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PubMed2002

Elevated platelet counts and thrombocytosis in erythema nodosum leprosum.

Changes in peripheral blood platelet counts associated with the onset of symptomatic erythema nodosum leprosum (ENL) were studied by comparing, in each patient, the value obtained on the day thalidomide therapy commenced with the average of the three preceding values. In the 11 patients studied, the mean platelet count rose from 235 to 322 x 10(3)/mm3, p < 0.001. In 3, the platelet count was above the normal limit, qualifying as thrombocytosis, in 7 the rise was appreciable, and in 2 it was negligible. In the 3 patients studied 1-2 weeks after beginning thalidomide, the mean count was 414 x 10(3)/mm3. Counts obtained after 3 or more weeks of thalidomide therapy were within normal limits. This study provided no direct evidence as to the mechanism responsible for the elevated platelet count, but mediation by interleukin-6 (IL-6) was concluded to be an attractive hypothesis, consistent with prior studies of IL-6 in reactive thrombocytosis and of IL-6 in ENL.

Adult↗
PubMed1976

Erythema nodosum.

Erythema nodosum (EN) is now more commonly associated with streptococcal disease than with tuberculosis. Sarcoidosis is the third most frequently associated systemic disease. Oral contraceptives now lead the list of drugs associated with EN. The appearance and disappearance of crops of nodules is extremely variable. Salicylates and rest are useful. Steroids are not recommended because they may compromise defenses against an underlying disease process.

Diagnosis, Differential↗
PubMed2003

Pentoxifylline in the treatment of erythema nodosum leprosum.

Erythema nodosum leprosum (ENL) is a well-known serious complication affecting 10% of lepromatous multibacillary leprosy patients. In the chronic form, its morbidity may be considerable. Thalidomide and systemic steroids are the two current effective drugs for the management of ENL. However, their use in endemic countries is often difficult and hazardous, and a search for new therapies is needed. We report our experience on the effects of pentoxifylline, a methylxanthine derivative, which has recently been suggested as a possible effective treatment for ENL attacks.

Administration, Oral↗
PubMed2003

Herpes simplex virus (HSV)-associated erythema multiforme (HAEM): a viral disease with an autoimmune component.

Erythema multiforme (EM) is a clinical conundrum the name of which reflects the broad morphological spectrum of the lesions. Molecular and immunologic evidence that herpes simplex virus (HSV) causes a subset of EM lesions [herpes-associated EM (HAEM)] is reviewed, and new data are presented which suggest that autoreactive T-cells triggered by virus infection play an important role in HAEM pathogenesis. Disease development begins with viral DNA fragmentation and the transport of the DNA fragments to distant skin sites by peripheral blood mononuclear cells (PBMCs). HSV genes within DNA fragments deposited on the skin [notably DNA polymerase (Pol)] are expressed, leading to recruitment of HSV-specific CD4+ Th1 cells that respond to viral antigens with production of interferon-gamma (IFN-gamma). This step initiates an inflammatory cascade that includes expression of IFN-gamma induced genes, increased sequestration of circulating leukocytes, monocytes and natural killer (NK) cells, and the recruitment of autoreactive T-cells generated by molecular mimicry or the release of cellular antigens from lysed cells. The PBMCs that pick up the HSV DNA [viz. macrophages or CD34+ Langerhans cells (LC) precursors], their ability to process it, the viral proteins expressed in the skin and the presence of epitopes shared with cellular proteins may determine whether a specific HSV episode is followed by HAEM development. Drug-associated EM (DIEM) is a mechanistically distinct EM subset that involves expression of tumor necrosis factor alpha (TNF-alpha) in lesional skin. It is our thesis that the polymerase chain reaction (PCR) assay for HSV DNA detection in lesional skin and staining with antibodies to IFN-gamma and TNF-alpha, are important criteria for the diagnosis of skin eruptions and improved patient management.

Antigens, Surface↗
PubMed2003

Erythema elevatum diutinum in an HIV-positive patient.

A 53-year old man with HIV infection and hepatitis C infection presented with multiple, firm nodules on the hands and ankles. Erythema elevatum diutinum is a rare, chronic cutaneous vasculitis that may be associated with hematologic, autoimmune, and infectious diseases, which include HIV infection. First-line therapy includes dapsone, as well as treatment of any underlying cause or infection.

Anti-HIV Agents↗
PubMed1992

Epstein-Barr virus-related persistent erythema multiforme in chronic fatigue syndrome.

