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Clinical trial with clofazimine for treating erythema dyschromicum perstans. Evaluation of cell-mediated immunity.

Eight patients were studied to determine the possible use of clofazimine for treating erythema dyschromicum perstans (EDP). The T-helper/T-suppressor cytotoxic ratio (CD-4/CD-8) and the in vitro lymphoproliferative response on stimulation with phytohemaglutin (PHA) and concanavalin A (Con A) were determined in peripheral blood before and after treatment. Of the eight patients studied, seven had excellent to good responses, whereas only one had a marginal response. The immunologic evaluation before and after treatment showed a significant change in the CD-4/CD-8 ratio, a decrease of the response to PHA, and no change in the response to Con A. The results obtained show that clofazimine is useful for treating this nosologic entity because of its cosmetic effect, and also because it induces changes in cell-mediated response, which could be very important therapeutically.

Adolescent↗

Erythema nodosum versus nodular vasculitis.

BACKGROUND: Erythema nodosum (EN) is usually considered to be a septal panniculitis, and nodular vasculitis (NV) a lobular panniculitis. We tested the usefulness of this histologic classification in clarifying a frequent clinical dilemma: EN versus NV. METHODS: Over 3 years 109 patients with panniculitis were included in this study. After a history and a physical examination, a clinical diagnosis was made according to well-established criteria. RESULTS: From the study of 88 patients, we concluded that in the clinically typical cases, the clinico-pathologic agreement was 93% and 94% for EN and NV respectively, whereas it diminished to 79%, 72%, and 67% in the cases clinically diagnosed as EN migrans, atypical EN, and atypical NV, respectively. Moreover, septal and lobular panniculitis were always two clear-cut, different, opposite patterns of hypodermal inflammation to these conditions. CONCLUSIONS: In the absence of a known pathogenetic mechanism, the histopathology remains the most objective discriminating marker between EN and NV, mainly in the clinically atypical and doubtful cases.

Adolescent↗

Fat necrosis with features of erythema nodosum in a patient with metastatic pancreatic carcinoma.

A 59-year-old man presented with painful subcutaneous nodules on the anterior surfaces of the legs. He had received oral antibiotics and supportive care for presumed cellulitis and thrombophlebitis, but had minimal improvement. Five months earlier, he had undergone pancreaticoduodenectomy for acinar pancreatic carcinoma; at that time, the serum level of amylase had been normal, but the level of lipase was elevated. The patient denied fever, rigors, arthritis/arthralgia, or pleuritic pain. His medications included aspirin, furosemide, ranitidine, and nortriptyline. He denied any allergies. Physical examination revealed numerous firm, tender, erythematous and violaceous, subcutaneous nodules on the lower extremities, with marked bilateral pitting edema (Fig. 1). Skin biopsy of a representative lesion revealed septal panniculitis, consistent with erythema nodosum (Fig. 2). None of the characteristic changes of pancreatic fat necrosis was present. The patient was treated with aspirin, 650 mg orally, q 6 h, and indomethacin, 50 mg orally, q 12 h, but he continued to develop new nodules; prednisone, 60 mg orally was begun. Although he reported improvement in symptoms, the nodules failed to respond clinically and older nodules ulcerated along the medical aspect of the right leg (Fig. 3). The complete blood count was normal, except for hemoglobin, 10.9 mg per dL. Routine serum biochemical studies were also normal, except for albumin, 3.1 mg per dL, LDH, 312 U per L, and SGOT, 51 U per L. Serum amylase was 14 U per L (normal per 30 to 115 U per L) and serum lipase was 54,160 U per L (normal 0 to 200 U per L). Chest roentgenogram and tuberculin skin test were negative. A CT scan of the abdomen revealed extensive liver metastases. A second biopsy of the skin and subcutis of a necrotic nodule revealed lobular panniculitis with the characteristic picture seen in pancreatic fat necrosis (Fig. 4). The patient was presumed to have metastatic pancreatic carcinoma and pancreatic fat necrosis. Nodules subsequently developed on the thighs, arms, hands, wrists, and fingers. He developed arthritis and arthralgias of the ankles, wrists, and hands, bilaterally, and the right knee. Aspiration of a right knee effusion revealed numerous neutrophils, but no evidence of infection. Treatment was begun with the somatostatin analog, octreotide, in increasing doses. During this therapy, the lesions did not progress and new lesions did not appear. There was no change in the lipase level. Inadvertently, octreotide was omitted at discharge, but reintroduction of octreotide was associated with lack of further progression of the nodules, according to the patient's spouse; however the patient became progressively debilitated and his abdominal pain worsened, requiring continuous sedation. His condition deteriorated and he died several weeks after hospital discharge.

