REFERENCIAS BIBLIOGRÁFICAS / BIBLIOGRAPHICAL REFERENCES

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A.- Erythematous Plaques and Nodules in a Child (2025).

B.- Novel Use of Topical Dapsone 5% Gel for Erythema Elevatum Diutinum: Safer and Effective (2013).

C.- Erythema Elevatum Diutinum: Skin and Systemic Manifestations, Immunologic Studies, and Successful Treatment With Dapsone (1977).

D.- Erythema Elevatum Diutinum: A Case Report and Review of Literature (2019).

E.- Erythema Elevatum Et Diutinum as a Systemic Disease (2019).

F.- Infantile Erythema Elevatum Diutinum: Report of a Vesiculo-Bullous Case (2006).

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1.) [Erythema elevatum and diutinum in Crohn disease]

2.) Erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with intermittent plasma exchange.

3.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases.

4.) Terrien's marginal degeneration associated with erythema elevatum diutinum.

5.) Erythema elevatum Diutinum.

6.) Nodular erythema elevatum diutinum mimicking cutaneous neoplasms.

7.) Erythema elevatum diutinum: a clinicopathological study of eight cases.

8.) Erythema elevatum diutinum with extensive acro-osteolysis.

9.) Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale.

10.) Erythema elevatum diutinum in association with coeliac disease.

11.) Erythema elevatum diutinum complicated by rheumatoid arthritis.

12.) Erythema elevatum diutinum in human immunodeficiency virus-infected patients--report of a case and review of the literature [see comments]

13.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation.

14.) POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.

15.) A continuum of neutrophilic disease occurring in a patient with ulcerative colitis.

16.) Granuloma faciale with extrafacial lesions.

17.) Erythema elevatum diutinum and HIV infection: a report of five cases.

18.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases.

19.) Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale.

20.) [Erythema elevatum diutinum. A rare dermatosis with a broad spectrum of associated illnesses].

21.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation.

22.) Erythema elevatum diutinum in human immunodeficiency virus-infected patients--report of a case and review of the literature.

23.) Cutaneous vasculitis.

24.) Pyoderma gangrenosum, erythema elevatum diutinum and IgA monoclonal gammopathy.

25.) Simultaneous occurrence of two rare cutaneous markers of poor prognosis in myelodysplastic syndrome: erythema elevatum diutinum and specific lesions.

26.) Erythema elevatum diutinum: case report showing an unusual distribution.

27.) Ultrastructural study of chronic lesions of erythema elevatum diutinum: "extracellular cholesterosis" is a misnomer.

28.) Nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus.

29.) Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet.

30.) Association of hyperimmunoglobulinaemia D syndrome with erythema elevatum diutinum.

31.) Erythema elevatum diutinum associated with Wegener's granulomatosis and IgA paraproteinemia.

32.) Pyoderma gangrenosum in association with erythema elevatum diutinum: report of two cases.

33.) Erythema elevatum diutinum: a clinicopathological study.

34.) Erythema elevatum diutinum in a patient with relapsing polychondritis.

35.) Erythema elevatum diutinum: a clinical and histopathologic study of 13 patients.

36.) Erythema elevatum diutinum in a patient with acquired immunodeficiency syndrome. Another clinical simulator of Kaposi's sarcoma.

37.) Erythema elevatum diutinum--an unusual association with ulcerative colitis.

38.) Erythema elevatum diutinum mimicking porphyria cutanea tarda.

39.) Cutaneous manifestations of neutrophilic disease. A study of seven cases.

40.) Erythema elevatum diutinum--a solitary lesion in a patient with rheumatoid arthritis.

41.) Erythema elevatum diutinum in a patient with Crohn's disease.

42.) The natural history of vasculitis. What the histology tells us about pathogenesis.

43.) Pleural effusions in an overlap syndrome of idiopathic hypereosinophilic syndrome and erythema elevatum diutinum.

44.) Erythema elevatum diutinum: an ultrastructural case study.

45.) [Erythema elevatum diutinum--a rare variant of skin changes in systemic lupus erythematosus].

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1.) [Erythema elevatum and diutinum in Crohn disease]

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Author

Elsner J; Kiehl P; Kapp A; Weiss J

Address

Dermatologische Klinik und Poliklinik, Medizinische Hochschule Hannover.

Source

Hautarzt, 47(9):701-4 1996 Sep

Abstract

A 35-year-old woman suffering from Crohn's disease for 3 years developed painful erythematous nodular lesions predominantly on the dorsa of her hands and feet, as well as on her elbows and wrists. The clinical features and histopathological picture of leukocytoclastic vasculitis were both highly suggestive of erythema elevatum diutinum. No improvement of the skin lesions was observed during treatment with high doses of oral steroids. In contrast, treatment with dapsone resulted in a marked reduction of skin lesions. During a new attack of her Crohn's disease the erythema elevatum diutinum lesions flared again.

The parallel clinical course of erythema elevatum diutinum and Crohn's disease, as well as the detection of immune complexes and neutrophilic granulocytes in lesions of both diseases, strongly suggest a common etiology. This hypothesis is discussed in context with this case and the recent literature.

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2.) Erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with intermittent plasma exchange.

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Author

Chow RK; Benny WB; Coupe RL; Dodd WA; Ongley RC

Address

Division of Dermatology, University of British Columbia, Vancouver.

Source

Arch Dermatol, 132(11):1360-4 1996 Nov

Abstract

BACKGROUND: We review the literature and report a case of refractory erythema elevatum diutinum associated with IgA paraproteinemia that was successfully controlled with intermittent plasma exchange (PLEX).

