Pemphigus vulgaris with involvement of the cervix treated using thalidomide therapy

A 55‐year‐old White woman was first seen in July 1995 with ulceration of the oral mucosa, including the gingiva, labial mucosa, gums, palate and tongue, as well as erosions and blisters on the trunk. A biopsy showed a suprabasal blister with acantholysis. Direct immunofluorescence was positive for intercellular deposits of IgG and C3, and indirect immunofluorescence was positive for intercellular antibodies at a titer of 160. The patient was diagnosed as having pemphigus vulgaris and treated with prednisone 1–1.5 mg/kg/day from 1995 until 1998, but no response was observed (the disease continued to be active with the formation of new lesions). The patient developed a number of steroid‐induced complications including diabetes, osteoporosis, Cushing syndrome, hypertension and high‐output cardiac failure, and required repeated hospitalization. In August 1998 she was started on azathioprine 100–150 mg/day and continued on prednisone 1 mg/kg/day. One year later, in August 1999, the disease was still active with new lesion formation and persistent oral erosions and dysphagia, despite persistent therapy with azathioprine 150 mg/day and prednisone 1 mg/kg/day. Because of the disease severity and the lack of a response to treatment, in October 1999 the patient was started on thalidomide therapy 100 mg/day (1.7 mg/kg/day) and continued the prednisone treatment at 1 mg/kg/day together with azathioprine 150 mg/day (2.7 mg/kg/day). There was a good response, with clearing of all oral lesions in 20 days. The prednisone dose was gradually reduced and was discontinued in May 2000; azathioprine was gradually reduced to 50 mg 3 times/week (0.35 mg/kg/day). Thalidomide was continued at a dose of 100 mg/day. The patient was in total clinical remission, being free of both old and new lesions, for the next 14 months, when the thalidomide treatment was discontinued as a result of side‐effects, including weakness in the legs and paresthesis of the fingers. After the discontinuance of treatment with thalidomide, severe lesions returned. Thalidomide was reintroduced, at 100 mg/day. After 2 months the patient entered total clinical remission and on the last occasion on which she was seen, in May 2003, was found to have remained clear of lesions. The patient continues to take thalidomide at 100 mg/day.     

Treatment of bullous pemphigoid with prednisolone only: 0.75 mg/kg/day versus 1.25 mg/kg/day. A multicenter randomized study

Systemic corticosteroids for the treatment of bullous pemphigoid are an accepted therapeutic measure among dermatologists. Nevertheless, the best initial dosage is still unknown. The purpose of the present study was to compare the efficacy and safety of two dosages of prednisolone used as a single therapeutic agent: 0.75 mg/kg/day versus 1.25 mg/kg/day for three weeks. Fifty patients with bullous pemphigoid confirmed by direct cutaneous immunofluorescence were included in this study in different centers. They were randomly assigned to one of two groups: 24 patients were treated with prednisolone 0.75 mg/kg/day (group I) and 22 patients were treated with 1.25 mg/kg/day (group II). Four patients had to be excluded from this study. The low and high dosage prednisolone groups do not show a statistically significant difference from each other after 51 days. At day 21, 58 p. 100 of the patients in group I were disease-free, and 64 p. 100 in group II. At day 51, after a slow decrease of prednisolone therapy (half of the initial dosage per day), 33 p. 100 of the patients in group I were still free of skin lesions, and 55 p. 100 in group II.     

Comparative study of efficacy and effect on plasma cortisol levels of micronised desonide cream 0.1 p. 100 versus betamethasone dipropionate cream 0.05 p. 100 In the treatment of childhood atopic dermatitis

OBJECTIVE: Systemic side effects of local corticosteroid therapy may occur when treating chronic inflammatory dermatoses in children. We compared the effect of micronized desonide cream 0.1 p.100 versus betamethasone dipropionate cream 0.5 p.100. PATIENTS AND METHODS: A randomized double-blind trial was conducted to assess the efficacy of micronized desonide cream 0.1 p.100 (group 1) versus bethamethasone cream dipropionate 0.05 p.100 (group 2) in children treated for atopic dermatitis and to compare their effects on serum cortisol levels 8 hours after administration. Twenty-nine patients, mean age 13.8 months were included (15 in group 1 and 14 in group 2). The creams were applied twice a day from day 1 to 5 then once a day from day 6 to 7 and finally once every two days to day 15. RESULTS: The two treatments were effective with a decrease in body surface area involved and an improvement in lesion score from day 5 to day 20. Cortisolemia fell off significantly for both treatments between day 0 and day 5 (group 1: Deltad5=-4.74 mg/ml, p=0.01; group 2: Deltad5=-13.06 mg/ml, p<0.0001), only for group 2 between day 0 and day 20 (Deltad20=-7.38 mg/ml, p=0.02) and to a lesser degree between day 0 and day 30 (Deltad30=-3.18 mg/ml, p=0.06). The decrease was greater in group 2 than in group 1 on day 5 (p=0.01) and to a lesser degree at day 20 (p=0.06). CONCLUSIONS: Micronized desonide cream 0.1 p.100 has less potential for suppressing the adrenal cortisol axis than betamethasone dipropionate cream 0.05 p.100 while the therapeutic effect on childhood atopic dermatitis is the same.     