BACKGROUND: Erythema multiforme (EM) has been rarely reported in Epstein-Barr virus (EBV)-associated diseases; this includes patients with chronic fatigue syndrome who have chronic or recurrent and disabling illness and an abnormal antibody reactivity to EBV. We describe a patient fulfilling the chronic fatigue syndrome diagnostic criteria who had developed an unusually persistent EM resistant to corticosteroids therapy. The EBV DNA was studied in skin EM lesions, throat washings, peripheral mononuclear cells, and plasma. The EBV antigens were studied in skin EM lesions and in mononuclear cells. The patient was followed up to 2 years. OBSERVATIONS: The patient had abnormal titers of antibodies against various EBV antigens and by immunofluorescence she disclosed the EBV nuclear antigen and the viral capsid antigen in the blood vessels of the affected skin. The dot blot hybridization assay detected viral DNA in throat washings and mononuclear cells, but not in plasma. The presence of the viral genomic content in lesional skin is suggested by the autoradiographic signal and by the difference from appropriate control specimens. Skin lesions and constitutional symptoms cleared after acyclovir sodium therapy and recurred after discontinuation of this therapy. CONCLUSIONS: This is the first EM case in which evidence of the EBV causal role has been provided. The association with chronic fatigue syndrome suggests the EBV role in selected cases of this syndrome.

Adult↗
PubMedDate not supplied

Topical chloroquine applied before irradiation protects against ultraviolet B (UVB)- and UVA-induced erythema but not against immediate pigment darkening.

Seventeen healthy volunteers were phototested with ultraviolet B (UVB) and UVA before and after topical treatment with chloroquine phosphate. The skin areas treated before but not after irradiation showed higher minimal erythema dose values for UVB and UVA than control skin. The effect was clearly spectral with greater protection afforded against UVB than UVA. The immediate pigment darkening after irradiation with UVA, however, was not affected by pretreatment with the drug. The mechanism of action for this protective effect did not seem to be related to merely absorption and screening, inhibition of the inflammatory reaction or a UV-induced effect on the stereo-isomerization of the drug.

Administration, Cutaneous↗
PubMed1992

T lymphocytes expressing HECA-452 epitope are present in cutaneous acute graft-versus-host disease and erythema multiforme, but not in acute graft-versus-host disease in gut organs.

Lymphocytes in formalin-fixed skin biopsies from patients with cutaneous acute graft-versus-host disease (aGVHD) were studied with HECA-452 (an antibody recognizing lymphocytes with skin-homing properties) and a panel of antibodies recognizing pan-B (L26 [CD20]), pan-T (L60 [CD43] and A6 [CD45RA]), and T-helper subset (OPD4) antigens in paraffin sections. Biopsies from patients with erythema multiforme (EM) were similarly studied for comparison. In both conditions, T lymphocytes stained by OPD4 were predominantly confined to the dermis, whereas those stained by HECA-452 were concentrated in the epidermis; however, there was considerable variation between cases, and overlap between findings in the dermis and epidermis. Lymphocytes similarly studied in paraffin sections of liver, salivary gland, and gut affected by aGVHD were essentially unreactive with HECA-452, although they were largely stained by pan-T markers and showed some comparable reactivity with OPD4. The findings suggest that aGVHD of the skin is mediated by a different set of lymphocytes than in gut organs, and may have a similar immunologic mechanism to EM.

Acute Disease↗
PubMed1992

[Erythema nodosum and non-Hodgkin lymphoma].

A 36-year-old woman presenting with erythematous painful tender inflammatory nodular lesions on both knees, shins and ankles is reported. Erythema nodosum (EN) was diagnosed. Further clinical examination revealed hilar lymphadenopathy. A lymph node was removed for histological examination, and a centroblastic non-Hodgkin lymphoma was diagnosed. Remarkably, in this patient EN occurred before the haematological malignancy was detected.

Adult↗
PubMed1977

Erythema elevatum diutinum: skin and systemic manifestations, immunologic studies, and successful treatment with dapsone.

Erythema elevatum diutinum is a disease characterized by red, pink, purple and yellow cutaneous papules, nodules, and plaques distributed mainly over extensor surfaces. Histologically, there is leukocytoclastic vasculitis with fibrinoid necrosis of the upper and mid dermal vessel walls. Two of the five patients presented had moderately severe arthralgias and, unlike previously reported cases, three of them had a long history of bacterial infections and one had a coincident IgA monoclonal gammopathy. Each of the four patients treated with dapsone responded dramatically with rapid resolution of existing lesions and marked diminution of systemic symptoms. Skin lesions and systemic complaints recurred within 12 to 48 hours after discontinuing dapsone. Four of the patients had Arthus-like reactions to SKSD skin tests prior to therapy, with a marked decrease in the response after institution of therapy. Neutrophil chemotactic responsiveness of two patients was impaired. The sera of three patients had significant C1q binding activity suggesting that they might have circulating immune complexes.

Adolescent↗
PubMed2003

Erythema dyschromicum perstans and vitiligo.

We report an East Indian man with simultaneous lesions of erythema dyschromicum perstans and vitiligo. This report is only the second to present these two pigmentary disorders occurring in the same patient. Although the exact cause of both disorders is unknown, a pathogenetic role for cytotoxic T-cell responses has been hypothesized in each condition.