OBSERVATIONS: Typical lesions of erythema elevatum diutinum developed in a 72-year-old patient with IgA paraproteinemia; the condition predictably flared whenever IgA levels reached a threshold of 8 g/L. After 8 years of unsuccessful treatment with various agents, we instituted a trial of PLEX during an acute flare. Following 6 exchanges over a period of 2 weeks, the IgA level decreased from 8 to 2 g/L and the skin lesions cleared. Three weeks later, new skin lesions developed and the IgA level had rebounded from 2 to just over 8 g/L.

A second course of PLEX was administered, with excellent results, and a 3-month course of oral chlorambucil (2 mg/d) was initiated. The patient's condition remained in clinical remission for 10 months.

Over the ensuing 9 years, she suffered 11 further flares, each of which was associated with IgA levels of 8 to 10 g/L and each responding dramatically to 3 to 5 PLEXs followed by a consolidative dose of intravenous cyclophosphamide (250-500 mg).

CONCLUSION: We believe that PLEX may have an important role in the management of severe erythema elevatum diutinum associated with monoclonal paraproteinemia refractory to other

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3.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases

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Author

Takiwaki H; Kubo Y; Tsuda H; Arase S; Shiota H

Address

Department of Dermatology, School of Medicine, University of Tokushima, Japan.

Source

Br J Dermatol, 138(5):893-7 1998 May

Abstract

Peripheral ulcerative keratitis (PUK) is a complication of collagen-vascular diseases such as rheumatoid arthritis (RA) and other systemic vasculitides. We report three cases of erythema elevatum diutinum with PUK.

These patients presented with nodules and plaques consistent with erythema elevatum diutinum on the extremities and crusted or ulcerated purpuric lesions on the soles. Histopathological examination of these lesions revealed a dense neutrophilic infiltrate with nuclear dust and fibrin around blood vessels. All the patients developed PUK concomitant with the development of the skin lesions. The rheumatoid factor was positive at high titre in all three patients; this was associated with probable RA in one.

Cutaneous lesions were dramatically improved by administration of dapsone in all cases. Dapsone was also effective in treating the ocular lesions in two patients.

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4.) Terrien's marginal degeneration associated with erythema elevatum diutinum.

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Author

Shimazaki J; Yang HY; Shimmura S; Tsubota K

Address

Department of Ophthalmology, Tokyo Dental College, Chiba, Japan.

Source

Cornea, 17(3):342-4 1998 May

Abstract

PURPOSE: To describe a patient with Terrien's marginal degeneration associated with a chronic skin disease, erythema elevatum diutinum.

METHODS: A 27-year-old woman with peripheral thinning of her left cornea associated with adjacent conjunctival and episcleral inflammation was clinically examined.

RESULTS: Episodes of worsening skin lesions were associated with development of inflammation in her left eye. Administration of sulfones was effective for reducing both skin and ocular inflammation.

CONCLUSION: Erythema elevatum diutinum should be considered an underlying systemic disease of Terrien's marginal degeneration.

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5.) Erythema elevatum diutinum.

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Author:

Croker H W, Williams C.

Br J Dermatol 1984;6:1-9,33,8

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6.) Nodular erythema elevatum diutinum mimicking cutaneous neoplasms.

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Author

Shanks JH; Banerjee SS; Bishop PW; Pearson JM; Eyden BP

Address

Department of Histopathology, Christie Hospital NHS Trust, UK.

Source

Histopathology, 31(1):91-6 1997 Jul

Abstract

AIMS: We describe the cutaneous pseudoneoplastic lesions in two patients with nodular erythema elevatum diutinum, a rare chronic disorder in which polymorph nuclear fragmentation (leukocytoclasis) is present within dermal nodules showing spindle cells and fibrosis.

In both cases diagnostic difficulty was encountered clinically and pathologically and various benign and malignant neoplasms were considered in the differential diagnosis.

METHODS AND RESULTS: Immunohistochemically the spindle cells were negative for CAM5.2, AE1/3, S100 protein and desmin (D33). They were positive for vimentin and focally positive for CD34 and alpha-smooth muscle actin. Some of the spindle cells were positive for Mac 387 and KP1(CD68). By electron microscopy, the lesions were shown to consist of fibroblasts/myofibroblasts and fusiform macrophages.

CONCLUSIONS: Increased awareness of the features described will help to avoid misdiagnosis as a neoplastic process.

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7.) Erythema elevatum diutinum: a clinicopathological study of eight cases.

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Author

Sang¨ueza OP; Pilcher B; Martin Sang¨ueza J

Address

Department of Pathology, Medical College of Georgia, Augusta 30912, USA.

Source

Am J Dermatopathol, 19(3):214-22 1997 Jun

Abstract

Erythema elevatum diutinum (EED) is a rare cutaneous condition that initially presents as leukocytoclastic vasculitis (LCCV) of the skin and later resolves with fibrosis.

In addition to the LCCV, EED may show features reminiscent of other entities. For example, it may mimic lesions of dermatofibroma, granuloma annulare, granuloma faciale, or dermatitis herpetiformis.

For this study, we reviewed the clinical records and 13 skin biopsies in eight patients with EED. One of the patients had concurrent pityriasis rubra pilaris, and another developed lesions of EED following and at the sites of mosquito bites; these associations have not been noted previously.

In addition to such typical histopathological features as diffuse dermal involvement by neutrophils, eosinophils, and leukocytoclastic vasculitis, we also found two unusual patterns. The first was characterized by palisaded necrotizing granulomas, as previously described and associated with Churg-Strauss granuloma; the second condition simulated a pyogenicgranuloma--like lesion.

