Etretinate and AIDS-related Reiter's disease

We report a case of a 34-year-old homosexual man who developed Reiter's disease with severe cutaneous and joint involvement shortly after he was diagnosed as having acquired immunodeficiency syndrome (AIDS). Therapy with potent topical steroids and oral methotrexate was ineffective, but etretinate at a dose of 0.75 mg/kg resulted in rapid clearing of the skin lesions and a marked improvement of his arthralgia.     

Long-term cefuroxime axetil in subacute cutaneous lupus erythematosus. A report of three cases

BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) is a subset of lupus erythematosus characterized mainly by prominent photoaggravated cutaneous manifestations. Standard therapies for SCLE include topical or systemic steroids and antimalarial drugs. Both methods show limited efficacy in clearing cutaneous lesions and occasionally produce serious side effects. AIM: To assess the efficacy of cefuroxime axetil, an oral cephalosporin with antibacterial and immunosuppressive activity, in patients with SCLE. METHODS: Three patients with SCLE were treated with cefuroxime axetil at a daily dose of 500 mg for 30-60 days. RESULTS: In all patients complete clearing of skin lesions was achieved and no side effects were observed. CONCLUSION: We suggest that long-term cefuroxime axetil administration might be an alternative treatment for patients with SCLE skin lesions.     

One-week therapy with oral albendazole in hookworm-related cutaneous larva migrans: a retrospective study on 78 patients

We evaluated retrospectively the efficacy and tolerability of oral albendazole (400 mg/day for 1 week) in 78 patients with hookworm-related cutaneous larva migrans characterized by multiple and/or extensive lesions. The diagnosis was based on history and the clinical picture. Neither topical or systemic drugs nor physical treatments were used. All patients were followed-up for at least 3 months after the therapy. All patients were cured at the end of the therapy. The disappearance of pruritus was reported after 2-3 days and skin lesions after 5-7 days of therapy. One patient reported nausea and abdominal pain; another patient reported worsening of pruritus: in both cases it was not necessary to stop the therapy. No recurrences were observed during follow-up. One week of therapy with 400 mg/day oral albendazole is very effective (cure rate: 100%) in patients with cutaneous larva migrans characterized by multiple and/or extensive lesions. This therapeutical regimen is not accompanied by the appearance of new and/or more severe side effects.     

Efficacy and safety of cyclosporine versus methotrexate in severe psoriasis: a study from north India

Treatment of patients with severe psoriasis is difficult. Among the number of systemic drugs that are available, methotrexate has long been used, but cyclosporine has been recently recommended for the management of severe psoriasis. The purpose of this study was to compare the efficacy and safety of daily cyclosporine with weekly methotrexate in the management of severe psoriasis. Thirty consecutive patients with severe psoriasis were randomly assigned to treatment with cyclosporine or methotrexate. The initial dose of cyclosporine was 3 mg/kg/day, which was increased to a maximum of 4 mg/kg after two weeks of therapy when the response was not adequate. Methotrexate was administered weekly at a dose of 0.5 mg/kg. Clinical response was assessed by calculating PASI score in all patients at biweekly intervals. Patients were followed up fortnightly up to a maximum of 12 weeks. The doses of both drugs were gradually tapered once >75% reduction in disease severity was attained. Marked improvement (>75%) reduction in PASI was noted in all patients except for one in the cyclosporine group. The median time for marked improvement was 5.3 weeks with methotrexate and 6.8 weeks with cyclosporine. Patients on methotrexate were found to have more rapid and complete clearance than those on cyclosporine. Both drugs were well tolerated. Side effects in both the treatment groups were minor, transient, and manageable. At doses with comparable safety profiles, methotrexate resulted in more rapid and cost effective clearance of patients with severe psoriasis. Cyclosporine can provide an effective and safe alternative.     

An AIDS patient with atopic dermatitis-like eruption responsive to systemic anti-fungal treatment.