Carcinoma, Acinar Cell↗

Erythema dyschromicum perstans: response to dapsone therapy.

Erythema dyschromicum perstans (EDP) is a rare disorder characterized by asymptomatic, slowly progressive, ash-gray macular pigmentation of the skin, which usually occurs from age 5 years through adult life. Most cases reported to date are of Latin American and Indian patients. Rare cases have been reported from Turkey. No treatment of choice is presently available. Various therapies have been tried, including sun protection, chemical peels, antibiotics, corticosteroids, vitamins, isoniazid, griseofulvin, and chloroquine, without any benefit. Some authors have suggested the therapeutic efficacy of clofazimine and dapsone on EDP. We report a case of EDP that responded remarkably well to treatment with dapsone.

Adolescent↗

Immunopathologic study of erythema dyschromicum perstans (ashy dermatosis).

Erythema dyschromicum perstans (EDP) is a pigmentary disease of unknown etiology in which damage to basal cells is thought to be mediated by adhesion molecules. The aim of this study was to characterize the histopathology and immunopathology of EDP. Forty-three patients from Medellín, Colombia, with the diagnosis of EDP were evaluated. Skin biopsy specimens were obtained for histopathology and immunohistochemistry, using monoclonal antibodies directed against the following markers: CD4, CD8, CD56, CD1a, CD68, CLA, HLA-DR, ICAM-1 and LFA-1alpha. A dermal lymphocytic infiltrate was observed in all cases, with a perivascular location in 86%. Other histologic features included melanophages in all specimens, vacuolization of the basement membrane zone (BMZ) 58% and exocytosis of lymphocytes (53.5%). The mean number of total leukocytes was 1510 cells mm-2 of tissue. There was a predominance of CD8+ T lymphocytes in the dermis and HLA-DR+, ICAM-1+ keratinocytes in the epidermis. Exocytosis of cutaneous lymphocyte antigen (CLA)+cells was observed in areas of BMZ damage, suggesting that response to antigenic stimulation may play a role in the development of EDP.

Adolescent↗

Erythema multiforme is associated to HLA-Aw33 and DRw53.

Erythema multiforme is an acute eruption of the skin and mucous membranes of various aetiologies. Forty-one unrelated patients were HLA typed for 53 specificities of the HLA-A, B, C, DR and DQ series. Frequencies of Aw33 and DRw53 were significantly increased: Aw33, 17.0% in patients vs 2.8% in controls (corrected p = 0.01, relative risk = 7.2); DRw53, 70.7% in patients vs 30.5% in controls (corrected p = 0.0005, relative risk = 5.5).

Adult↗

Atypical presentation of erythema elevatum diutinum.

A 47-year-old man presented with two red-brown plaques on his back. The histology was consistent with erythema elevatum diutinum. The case was unusual in the site of presentation and in the paucity of lesions. Investigations were aimed at screening for associations such as infections, malignancies and inflammatory diseases. Dapsone remained an effective treatment.

Back↗

Parvovirus B19 infection presenting as 'bathing trunk' erythema with pustules.

A 7-year-old girl presented with acute vulval erythema and pustules, associated with a petechial eruption in her flexures and over her feet. There was a mild prodromal illness and the patient was afebrile. There were minimal symptoms associated with the rash. Skin and throat swabs were negative and blood examination showed mild neutrophilia and lymphopaenia. Parvovirus B19 IgM was detected on serology and cutaneous features resolved within 4 days. This is a further case of parvovirus B19 infection presenting as a 'bathing trunk' exanthem that has unique dermatologic features, including the presence of pustules and distant petechiae.

Acute Disease↗

Neonatal erythema infectiosum.

A report is presented of a patient with neonatal erythema infectiosum who developed petechiae, transient thrombocytopenia and transient cardiac failure due to transplacental transmission of human parvovirus B19 (HPV B19) infection. It is suggested that the thrombocytopenia was caused by platelet-associated IgG produced by the patient, and that the cardiac failure may have been caused by direct entry of HPV B19 into the cardiac tissue.

Adult↗

Bullous erythema multiforme following herpes zoster and varicella-zoster virus infection.

Four cases of herpes zoster-induced bullous erythema multiforme (EM) are reported. Three patients presented with widespread skin lesions 10 to 14 days after an episode of thoracic herpes zoster. In these patients a high increase in varicella-zoster virus (VZV) antibody titer was detected, indicating secondary VZV infection. Histologic examinations of skin biopsy from a patient with widespread lesions (case 4) revealed a mixture of EM, toxic epidermal necrolysis and herpetic virus infection. VZV should be included in the list of infectious agents able to trigger EM and Stevens-Johnson syndrome.