Palmoplantar pustulosis treated with itraconazole: a single, active-arm pilot study

Pustulosis palmoplantaris (PPP; synonyms: pustulosis palmaris et plantaris, palmoplantar amicrobic pustulosis) is a common chronic, relapsing, pustular eruption affecting the palms and soles. The authors report the successful treatment of six therapy-experienced patients with histologically confirmed PPP with oral itraconazole (100 mg/day for 1 month, followed by a month of 100 mg/day every other day). Three of six patients showed complete clearance of pustules, significant reduction of erythema, and unnoticeable desquamation, whereas the other three patients had no new pustules appearing and had modest reduction of erythema and desquamation. All patients experienced relapses within a month of therapy cessation. Two of the three complete responders reinitiated itraconazole therapy at 100 mg/day for another 2 weeks, followed by a maintenance dose of 50 mg/day until achieving remission. As complete responses are not commonly observed in placebo treatments in placebo-controlled trials for PPP, the authors believe that the present study shows that itraconazole is an effective treatment for treatment-resistant PPP.     

Childhood leprosy: Report of a case

Childhood leprosy is very common, especially in tropical and subtropical areas, such as Paraguay. Early symptoms can be missed in a routine examination and the diagnosis can pass unnoticed. Pediatricians and dermatologists should remember the manifestations of this disease in order to make an early diagnosis. We present the case of a 10-year-old child with borderline Hansen disease, considered unusual in children. She was treated with multibacillary therapy (MB-WHO) with the combination of Rifampicin 600 mg, clofazimine 300 mg, and dapsone 100 mg once a month (the three drugs together on the same day once a month for 18 months); the remaining 28 days of the month, the child received clofazimine 50 mg/day and dapsone 100 mg/day (the two drugs together on the same day 28 days of the month for 18 months). This therapy produced complete remission of the lesions without reactional states.     

Eosinophilic Pustular Folliculitis

Eosinophilic pustular folliculitis (EPF), also known as Ofuji disease, is a disease that manifests with follicular papules or pustules. Its variants include a classic type that occurs most commonly in Japan, an HIV-associated type, an infantile type, a type that occurs on the palms and soles, a rare medication-associated variant, and a rare neoplasia-associated variant.A wide range of medications has been used to treat EPF. Topical corticosteroids are the first-line treatment option for EPF. Topical tacrolimus seems to be useful initial therapy as well. Oral indometacin (50-75 mg/day) is an effective treatment of classic EPF although it can induce peptic ulcers. For treatment of HIV-associated EPF when topical corticosteroids and indometacin do not work, various other treatments should be considered. These treatment options include cetirizine 20-40 mg/day, metronidazole 250 mg three times a day, itraconazole starting at a dosage of 200 mg/day and increasing to 300-400 mg/day, and topical permethrin. If these treatments do not work phototherapy with UVB is the 'gold standard' of treatment and is often curative. Treatments with less certain risk-benefit ratios but with some efficacy include PUVA (psoralen + UVA) photochemotherapy, oral corticosteroids, synthetic retinoids (i.e. isotretinoin 1 mg/kg/day), and acitretin (0.5 mg/kg/day), oral cyclosporine (ciclosporine) 5 mg/kg/day, interferon (IFN)-alpha-2b, and IFNgamma. Minocycline 100mg twice daily and dapsone 50-100mg twice daily have been used with some effect. The use of highly active antiretroviral therapy for HIV has resulted in the amelioration of EPF as CD4 cell counts rise above 250/mm(3). The diversity of clinical presentations and affected populations make it seem that EPF is a reaction pattern as much as a disease and that therapy should be tailored to the variant of EPF and the underlying etiology.     

