1/16. Pseudoporphyria induced by propionic acid derivatives.BACKGROUND: Pseudoporphyria is a photosensitive bullous skin disease that is distinguished from porphyria cutanea tarda (PCT) by its normal porphyrin profile. Drugs are a major cause of this disease, and the list of culprits is continually expanding. Nonsteroidal antiinflammatory agents (NSAIDs), especially naproxen and other propionic acid derivatives, appear to be the most common offenders. OBJECTIVE: The study was carried out to increase awareness about the etiology and characteristic features of pseudoporphyria. methods: We report two cases of pseudoporphyria caused by naproxen and oxaprozin. We review the current English language literature on this entity and discuss its clinical features, histology, ultrastructure, etiology, and pathophysiology. RESULTS: A 44-year-old man taking naproxen for chronic low back pain and a 20-year-old woman on oxaprozin for rheumatoid arthritis presented with tense bullae and cutaneous fragility on the face and the back of the hands. In both, skin biopsy showed a cell-poor subepidermal vesicle with festooning of the dermal papillae. Direct immunofluorescence revealed staining at the dermal-epidermal junction and around blood vessels with IgG in the first case and with IgG, IgA, and fibrin in the second case. urine collections and serum samples yielded normal levels of uro- and coproporphyrins. CONCLUSIONS: Most cases of pseudoporphyria are drug-induced. naproxen, the most common offender, has been associated with a dimorphic clinical pattern: a PCT-like presentation and one simulating erythropoietic protoporphyria in the pediatric population. Other NSAIDs of the propionic acid family can also cause pseudoporphyria.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/16. Pustular vasculitis of the hands.A case of pustular vasculitis of the hands with evidence of systemic involvement is described. A 64-year-old woman presented with a 2-day history of large, tense bullae arranged symmetrically over the dorsum of the three radial digits and extending on to the radial aspect of the dorsum of each hand. The bullae caused some discomfort and prevented normal use of her hands. There was no response to antibiotic therapy initiated prior to referral to hospital. Initial investigations revealed a raised white cell count with a neutrophilia, a raised erythrocyte sedimentation rate and a raised c-reactive protein. Abnormalities of liver function were detected. Aspirates from the bullae and blood cultures were sterile. The histology of debrided tissue demonstrated a florid neutrophilic dermal infiltrate with many blood vessels associated with prominent fibrin. A diagnosis of pustular vasculitis of the hands was made. The bullae were surgically debrided and treatment with oral corticosteroids was started. Two days after commencement of oral prednisolone, a crusted pustule appeared on her upper cutaneous lip. There was rapid resolution of both the vasculitis of the hands and the pustule on her upper lip following an increase in the dose of oral prednisolone. The patient was discharged on the seventeenth day following admission.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/16. Bullosis diabeticorum in a newly discovered type 2 diabetes mellitus.Bullosis diabeticorum (BD), although a rare manifestation, is characteristic of diabetes mellitus. The exact origin of BD is unknown. We report another case of a 54-year-old man who developed a blistering lesion on his left leg. Histopathological examination revealed a microangiopathy with hyalinosis of the blood vessel walls.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/16. Atypical hemorrhagic bullous pyoderma gangrenosum.A 55-year-old woman was seen in the Plastic and Reconstructive Surgery Clinic because of a nonhealing wound on her left leg of approximately 2 months' duration. She had an 8-month history of multiple wounds appearing on her arms and legs. The patient noted that the majority of the wounds had been treated and healed with the use of topical medications. One wound on her left leg had continued to enlarge rapidly despite topical treatments. Therefore, hyperbaric oxygen therapy had been administered for 15 sessions. No additional healing had occurred with this treatment. skin grafting was performed on the affected area. She developed ulcers and blistering lesions at surgical and nonsurgical sites after skin grafting. The patient was referred to the dermatology Department. Dermatologic examination revealed a deep, necrotic ulcer, 30 cm x 10 cm, with surrounding violaceous erythema on the donor area, ulceration (18 cm x 8 cm) on the graft area, a hemorrhagic bullous plaque (5 cm x 15 cm) over the right malleolus, scattered ecchymotic lesions and small hemorrhagic bullae on both legs, and small pustules around the staplers (Fig. 1a,b). Cutaneous biopsy of a new lesion revealed a focal, dense neutrophilic infiltrate, liquefaction degeneration in the center, lymphocytic and mild plasmacytic infiltration around the venules, and fibrinoid deposits in the walls and lumen of the vessels (Fig. 2a). In addition, excessive polymorphonuclear leukocytes and extravasated erythrocytes were present in the papillary and reticular dermis (Fig. 2b). The patient had a 14-year history of asthma bronchiale. physical examination did not reveal any abnormality, except for crackling rales at the base of each lung. Laboratory examinations were within normal limits, except for the sedimentation rate (55 mm/h). Protein electrophoresis, peripheral blood smear, abdominal ultrasound, and thorax and abdominopelvic computed tomography scans were all normal. Swab cultures from the ulcers were negative. Bullous pyoderma gangrenosum was diagnosed on clinical and histopathologic grounds. prednisolone 80 mg/day was started. Rapid epithelialization was observed within 2 months of treatment. The dose of prednisolone was gradually decreased to 20 mg/day, and was used as a maintenance dose for an additional 6 months. Complete improvement was achieved in 8 months. The patient has been followed up for approximately 1 year. There were no side effects observed during the treatment and in addition no new lesions developed at the follow-up.