Blepharochalasis associated with dermatomyositis and acute lymphocytic leukemia.
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Publications and source records attributed to L E Gibson.
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BACKGROUND: Erythema elevatum diutinum is a rare condition representing a chronic leukocytoclastic vasculitis. OBJECTIVE: Clinical and laboratory features of the disease were reviewed to better understand the disease. METHODS: The medical records and histopathologic slides of 13 patients with erythema elevatum diutinum were studied. RESULTS: The lesions were violaceous, deep red, or brown and typically were papules or plaques. Lesions were most often located on the extensor surfaces of the extremities. Associated medical problems included hematologic abnormalities in six patients: IgA clonal gammopathies (four), multiple myeloma (one), and myelodysplasia (one). Erythema elevatum diutinum preceded the myeloproliferative disorders by an average of 7.8 years. All patients showed vasculitis. Leukocytoclasia was present in 27 of 35 specimens. The predominant cell type in the inflammatory infiltrate was polymorphonuclear leukocytes or a mixture of polymorphonuclear leukocytes and lymphocytes. CONCLUSION: The most significant finding of this study is the association of erythema elevatum diutinum with hematologic disease, most frequently an IgA monoclonal gammopathy.
BACKGROUND: Urticarial vasculitis is a subset of vasculitis characterized clinically by urticarial skin lesions and histologically by necrotizing vasculitis. OBJECTIVE: A review of patients with urticarial vasculitis was undertaken to further characterize the clinical and histologic findings and to differentiate this disorder from urticaria and other types of cutaneous vasculitis. METHODS: Seventy-two cases of biopsy-proven urticarial vasculitis were selected for a review of medical records, laboratory data, and histologic findings. Fifty cases of simple urticaria were also reviewed for purposes of comparison. RESULTS: Systemic symptoms in patients with urticarial vasculitis included angioedema in 30 patients (42%), arthralgias in 35 (49%), pulmonary disease in 15 (21%), and abdominal pain in 12 (17%). Twenty-three patients (32%) had hypocomplementemia. Forty-six of 72 patients (64%) had lesions that lasted more than 24 hours, 23 of 72 (32%) had painful or burning lesions, and 25 of 72 (35%) had lesions that resolved with purpura. Sixteen biopsy specimens from the 23 patients with hypocomplementemia showed dermal neutrophilia in addition to the perivascular infiltrate. Of the 23 patients with hypocomplementemia, 20 (87%) had fluorescence of the blood vessels and 16 (70%) had fluorescence of the basement membrane zone as determined by routine direct immunofluorescence. CONCLUSION: Patients with hypocomplementemia were more likely to have systemic symptoms such as urticaria that resolved with purpura, arthralgias, abdominal pain, and chronic obstructive pulmonary disease. The histologic pattern associated with hypocomplementemia is interstitial neutrophilic infiltrate of the dermis and an immunofluorescent pattern of immunoglobulins or C3 in the blood vessels and along the basement membrane zone.
Malignant blue nevus is uncommon compared to its benign counterpart and is regarded as a rare form of malignant melanoma. We report the clinical and histological findings in eight cases. Histologically, all eight specimens showed no epidermal involvement and had contained within or were adjacent to portions of blue nevus or cellular blue nevus. Proliferation of bundles of bipolar spindle shaped cells with marked cellular atypia, mitotic figures, foci of necrosis, and inflammatory cell infiltrate were noted. Two of the cases were studied by DNA flow cytometry and the populations of tumor cells were found to be diploid. Two cases have died secondary to metastasis. Although malignant blue nevi may not behave as aggressively as nodular malignant melanoma, they have definite potential to do so and therefore should be removed by wide surgical excision.
A retrospective clinical and histopathologic study of 53 patients with granulomatous rosacea was undertaken. The patients had a broad clinical spectrum of lesions that ranged from primarily erythema to papulonodular lesions. Extrafacial lesions occurred in 15% of patients. Histologic examination showed mixed lymphohistiocytic inflammation (primarily lymphocytic inflammation in 40% of patients and primarily histiocytic with a few giant cells in 34%), epithelioid granulomas in 11% of patients, and epithelioid granulomas with caseation necrosis in 11%. Most patients had a good response to oral antibiotic therapy. Granulomatous rosacea is not a distinct disease but can be regarded and treated as a subtype of rosacea.