Adult↗
PubMed2003

Erythema elevatum diutinum.

A 53-year-old man with human-immunodeficiency-virus infection presents with a 2-year history of nodules on the extensor surfaces of his extremities. A biopsy confirmed the diagnosis of erythema elevatum diutinum. The clinical presentation, histopathology, etiology, and treatment options are reviewed.

Erythema↗
PubMedDate not supplied

A case of hereditary angioedema with recurrent arthritis, erythema marginatum-like rash and chest pain.

Hereditary angioedema (HAE) results from a congenital deficiency of C1 inhibitor and is characterized by submucosal and subcutaneous edema of skin, larynx and abdomen. Occasional reports have appeared linking HAE with autoimmune diseases. We report a case of HAE presenting recurrent nondeforming polyarthritis, erythema marginatum-like rash and chest pain. There were no significant radiographic joint changes. Serologic tests for rheumatologic and autoimmune diseases were negative. After danazol treatment, physical examination and laboratory findings were normal over five years. We suggest that pediatricians should be aware of this rare disease and treat patients accordingly.

Adolescent↗
PubMed1992

The effect of topical L-selenomethionine on minimal erythema dose of ultraviolet irradiation in humans.

The purpose of this study was to determine whether topical L-selenomethionine reduces the degree of acute damage to the skin (i.e., sunburn) induced by ultraviolet (UV) irradiation in humans. Eight women volunteers were treated for 2 weeks first with lotion vehicle then with topical L-selenomethionine for two weeks at each of three concentrations (0.002%, 0.02%, and 0.05%). At the end of each 2-week treatment period, the minimal erythema dose (MED) of UV irradiation was measured using a Multiport Solar Ultraviolet Simulator. It was found that topical L-selenomethionine was effective in protecting against acute UV damage to the skin. A response curve demonstrated that increasing concentrations of topical L-selenomethionine gave increasing MED, with a plateau of maximal protection attained at a concentration between 0.02% and 0.05%. Although previous studies in rats and mice demonstrated percutaneous absorption of selenium after application of L-selenomethionine, in the concentrations used here, the topical L-selenomethionine did not result in increased levels of selenium in the blood plasma of the patients tested.

Administration, Cutaneous↗
PubMed1992

[Study of 94 cases of sarcoidosis with special reference to erythema nodosum].

We studied 94 patients diagnosed of sarcoidosis in order to establish the incidence of erythema nodosum (EN), in the course of sarcoidosis, in our setting and to check if it induces different clinical and analytical characteristics in patients. 37 patients (39%) showed EN with a mean age of 41 +/- 1.62 years and with a marked female predominance (81%). Its presence was associated with a higher frequency of general symptoms (p less than 0.01), articular manifestations (p less than 0.001), specially in knees and ankles (p less than 0.01), subacute evolution (p less than 0.001), raise in GSR and of alpha-one-globulin (p less than 0.01) and hypoalbuminemia (p less than 0.05). On the other hand, those patients without EN showed higher frequency of dermal lesions different from EN (p less than 0.001) and the articular manifestations were localized preferentially in knees and proximal interphalangeal articulations of hands (p less than 0.05). We underline the high incidence of EN in sarcoidosis in our setting, which originates a more benign clinical type with a more favorable prognostic, embedded in the course of sarcoidosis, to which EN would transfer its clinical, analytical and immunopathogenic characteristics.

Adolescent↗
PubMed2003

[Necrolytic migratory erythema revealing glucagonoma without diabetes].

We report a case of glucagonoma syndrome, revealed by a necrolytic migratory erythema that had developed for four Years, associated with anorexia, severe weight loss, anemia, hypoprotidemia, and hypoaminoacidemia. The fasting blood glucose level tended paradoxically to be low (0.6 g/l). Elevated plasma glucagon levels confirmed our diagnosis. The absence of diabetes was explained by an independent insulin secretion derived from this composite pancreatic tumor, authenticated by the histological analysis and the proinsulin level. This level was similar to those typically observed in insulinomas. Six Months after a complete surgical exeresis, symptoms disappeared and biological results returned to normal values.

Aged↗
PubMed2003

[Histoid leprosy with erythema nodosum leprosum].

Histoid leprosy is a particular variant of lepromatous leprosy presenting as cutaneous or subcutaneous nodular and/or plaque-like lesions arising form apparently normal skin. It is characterized histologically by spindle-shaped histiocytes in interlacing bundles and whorls, containing numerous intact and rod-shaped Mycobacterium leprae. It can occur de novo or secondary in patients treated for a long course by dapsone alone. We describe a case of lepromatous leprosy treated according to the national Moroccan protocol who developed histoid lesions during his treatment by dapsone. The patient responded well to fluoroquinolone, rifampicin and clofazimine, with however, the occurrence of erythema nodosum leprosum.

Adult↗