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/16. Noninflammatory bullae associated with epsilon-aminocaproic acid infusion.Three patients who had cardiac surgery developed a transient, noninflammatory subepidermal bullous eruption on the legs after epsilon-aminocaproic acid infusion. Fibrin thrombi were demonstrated in papillary dermal vessels. The use of epsilon-aminocaproic acid as an antifibrinolytic agent may predispose patients to cutaneous vascular thromboses.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/16. Idiopathic atrophie blanche.A 41-year-old woman presented with a 3-year history of purpuric lesions followed by superficial, painful ulcers and development of lesions on the lower legs and on the dorsa of the feet, particularly in the summer. The patient was asymptomatic during the winter months. On physical examination she had irregular, scleroatrophic, white-ivory, coalescent lesions on a livedoid basis, with purpuric and, in some lesions, pigmented borders with numerous telangiectatic capillaries. These lesions were localized on the medial sides of the lower legs and on the dorsa of the feet (Figure 1). Laboratory investigations were normal or negative, including complete blood cell count, platelets, coagulation indexes, erythrocyte sedimentation rate, serum immunoglobulins, antinuclear antibodies, anti-double-stranded dna, anticardiolipin, antiphospholipids, antineutrophilic cytoplasmic antibodies, circulating immunocomplexes, complement fractions (C3, C4), cryoglobulins, rheumatoid factor, and Rose-Waaler reaction. The only laboratory abnormality was an elevated fibrinogen level (472 mg/dL). Doppler velocimetry excluded a chronic venous insufficiency. Thoracic x-ray and abdominal ultrasound were normal. A digital photoplethysmograph revealed functional Raynaud's phenomenon. A biopsy specimen taken from a purpuric lesion showed an atrophic epidermis with parakeratosis and focal spongiosis. An increased number of small-sized vessels were observed within a sclerotic dermis. Most of the vessels in the upper dermis were dilated and showed endothelial swelling; some were occluded due to amorphous hyaline microthrombi (Figure 2). There were fibrinoid deposits around the vessels with thickening of the vessel walls. Extravasated erythrocytes were found throughout the upper and mid-dermis. There was a sparse perivascular lymphocytic infiltrate but no vasculitis. Direct immunofluorescence showed a perivascular microgranular deposit of IgM ( ), C3 ( ), and fibrinogen/fibrin ( ). On the basis of clinical, serologic, histopathologic, and immunopathologic findings, a diagnosis of idiopathic atrophie blanche was made. The patient was treated with dapsone (50 mg p.o. q.d.) and pentoxifylline (400 mg p.o. t.i.d.) with pain relief and complete resolution of the ulcerations after 6 weeks of therapy.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
7/16. Multiple bullae and paresis after drug-induced coma.Two cases of bullous skin lesions and paresis following coma due to the ingestion of many antipsychotic drugs were reported. Histological examination showed an intra-epidermal blister in case 1 and degeneration of sweat glands in both cases. An immunofluorescence study showed massive deposits of IgM and C3 in the dermal vessels. As similar deposits of immunoglobulin and complement were not observed in patients with ordinary lesions such as decubitus, a different mechanism in the formation of the bullous skin lesion other than pressure is suggested.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/16. Vesiculobullous cutaneous lymphatic reflux.Although lymphedema is a fairly common condition, cutaneous vesicles and bullae due to reflux of lymphatic fluid are rare. We describe a patient with adenocarcinoma of the cervix treated by radiation in whom acute secondary lymphedema with overlying clear vesicles and bullae developed. Histopathologic examination revealed a subepidermal vesicle communicating directly with a dermal lymphatic vessel. Vesicles occur only in lymphedema of acute onset. Vesiculobullous cutaneous lymphatic reflux should be recognized as a rare complication of lymphedema and included in the differential diagnosis of cutaneous blistering disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/16. A bullous variant of Kaposi's sarcoma in an elderly female.We report the case of Kaposi's sarcoma (KS) in an elderly Italian female that has slowly progressed and evolved clinically with bullous formation. The skin biopsy was diagnostic of KS, showing the typical spindle cell formations, but at the same time showing a labyrinth-like collection of vessels that correlated with the bullous nature of the clinical lesions. Immunohistochemical staining for factor viii antigens demonstrated strong reactivity of non-neoplastic blood vessel endothelium but virtually no staining of tumor cells. This is similar to the reactivity of normal lymphatic endothelial cells and supports the notion that these labyrinth-like vessels are derived from lymphatic endothelium.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
10/16. Hemorrhagic bullae associated with vibrio vulnificus septicemia. Report of two cases.Bullous lesions associated with vibrio vulnificus infection developed in two patients, both of whom had hepatic cirrhosis. One patient had a recent history of ingestion of raw oysters, while the other patient had recently exposed skin lacerations to sea water. Both patients died within 24 hours of hospitalization, in spite of antibiotic treatment. vibrio vulnificus was isolated from blood and bullae in both patients. Histologic examination of skin biopsy specimens revealed epidermal/dermal separation and clusters of bacteria within dermal vessels with a negligible inflammatory response.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
| | Next -> |