Favorable clinical response by pre-prandial administration of low-dose ciclosporin to severe adult atopic dermatitis

Although ciclosporin is useful for atopic dermatitis (AD), appropriate dosage and therapeutic drug monitoring (TDM) has been performed only by post-prandial ciclosporin administration. We administered ciclosporin pre-prandially to eight severe adult AD patients (four cases of erythrodermic AD, three cases of AD recalcitrant to standard therapy, and one AD case with numerous pruriginous lesions). Blood concentrations of ciclosporin at various dosages were measured and appropriate dosage in terms of therapeutic efficacy was analyzed by using the area under the concentration curve (AUC). AUC was estimated by the C1 (obtained serum concentration of ciclosporin at 1 hour after ciclosporin administration), C2 (concentration of ciclosporin at 2 hours) and C4 (concentration of ciclosporin at 4 hours) concentrations of ciclosporin. The trough levels of ciclosporin with 200 mg/day, 150 mg/day, and 100 mg/day administration were 96.5 ng/ml, 66.4 ng/ml, and 75.3 ng/ml, respectively. The peak serum concentration (C(max)) was obtained at 1 hour (C1) in most cases. The AUC of 0-4 hours (AUC 0-4) were 2099.5 ng · h/ml (200 mg/day), 1782.6 ng · h/ml (150 mg/day) and 1696.2 ng · h/ml (100 mg/day). VAS scores of itching and blood eosinophil counts were decreased significantly by the ciclosporin treatment. Pre-prandial administration of a relatively low dose of ciclosporin for severe atopic dermatitis resulted in a favorable subjective and objective clinical response and the measurement of blood concentration mostly correlated with the effective dosage assessment.     

达那唑联合低分子肝素/雷公藤多甙治疗难治性青斑性血管病12例

目的评价达那唑片联合低分子肝素针/雷公藤多甙片治疗青斑性血管病的临床疗效和安全性。方法对常规治疗无效的12例青斑性血管病患者,予达那唑片联合低分子肝素针/雷公藤多甙片治疗。急性期予达那唑片100 mg口服,2次/d,低分子肝素针0.3 mL皮下注射,1次/d,雷公藤多甙片20 mg口服,3次/d。维持期予达那唑片100 mg口服,1次/d,低分子肝素针0.3 mL皮下注射,隔日1次,雷公藤多甙片20 mg口服,2次/d。随访0.5~3年。结果 2周后,10例溃疡开始结痂、变浅、面积缩小,疼痛减轻,无新发皮疹;治疗8~16周后,12例痊愈。疗程中不良反应少且轻微。结论达那唑片联合低分子肝素针/雷公藤多甙片对于常规治疗疗效差的青斑性血管病安全有效。     

Seborrhoea—an indicator for poor clinical response in acne patients treated with antibiotics

The relationship between sebum excretion rate (SER) and clinical improvement was investigated in 255 acne patients treated for 6 months with either oral erythromycin (1 g/day), minocycline (100 mg/day), oxytetracycline (1 g/day) or cotrimoxazole (400 mg/day); topical therapy was 5% benzoyl peroxide. In all but the cotrimoxazole treated group, there was a significant correlation between a high SER and reduced clinical response. This was particularly evident in those patients with an SER of greater than 2.5 micrograms/cm2/min. These patients showed only 17% improvement compared with 100% improvement in those subjects with an SER of 1.0 micrograms/cm2/min or less. The presence of obvious seborrhoea in a patient who has failed to respond to an adequate 6-month course of antimicrobial therapy, should indicate the earlier rather than later use of isotretinoin for their acne.     

Peroxisomal proliferator-activated ligand therapy for HIV lipodystrophy

Lipodystrophies associated with HIV disease have been reported in recent years and have included a general redistribution of fat with more central fat and increased dorsocervical fat. These lipodystrophies are commonly associated with hyperlipidemia and in some cases with insulin resistant diabetes. Although a similar redistribution of fat is seen in hypercortisolism, in general, serum and urinary cortisol levels are normal in these HIV-positive patients. However cortisol/dehydroepaindrosterone (DHEA) ratios are increased in HIV disease and may result in a relative hypercortisolism. Seven HIV-positive male patients on multidrug antiviral therapy including HIV protease inhibitors had developed increased central and dorsocervical fat over 1 year. All patients had increased serum lipids and three had insulin resistant diabetes. Four patients were treated initially with DHEA 100-200 mg/day, with addition of a cyclo-oxygenase (COX) inhibitor (indomethacin 100 mg/day) and three others were treated from the onset with a combination of DHEA 200 mg/day and a COX inhibitor (indomethacin 100 mg/day or naprosyn 1000 mg/day). All patients reported moderation or normalization of their serum lipids and some moderation of blood sugars while on DHEA alone. More marked improvement in blood sugar and noticeable decreases in the dorsocervical fat; however, occurred only with addition a COX inhibitor. Both DHEA and COX inhibitors have a number of mechanisms of action; among these is their role as a peroxisome proliferator-activator receptor ligand. Dysregulation of peroxisome function is associated with the spectrum of biochemical changes seen within these HIV associated lipodystrophies. Use of HIV protease inhibitors is reported in the majority of patients with these lipodystrophies, and protease inhibitors may accentuate the underlying peroxisome dysregulation. Supplementation with DHEA and a COX inhibitor may improve peroxisomal function.     