Acyclovir↗

Erythema multiforme during cytomegalovirus infection and oral therapy with terbinafine: a virus-drug interaction.

The authors report a case of erythema multiforme in a 32-year-old woman who was also taking oral terbinafine for an onychomycosis. The patient data analysis showed serological positivity for cytomegalovirus (IgM and IgG) and hepatitis C virus and serological titre of antinuclear antibody was elevated. After a brief review of the literature the authors propose the possibility of virus-drug interaction as a model of adverse drug reactions.

Adult↗

Hypereosinophilic dermatitis-like erythema annulare centrifugum in a patient with chronic lymphocytic leukaemia.

A 65-year-old male presented with a history of disseminated erythema annulare centrifugum lesions and a high peripheral blood eosinophilia (55%). Histopathology of a lesion revealed a superficial and deep perivascular and interstitial inflammatory eosinophilic infiltrate. The bone marrow aspirate showed a heavy eosinophilic infiltrate. There was no evidence of any other systemic involvement except for bone marrow eosinophilia. The patient had a 6-year history of chronic lymphocytic leukaemia. The disease is in complete remission for the last 3 years. The clinical and histopathological findings in the reported patient correspond to the diagnosis of hypereosinophilic dermatitis. Topical treatment with corticosteroid creams was successful. The cutaneous lesions gradually resolved in 8 weeks, within 7 months the peripheral blood eosinophilia slowly declined from 55% to 7%.

Aged↗

Erythema dyschromicum perstans in children: a report of 14 cases.

BACKGROUND: Erythema dyschromicum perstans (EDP) or ashy dermatosis is a peculiar, slowly progressive, macular hyperpigmentation, which leaves a permanent discoloration. It is an acquired dermatosis that occurs most frequently in Central and South America. EDP usually appears in adults, but some isolated cases and small series have been reported in prepubertal children. METHODS: A retrospective review of cases of EDP in 10-year-old children or younger, attended in a pediatric hospital between 1990 and 2003. RESULTS: We present 14 cases of EDC in children 10 years and younger. With an additional 25 cases reported so far in the English language literature, a total of 39 children have been described. Unlike adult patients, who are most commonly of Hispanic origin, children with EDP are usually Caucasian. Other important facts in children with EDP are the absence of consistent trigger factors and an eventual improvement or resolution of the lesions in 50% of prepubertal patients. CONCLUSION: We suggest that EDP is a distinctive clinical entity, different from lichen planus and lichen planus pigmentosus, which may be identical to the so-called idiopathic eruptive macular pigmentation.

Child↗

Erythema figuratum in septic babesiosis.

Babesiosis is a rare worldwide-distributed protozoal zoonosis caused by a haemoprotozoan of the genus Babesia, transmitted through bites of tick of the genus Ixodes. The first demonstrated case of human babesiosis in the world was discovered in Europe, in 1957. However, most of the cases were reported later in the north-east of the United States where Babesia microti has been the cause of over 300 cases of human babesiosis since 1969. In Europe, the most severe cases are observed in asplenic patients infected by a parasite of cattle, the Babesia divergens. Only two cases of babesiosis have been reported in Spain. We present a case of erythema figuratum associated to septic babesiosis in a non-splenectomized man, which is currently the third case of babesiosis in Spain.

Aged↗

Bullous mastocytosis: diffuse cutaneous mastocytosis with extensive blisters mimicking scalded skin syndrome or erythema multiforme.

Bullous mastocytosis (diffuse cutaneous mastocytosis) is a rare form of mast cell disease that begins during the first month of life and causes extensive blisters that mimic scalded skin syndrome or bullous erythema multiforme. Discrete pigmented macules, papules, and nodules are absent and the characteristic leathery induration of skin may not develop until 6 months of age. Skin biopsy shows a subepidermal blister with mast cells at the base. The most serious complications are gastrointestinal hemorrhage and shock. The symptoms of bullous mastocytosis may be modified by a number of new therapeutic agents.

Burns↗

Erythema multiforme in childhood and early infancy.

Erythema multiforme is said to be rare in childhood and especially in early infancy. Three infants, none older than 1 year, were seen with this condition; all showed typical clinical features. In one of the three infants, no distinctive etiological factor(s) could be found. One was suffering from congenital hepatitis (cause unknown) and another from a staphylococcal infection.

Amoxicillin↗