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8.) Erythema elevatum diutinum with extensive acro-osteolysis.

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Author

Ellabban A; Schumacher HR Jr Address

Division of Rheumatology, University of Pennsylvania School of Medicine, Philadelphia, USA.

Source

J Rheumatol, 24(6):1203-5 1997 Jun

Abstract

Erythema elevatum diutinum (EED) is a rare chronic skin disease with cutaneous vasculitis, characterized by the absence of systemic vasculopathy. We describe a 79-year-old white woman who has been followed with the diagnosis of EED for 27 years. Our patient has a previously unreported combination of EED and acro-osteolysis. After reviewing possible reasons for the association, her vasculitis seems the most likely factor.

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9.) Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale.

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Author

Carlson JA; LeBoit PE

Address

Department of Pathology, Albany Medical College, New York, USA.

Source

Am J Surg Pathol, 21(6):698-705 1997 Jun

Abstract

Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic, localized forms of cutaneous leukocytoclastic vasculitis that result in patterned (storiform or concentric) fibrosis. EED often occurs in systemically ill patients as bilaterally symmetrical plaques, papules, or nodules, often over the dorsa of joints. GF occurs as one or a few plaques on the face. Eosinophils and plasma cells are prominent in GF, whereas neutrophils are plentiful in EED.

Rarely, extrafacial lesions accompany facial ones in GF, and there are a few reports of upper respiratory tract masses with GF-like histologic features. We report on eight patients with solitary cutaneous lesions with histologic features similar to those of EED or GF, but whose clinical picture was not that of either disease.

One, whose histology resembled GF, had a large multinodular dermal and subcutaneous mass that persisted despite attempted resection. Unusual histologic findings in other cases included storiform fibrosis with dense infiltrates of plasma cells, branching nerve fascicles admixed with EED-like changes, and EED-like areas adjacent to zones mimicking a sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the stereotypic settings of GF and EED and is an inflammatory reaction pattern that does not signify a specific diagnosis.

Because of transitions between EED or GF-like areas and those of patterned sclerosis with plasma cell-rich infiltrates, we believe that some "inflammatory pseudotumors" of the skin, and perhaps of other sites could be the result of localized vasculitis.

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10.) Erythema elevatum diutinum in association with coeliac disease.

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Author

Tasanen K; Raudasoja R; Kallioinen M; Ranki A

Address

Department of Dermatology, Oulu University Hospital, Finland.

Source

Br J Dermatol, 136(4):624-7 1997 Apr

Abstract

Erythema elevatum diutinum (EED) has been described in association with several immunological or infectious diseases. We describe a female patient who presented with clinically and histologically typical EED in whom previously undiagnosed coeliac disease was found. Appearance of EED lesions was preceded by widespread joint pains. In extensive laboratory tests, the only abnormal findings were an elevated erythrocyte sedimentation rate (ESR) and decreased haemoglobin and folic acid levels.

Later, IgA and IgG type antireticulin and antigliadin antibodies were detected. Serum total IgA was elevated but no paraproteinaemia was found. In lesional skin, granular deposits of IgA and C3 were seen at the dermo-epidermal junction. A duodenal biopsy revealed total villous atrophy.

Dapsone treatment was partly effective but complete healing of the EED lesions was achieved only after the introduction of a strict gluten-free diet. The patient has now remained symptom-free on the diet for 1.5 years.

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11.) Erythema elevatum diutinum complicated by rheumatoid arthritis.

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Author

Nakajima H; Ikeda M; Yamamoto Y; Kodama H

Address

Department of Dermatology, Kochi Medical School, Japan.

Source

J Dermatol, 26(7):452-6 1999 Jul

Abstract

A 53-year-old female developed erythema elevatum diutinum (EED) twelve years after the onset of rheumatoid arthritis. The arthritis had been well controlled for the last several years.

Annular purpuric macules were characteristically complicated by common nodular and plaque lesions. Both leukocytoclastic vasculitis and fibrosis were observed in the macular lesions, indicating that the lesions were a manifestation of an early phase of EED.

Both types of skin lesions disappeared with treatment with dapsone. They have not relapsed for two years after stopping the dapsone. The leukocytoclastic vasculitis was thought to have developed independently of the rheumatoid arthritis. She had noticed sicca symptoms two years before the appearance of EED, but she did not satisfy the diagnostic criteria for Sj¨ogren's syndrome.

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12.) Erythema elevatum diutinum in human immunodeficiency virus-infected patients--report of a case and review of the literature [see comments]

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Author

Dronda F; Gonz´alez-L´opez A; Lecona M; Barros C

Address

Hospital General Penitenciario, Madrid, Spain.

Source

Clin Exp Dermatol, 21(3):222-5 1996 May

Abstract

Erythema elevatum diutinum (EED) is a rare chronic disease of unknown origin, part of the spectrum of cutaneous leucocytoclastic vasculitis. A case of EED in a 32-year-old HIV-infected male patient, with no previous opportunistic infections and a CD4+ cell count of less than 200/mm3, is reported.

Therapy with oral dapsone (100 mg/day for 15 days) resulted in clinical cure with no relapse after 6 months of follow-up. To our knowledge, only six cases of EED in HIV-positive patients have been reported to date. A brief review of these seven cases is described.

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13.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation.

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Author

Porneuf M; Duterque M; Sotto A; Jourdan J

Address

Department of Internal Medicine, University of Nimes, France.