Patients with the acquired immunodeficiency syndrome (AIDS) often develop unusual skin complications. We describe a case of a 58-year-old man with AIDS who had a history of multiple transfusions with anti-hemophilic factor A. He developed papulovesicular and lichenified skin lesions on his head, face, neck and the extensor aspects of his extremities accompanied by severe pruritus. Atopic dermatitis was suspected; however, intensive treatment with a potent topical corticosteroid and a systemic antihistamine failed. In addition to the decreased subset of CD4-positive lymphocytes characteristic of AIDS, this patient showed an elevated level of serum IgE particularly specific for Candida albicans, probably because he had a chronic candidial infection of the digestive tract. Oral administration of anti-fungal agents Diflucan and Fungizone produced almost complete relief from the atopic dermatitis-like skin disease within 2 weeks.     

Successful treatment of adolescent pemphigus vulgaris by immunoadsorption method.

A 15-year-old girl with pemphigus vulgaris did not respond to oral administration of prednisolone at 45 mg/day. The skin and oral mucous membrane lesions recurred after effective treatments with methylprednisolone pulse therapy and combination therapy with prednisolone and cyclosporine. The finally successful treatment involved eleven cycles of immunoadsorption using a tryptophan column and administration of a moderate dose of prednisolone. Serum gamma-globulin level and anti-intercellular antibody titer decreased from 1.08 g/dl to 0.5 g/dl and 1:320 to 1:20, respectively. She has been well controlled with 21.5 mg/day prednisolone for 8 months after the final adsorption. Considering the physical, mental and social situation of adolescent student patients, immunoadsorption is a highly preferable choice among a variety of treatment modalities for pemphigus vulgaris because it makes the term of hospitalization shorter and avoids undesirable side effects from initial high dose corticosteroids.     

Successful Treatment of Hydroxychloroquine-Induced Recalcitrant Acute Generalized Exanthematous Pustulosis with Cyclosporine: Case Report and Literature Review

Acute generalized exanthematous pustulosis (AGEP) is a cutaneous reaction principally induced by drugs. Spontaneous resolution is observed in most patients. However, severe cases required systemic corticosteroid administration. Hydroxychloroquine, which is used to treat some dermatologic and rheumatologic diseases because of its anti-inflammatory and immunosuppressive effects, is an uncommon cause of AGEP. A 67-year-old female patient presented with severe AGEP due to hydroxychloroquine treatment. She was recalcitrant to supportive care and systemic corticosteroid treatment butwas successfully treated with cyclosporine. Hydroxychloroquine-induced AGEP occurs in women with underlying rheumatologic diseases, has a longer latent period, and has a severe course usually requiring systemic treatment.     

Erythema annulare centrifugum as the presenting sign of the hypereosinophilic syndrome: observations on therapy

A 79-year-old man with severe generalized pruritus and erythema annulare centrifugum as manifestations of his idiopathic hypereosinophilic syndrome slowly responded to long-term therapy with ketoconazole and dapsone. The skin lesions cleared within one month but the pruritus and eosinophilia required seven full months of therapy.     

TREATMENT OF ORAL PEMPHIGUS VULGARIS

Background. Oral pemphigus is considered to represent either an initial stage of pemphigus vulgaris that should be treated with high doses of immunosuppressive drugs to prevent its spread to the skin or a variety of the disease that does not need aggressive treatment. The absence of a widely accepted therapeutic method and the obscure nature of this disease prompted this study. Methods. Twenty-eight patients with oral pemphigus were randomly divided into three groups. The first group received only steroids at a dose of 40 mg of prednisone equivalent. In the other two groups the same dose of steroids was administered, but combined with either 100 mg of cyclophosphamide or 5 mg/kg of cyclosporine as an adjuvant. Direct immunofluorescence studies were performed on specimens obtained from both buccal mucosa and oral skin. Results. There was no significant difference in the duration of treatment required to achieve remission and in the relapse rate among the three groups. The incidence of complications was higher with combination treatment. Deposited immunoreactants were detected with equal frequency in specimens obtained from the buccal mucosa and normal skin. Conclusions. Oral pemphigus most likely represents an initial stage of a disease that can become generalized. Administration of moderate doses of steroids alone is effective in controlling the disease.     