Treatment of pyoderma gangrenosum with cyclosporin A

Two patients with recalcitrant pyoderma gangrenosum were treated with oral cyclosporin A (5 mg/kg body-weight/day). Healing of the lesions was achieved in Patient 1 within 1 month of starting treatment, but new areas of ulceration appeared when the dose was reduced to 3 mg/kg body-weight/day. The ulcers showed marked improvement by 3 weeks after the start of treatment in Patient 2 and remained inactive at a maintenance dosage of 100 mg/day, but there was no change in the associated seronegative arthritis. A steroid-sparing effect of CyA was evident in both patients. It is suggested that a lower dose of cyclosporin A than doses used previously in the treatment of pyoderma gangrenosum may be equally effective.     

Perianal Crohn's disease

A 13-year-old girl with a history of 4 months of perianal skin lesions is described. Physical examination revealed three 0.5 I 1-cm red, swollen, fleshy, skin tags extending from the perianal area to the perineum (Fig. 1). The patient reported intermittent fever, diarrhea, and abdominal pain, and her body weight was below the third percentile for her age. Laboratory studies showed an erythrocyte sedimentation rate of 101 mm/h; hematocrit of 26%; white blood cell count of 9800/mm3; serum iron of 15 mg/L (normal value (NV), 60-160 mg/L); ferritin of 43.4 microg/L (NV, 12-150 microg/L); transferrin of 203 mg/100 mL (NV, 200-400 mg/100 mL); transferrin saturation of 6% (NV, 20-50%); hypoalbuminemia; negative purified protein derivative (PPD), cytomegalovirus (CMV), human immunodeficiency virus (HIV), venereal disease research laboratory (VDRL), and antinuclear antibody tests; and Toxoplasma titers of 1/16, Van de Kamer 1.67 g/day. A barium examination revealed marked irregularity of the descending colon, and a colonoscopy showed uneven areas of mucosal edema and pseudopolyps in the transverse and descending colon, associated with irregular thickening and stenosis. Histopathologically, large intestine and skin lesions consisted of noncaseating epithelioid and giant cell granulomas (Fig. 2). Cultures for acid-fast bacilli and fungi were negative, and under polarized light no foreign bodies were seen. Treatment with metronidazole (250 mg three times a day), prednisone (0.5 mg/kg/day), and acetylsalicylic acid (75 mg/kg/day) was moderately effective. Vitamin, folic acid, and iron supplements were also added.     

Juvenile generalized circinate pustular psoriasis treated with oral cyclosporin A

We report on a 17-year-old boy presenting with relapsing generalized circinate pustular psoriasis exacerbated by streptococcal angina. Because of the severe course in his case, we started systemic treatment with cyclosporin A and corticosteroids. Corticosteroids could easily be tapered down. Cyclosporin A maintenance therapy was realized with 100 mg/day (1.6 mg per kg body weight and day). Side effects were a temporary increase in blood pressure during initiation with 200 mg cyclosporin A/day. After dose reduction no side effects were seen. The pustular lesions disappeared and the PASI score decreased from 40.7 to 4.8. The treatment was found to be well tolerated and effective.     

Safety and efficacy of a fixed-dose cyclosporin microemulsion (100 mg) for the treatment of psoriasis

Cyclosporin is a second-line modality for the treatment of psoriasis. The long-term efficacy of cyclosporin and potential adverse side-effects, however, are a concern to patients. Therefore, a cyclosporin microemulsion (Neoral), which is steadily absorbed at an ultra-low dosage (1-2 mg/kg per day) or low dosage (2-3 mg/kg per day), is currently recommended. The dose must be calculated based on patient bodyweight and the blood concentration monitored regularly, which is time-consuming. Furthermore, the concentration is related to the safety profile, but not to efficacy. We examined whether a fixed-dose cyclosporin microemulsion (100 mg/day) is effective for treating psoriasis. Enrolled patients (n = 40) were given either 100 mg cyclosporin emulsion once daily (group A) or 50 mg twice daily (group B), regardless of patient weight and condition, before meals in a randomized controlled study. Patient bodyweight ranged 50-80 kg. We assessed the serum cyclosporin concentration 1 h after administrating the medicine (C1 score), Psoriasis Area and Severity Index (PASI) score, quality of life, and the results of regular blood examinations. The improvement rate was 69.4 ± 4.8% in group A and 73.4 ± 4.3% in group B. PASI-50 was achieved by 82% in group A and 84% in group B. At 6 weeks, the number of patients with PASI-50 was significantly higher in group A than in group B. PASI-75 and -90 were also achieved in both groups with no significant difference between groups. Administration of a fixed-dose cyclosporin microemulsion (100 mg/day) is practical for second-line psoriasis treatment.     