Among 33 patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) made at our institution between 1982 and 1989, 9 had mycosis fungoides diagnosed concomitantly. Three other patients had lymphoproliferative disorders, and two had Kaposi's sarcoma. Analysis of biopsy features such as epidermal lymphocytic exocytosis, periappendageal infiltrate, and deposition of mucin revealed no predominant finding that distinguished a benign course from mycosis fungoides. A predominance of eosinophils in the infiltrate was suggestive of benign follicular mucinosis rather than mycosis fungoides. Gene rearrangement studies detected three clones in three patients with follicular mucinosis; two were in patients with mycosis fungoides, and one was in a patient with dermatitis. The outcome of these three patients is pending further follow-up. No histopathologic or clinical features distinguished these patients from the others.
We performed a retrospective study of 52 children and young adults (average age 18 yrs) with lichen sclerosus et atrophicus. In 56% of patients the eruption was still present after a follow-up of 7.5 years. Younger patients were most likely to show improvement. The histologic appearance of both groups (patients with persistent lesions and those with resolution of lesions) showed classic features of lichen sclerosus et atrophicus: homogenized collagen with an underlying bandlike lymphocytic infiltrate. Epidermal changes such as atrophy, hyperplasia, and follicular plugging were slightly more prominent in patients with persistent lesions. Menarche, pregnancy, and the presence of extragenital lesions had no effect on the prognosis, but since the condition resolved, on average, during adolescence, unknown developmental factors may be implicated.
A retrospective review of 47 cases of lymphomatoid granulomatosis encountered between 1977 and 1990 revealed that 24 patients had skin involvement. Clinical and histologic features were noted. The dominant and most characteristic cutaneous finding was scattered nodules. Other cutaneous changes included eroded and crusted lesions, facial edema, papules, and folliculitis-like eruptions. The nodular skin lesions most often had an angiocentric and angiodestructive dense, polymorphous, lymphohistiocytic infiltrate in the dermis and fat. A minority of the cases had a lymphoma cutis type of infiltrate. Immunophenotypic analysis showed a mixed population of T cells, with helper T cells (CD4+) predominant. Our results emphasize the usefulness of the clinical and histologic examination of the skin in lymphomatoid granulomatosis. The histologic pattern of lymphomatoid granulomatosis was seen in the skin in more than half of the biopsy specimens.
Multiple eruptive keratoacanthoma of Witten and Zak is a rare disorder characterized by numerous small, eruptive tumors and larger, more typical keratoacanthomas. Affected patients have features of Grzybowski-type keratoacanthomas and Ferguson Smith type. Two patients with multiple keratoacanthomas were treated with oral retinoids. Both patients had hundreds of follicular papules on the trunk and extremities. Less common lesions included nodules with central horn-filled craters more characteristic of classic keratoacanthomas. Retinoid therapy resulted in regression of the larger, more typical keratoacanthomas in both patients. The small follicular keratoacanthomas remained unaffected. Thus oral retinoids are only partially beneficial for the treatment of the Grzybowski type or the Witten and Zak type of multiple eruptive keratoacanthomas.
The starting point in the evaluation of vasculitis is the clinical examination. The character and location of the primary lesions determine the type of biopsy specimens and additional tests needed to classify vasculitis. The most common causes of cutaneous vasculitis are drug reactions, infectious diseases, reactions to inflammatory diseases such as rheumatoid arthritis or inflammatory bowel disease, or association with malignant disease, especially lymphoproliferative disorders. Direct immunofluorescent techniques and leukocyte monoclonal antibody studies are useful for the diagnosis of selected cases of vasculitis. The clinical and histopathologic data help delineate an approach for further investigation of potentially associated systemic disorders or underlying causes. Although some cases of cutaneous vasculitis are not associated with systemic disorders, this possibility should never be assumed but considered only as a diagnosis of exclusion after careful examination of each patient.
Cutaneous vasculitis comprises a wide spectrum of clinical syndromes and histopathologic findings which share the common theme of vascular inflammation and changes in the adjacent tissue. This article discusses several types of cutaneous vasculitides including leukocytoclastic vasculitis, Henoch-Schönlein purpura, urticarial vasculitis, livedoid vasculitis, and granulomatous vasculitides. The clinical patterns of these types of vasculitic syndromes as well as associated diseases and histopathology are discussed.