Successful treatment of Rowell syndrome using oral cyclosporine A

Background To present and discuss successful treatment of Rowell syndrome with low dose orally administered cyclosporine in a male patient with refractory course of disease. Methods A 63‐year‐old male patient presented with a five‐week history of widespread, severely pruritic erythematous skin changes that were first seen on the back. Within a few weeks, skin rashes extended to the whole body recessing the face. Because treatment with topical and orally administered corticosteroids was ineffective, we began an immunosuppressive therapy using cyclosporine. Treatment was initiated with an orally administered dosage of 100mg/day and subsequent dose reduction to 50mg/day after 14 days. Result After four weeks of treatment, all clinical signs were resolved; only postinflammatory hyperpigmentation persisted for several weeks. Treatment‐related side effects included arterial hypertension that could be controlled with antihypertensive drugs. Conclusion Primarily described in 1963, Rowell syndrome represents the combination of cutaneous lupus erythematosus and erythema multiforme in a single individual. Controversial discussion concerning the genuine character of the disease is still going on, and even the existence of this entity remains unclear. Several therapeutic regimes are known, including corticosteroids, methotrexate, or dapsone. To the best of our knowledge, this is the first report of successful treatment of Rowell syndrome with low doses of cyclosporine. Orally administered low‐dose cyclosporine seems to be a potent alternative treatment option for patients with Rowell syndrome refractory to standard therapies.     

Toxic epidermal necrolysis as a dermatological manifestation of drug hypersensitivity syndrome

Drug hypersensitivity syndrome (DHS) is believed to be an adverse idiosyncratic drug reaction associated mainly with administration of aromatic antiepileptic drugs, such as phenytoin, carbamazepine, phenobarbital, lamotrigine. The syndrome is defined by the clinical triad of fever, skin rash and internal organ involvement and can be life-threatening condition. We describe three patients treated in our institution. The first was a 32-year-old man who developed toxic epidermal necrolysis (TEN) with pulmonary and liver involvement after initiation of lamotrigine therapy for concomitant epilepsy. The second 32-year-old man was treated with salazopyrine and omeprazole in order to relief the symptoms of inflammatory bowel disease, but as a result developed toxic epidermal necrolysis with elevated liver enzymes. The third patient was a 28-year-old man with long history of alcohol abuse who began treatment with carbamazepine and a few days later he was admitted to the clinic with symptoms of severe disseminated skin rash. The patients had peripheral eosinophilia. All the patients needed urgent life-saving therapy, intensive care and nursing. The culprit drug was discontinued and prompt systemic therapy with corticosteroids at an initial dose of 2 mg/kg/d and with broad spectrum antibiotics was started. Topical therapy included spraying Avène thermal water and local antiseptics. Resolution and epithelization of skin erosions were observed in about 4 weeks after the initiation of the therapy. Medications can give rise to certain adverse reactions including serious cutaneous and systemic involvement. TEN is a rare complication of DHS. Patients who develop DHS need optimal and adequate treatment. The concomitant use of corticosteroids and broad spectrum systemic antibiotics is essential. The local therapy plays an important part in relieving symptoms and should consist of mild preparations with minimally sensitizing potential.     