Source

Br J Dermatol, 134(6):1131-4 1996 Jun

Abstract

A 78-year-old woman with a history of symmetrical erythematous plaques on the arms, and a monoclonal gammopathy, developed a strange striped reticulate papular dermatosis with central atrophy. Histological examination was compatible with a very late stage of erythema elevatum diutinum (EED), showing a fibrohistiocytic proliferation with areas of granulation tissue.

This fibrosis may result from the chronic dermal injury of leucocytoclastic vasculitis and is sometimes the predominant histology of EED. Investigations for underlying haematological anomalies, such as paraproteinaemia and infection with human immunodeficiency virus, must be performed. Dapsone is ineffective once the fibrous nodules have appeared.

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14.) POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.

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Author

Albitar S; Bourgeon B; Genin R; Jacquesson M; Riviere JP; Serveaux MO; Ribera A; Serveaux JP

Address

Hemodialysis Center, AURAR, 6 Avenue Stanislas Gimart, F-97490 Ste Clotilde de La Reunion, France.

Source

Am J Kidney Dis, 31(4):E3 1998 Apr

Abstract

POEMS syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, POEMS syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically.

Skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced.

However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that POEMS syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.

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15.) A continuum of neutrophilic disease occurring in a patient with ulcerative colitis.

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Author

Salmon P; Rademaker M; Edwards L

Address

Department of Dermatology, Waikato Hospital, Health Waikato, Hamilton, New Zealand.

Source

Australas J Dermatol, 39(2):116-8 1998 May

Abstract

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurring concurrently with bullous pyoderma gangrenosum is reported to emphasize the close relationship between these two disorders.

Atypical pyoderma gangrenosum and Sweet's syndrome have been described as occurring simultaneously in haematological dyscrasias but not, to our knowledge, in ulcerative colitis. It has been proposed that pyoderma gangrenosum, Sweet's syndrome, erythema elevatum diutinum and subcorneal pustular dermatosis may represent manifestations along a continuum of neutrophilic dermatoses.

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16.) Granuloma faciale with extrafacial lesions.

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Author

Roustan G; S´anchez Yus E; Salas C; Sim´on A

Address

Department of Dermatology, Cl´inica Universitaria Puerta de Hierro, Madrid, Espana.

Source

Dermatology, 198(1):79-82 1999

Abstract

Extrafacial involvement in granuloma faciale (GF) is rather exceptional. We report herein a patient with GF associated with lesions on the trunk and the forearm. Histological studies of facial and extrafacial lesions shared similar characteristics: a mixed inflammatory infiltrate with abundant eosinophils in the superficial and middle dermis with a narrow grenz zone of uninvolved dermis between the epidermis and the infiltrate.

Evidence of vasculitis was clearly observed in both biopsy specimens. Treatment with dapsone did not alter the course of the disease. We review the 12 cases of extrafacial GF that have been reported in the English and Spanish literature.

In these cases a diagnosis of erythema elevatum diutinum (EED) may be suggested. Although GF and EED may share some pathogenic mechanisms, there are several clinical and histological differences between them that make us consider EED and GF as distinct entities.

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17.) Erythema elevatum diutinum and HIV infection: a report of five cases.

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Br J Dermatol 1999 Aug;141(2):335-338

Muratori S, Carrera C, Gorani A, Alessi E

Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore, Via Pace 9, 20122 Milan, Italy.

Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated dermatosis, 11 cases having so far been reported in the medical literature and five patients with the disease having been seen by us during the last 4 years. As the disease is poorly known, it is easily confused with Kaposi's sarcoma or bacillary angiomatosis, but the histopathological features are diagnostic.

EED is considered to be an immune complex-mediated vasculitis. A streptococcal infection seemed to be the trigger factor in four of our patients. Partial control of the cutaneous lesions was achieved by the use of antibiotics.

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18.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases.

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Br J Dermatol 1998 May;138(5):893-7

Takiwaki H, Kubo Y, Tsuda H, Arase S, Shiota H

Department of Dermatology, School of Medicine, University of Tokushima, Japan.

Peripheral ulcerative keratitis (PUK) is a complication of collagen-vascular diseases such as rheumatoid arthritis (RA) and other systemic vasculitides. We report three cases of erythema elevatum diutinum with PUK. These patients presented with nodules and plaques consistent with erythema elevatum diutinum on the extremities and crusted or ulcerated purpuric lesions on the soles. Histopathological examination of these lesions revealed a dense neutrophilic infiltrate with nuclear dust and fibrin around blood vessels.

vAll the patients developed PUK concomitant with the development of the skin lesions. The rheumatoid factor was positive at high titre in all three patients; this was associated with probable RA in one.

Cutaneous lesions were dramatically improved by administration of dapsone in all cases. Dapsone was also effective in treating the ocular lesions in two patients.

============================================================

19.) Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale.

============================================================

Am J Surg Pathol 1997 Jun;21(6):698-705

Carlson JA, LeBoit PE

Department of Pathology, Albany Medical College, New York, USA.

Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic, localized forms of cutaneous leukocytoclastic vasculitis that result in patterned (storiform or concentric) fibrosis.

EED often occurs in systemically ill patients as bilaterally symmetrical plaques, papules, or nodules, often over the dorsa of joints. GF occurs as one or a few plaques on the face. Eosinophils and plasma cells are prominent in GF, whereas neutrophils are plentiful in EED.

