1/49. pemphigus vulgaris preceded by herpetiform-like skin lesions with negative immunofluorescence findings.We report a case of pemphigus vulgaris in a 63-year-old woman. At the first onset, she developed herpetiform-like skin lesions. Their histology showed eosinophilic spongiosis and intraepidermal blister formation with an infiltrate of eosinophils. Both direct and indirect immunofluorescence studies were negative. She remained in remission for 6 years. At the second onset, she developed bullous skin lesions; histological examination disclosed suprabasal acantholysis and infiltration of eosinophils. Direct immunofluorescence revealed IgG and C3 deposits at the cell surface, predominantly in the lower epidermis. Indirect immunofluorescence using normal human skin as a substrate demonstrated anti-cell surface IgG antibodies at a titer of 1:160. Immunoblot analysis showed that the patient's serum reacted only with a 130 kD protein. This case indicates that pemphigus vulgaris can follow herpetiform-like skin lesions with negative immunofluorescence findings after a long remission time.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/49. Chronic radiodermatitis following repeated percutaneous transluminal coronary angioplasty.We review three patients who developed chronic radiodermatitis subsequent to undergoing multiple percutaneous transluminal coronary angioplasties (PTCAs). All patients had had chronic ischaemic heart disease (IHD) and had undergone lengthy PTCA on several occasions. The skin eruption was characterized by an atrophic rectangular plaque on the left upper back, presenting as mottled hyper- and hypopigmentation with reticulate telangiectasia. Histologically, the eruption demonstrated epidermal atrophy, hyalinized and irregularly stained collagen, and telangiectasia of superficial vessels in the dermis. Although the risk of radiation injury in most patients undergoing cardiac catheterization is low, this danger should not be ignored. In particular, patients with long-standing IHD and numerous repeated catheterizations to only one or two occluded coronary arteries should be considered at high risk.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
3/49. Differences between scar and dermal cultured fibroblasts derived from a patient with recurrent abdominal incision wound herniation.fibroblasts were derived from dermis and scar of a 47-year-old white man with a recurrent incisional hernia as a result of fractured ribs. The scar was thin and stretched, suggesting a defect in the maturation of granulation tissue. After surgical repair, biopsy specimens of discarded scar and skin were used to generate fibroblast cell lines. fibroblasts maintained in medium containing 10% fetal bovine serum and antibiotic were studied between their third and eighth passage. By phase contrast microscopy, no structural differences were obvious, but it was noted that to pass scar fibroblasts, a more aggressive trypsin regimen was required. Immunohistologic and Western blot analysis of patient scar fibroblasts showed (1) more a smooth muscle actin within stress fibers, (2) increased expression of the vitronectin integrin receptor alpha(v) (CD 51), and (3) reduced expression of the collagen integrin receptor alpha2 (CD 49b). The expression of vinculin from focal adhesions or a tubulin from microtubules was the same among cell lines. Contractions of scar and dermal fibroblast-populated collagen lattice were compared. At 24 hours, contractions were 69 percent with newborn fibroblasts (normal); 68 percent for patient dermal fibroblasts; and only 48 percent for patient scar fibroblasts. The retarded contraction of scar fibroblast-populated collagen lattice was significant (p > or = 0.002). Myosin ATPase activity, critical for lattice contraction, and cell migration were equivalent among all cell lines. A plausible mechanism for the retardation of scar lattice contraction is disruption of fibroblasts and collagen interactions, for which the attachment of cells to collagen is altered. It is proposed that either the decrease in the expression of collagen integrin receptor alpha2 (CD 49b), an increase in the expression of the vitronectin receptor alpha(v) (CD 51), or a combination of both is responsible for disruption of collagen fibroblast interactions.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
