmar keratoderma describes an acquired and transient condition which occurs after brief exposure to water and disappears after drying within minutes to an hour. Only rarely may a slight hyperkeratosis remain for a longer time. The possible pathophysiology and treatment options are discussed.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
6/16. Papular elastorrhexis. report of five cases.Papular elastorrhexis is a rare disorder that occurs predominantly during adolescence. We report 5 patients with asymptomatic white, nonfollicular, firm papules scattered over the trunk and extremities. Histologically, the papules demonstrate focal areas of collagen homogenization with decreased and fragmented elastic fibers. The clinical differential diagnosis includes papular acne scars, dermatofibrosis lenticularis disseminata (Buschke-Ollendorff syndrome), cutaneous collagenoma and nevus anelasticus, but histology clearly separates papular elastorrhexis from the other entities.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/16. Benign Degos' disease developing during pregnancy and followed for 10 years.Degos' disease, or malignant atrophic papulosis, is a rare and often fatal multisystem vasculopathy of unknown etiology. The cutaneous manifestations comprise erythematous papules, which heal to leave scars with a pathognomonic central porcelain-white atrophic area and a peripheral telangiectatic rim. Involvement of the gastrointestinal tract is observed in 50% of cases, with intestinal perforation being the most common cause of death. Other organ systems can also be affected; 20% of cases involve the central nervous system. Systemic manifestations usually develop from weeks to years after onset of skin lesions or, in rare instances, may precede skin lesions. In the patient with Degos' disease reported in this article, the characteristic skin lesions developed during pregnancy, a precipitating event not previously reported. She has survived an unusually long time (10 years) without visceral or neurological involvement, despite florid cutaneous lesions. Moreover, we could detect the presence of antiphospholipid antibodies, the significance of which are currently unclear. These observations therefore confirm that there may be a strictly cutaneous form of Degos' disease with a favourable prognosis.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
8/16. Degos disease with an inconspicuous clinical course.BACKGROUND: Degos disease is a rare systemic disorder with involvement of the skin and visceral organs, leading to death in about 50% of cases within 1 or 2 years. In recent years, several cases with cutaneous lesions only have been recognized. methods: We report on a young male patient presenting with single inconspicuous papules with bluish/black centres on the trunk and the upper limbs that, upon healing turn white. These lesions recurred on different locations over the past 6 years, and were never more than two to three at one time. RESULTS: Histopathological examinations revealed archetypal features for Degos disease. The patient had no other complaints, neither visceral organs nor the central nerve system were involved. Laboratory examinations were within normal range. CONCLUSIONS: This case increases the number of reports on a benign course of Degos disease. It raises the question if the 'malignant' and the 'benign' course of the disease represent two distinct diseases or variants of a systemic vasculitis with unknown cause.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
9/16. White fibrous papulosis of the neck: a chinese case.A 48-year-old man who presented with papules on his neck was reported. The patient had multiple, discrete, non-follicular papules on the neck that were round-to-oval, 2--3 mm in diameter and pale. They had remained entirely asymptomatic. On histologic examination, there were circumscribed areas of thickened collagen bundles in the papillary to mid-dermis. This clinical and histologic picture suggested the diagnosis of "white fibrous papulosis of the neck". As far as we are aware, this is the first report of the condition from china.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/16. Benign cutaneous Degos disease.A 24-year-old woman presented with an 8-year history of a recurrent asymptomatic rash characterized by small erythematous papules which evolved to form atrophic porcelain white scars with a telangectatic rim. She had never had gastrointestinal or neurological symptoms. A short trial of aspirin did not alter the behavior of the disease. histology confirmed the clinical diagnosis of Degos disease. Degos disease is a rare disorder that has been classified into the benign or malignant variety. The malignant type has a poor prognosis. Gastrointestinal involvement is the most frequent cause of death. The existence of patients with a prolonged, purely cutaneous or benign form has been increasingly recognized. It may be impossible to classify a patient at the time of initial presentation. Her progress is consistent with the benign form.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
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