UV-B phototherapy clears psoriasis through local effects

OBJECTIVE: To determine if UV-B phototherapy clears psoriasis through systemic effects. DESIGN: Randomized, within-subject comparison of change in psoriasis in 3 plaques in patients attending for whole-body UV-B therapy. Change in patients' psoriasis plaques covered during UV-B treatment was compared with plaques in an untreated control group. SETTING: University hospital phototherapy unit. PATIENTS: The study population comprised 17 patients with chronic plaque psoriasis treated with UV-B and 24 psoriasis control patients awaiting UV-B phototherapy. INTERVENTIONS: Treatment with a standard 3-times weekly narrowband TL-01 UV-B regimen. Three similar plaques were randomly allocated to be covered every treatment, covered for 2 of 3 weekly treatments, and exposed to local UV-B every treatment. Similar plaques were selected in control patients (awaiting but not yet started UV-B therapy). Severity of psoriasis plaques was assessed using a scaling, erythema, and induration (SEI) scoring system. MAIN OUTCOME MEASURES: Change in SEI score of the selected plaques over the complete treatment course for UV-B-treated patients and change over 3 weeks in SEI score of plaques covered during UV-B treatment compared with that of plaques in controls. RESULTS: There was a significant (P<.001) difference in how much the SEI score changed in the 3 plaques in UV-B-treated patients. It fell by a mean of 7.6 for uncovered plaques compared with 3.2 for plaques covered during each UV-B exposure (95% confidence interval for difference, 3.0 to 5.8). In patients awaiting UV-B, SEI score of plaques fell by a mean of 0.4 over 3 weeks, compared with a mean fall of 1.4 for covered plaques in UV-B-treated patients (95% confidence interval for difference in means, 0.1 to 2.0). CONCLUSIONS: If UV-B therapy has any systemic effect capable of improving psoriasis, this effect is small and unlikely to be of clinical importance. It is insufficient to alter interpretation of findings of within-subject comparative phototherapy studies. UV-B phototherapy works for chronic plaque psoriasis through local effects.     

Therapie der lymphomatoiden Papulose mit Bade-PUVA-(Balneo-)Photochemotherapie

Lymphomatoid papulosis is a rare lymphoproliferative disorder of the skin. The standard therapeutic regimen is systemic (oral) photochemotherapy (PUVA). We examined the efficacy of PUVA-bath photochemotherapy in a patient requiring heart transplantation because of idiopathic dilatated cardiomyopathy and a relative contraindication against systemic 8-methoxypsoralen. The 42-years old male patient had suffered for 15 years with itching papules and plaques which clinically, immunohistochemically and molecular biologically were diagnosed as lymphomatoid papulosis. PUVA-bath photochemotherapy with 8-MOP was initiated. After 29 treatments the plaques disappeared completely. After 44 sessions (cumulative UV-A dose 206 J/cm2) the patient's skin almost was clear. PUVA-bath photochemotherapy proved to be a therapeutic alternative to systemic PUVA-treatment in this case of lymphomatoid papulosis.     

Olmsted syndrome: report of a new case

We report the case of a 20-year-old man, who was born with an intense erythema of the genital area, unresponsive to any treatment employed. When he was 9 months old, he presented with well-defined hyperkeratotic erythematous plaques around the mouth, eyes, nose, and perianal area, with similar plaques on the lateral aspect of the neck and axillae. At the same time the erythema of the genital area became hyperkeratotic. When he was 2 years old, he presented with a disabling palmoplantar keratoderma, initially focal, and later diffuse, also unresponsive to local or systemic treatments employed. The lesions have varied during the course of the disease without ever clearing completely. The axillary and inguinal plaques have shown spontaneous resolution on occasion. Six skin biopsies have been performed with no conclusive histological diagnosis of any of the typical disorders of keratinization. All treatments, topical and systemic, including etretinate and acitretin, have failed to improve the condition. We believe that this patient has Olmsted syndrome, a rare form of palmoplantar keratoderma with periorificial keratotic plaques.     

Papulonodular mucinosis in systemic lupus erythematosus

A 41-year-old man with systemic and serological manifestations of systemic lupus erythematosus presented with a diffuse eruption comprising annular plaques. Histopathology revealed diffuse deposition of mucin throughout the dermis, consistent with papulonodular mucinosis. This uncommon entity of unclear pathogenesis has been described in systemic lupus erythematosus, discoid lupus erythematosus, and subacute cutaneous lupus erythematosus.     