4/49. Generalized pruritus without primary lesions. Differential diagnosis and approach to treatment.A 65-year-old man presented with recurrent generalized pruritus and excoriations of many years' duration. He had been treated with antihistamines, topical corticosteroids, and antibiotics for secondary wound infections, but improvement was only temporary. He had also been hospitalized for leg ulcers complicated by cellulitis. Examination revealed multiple oval and linear red papules and nodules measuring 0.5 to 2 cm in diameter. Some of the lesions were eroded and had a central crater and yellowish crust. The patient also had hypopigmented linear scars localized to the posterior scalp, neck, upper back, chest, abdomen, arms, and legs with sparing of the middle and lower back (figures 1 and 2). An ulcer measuring 1.5 x 2 cm that was surrounded by indurated skin was present on the medial aspect of his right ankle. The ulcer was partially covered by yellow exudate. There was no evidence of cellulitis. liver enzyme, serum creatinine, and thyrotropin levels, as well as a chest roentgenogram, were normal. Wound cultures for bacteria and fungi were nonsignificant. A punch biopsy from a representative lesion showed an abrupt epidermal defect with sparse superficial lymphocytic infiltrate in the dermis. The patient was admitted to the hospital to isolate him from his home environment. He received a 10-day course of systemic cephalexin, topical clobetasol propionate ointment for the affected skin areas, and oral hydroxyzine for pruritus. Ultraviolet light therapy was instituted once daily and was to continue for 2 months. His lesions had improved moderately by the time he was discharged from the hospital. On follow-up 2 weeks later, his lesions were flat and had resulted in hypopigmented scars. Three months later, however, he had persistent, intense pruritus, and new excoriations had developed on his forearms and back. He improved after receiving treatment with oral doxepin hydrochloride.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/49. Large annular purpura and paraneoplastic purpura in a patient with sjogren's syndrome and cervical cancer.We report a 79-year-old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by sjogren's syndrome and advanced cervical cancer. The annular purpura spontaneously resolved in a week and did not recur. However, the anaphylactoid purpura relapsed more frequently and spread more widely following the elevation of her serum SCC antigen levels from the onset of purpura until her death. We consider that the characteristic annular configuration was caused by the complication of sjogren's syndrome and that the recurrent anaphylactoid purpura indicated paraneoplastic vasculitis primarily caused by the tumor specific protein immune complexes. Complication by Sjogren's syndrome many also play a role in the development of allergic vasculitis.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
6/49. Treatment of birt-hogg-dube syndrome with erbium:YAG laser.We describe a female patient with birt-hogg-dube syndrome presenting with multiple, disfiguring papules on the cheeks, nose, and ears. In addition, there was evidence of photoaggravation and inflammatory processes in this case. The patient was treated with an erbium:YAG laser. Although the lesions could be successfully ablated up to the papillary dermis without scar formation, a relapse was observed after 6 months. Thus deeper laser ablations may possibly prevent relapses from dermal residual lesions.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
7/49. Late recurrence of inflammatory first-stage lesions in incontinentia pigmenti: an unusual phenomenon and a fascinating pathologic mechanism.BACKGROUND: incontinentia pigmenti (IP) is an X-linked genodermatosis that is manifested by neonatal inflammatory vesicles localized along the lines of Blaschko. These lesions usually clear spontaneously within a few months, leaving hyperpigmentation. Ophthalmologic and neurologic symptoms can be associated with IP. Late recurrences of the first-stage inflammatory lesions after the initial rash are uncommon and have been reported infrequently. The mechanism involved in this phenomenon is unclear. However, the recent identification of NEMO/IKKgamma as the gene responsible for IP sheds new light on its pathophysiologic origins. OBSERVATIONS: We report 5 cases of children who experienced episodes of late reactivation of IP. In all cases, the recurrences occurred on the previously hyperpigmented streaks several months or years after resolution of the initial eruptions. In most cases, the recurrences were preceded by an infectious episode. CONCLUSIONS: These IP recurrences suggest that mutated cells can persist a long time in the epidermis. We theorize that infections trigger the reactivations. The NEMO/IKKgamma gene encodes a protein essential in nuclear factor kappaB activation, which is required for resistance to tumor necrosis factor alpha-induced apoptosis. We discuss the role of a proinflammatory cytokine such as tumor necrosis factor alpha as a triggering factor for the reactivation.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
