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PubMed2003

[Pentoxifylline in the treatment of erythema nodosum leprosum: results of an open study].

Erythema nodosum leprosum (ENL) is a well-known immunological serious complication affecting lepromatous multibacillary leprosy patients. For a long time, ENL has been regarded as an immune complex-mediated disease or Arthus phenomenon. Recently, it has been reported that ENL was associated with high serum tumor necrosis factor-alpha (TNFa) levels, suggesting that this cytokine could also play a central role in the manifestations of ENL. Thalidomide (TH) and systemic steroids (S), both TNFa production inhibitors, are the two current effective drugs for the management of ENL. However, TH is rarely available in leprosy endemic countries, and its teratogenicity and neurotoxicity strongly limit its use. Moreover, the morbidity of S and the frequent steroid-dependence of ENL also create real therapeutic problems. Recently, the efficacy of pentoxifylline (PTX), which also inhibits in vitro and in vivo production of TNFa, has been suggested for ENL treatment. We report our experience on its use for the treatment of 15 leprosy patients suffering from a first ENL. attack. (11 cases), a chronic steroid-dependent ENL (3 cases) or chronic steroid- and thalidomide-dependent ENL (1 case). PTX has been given at 800 mg t.i.d, (2 cases) or 400 mg t.i.d. (13 cases) doses. The patients received PTX at the initiating dosage until complete clinical cure. At the end of ENL attacks, PTX was either abruptly stopped or tapered down over the next 4 months. In ten of 11 patients who developed ENL for the first time, the systemic symptoms and neuritic pains disappeared within one week; at three weeks, half of the patients were cured and the other half had striking clinical improvement; complete cure was obtained within 7 to 35 days (mean: 27 days). A relapse occurred within 2-3 months in the 5 patients, in which PTX was abruptly stopped. In contrast, no relapse occurred in the patients who benefited from decreasing doses of PTX. Recurrent ENL episodes also responded well to PTX. The 3 patients who had chronic steroid-dependent ENL failed to show any improvement after 3 to 6 weeks of PTX. In contrast, steroid therapy could be stopped in the steroid- and thalidomide-dependent patient. Our results confirm the action of PTX if it is slowly tapered down (4 months seem sufficient) and not abruptly to avoid relapses. As it is safe use, PTX could constitute the first line of ENL attack treatment.

Adolescent↗
PubMedDate not supplied

Palmar erythema due to topiramate.

Topiramate is an anti-convulsant often used off-label for the treatment of several conditions, including obesity and schizophrenia. This case report describes palmar erythema as a side effect in a patient using topiramate for paranoid schizophrenia.

Adult↗
PubMed1992

[Pigmented urticarial erythema with flaccid atrophy as expression of an unusual manifestation of Lyme borreliosis].

Unusual courses of infections with borrelia burgdorferi can make diagnosis problematic, but early and adequate therapy is mandatory to avoid further complications. In this paper a patient is presented who developed urticarial erythemas and hyperpigmentation with slack skin atrophy. Positive serological findings and the regression of the skin eruptions under antibiotic therapy confirmed the diagnosis of an unusual manifestation of Lyme borreliosis.

Atrophy↗
PubMed2004

Etiology and pathogenesis of necrolytic migratory erythema: review of the literature.