Rarely, extrafacial lesions accompany facial ones in GF, and there are a few reports of upper respiratory tract masses with GF-like histologic features. We report on eight patients with solitary cutaneous lesions with histologic features similar to those of EED or GF, but whose clinical picture was not that of either disease. One, whose histology resembled GF, had a large multinodular dermal and subcutaneous mass that persisted despite attempted resection. Unusual histologic findings in other cases included storiform fibrosis with dense infiltrates of plasma cells, branching nerve fascicles admixed with EED-like changes, and EED-like areas adjacent to zones mimicking a sclerotic fibroma.

Chronic fibrosing venulitis can be seen outside the stereotypic settings of GF and EED and is an inflammatory reaction pattern that does not signify a specific diagnosis. Because of transitions between EED or GF-like areas and those of patterned sclerosis with plasma cell-rich infiltrates, we believe that some "inflammatory pseudotumors" of the skin, and perhaps of other sites could be the result of localized vasculitis.

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20.) [Erythema elevatum diutinum. A rare dermatosis with a broad spectrum of associated illnesses].

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Hautarzt 1997 Feb;48(2):113-7

Gerbig AW, Zala L, Hunziker T

Dermatologische Universitatsklinik, Bern.

Erythema elevatum diutinum (EED) is a rare disease presenting with persistent red to yellow-brown papules or plaques which are mainly localized symmetrically on the extensor aspects of the hands and fingers, the elbows and the knees.

The histology shows a leucocytoclastic vasculitis in early lesions and fibrosis of the dermis later on. Dapsone is the treatment of choice. Today, EED is usually assigned to the neutrophilic dermatoses in which an association with hematological disturbances is well documented. We report on a patient with EED and glioma WHO grade IV, a coincidence unreported hitherto. Additionally, we review the literature on diseases associated with EED.

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21.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation.

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Br J Dermatol 1996 Jun;134(6):1131-4

Porneuf M, Duterque M, Sotto A, Jourdan J

Department of Internal Medicine, University of Nimes, France.

A 78-year-old woman with a history of symmetrical erythematous plaques on the arms, and a monoclonal gammopathy, developed a strange striped reticulate papular dermatosis with central atrophy. Histological examination was compatible with a very late stage of erythema elevatum diutinum (EED), showing a fibrohistiocytic proliferation with areas of granulation tissue.

This fibrosis may result from the chronic dermal injury of leucocytoclastic vasculitis and is sometimes the predominant histology of EED. Investigations for underlying haematological anomalies, such as paraproteinaemia and infection with human immunodeficiency virus, must be performed. Dapsone is ineffective once the fibrous nodules have appeared.

============================================================

22.) Erythema elevatum diutinum in human immunodeficiency virus-infected patients--report of a case and review of the literature.

============================================================

Clin Exp Dermatol 1996 May;21(3):222-5

Dronda F, Gonzalez-Lopez A, Lecona M, Barros C

Hospital General Penitenciario, Madrid, Spain.

Erythema elevatum diutinum (EED) is a rare chronic disease of unknown origin, part of the spectrum of cutaneous leucocytoclastic vasculitis. A case of EED in a 32-year-old HIV-infected male patient, with no previous opportunistic infections and a CD4+ cell count of less than 200/mm3, is reported.

Therapy with oral dapsone (100 mg/day for 15 days) resulted in clinical cure with no relapse after 6 months of follow-up. To our knowledge, only six cases of EED in HIV-positive patients have been reported to date. A brief review of these seven cases is described.

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23.) Cutaneous vasculitis.

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Rheum Dis Clin North Am 1995 Nov;21(4):1097-113

Gibson LE, Su WP

Mayo Clinic, Rochester, Minnesota, USA.

Cutaneous vasculitis comprises a wide spectrum of clinical syndromes and histopathologic findings that share the common theme of vascular inflammation and blood vessel damage. Clinical pattern and proper histopathologic studies are essential to the proper classification and evaluation of vasculitis.

This article discusses the major types of cutaneous vasculitis typified by necrotizing or leukocytoclastic vasculitis and includes discussions of Henoch-Schonlein purpura, urticarial vasculitis, erythema elevatum diutinum, cutaneous polyarteritis nodosa, livedoid vasculitis, and cutaneous granulomatous vasculitis.

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24.) Pyoderma gangrenosum, erythema elevatum diutinum and IgA monoclonal gammopathy.

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Australas J Dermatol 1995 Feb;36(1):21-3

Wayte JA, Rogers S, Powell FC

Regional Centre of Dermatology, Mater Misericordiae Hospital, Dublin, Ireland.

A patient with erythema elevatum diutinum (EED) developed pyoderma gangrenosum (PG). Investigation revealed an IgA kappa monoclonal gammopathy. Previous reports of PG in association with EED are reviewed and the spectrum of the neutrophilic dermatoses discussed.

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25.) Simultaneous occurrence of two rare cutaneous markers of poor prognosis in myelodysplastic syndrome: erythema elevatum diutinum and specific lesions.

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Br J Dermatol 1994 Jul;131(1):112-7

Aractingi S, Bachmeyer C, Dombret H, Vignon-Pennamen D, Degos L, Dubertret L

Department of Dermatology, H pital Saint-Louis, Paris, France.

We report the concomitant occurrence of erythema elevatum diutinum and specific skin lesions in a patient with a myelodysplastic syndrome (MDS).

This patient's course, and review of other reported cases, support the opinion that neutrophilic dermatoses are associated with a poor prognosis of MDS. The simultaneous appearance of these manifestations could be the consequence of a particular chemotactism of myeloid cells, expressed after acute transformation.

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26.) Erythema elevatum diutinum: case report showing an unusual distribution.