结节性硬皮病

报告1例结节性硬皮病,患者在诊断为系统性硬皮病4年后,胸、背部出现瘢痕疙瘩样皮损,根据病史和组织病理学改变诊断为结节性硬皮病。该型硬皮病多继发于系统性硬皮病,治疗困难。     

变应性肉芽肿病1例

报告1例变应性肉芽肿病。患者男,71岁。躯干、四肢红斑、瘙痒伴双下肢麻木1年。皮肤科检查见躯干、四肢泛发性红色斑块。组织病理示真皮血管周围及胶原束间多数中性粒细胞、嗜酸性粒细胞浸润。经强的松治疗后,皮损明显消退。     

Skin lesions serve as clues to relapse of pediatric blastic plasmacytoid dendritic cell neoplasm

A 10‐year‐old girl with a history of blastic plasmacytoid dendritic cell neoplasm, a rare malignancy in children, presented with recurrent skin eruptions beginning while on maintenance chemotherapy, including mildly pruritic skin‐colored plaques, tender indurated nodules, and violaceous bound‐down plaques. This case highlights an unusual presentation of relapsed blastic plasmacytoid dendritic cell neoplasm on chemotherapy, with skin lesions providing important clues to the progression of systemic disease.     

Exudative discoid and lichenoid chronic dermatosis (Sulzberger-Garbe): presentation with a puzzling penile plaque*

Objective Report of two cases of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), with typical penile lesions. Differential diagnosis of persistent penile plaques. Background Following remission of the generalized eruption of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), it is not unusual to see a single remaining lesion localized on the penis. Since Sulzberger et al. (Sulzberger MB, Witten VH, Hunt JA. Puzzling persistent penile plaques. Arch Dermatol 1956:73:101-109) reviewed dermatoses presenting with puzzling persistent penile plaques in 1955, further dermatoses have evolved as important differential diagnoses. Much controversy has arisen as to the justification of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe) as a disease entity. Conclusions Diagnosis of most cases of persistent plaques of dermatitis on the penis is possible with a careful examination of the entire skin, and a thorough history to discover past lesions. The possibility of malignancy makes a biopsy mandatory. Exudative discoid and lichenoid chronic dermatosis (Sulzberger-Garbe) typically responds well to systemic steroids. Intrale-sional triamcinolone acetonide has been demonstrated to be effective in the treatment of a persistent penile plaque in exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), and may be helpful in discontinuing or reducing systemic therapy to a level compatible with long-term treatment.     

SWEET''S PANNICULITIS

We report the development of acute, tender, erythematous plaques in a 65 year old female with Myelodysplastic Syndrome transforming to Acute Myeloid Leukaemia. The clinical presentation strongly suggested Sweet's syndrome. Histopathological examination of the plaques showed a normal epidermis, dermal and subcutaneous oedema, and large numbers of polymorphs in the panniculus. The eruption responded quickly to systemic steroids, with recrudescence when steroid dosage was reduced. She remained symptom free when prednisolone dosage was reduced more slowly.     

Systemic sclerosis, localized morphea, en coup de sabre and aortic regurgitation: a rare association

A 24-year-old male presented with Raynaud's phenomenon, digital infarcts, salt and pepper pigmentation and characteristic facies. There was gastrointestinal involvement clinically, endoscopically as well radiologically. In view of these findings and the demonstration of anti-nuclear antibodies with a homogeneous pattern of immunofluorescence and ScL-70 antibodies, he was diagnosed as a case of diffuse cutaneous systemic sclerosis. In addition, the patient had plaques of localized morphea, en coup de sabre with facial hemiatrophy on the left side, and mild aortic regurgitation (detected on echocardiography). The occurrence of these rare associations of localized plaques of morphea, en coup de sabre and aortic regurgitation in a single case of systemic sclerosis is quite an exceptional and interesting occurrence.     

Topisches Interferon-βEin Vorschlag zur additiven Therapie der ulzerierten Mycosis fungoides

The combination of systemic interferon-alpha and systemic photochemotherapy is one of the most effective and most frequently administered treatment regimens for mycosis fungoides. Two patients with mycosis fungoides stage IIb with ulcerated tumors were treated with this regimen. While the plaques responded favorably to the combination therapy, the ulcerated tumors were quite resistant despite treatment for several weeks. When topical interferon-beta in a gel base was added to the regimen, a rapid resolution of the tumors was noticed. This observation suggests topical interferon-beta may be an effective adjuvant strategy to be combined with systemic therapy.     