Guidelines for management of atopic dermatitis

Atopic dermatitis (AD) is a chronic relapsing eczematous skin disease characterized by pruritus and inflammation and accompanied by cutaneous physiological dysfunction (dry and barrier-disrupted skin). Most of the patients have atopic diathesis. A standard guideline for the management (diagnosis, severity classification and therapy) of AD has been established. In our guideline, the necessity of dermatological training is emphasized in order to assure diagnostic skill and to enable evaluation of the severity of AD. The definitive diagnosis of AD requires the presence of all three features: (i) pruritus; (ii) typical morphology and distribution; and (iii) chronic and chronically relapsing course. For the severity classification of AD, three elements of eruption (erythema/acute papules, exudation/crusts and chronic papules/nodules/lichenification) are evaluated in the most severely affected part of each of the five body regions (head/neck, anterior trunk, posterior trunk, upper limbs and lower limbs). The areas of eruption on the five body regions are also evaluated, and both scores are totaled (maximum 60 points). The present standard therapies for AD consist of the use of topical corticosteroids and tacrolimus ointment as the main treatment for the inflammation, topical application of emollients to treat the cutaneous physiological dysfunction, systemic antihistamines and anti-allergic drugs as adjunctive treatments for pruritus, avoidance of apparent exacerbating factors, psychological counseling and advice about daily life. Tacrolimus ointment (0.1%) and its low-density ointment (0.03%) are available for adult patients and 2–15-year-old patients, respectively. The importance of the correct selection of topical corticosteroids according to the severity of the eruption is also emphasized. Furthermore, deliberate use of oral cyclosporine for severe recalcitrant adult AD is referred.     

Remarkable response of lipoid proteinosis to oral dimethyl sulphoxide

We report the successful treatment of lipoid proteinosis in a 41-year-old man using oral dimethyl sulphoxide. The initial dosage was 40 mg/kg/day, which was then increased progressively to 60 mg/kg/day. After 3 years of treatment, the patient's skin lesions, hoarseness of voice and abnormal oesophageal function improved remarkably. No side-effects were noted except for a garlic-like smell on the patient's breath.     

Mirtazapine for reducing nocturnal itch in patients with chronic pruritus: a pilot study

Nocturnal pruritus is a significant problem for patients with inflammatory skin diseases and many systemic diseases. The oral therapies currently available have a limited effect. We present an open, uncontrolled pilot study of 3 patients with inflammatory skin diseases and severe nocturnal pruritus who underwent treatment with mirtazapine (Remeron), a noradrenergic and specific serotonergic antidepressant. Mirtazapine is a safe medication without serious side effects and may be an effective alternative for the treatment of nocturnal pruritus.     

Palmoplantar pustulosis with pustulotic arthroosteitis involving temporal osteomyelitis and meningitis

A 46-year-old man who had been suffering from palmoplantar pustulosis (PPP) for 3 years had anterior chest pain and left temporal pain from six months after the onset of his disease. A bone scan revealed abnormal uptake at the sternoclavicular joint and left temporal region. The head CT and MRI gave the diagnosis of temporal osteomyelitis with meningitis and myositis. His headache continued even after tonsillectomy and was effectively treated with cyclosporine A (3 mg/kg/day). Oral cyclosporine A was beneficial for the osteomyelitis and skin lesions. Sterile lytic bone lesions occurring most often at the sternocostoclavicular joint have been associated with PPP. However, there have been no reports of a PPP patient with temporal osteomyelytic involvement.     

DRESS syndrome caused by efalizumab

We report a case of drug reaction with eosinophilia and systemic symptoms (DRESS) to efalizumab. A 52-year-old man developed a widespread papulovesicular rash after 4 weeks of treatment with efalizumab (1.0 mg/kg/week) for treatment-resistant severe psoriasis. Histology revealed a subepidermal blister with eosinophil-rich inflammatory cell infiltrate. Subsequently, the patient developed high peripheral eosinophilia, abnormal liver function, malaise and fever, all requiring inpatient admission. Efalizumab was discontinued immediately, but the rash persisted for 4 months and was only controlled by oral prednisolone at a dose of 30 mg/day. To our knowledge, this is the first reported case of DRESS caused by efalizumab.     

Systemic isotretinoin treatment of oral and cutaneous lichen planus

Lichen planus of the skin and mucous membranes may be disabling. Severe pruritus or bullous lesions may be incapacitating when they occur while erosive oral lesions may be extremely painful. Various treatment modalities have been attempted including corticosteroids (parenteral, intralesional, and topical) and photochemotherapy. Recent successful therapeutic trials of topical retinoic acid and oral etretinate have been completed. Two patients with cutaneous and severe erosive oral lichen planus unresponsive to conventional therapies responded to a trial of oral isotretinoin with prompt and successful remission of cutaneous and oral lesions. This suggests that systemic isotretinoin may have a unique position in the treatment of mucous membrane lichen planus that is refractory to conventional therapies.     