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Cutis 1994 Mar;53(3):124-6

Hansen U, Haerslev T, Knudsen B, Jacobsen GK

Department of Pathology, Gentofte Hospital, University of Copenhagen, Denmark.

A case of erythema elevatum diutinum showing an unusual distribution is reported. Hard nodules were located on the patient's fingertips and pulps while the extensor surfaces were not involved.

The disease was initially misinterpreted based on both clinical and histologic examination. Dapsone therapy was followed by a remarkable recovery. The clinical and histologic features of erythema elevatum diutinum are summarized.

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27.) Ultrastructural study of chronic lesions of erythema elevatum diutinum: "extracellular cholesterosis" is a misnomer.

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J Am Acad Dermatol 1993 Aug;29(2 Pt 2):363-7

Kanitakis J, Cozzani E, Lyonnet S, Thivolet J

Department of Dermatology, Ed. Herriot Hospital, Lyon, France.

Erythema elevatum diutinum (EED) is a rare disease of unknown origin that belongs to the spectrum of leukocytoclastic vasculitis. Chronic lesions of EED contain lipid deposits, for which the term extracellular cholesterosis has been coined. We studied a typical case of EED with long-standing lesions.

Findings of electron microscopic examination revealed a heavy, exclusively intracellular lipid deposition that consisted of lipid droplets, myelin figures, and rare cholesterol clefts within histiocytes but also within epidermal keratinocytes, mast cells, pericytes, and lymphocytes.

These findings are in keeping with the results of previous ultrastructural studies and suggest that the term extracellular cholesterosis is a misnomer; intracellular lipidosis would more accurately describe the lipid deposition.

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28.) Nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus.

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J Am Acad Dermatol 1993 Jun;28(6):919-22

LeBoit PE, Cockerell CJ

Department of Pathology, School of Medicine, University of California, San Francisco 94143-0506.

BACKGROUND: Erythema elevatum diutinum (EED) is a rare, chronic form of cutaneous vasculitis that can result in fibrosis. Four patients infected with the human immunodeficiency virus (HIV) had unusual nodular lesions of EED.

OBJECTIVE: Our purpose was to document the occurrence of these lesions in HIV-infected patients and to characterize the histopathologic and immunophenotypic features of nodular EED. METHODS: Clinicopathologic studies were supplemented by special stains of biopsy specimens.

RESULTS: EED may be a complication of HIV infection. Nodules of EED are composed of small aggregates of Mac-387+ spindled cells. Within the aggregates can be seen neutrophils, nuclear dust, and fibrin. The marked fibrosis of the lesions may account for the lack of response to dapsone.

CONCLUSION: EED should be considered in the differential diagnosis of juxta-articular nodules in HIV-infected patients; its diagnosis can be confirmed by biopsy.

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29.) Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet.

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Pediatr Dermatol 1993 Jun;10(2):125-8

Rodriguez-Serna M, Fortea JM, Perez A, Febrer I, Ribes C, Aliaga A

Department of Dermatology, Hospital General, Valencia, Spain.

An 11-year-old girl with a history of insulin-dependent diabetes mellitus had erythema elevatum diutinum (EED) associated with a celiac disease related to a possible kidney disease.

Dapsone did not improve the skin manifestations. However, the lesions disappeared after a gluten-free diet was begun. To our knowledge, this report describes the first case of EED in a patient with celiac disease.

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30.) Association of hyperimmunoglobulinaemia D syndrome with erythema elevatum diutinum.

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Br J Dermatol 1993 May;128(5):572-4

Miyagawa S, Kitamura W, Morita K, Saishin M, Shirai T

Department of Dermatology, Nara Medical University, Japan.

Hyperimmunoglobulinaemia D and periodic fever syndrome was observed in a female patient with erythema elevatum diutinum. The association of this skin disease with hyperimmunoglobulinaemia D may indicate a pathogenetic relationship. The skin lesions responded to dapsone therapy.

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31.) Erythema elevatum diutinum associated with Wegener's granulomatosis and IgA paraproteinemia.

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J Am Acad Dermatol 1993 May;28(5 Pt 2):846-9

Kavanagh GM, Colaco CB, Bradfield JW, Archer CB

Department of Dermatology, Bristol Royal Infirmary, University of Bristol, United Kingdom.

A 69-year-old man had erythema elevatum diutinum for several years before he developed IgA paraproteinemia and a limited form of Wegener's granulomatosis. This is the first report of an association between erythema elevatum diutinum and Wegener's granulomatosis. IgA paraproteinemia has been reported in association with erythema elevatum diutinum but not with Wegener's granulomatosis.

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32.) Pyoderma gangrenosum in association with erythema elevatum diutinum: report of two cases.

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Cutis 1992 Mar;49(3):261-6

Planaguma M, Puig L, Alomar A, Matias-Guiu X, de Moragas JM

Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Autonomous University of Barcelona, Spain.

We report on the appearance of centrifugally spreading ulcers with undermined borders in two patients with chronic recurrent erythema elevatum diutinum controlled with dapsone.

The ulcerated lesions were consistent on clinical and pathologic examination with the diagnosis of pyoderma gangrenosum. They eventually responded to treatment with oral corticosteroids. The addition of cyclosporine was required in one case. No associated disease was found in any of the patients. The possible pathophysiological mechanisms of this uncommon association are reviewed.

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33.) Erythema elevatum diutinum: a clinicopathological study.

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Clin Exp Dermatol 1992 Mar;17(2):87-93

Wilkinson SM, English JS, Smith NP, Wilson-Jones E, Winkelmann RK

Department of Dermatology, North Staffordshire Hospital Centre, Stoke-on-Trent, U.K.