Nephrogenic fibrosing dermopathy in a patient with systemic lupus erythematosus and acute lupus nephritis

Abstract:  Nephrogenic fibrosing dermopathy is a recently recognized skin disorder similar in appearance to scleromyxedema but without the systemic involvement. We describe a 14-year-old girl with new-onset systemic lupus erythematosus and acute lupus nephritis who developed on the lower extremities confluent hyperpigmented, woody, indurated plaques that contained groups of coalescing erythematous papules. Nephrogenic fibrosing dermopathy was diagnosed histologically. Possible etiologies are discussed.     

Necrobiosis Lipoidica-like Skin Lesions in Systemic Sarcoidosis

A 62-year-old woman with systemic sarcoidosis developed erythematous plaques on her lower legs. Clinically, two kinds of skin lesions were distinguished; one type formed brownish-red plaques with induration suggesting plaque-type skin sarcoid, and the other formed purplish erythematous plaques with atrophic centers resembling necrobiosis lipoidica. In spite of this clinical appearance, a biopsy specimen from one of the latter lesions revealed typical skin sarcoid histology composed of discrete non-caseating granulomas, while that from one of the other lesions showed necrobiotic changes of collagen bundles surrounded by epitheloid histiocytes and foreign-body giant cells. Because cutaneous involvement of sarcoidosis may mimic necrobiosis lipoidica clinically and/or histologically, we diagnosed her skin lesions as necrobiosis-like skin sarcoid.     

Atypical severe progressive perioral ulceration due to herpes simplex virus on the background of undiagnosed HIV/AIDS

Perioral ulcerative plaques have a broad list of differential diagnoses. We describe an unusual presentation of chronic progressive perioral ulceration due to herpes simplex type (HSV)-1 on a background of undiagnosed human immunodeficiency virus infection with acquired immunodeficiency syndrome. Whilst chronic mucocutaneous HSV is an AIDS-defining condition with both HSV-1 and HSV-2 implicated, typical reported cases describe vesicular eruptions rather than perioral ulcerative plaques. This case highlights that common infections may present atypically in immunocompromised individuals and may be a clue to underlying systemic illness.     

Localized axillary milia en plaque: a rare cutaneous case presentation of systemic amyloidosis

Systemic AL amyloidosis is known to be associated with plasma cell dyscrasias, including multiple myeloma. The cutaneous manifestations of systemic AL amyloidosis are varied, but typically include waxy plaques or subcutaneous nodules. We report a woman who presented with bilateral eruptions of hyperpigmented plaques in her axillae, which were diagnosed as milia en plaque. She had a history of multiple myeloma, for which she was under the care of a haematologist. This is the first documented case, to our knowledge, of an eruption in the axillae being milia en plaque.     

结节性脂膜炎78例临床分析

总结了78例经病理证实的脂膜炎患者的临床资料。本病多发生在中青年女性,分皮肤型和系统型,主要的临床表现有发热(70.51%)、关节痛(66.67%)、皮下浸润性伴有自觉痛及触痛的结节或斑块(100%)。系统型病人可伴有呼吸、循环、消化系统脏器受累及实验室检查改变。对其诊断名称、病因、早期诊断、易误诊疾病的鉴别诊断等进行了探讨。认为此病临床上并非罕见,可因其临床表现多样化不被认识而误诊,因而临床上对中青年女性、不明原因发热伴非游走性关节疼痛、皮下出现伴有自觉痛及触痛的浸润性结节或斑块应高度警惕脂膜炎的可能,须早期行病理检查。     

Palmoplantar keratoderma and skin grafting: postsurgical long-term follow-up of two cases with Olmsted syndrome

Abstract:  Olmsted syndrome is a rare congenital mutilating palmoplantar keratoderma associated with periorificial keratotic plaques. Treatment options include topical keratolytics, systemic retinoids, and debulking procedures. Full-thickness excision of hyperkeratotic plaques followed by skin grafting has been reported in the medical literature, although long-term results have not been evaluated. We present two cases of Olmsted syndrome with severe palmoplantar keratoderma treated with excision and skin grafting, along with long-term clinical results 11 years (patient 1) and 6 years (patient 2) following the initial surgery.     