CONTEXT: Necrolytic migratory erythema (NME) is a characteristic skin condition seen in the presence of a pancreatic glucagonoma. The presence of NME in the absence of a pancreatic tumor has been termed the pseudoglucagonoma syndrome. In such cases, NME is commonly associated with conditions, such as liver disease, inflammatory bowel disease, pancreatitis, malabsorption disorders (ie, celiac sprue), and other malignancies. There are many theories on the pathogenesis of NME, which include the direct action of glucagon in inducing skin necrolysis, hypoaminoacidemia-inducing epidermal protein deficiency and necrolysis, a nutritional or metabolic deficiency of zinc or essential fatty acids, liver disease, glucagon induction of inflammatory mediators, a substance secreted from pancreatic and other visceral tumors associated with NME, and generalized malabsorption. OBJECTIVE: To present a review of the literature on the clinical presentation, etiology, pathogenesis, and treatment of NME. DESIGN: Review of the literature on NME occurring in patients both with and without a pancreatic glucagonoma. METHODS: We performed a PubMed review of the literature on the etiology and pathogenesis of NME to identify case reports and reviews published in both the internal medicine and dermatology literature. RESULTS: Our literature review encompassed 17 primary case reports and literature reviews published in the dermatologic and internal medicine literature on NME in patients both with and without a pancreatic glucagonoma. Although we found no clear consensus among the investigators of a universally accepted pathogenesis for NME, we did identify 4 main categories of etiologic/pathogenetic mechanisms for NME (glucagon excess, nutritional deficiencies, inflammatory mediators, and liver disease) that were discussed by many of the investigators and validated by both clinical and scientific evidence. CONCLUSION: The exact pathogenesis and treatment of NME remain ill-defined despite many case reports and studies on NME in the literature. The many systemic diseases and nutritional deficiencies that have been found to be associated with NME suggest a multifactorial model for the pathogenesis of the disease. The most comprehensive, postulated mechanism for NME involves a combination of zinc, amino acid, and fatty acid deficiencies (arising from a wide variety of causes, such as dietary insufficiency, malabsorption syndromes, liver disease, elevated glucagon levels, and disorders of metabolism) that contributes to increased inflammation in the epidermis in response to trauma and to the necrolysis observed in NME. The importance of gaining an understanding of the etiology and pathogenesis of NME lies in the fact that there is no universally accepted mechanism of pathogenesis for NME, and that the only treatment reported to resolve the rash in these patients is to adequately identify and treat the underlying associated systemic condition or nutritional deficiency.

Erythema↗
PubMed2004

Clinical characteristics of childhood erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in Taiwanese children.

Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are mucocutaneous diseases associated with significant morbidity and mortality. This study compared childhood EM, SJS and TEN in terms of clinical courses, laboratory data, etiologies and outcomes in Taiwan. The initial laboratory findings, clinical presentations, etiologies and subsequent clinical courses of 30 patients with a diagnosis of EM, SJS or TEN, who were admitted between 1995 and 2003 at National Taiwan University Hospital were included and analyzed. There were 19 cases of EM, 8 cases of SJS, 2 cases of SJS/TEN and 1 case of TEN. The most common etiology in EM was infection (84.2%), and the most common implicated organism was Mycoplasma pneumoniae (42.1%). In contrast, 75% of SJS and 100% of TEN were induced by drugs. The most common offending drug was carbamazepine. Those patients with underlying diseases had more protracted courses and longer hospitalization stays. No mortalities were found in our cases. Early short-term steroid equivalent to 1-2 mg/kg/day of prednisolone for 3-5 days was used in 87.5% of SJS patients, without any significant side effects. Those with poor responsiveness to steroids and protracted courses were treated with additional intravenous immunoglobulin (IVIG) [1 g/kg/day], with satisfactory results. Early ophthalmic consultations were performed in all cases. No ocular complications were found in our cases. In conclusion, EM, SJS and TEN were associated with significant morbidity. Early ophthalmic consultations and withdrawal of the offending medication was necessary. Early short-term use of steroids in SJS showed promising results without significant side effects. The additional IVIG in those who had a poor response to steroid treatment may be helpful.

Adolescent↗
PubMed2004

A case of recurrent erythema multiforme and its therapeutic complications.

INTRODUCTION: We report a patient with recurrent erythema multiforme (recurrent EM) who developed iatrogenic Cushing's syndrome due to prolonged corticosteroid use. CLINICAL PICTURE: The patient had been treated with multiple courses of oral and intramuscular prednisolone over a 10-year period to suppress his recurrent and episodic symptoms. This resulted in the development of iatrogenic Cushing's syndrome with secondary adrenal suppression and steroid-induced osteoporosis. TREATMENT: The patient was treated with continuous acyclovir therapy in addition to azathioprine. This combination controlled his disease and enabled us to stop his requirement for high-dose prednisolone. OUTCOME: The patient responded well to this treatment regimen and has been in remission to date. CONCLUSION: This represents a severe case of recurrent EM and the side effects associated with years of chronic high-dose steroid usage. We discuss the therapeutic options to aid physicians in treating this disabling condition.

Adult↗
PubMedDate not supplied

Simultaneous acral nodular eruption and flagellate erythema caused by bleomycin.