8/49. aspirin-responsive painful red, blue, black toe, or finger syndrome in polycythemia vera associated with thrombocythemia.Five patients with red, purple blue, or black toes or fingers due to thrombocythemia associated with polycythemia vera (polycythemia and thrombocythemia vera) in four and essential thrombocythemia (thrombocythemia vera) in one are described. The microvascular erythromelalgic syndrome of thrombocythemia was overlooked and progressed to cold blue swollen and painful fingers or black toes in three patients with polycythemia and thrombocythemia vera due to arteriographically documented occlusions of digital or large peripheral arteries with no evidence of preexistent atherosclerotic vascular disease. Concomitant erythromelalgia of the hand palm could be confirmed by the histopathological findings of arteriolar thrombotic lesions in the reticular dermis in two patients with polycythemia and thrombocythemia vera. The increased hematocrit in the presented patients with polycythemia and thrombocythemia vera contributed to the progression of the microvascular syndrome of thrombocythemia to major occlusive ischemic events of the extremities. Standard therapy with oral anticoagulants and reduction of the hematocrit to normal by bloodletting did not affect the platelet-mediated microvascular erythromelalgic, ischemic symptoms in the patients with polycythemia vera because thrombocythemia vera persisted. Complete relief of pain and restoration of the ischemic acral circulation disturbances in patients with thrombocythemia vera or thrombocythemia associated with polycythemia vera in maintained remission by bloodletting could be obtained by long-term treatment with low-dose aspirin.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
9/49. Sweet's syndrome associated with recurrent fever in a patient with trisomy 8 myelodysplastic syndrome.In the case of a 69-year-old man suffering from recurrent high fever, laboratory data demonstrated elevated inflammatory findings such as neutrophilic leukocytosis and an increase in serum level of c-reactive protein. A nonsteroidal antiinflammatory drug or a low dose of prednisolone proved to be temporarily effective. A bone marrow specimen showed myelodysplastic syndrome (MDS) of the refractory anemia (RA) type with trisomy 8. Seven months after onset, painful erythematous eruptions were found on both legs. biopsy of a skin lesion demonstrated neutrophic infiltration into the dermis. As a result, Sweet's syndrome (SS) was diagnosed on the basis of clinical and histopathological findings. Administration of prednisolone (30 mg/d) was started and resulted in prompt defervescence and resolution of the lesions within 1 week. We also investigated serum levels of 3 cytokines: interleukin 1beta (IL-1beta), IL-6, and granulocyte colony-stimulating factor (G-CSF). Levels of IL-6 and G-CSF were elevated during the active phase, but the level of IL-1beta did not increase. Because cases of MDS with trisomy 8 and SS or Behcet disease have been reported recently, our findings suggested that cytokine production, which enhances neutrophil function, is elevated in some MDS patients with trisomy 8; that is, not only cytokines but also trisomy 8 may be related to the pathogenesis of SS in MDS. It is thus advisable to watch for development of SS during follow-up of MDS patients with recurrent fever or trisomy 8.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
10/49. Normolipemic plane xanthoma associated with adenocarcinoma and severe itch.Normolipemic plane xanthomas are yellow-red-colored flat patches or plaques with barely palpable borders, under normolipemic conditions usually involving the eyelids, the lateral sides of the neck, the upper aspect of the trunk, or the flexural folds. Histologically the lesions are characterized by an infiltrate consisting of foamy macrophages in the papillary and middermis with a distinct perivascular localization. Plane xanthoma has been associated with monoclonal gammopathy, cryoglobulinemia, and myeloproliferative disorders. We present a patient in whom plane xanthoma developed on the upper aspect of the back, which was accompanied by severe itch in the affected area. These symptoms started 1 month after resection of an adenocarcinoma of the rectum that was complicated by recurrent abdominal abscesses and, currently, by ongoing inflammatory bowel disease. A hypothetic pathophysiologic scheme of events leading to xanthoma formation in this patient is presented.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
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