Erythema elevatum diutinum is a syndrome of vasculitis in which lesions, typically over the extensor surfaces, showed a mixed inflammatory infiltrate on biopsy. We describe a series of 13 patients.

The most common association in our series was with hypergammaglobulinemia; both mono and polyclonal. Chronic infection, not streptococcal, was a less frequent finding although two of three patients had a positive reaction to the intradermal injection of streptococcal antigen. Dapsone remains the initial treatment of choice.

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34.) Erythema elevatum diutinum in a patient with relapsing polychondritis.

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J Am Acad Dermatol 1992 Feb;26(2 Pt 2):312-5

Bernard P, Bedane C, Delrous JL, Catanzano G, Bonnetblanc JM

Department of Dermatology, CHRU Dupuytren, Limoges, France.

A 69-year-old man with a history of relapsing polychondritis developed a widespread cutaneous eruption that was clinically and histologically typical of erythema elevatum diutinum. This eruption was accompanied by a recurrence of arthralgias. The significance of this association between relapsing polychondritis and erythema elevatum diutinum, which has not been previously reported, is discussed.

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35.) Erythema elevatum diutinum: a clinical and histopathologic study of 13 patients.

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J Am Acad Dermatol 1992 Jan;26(1):38-44

Yiannias JA, el-Azhary RA, Gibson LE

Department of Dermatology, Mayo Clinic, Rochester, MN 55905.

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.

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36.) Erythema elevatum diutinum in a patient with acquired immunodeficiency syndrome. Another clinical simulator of Kaposi's sarcoma.

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Arch Dermatol 1991 Dec;127(12):1819-22

Requena L, Sanchez Yus E, Martin L, Barat A, Arias D

Department of Dermatology, Jimenez Diaz Foundation, Nuestra Senora de la Concepcion Clinic, Universidad Autonoma, Madrid, Spain.

Several types of vasculitis have been described in patients with human immunodeficiency virus infection. Erythema elevatum diutinum is a rare variant of cutaneous leukocytoclastic vasculitis which, with the exception of the case reported herein, has been described only once in human immunodeficiency virus-infected patients.

Our male patient, a longtime intravenous drug abuser, had cutaneous lesions, closely resembling Kaposi's sarcoma, on the extensor surfaces of the lower extremities. Cutaneous biopsy specimens, however, demonstrated leukocytoclastic vasculitis with fibrinoid necrosis of the vessel walls and areas of basophilic degeneration of collagen bundles in early lesions, whereas late lesions showed dense diffuse fibrosis with proliferation of dermal spindle cells and some foci of residual leukocytoclastic vasculitis.

Oral therapy with dapsone resulted in marked clearing of the cutaneous lesions within few days. This case raises the necessity of histologic confirmation for all cases of suspected Kaposi's sarcoma in patients with acquired immunodeficiency syndrome. We discuss the possible pathogenesis of leukocytoclastic vasculitis in human immunodeficiency virus-infected patients.

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37.) Erythema elevatum diutinum--an unusual association with ulcerative colitis.

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Clin Exp Dermatol 1991 May;16(3):204-6

Buahene K, Hudson M, Mowat A, Smart L, Ormerod AD

Department of Dermatology, Aberdeen Royal Infirmary, UK.

We report the association of erythema elevatum diutinum (EED) with ulcerative colitis (UC). The patient, whose history included rheumatoid disease, diabetes mellitus (type I) and hypothyroidism developed EED during a severe acute exacerbation of UC which resolved following colectomy.

To our knowledge this is the first report of such an association. Also of interest was the observation of Koebner phenomenon occurring at the sites where blood vessels were damaged.

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38.) Erythema elevatum diutinum mimicking porphyria cutanea tarda.

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Br J Dermatol 1991 Jan;124(1):89-91

Requena L, Barat A, Hasson A, Arias D, Gutierrez MC, Martin L, De Castro A

Department of Dermatology, Universidad Autonoma, Madrid, Spain.

A case of erythema elevatum diutinum (EED) closely resembling porphyria cutanea tarda (PCT) is reported. The initial skin biopsies were suggestive for PCT but porphyrin levels in the urine, stool and plasma were normal. A further biopsy from an early cutaneous lesion showed a leucocytoclastic vasculitis with fibrinoid necrosis of the vessel walls.

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39.) Cutaneous manifestations of neutrophilic disease. A study of seven cases.

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Dermatologica 1991;183(4):255-64

Vignon-Pennamen MD, Wallach D

Department of Dermatology, Hopital Saint-Louis, Paris, France.

Seven patients with a complex form of neutrophilic dermatosis are reported. Clinically, they had variable associations of four types of lesions: blisters/pustules, plaques, nodules and ulcerations.

Histologically, a neutrophilic infiltrate was observed at variable levels in the epidermis, dermis and subcutis. Systemic manifestations were present in all cases (general symptoms, joint, renal, ocular and lung involvements). Three patients had an associated disease (myelodysplasia, metastatic carcinoma, IgG gammopathy).

Steroids were the most efficient treatment. These observations, as well as a review of the literature, support the opinion that the neutrophilic dermatosis represents a continuous spectrum encompassing four well-defined entities: subcorneal pustular dermatosis, Sweet's syndrome, erythema elevatum diutinum and pyoderma gangrenosum. We propose that the different patterns of the neutrophilic dermatosis are the most obvious manifestations of a potentially multisystemic neutrophilic disease and allow its recognition.

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40.) Erythema elevatum diutinum--a solitary lesion in a patient with rheumatoid arthritis.