A 29-year-old male with AIDS was treated with bleomycin and vincristine for visceral Kaposi's sarcoma. Three days later, he developed two distinct eruptions simultaneously. One eruption was characterized by tender, erythematous, edematous plaques and nodules on the palmar and dorsal surfaces of the hands, forearms, and elbows. The other consisted of grouped, erythematous, hyperpigmented streaks on the trunk, buttocks, and extremities. The patient subsequently received a second treatment consisting of vincristine with adriamycin, without bleomycin, and no cutaneous effects were seen. Diagnoses of flagellate erythema and acral nodular eruption secondary to bleomycin were made and confirmed histopathologically. To our knowledge, this is the first report of two cutaneous side effects of bleomycin appearing simultaneously in a patient with AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1992

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

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.

Adrenal Cortex Hormones↗
PubMed2005

Facial erythema associated with short stature, absent distal phalanx, dental and nail anomalies: case report and neuropsychological profile.

We describe a patient presenting with a combination of dental agenesis, agenesis of the distal phalanx of the fifth finger, small, dystrophic toenails, permanent facial erythema, short stature and mild mental retardation. The neuropsychological profile of this patient which was studied in detail and showed a significant discrepancy between verbal and performance IQ. We discuss the differential diagnosis of this case and conclude that our patient's condition could represent a new syndrome.

Abnormalities, Multiple↗
PubMed2004

[Review: update on erythema nodosum leprosum].

Erythema nodosum leprosum (ENL) is an immunological reaction corresponding to vascularitis due to circulating immune complexes usually occurring during treatment of lepromatous leprosy. The precise mechanism underlying ENL are unclear but involvement of tumor necrosis factor alpha (TNFalpha) is suspected. Based on this suspicion pentoxifylline has been used instead of conventional treatments such as corticosteroids and thalidomide.

Erythema Nodosum↗
PubMed2005

A randomized, double-blind, double-dummy, controlled dose comparison of thalidomide for treatment of erythema nodosum leprosum.

In a randomized, double-blind, double-dummy controlled study, 22 men with erythema nodosum leprosum (ENL) received six capsules containing either 100 mg (group A, n = 12) or 300 mg (group B, n = 10) of thalidomide daily for one week. A six-week, four capsules per day taper followed, in which group A received 50 mg/day of thalidomide in weeks 2 and 3, then dummy capsules in weeks 4 through 7, while group B had gradual decrements every two weeks. Both regimens caused comparable improvement in 19 patients at day 7 (group A [12 of 12] versus group B [7 of 10]; P = 0.08), but slower tapering in group B showed less re-emergence of ENL through week 7 (P = 0.02, versus group A). Most patients developed new lesions soon after stopping treatment. Slower tapering from a higher initial thalidomide dose may improve clinical ENL responses, but high recurrence rates after discontinuation indicates further assessment is needed to identify better tapering regimens.

Adolescent↗
PubMedDate not supplied

Necrotic erythema nodosum leprosum.

A 30 year-old farmer presented himself with mild degree fever, joint pain and multiple ulcers of 15 days' duration. Ulcers were multiple, well-defined, irregular in shape with necrotic base and elevated, sloppy margins over anus, forearms, wrists, buttocks, lower half of thighs and shins. A few of the ulcers were covered with black-coloured scab. They were tender and with serosanguinous discharge. Black atrophic scars and ill-defined, irregular, brown black-pigmented macules of various sizes were present over thighs and legs. Excision biopsy of the ulcer showed features of erythema nodosum leprosum. The patient was treated with systemic corticosteroids.

Adult↗
PubMed1992

Oculocutaneous manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.

Although they were once believed to be distinct diseases, many observers currently consider erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis to represent a mild-to-severe continuum of the same process. Each shares certain clinical, histologic, and etiologic characteristics; among the latter two are frequent ocular involvement. Because long-term ophthalmic sequelae may be catastrophic, early recognition and therapy of this acute oculocutaneous disease spectrum are critically important.

Conjunctivitis↗
PubMedDate not supplied

Palatal erythema in patients using Listerine Cool Mint PocketPaks Oral Care Strips: case reports.