Specific cutaneous lesions in patients with systemic sarcoidosis: relationship to severity and chronicity of disease

Background. Specific (granulomatous) cutaneous lesions are seen in 9–37% of cases of systemic sarcoidosis, and are usually classified into maculopapules, plaques, lupus pernio (LP), scar sarcoidosis, and subcutaneous sarcoidosis. Their prognostic significance has not been fully established. Aim. To analyse the relationship between the clinical type of granulomatous cutaneous lesions and the systemic features and prognosis of systemic sarcoidosis. Methods. The clinical charts of 86 patients (19 men, 67 women, mean age 46.82 years) with systemic sarcoidosis and granulomatous cutaneous involvement followed up for > 2 years at Bellvitge University Hospital were reviewed. Results. Cutaneous lesions developed before or at the time of diagnosis of systemic sarcoidosis in 80.23% of patients. The main cutaneous lesions were classified as maculopapules (28 patients), plaques (31), LP (6), scar sarcoidosis (7) and subcutaneous sarcoidosis (14). Erythema nodosum (EN) was seen in 30 patients. Radiological stage was 0 for 8 patients, I for 48, II for 24, III for 5 and IV for 1. Systemic sarcoidosis activity persisted for > 2 years in 47 patients, and 42 received systemic corticosteroid treatment for their disease. Maculopapular and subcutaneous sarcoidosis were mainly seen in patients with EN and radiological stage I. Plaques and LP were associated with chronic disease and requirement for systemic corticosteroids. Conclusions. Cutaneous granulomatous lesions are usually present at the diagnosis of systemic sarcoidosis, and the type of cutaneous involvement may have prognostic significance.     

Gadolinium-Associated Nephrogenic Systemic Fibrosis in a 9-Year-Old Boy

Abstract:   We describe a 9-year-old boy on chronic hemodialysis who presented with hyperpigmented, tightly bound-down, indurated plaques of his bilateral lower extremities. Pertinent history included a recent series of magnetic resonance imaging tests utilizing gadolinium-based contrast media. Histopathology showed widened septae with increased fibroblasts and collagen in the subcutis consistent with nephrogenic systemic fibrosis. Nephrogenic systemic fibrosis is a unique fibrosing disorder characterized by skin thickening of the extremities and trunk, resembling that seen in systemic sclerosis. The majority of cases have been reported in dialysis or renal transplant patients. This case of nephrogenic systemic fibrosis in a young child is presented to raise awareness in the pediatric community of nephrogenic systemic fibrosis and of gadolinium contrast being a possible trigger.     

Intralesional cyclosporine for psoriasis. Relationship of dose, tissue levels, and efficacy.

BACKGROUND AND DESIGN--To avoid systemic side effects, topical and intralesional administration of cyclosporine has been used; however, only intralesional administration has been successful. To understand more about the dosing requirements and resultant tissue levels of intralesional cyclosporine, we injected psoriasis plaques in a double-blind fashion with three different concentrations of cyclosporine (17 mg/mL in seven patients, 10 mg/mL in 13 patients, and 2.5 mg/mL in 11 patients) or matching vehicle three times weekly for 4 weeks. RESULTS--Statistically significant improvement was observed in plaques treated with 17 mg/mL (P = .003) compared with vehicle-treated plaques; the improvements in plaques treated with 10 mg/mL (P = .078) and 2.5 mg/mL (P = .054) achieved marginal statistical significance compared with vehicle treatment. Four weeks after discontinuation of therapy, the change from pretherapy in plaques that had received 17 mg/mL of cyclosporine was statistically significantly better (P less than .0001) than that with vehicle treatment. A similar finding but of marginal statistical significance (P = .059) occurred in the plaques that had received 10 mg/mL of cyclosporine. Throughout the study, untreated psoriasis plaques did not improve. Transient pain was the most common side effect noted with both cyclosporine and vehicle injections. Tissue levels of cyclosporine tended to be highest in plaques receiving the 17-mg/mL concentration; blood levels of cyclosporine were low throughout the study. CONCLUSIONS.--Intralesional cyclosporine requires a sufficient dosage to improve psoriasis, apparently by a local mechanism of action. Improvement may persist for 4 weeks or longer.