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Clin Exp Dermatol 1990 Sep;15(5):394-5

Collier PM, Neill SM, Branfoot AC, Staughton RC

Department of Dermatology, Westminster Hospital, London, UK.

Erythema elevatum diutinum is a rare disease of unknown aetiology. It is usually symmetrical with multiple lesions. An association with rheumatoid has previously been reported. We report a case of atypical erythema elevatum diutinum affecting the right elbow of a female patient with seropositive rheumatoid arthritis.

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41.) Erythema elevatum diutinum in a patient with Crohn's disease.

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J Am Acad Dermatol 1990 May;22(5 Pt 2):948-52

Walker KD, Badame AJ

Department of Dermatology, Tulane University Medical School, New Orleans, LA 70112.

A possible association between erythema elevatum diutinum and Crohn's disease is described. To our knowledge, erythema elevation diutinum has never been reported in a patient with Crohn's disease, nor has the disorder been considered one of the cutaneous manifestations of inflammatory bowel disease.

Erythema elevatum diutinum is believed to be mediated by the deposition of circulating immune complexes in the dermal perivascular spaces. Our patient showed clinical and histopathologic findings typical of both erythema elevatum diutinum and Crohn's disease. The patient also had a positive Raji cell assay, which demonstrates the presence of a circulating immune complex.

Increased levels of circulating immune complexes have been found in patients with inflammatory bowel disease, and the gastrointestinal inflammation in Crohn's disease could lead to enhanced antigenic exposure and increased formation of immune complexes. We contend that inflammatory bowel disease may have contributed to the development of erythema elevatum diutinum in our patient.

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42.) The natural history of vasculitis. What the histology tells us about pathogenesis.

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Arch Dermatol 1990 Jan;126(1):84-9

Smoller BR, McNutt NS, Contreras F

Department of Medicine (Dermatology), New York Hospital-Cornell University Medical Center, NY 10021.

While histopathologic analysis may offer some clues as to the pathogenesis of vasculitis, observations must be interpreted with caution, as there is considerable overlap in the histologic pattern. In most cases, a predominantly neutrophilic vasculitis affecting small dermal venules suggests a relatively acute, immune complex-mediated reaction. Less commonly, this histologic pattern may be seen in non-immunologically mediated processes, such as in the presence of bacterial toxins or malignant hypertension, or in more chronic disease states, such as granuloma faciale or erythema elevatum diutinum.

A predominantly lymphocytic vasculitis may represent several pathogenetic mechanisms. In lesions more than 24 to 48 hours old, a lymphocytic vasculitis may represent a resolving phase of an immune complex-mediated neutrophilic vasculitis. Alternatively, this histologic pattern may be seen de novo in conditions with a presumed cell-mediated immunologic pathogenesis. Lymphocytic vasculitis may also be seen in rickettsial infections such as Rocky Mountain spotted fever.

The pathogenesis of granulomatous vasculitis remains poorly understood and is thought to be induced by a combination of circulating immune complexes and a cell-mediated immune response.

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43.) Pleural effusions in an overlap syndrome of idiopathic hypereosinophilic syndrome and erythema elevatum diutinum.

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Eur Respir J 1990 Jan;3(1):115-8

Cordier JF, Faure M, Hermier C, Brune J

Dept. of Pneumology, Hopital Louis Pradel, Universite Claude Bernard, Lyon, France.

We report a patient with idiopathic hypereosinophilic syndrome presenting with bilateral eosinophilic pleural effusions. He also had erythema elevatum diutinum, a rare skin disease of the vasculitic type. No cardiomyopathy was present. Pleural effusions, skin lesions, and blood eosinophilia disappeared with prolonged corticosteroid treatment.

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44.) Erythema elevatum diutinum: an ultrastructural case study.

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J Cutan Pathol 1989 Aug;16(4):211-7

Lee AY, Nakagawa H, Nogita T, Ishibashi Y

Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.

An electron microscopic study was made of a patient with erythema elevatum diutinum, a rare variant of cutaneous leukocytoclastic vasculitis. Biopsies were obtained from early evolving and late fibroproliferative lesions. Early lesions showed vasculitis and a massive dermal infiltrate composed mainly of neutrophils, histiocytes/macrophages and apparently increased Langerhans cells.

Later lesions were characterized by a dense fibrosis with a proliferation of fibroblasts and myofibroblasts and a dermal infiltrate in which lymphocytes and histiocytes/macrophages predominated. Even in later lesions, dermal Langerhans cells were observed in great number. Langerhans cells may be involved in the evolution of this rare disease.

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45.) [Erythema elevatum diutinum--a rare variant of skin changes in systemic lupus erythematosus].

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Dermatol Monatsschr 1989;175(10):628-34

Schulze P, Audring H, Sonnichsen N

It is reported on 5 patients with erythema elevatum diutinum, in 2 of these cases exists a systemic lupus erythematosus and another patient suffers from cutaneous lupus erythematosus. This information can be considered as a possibility that cutaneous alterations similar to erythema elevatum diutinum could be the first symptoms of systemic lupus erythematosus when the usual treatment is inefficient.

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DATA-MÉDICOS/DERMAGIC-EXPRESS No 2-(81)  24/11/2000 DR. JOSÉ LAPENTA R. 

UPDATED 30 JUNE 2025

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Produced by Dr. José Lapenta R. Dermatologist

Venezuela 1.998-2.025

Producido por Dr. José Lapenta R. Dermatólogo Venezuela 1.998-2.025

Tlf: 0414-2976087 - 04127766810