The purpose of this report was to present 2 cases of similar erythematous lesions in patients using Listerine Cool Mint PocketPaks Oral Care Strips. The first case, a 44-year-old female presented with an erythematous, well-defined, macular lesion centrally located on the midhard palatal gingiva. The second case involved a healthy 7-year-old Hispanic female who presented with a similar lesion and the same clinical presentation. Both presented as solitary lesions involving the height of the palate, with bilateral congruency to the mid-palatal suture line. The lesions were square in shape, with 3 sides being distinctly demarcated. The fourth side feathered anteriorly, gradually disappearing as it approached the anterior third of the palate. On verbal questioning in both cases, no discomfort or irritation was indicated in the medical history or by the patient. Both patients indicated regular use of Listerine Cool Mint PocketPaks Oral Care Strips. Treatment consisted of a: (1) recommendation that the strips be discontinued; and (2) re-evaluation of the lesions at the follow-up appointments to make assessments for any possible changes in lesion color, size, or shape. Following discontinuance of the Oral Care Strips, the lesions disappeared. Follow-up appointments suggest an allergic contact focal erythema caused by prolonged contact with the irritant, in this case due to use of Listerine Cool Mint PocketPaks Oral Care Strips.

Adult↗
PubMed2005

Brucellosis with erythema nodosum-like manifestations diagnosed by isolated positivity of the ELISA test for anti-Brucella IgM.

Brucellosis is endemic in the Mediterranean area. In spite of the false negative results, the standard agglutination test remains the routine test for the diagnosis of brucellosis in southern Italy. We present a case of a patient with undulant fever and erythema nodosum-like skin lesions, with negative serum agglutination test, but isolated positivity of the ELISA test for anti-Brucella IgM. A diagnosis of brucellosis for this patient was supported by the anamnestic and clinical data, and by the response to therapy. This case and a review of the literature urge us to consider the ELISA test indispensable for the serological diagnosis of brucellosis.

Adult↗
PubMed2005

[Generalized erythema triggered by a rapid decrease of basophils in chronic myeloid leukemia treated with imatinib].

A 57-year-old woman with chronic myeloid leukemia showing severe basophilia (WBC 17.1 X 10(9)/L, basophils 23%) was treated with 400mg imatinib in June 2003. A high basophil count (WBC 10.6 X 10(9)/L, basophils 31%) was still observed after 1 week of therapy. After 9 days of therapy, she developed generalized pruritic skin erythema, chills and high fever. After terminating imatinib treatment, prednisolone therapy was initiated. The rash quickly disappeared. Four days after withdrawal of imatinib, leukocyte count was 13.0 X 10(9)/L with 3% of basophils, suggesting the possibility that rapid decrease in basophils following imatinib therapy may induce severe cutaneous reactions.

Antineoplastic Agents↗
PubMedDate not supplied

[Ixodes tick-borne borreliosis with erythema nodosum].

Ixodes tick-borne borrelioses (ITBB) is a group of natural-and-focal transmissible infections caused by Borrelia burgdorferi and characterized by multiple organ dysfunctions and a chronic progressive course. The pathognomonic marker of an acute period of the disease is migratory erythema at the site of tick suction. There may be other skin symptoms: rash on the face, urticaria, transient pointed and minor eruptions. Diagnosis presents some certain difficulties in these cases. The paper gives a description of rare eruptions in ITBB.

Acute Disease↗
PubMedDate not supplied

Skin manifestations in vasculitis and erythema nodosum.

Cutaneous lesions are frequent in medium-sized and small vessel systemic vasculitides. The classic cutaneous manifestation of vasculitis is palpable purpura; however the clinical manifestations greatly depend on the size of the vessels affected. They usually do not affect prognosis but relapsing or intractable forms have been described. When skin manifestations are only one of the clinical signs of vasculitis, treatment with corticosteroids and, when indicated, an immunosuppressant, is mandatory, which usually leads to the rapid disappearance of cutaneous lesions. Conversely, when skin lesions are isolated, the diagnosis can be more challenging, but initial treatment may be less aggressive, e.g., dapsone or colchicine, reserving corticosteroids only for those patients in whom the former are ineffective. Erythema nodosum (EN) is the most frequent septal panniculitis. In general it is characterized by the sudden eruption of one or more erythematous and tender nodules or plaques located mainly over the extensor sides of lower extremities. EN resolves with complete "restitutio ad integrum" of the skin in 3-6 weeks. Relapses are uncommon but in patients with idiophatic, streptococcal or EN associated with other upper respiratory tract infections they are more frequent. The main treatment of EN is that of the underlying associated conditions, if demonstrated. Aspirin and other NSAIDs in full doses are often sufficient.

Cryoglobulinemia↗