Treatment of pyoderma gangrenosum with cyclosporine.

BACKGROUND AND DESIGN--Pyoderma gangrenosum is a chronic inflammatory ulcerative skin disease of unknown origin, often associated with various diseases including inflammatory bowel disease, inflammatory arthritis, monoclonal gammopathies, hepatitis, and myeloproliferative disorders. Treatment of associated systemic disorders may improve the ulcers, but lesions may be recalcitrant and persist for months to years. Therapy for pyoderma gangrenosum includes high-dose systemic corticosteroids, sulfa drugs such as sulfasalazine, clofazimine, and immunosuppressive agents such as mercaptopurine and azathioprine; these drugs are sometimes ineffective. RESULTS--We present a series of 11 patients with pyoderma gangrenosum, with a wide range of underlying diseases, whose ulcers were refractory to usual therapy and who were treated with low-dose cyclosporine. Ten of the 11 patients cleared rapidly and completely with cyclosporine therapy. CONCLUSIONS--Cyclosporine should be seriously considered as a primary form of treatment for pyoderma gangrenosum.     

Lichenoid nail changes as sole external manifestation of graft vs. host disease

A 56-year-old-man who had refractory anemia with an excess of blasts underwent an allogeneic peripheral blood stem cell transplantation (PBSCT) from his brother after preparation with melphalan and fludarabin. He received GvHD (graft-vs.-host disease) prophylaxis with cyclosporine from day -1 at a daily dose of 5 mg/kg of body weight. The daily dosage was tapered gradually from day +20. On post-PBSCT day 68 he developed acute cutaneous GvHD grade 3 and acute gastrointestinal GvHD grade 2-3, which was resolved with a daily dose of 1 mg/kg of body weight of prednisone. The patient was discharged in good clinical condition and without signs of GvHD, and he started tapering his immunosuppressive treatment. By day 160 he developed oral lichen planus-like changes, with several reticulate white lesions on the oral mucosa. A biopsy specimen was microscopically consistent with lichenoid GvHD (Fig. 1). By day 150 after PBSCT, when he was being treated with CsA 100 mg once daily and prednisone 10 mg once daily, his fingernails started to grow abnormally and gradually became dystrophic and painful. Two months later his toenails became similarly affected. Although affecting all finger and toe nails, the lesions were especially important in both thumbs. Physical examination revealed multiple findings on his nails (Fig. 2): thickening, fragility, onycholysis, longitudinal striations, and even pterygium. The micological cultures were negative. A biopsy specimen showed an sparse papillary dermis lymphoid infiltrate with focal exocytosis and presence of isolated multiple necrotic keratinocytes (Fig. 3). These findings were interpreted as a lichenoid GvHD with oral and nail involvement. The patient did not have other associated cutaneous lesions. He did not develop signs or symptoms consistent with hepatic GvHD. In May 2000 thalidomide was added to the immunosuppressive therapy, at a daily dose from 100 to 300 mg according to tolerance (constipation, sedation, ...). The lesions on the oral mucous showed a substantial improvement, but the nail changes remained more or less stable. Thalidomide was discontinued after 7 months because the patient displayed numbness and tingling in the hands and feet consistent with a peripheral neuropathy. Twenty days later he stopped taking thalidomide and the oral lichenoid lesions worsened, resulting in difficulty in eating. He also developed periungueal erythema, swelling and intense pain after minimal trauma. The daily dose of prednisone increased to 20-30 mg with moderate improvement. However, the dose could not be increased because of the secondary immunosuppressive effects. Twenty-three months post-PBSCT the patient remains with intense oral and nail lichenoid lesions.