Sucessful treatment with clofazimine and itraconazole in a 46 year old patient after 32 years duration of disease

Lobomycosis, caused by the fungal pathogen Lacazia loboi, is a chronic deep mycosis and is only found in Central and South America. Clinically the disease is characterized by shiny keloidal nodules appearing mainly on the exposed parts such as face and the upper and lower extremities. Therapeutically the surgical removal of the lesions is considered as the only successful treatment. We describe the therapeutic response of a patient with Lobo's disease treated for one year with a combination of clofazimine (100 mg/day) and itraconazole (100 mg/day). A complete clinical and histopathological remission of the disease was observed. The patient has been followed for three years.     

The effect of long term tetracycline treatment for acne vulgaris on the occurrence of R factors in the intestinal flora of man

R factors are known to be the most important mechanism of antimicrobial resistance of intestinal flora. Short courses with therapeutic doses (1000 mg/day) of tetracycline select for strains containing transferable resistance factors to more than one antimicrobial agent. In this report we show that long term treatment with very low doses (100 mg/day) of tetracycline for acne vulgaris has an equally strong effect favouring establishment of resistent strains and R factors in the intestinal flora of patients.     

Treatment of diffuse cutaneous leishmaniasis with miltefosine: a case report

BACKGROUND: A 31-year-old man who has suffered since age 3 from diffuse cutaneous leishmaniasis (DCL), a disease with profound physical and psychosocial repercussions and no effective treatment at present, was treated with miltefosine. METHODS: The patient was treated for 120 days, 100 mg/day for 1 week, then 150 mg/day subsequently. RESULTS: Lesions were free of parasites at 43 days, and no signs of infiltration were present at day 76. No adverse side effects were observed. CONCLUSIONS: The dramatic clinical effect of miltefosine in this patient appears to fully justify further evaluation of this experimental therapy in DCL.     

A comparison of 2 weeks of terbinafine 250 mg/day with 4 weeks of itraconazole 1OO mg/day in plantar-type tinea pedis

Summary This double-blind, parallel group study compared a 2-week course of terbinafine 250 mg/day with a 4-week course of itraconazole 100 mg/day. A total of 190 patients were enrolled, of whom 129 were evaluable for efficacy. At week 8, 69% of patients treated with terbinafine were classified as effectively treated (mycological cure, and clinical assessment total score ≤2) vs. 67% in the itraconazole group. At week 16, however, the rating for effective treatment increased to 71% of the terbinafine group, but decreased to 55% of the itraconazole group. This difference was of borderline statistical significance ( P = 0.06). The results of this study demonstrate that both drugs can be used safely, and that 2 weeks' treatment with terbinafine 250 mg daily is as effective as 4 weeks' treatment with itraconazole 100 mg daily, but with fewer long-term relapses.     

Prurigo pigmentosa: a misdiagnosed dermatitis in Sicily

Prurigo pigmentosa is a papular pruriginous eruption that leaves a marble-like pigmentation. The majority of cases have been found in Japan. Three new female. Sicilian patients with prurigo pigmentosa were studied. All of them had previously been diagnosed as having different types of dermatitis. The administration of minocycline, at a dosage of 100 mg/day for 1 month, induced the disappearance of the papular eruption and pruritus in two patients, with an improvement of the gross reticular pigmentation. The third showed no modifications of the clinical picture after 2 months of minocycline treatment, but her condition significantly improved after 1 month of treatment with diaminodiphenylsulfone, 100 mg/day. These observations allow us to suggest that prurigo pigmentosa might be relatively frequent but misdiagnosed in the Sicilian population.     

Itraconazole versus griseofulvine in the treatment of tinea corporis and tinea cruris

126 patients (82 males and 44 females) aged above 12 years, suffering from tinea corporis and/or tinea cruris, were treated with either itraconazole (100 mg once a day for 2 weeks and then plecebo for 2 weeks) (63 patients), or griseofulvin (250 mg twice a day for 4 weeks). 90.47% of the patients treated with itraconzole improved whereas griseofulvin imporved 76.19% of patients, clinically. Mycological response was 72% with itraconazole and 57% with griseofulvin.