Wegener's granulomatosis, lymphomatoid granulomatosis, and Churg-Strauss granulomatosis may all have cutaneous involvement. The morphology of cutaneous lesions in these disorders varies from macular erythema to frank gangrenous ulceration. Most often lesions are located on the extremities; however, truncal or facial involvement has been reported, the latter especially in Wegener's granulomatosis. A common histologic finding in these cutaneous lesions is necrotizing vasculitis. However, it is also possible to see Churg-Strauss extravascular granulomas and even periarteritis. Cutaneous involvement with these three forms of systemic granulomatosis generally parallels the systemic course. The treatment for the cutaneous lesions is dictated by the treatment for the systemic vasculitis. It is important to recognize that the cutaneous extravascular granuloma and cutaneous granulomatous vasculitis can be associated with other disorders in addition to systemic granulomatosis. These disorders include most importantly lymphoproliferative diseases, inflammatory disorders such as arthritis, autoimmune diseases, and other inflammatory disorders such as sarcoidosis. Cutaneous involvement with giant cell or temporal arteritis is not common, but ulcerative temporal-parietal scalp lesions are distinctive. Although not common in the United States, Takayasu's arteritis may have several cutaneous manifestations, including erythema nodosum-like lesions. Granulomatous vasculitides have a myriad of cutaneous manifestations. Knowledge of these manifestations may allow for prompt diagnosis in many cases and increased surveillance in other cases for associated systemic illnesses.
Superficial granulomatous pyoderma is a form of pyoderma gangrenosum characterized by superficial ulceration and a chronic course. Histopathologic examination shows a granulomatous response. We report two new cases of superficial granulomatous pyoderma in detail and briefly review the clinical data of five previous cases included in a recent series. These lesions typically begin as single furunculoid papules that most commonly occur on the trunk and that may arise at sites of surgical treatment or other pathergic stimuli. The lesions progress to superficial ulcers with a relatively clean base and vegetative borders. Tetracycline, minocycline, sulfapyridine, dapsone, and intralesionally administered corticosteroids have been effective anti-inflammatory agents, producing healing and allowing avoidance of the use of systemic corticosteroids in the management of most patients with superficial granulomatous pyoderma.
Fifty-nine patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) were evaluated retrospectively. Thirty-seven were male and 22 were female; ages ranged from 10 to 76 years. Of 19 patients with mycosis fungoides, 16 had initial lesions of follicular mucinosis on the trunk or extremities. Two patients had Hodgkin's disease and follicular mucinosis; both were younger than 20 years of age. Seven other patients were 20 years of age or younger. Two of the nine adolescent patients had persistent plaques of follicular mucinosis up to 18 years after diagnosis. Evaluation of biopsy specimens for lymphocytes, eosinophils, nonlymphoid cell infiltration, epidermal lymphocytic exocytosis, mucin deposition, and epidermal hyperplasia revealed no predominant feature that differentiated the group with benign disease from the group with mycosis fungoides. We conclude that no single clinical or histopathologic observation predicts which patients with follicular mucinosis will have a benign course and that evaluation of multiple clinical and histologic variables is necessary.
Study of the histopathologic pattern of 28 cases of superficial granulomatous pyoderma demonstrated an ulcerative, verrucous pyoderma of the superficial dermis. Focal abscesses in the subepidermis or dermis were juxtaposed with granuloma formation and plasma cell inflammation. Hemorrhage and granulation tissue were present. The clinical lesions are indolent, and this feature is confirmed by chronic inflammatory elements, including sinus tract formation and focal fibrosis. Superficial granulomatous pyoderma must be added to the histologic differential diagnosis of the vegetative and ulcerative granulomas: blastomycosis, tuberculosis verrucosa cutis, and bromoderma.
The depth of wounds created by various concentrations and application techniques of trichloroacetic acid (TCA) in a porcine model is evaluated. The depth of tissue necrosis increased with the concentration of TCA. Tape occlusion did not deepen the wound but rather decreased its depth. An inverse relationship of wound depth to epidermal thickness is described.