Mild to moderate hidradenitis suppurativa treated with local excision and primary closure.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic skin disease with a great impact on quality of life. Treatment with antibiotics or anti-inflammatory drugs, such as prednisone or TNF-alpha-inhibitors usually achieves only temporary improvement. Surgical intervention is considered as the only curative treatment for recurrent lesions. OBJECTIVE: To determine the efficacy and patient satisfaction of local excision followed by primary closure. METHODS: Between 2005 and 2010, 92 local excisions with primary closure were performed in 57 patients with mild to moderate HS. All patients were treated on an outpatient basis, under local anaesthesia. Local excision was defined as complete excision of the affected tissue, beyond the borders of activity, leaving clear margins. The medical records were reviewed retrospectively in 2010. The final outcome of the procedure, cosmetic appearance and patient satisfaction was measured using a questionnaire. RESULTS: Successful treatment, without recurrence, was accomplished in 66% of the cases. The intervention was generally well tolerated: 84% of the patients stated that they would undergo the same surgical procedure again if necessary, and 89% would recommend the procedure to other patients. CONCLUSION: Local excision followed by primary closure is a valuable treatment for patients with mild to moderate HS (Hurly stage I & II), with low morbidity and a high patient satisfaction rate.

New-onset polyarthritis during successful treatment of hidradenitis suppurativa with infliximab.

BACKGROUND: Hidradenitis suppurativa (HS) can be associated with several forms of arthritis, usually considered as reactive arthritis. A new observation is that some patients with HS develop arthritis after treatment with infliximab (antitumour necrosis factor-α). OBJECTIVES: A retrospective study was performed to establish the frequency and clinical presentation of new-onset arthritis during infliximab treatment. METHODS: Between 2005 and 2009, 27 individuals with severe HS were treated with infliximab and followed up closely. Laboratory parameters and side-effects were recorded. The frequency of arthritis was compared with control groups consisting of 227 patients with HS not treated with any biological, 22 patients with HS treated with adalimumab and 28 patients with psoriasis treated with infliximab, in the same period at the same clinic. RESULTS: Five of the 27 patients with HS (18%) treated with infliximab developed an acute and painful polyarthritis during treatment. The arthritis occurred on average after 12 months of treatment, was not clearly associated with anti-infliximab antibodies and resolved on average after 4 months. Interestingly, none of the patients had suffered from arthritis before despite the long duration of HS and all showed a good skin response to infliximab. Moreover, arthritis was not observed in any of the control groups. Compared with the adalimumab group and the psoriasis group, odds ratios of 7·241 [95% confidence interval (CI) 1·15-45·6] and 9·025 (95% CI 1·45-55·82) were calculated. CONCLUSIONS: The five cases described in this article suggest that infliximab treatment in HS can induce a transient but severe polyarthritis. The underlying mechanisms remain to be investigated further.

Dermatologists are essential for quality of care in the general practice of medicine.

Dermatology is an increasingly growing specialty with several subspecialties that frequently overlap with other disciplines. Dedication to specific areas varies widely between countries, even within the European Union. The lack of uniform criteria that regulate the practice of dermatology and its subspecialties has a negative impact on the distribution of resources. Consequently, this may impair adequate patient care as access to dermatologists, who are the best trained physicians to recognize and treat skin disorders, may be delayed or unavailable. Not uncommonly, especially in the hospital setting, many specialists are consulted for a skin condition before a referral is made to a dermatologist. In this article, through a case series from daily practice, we illustrate the need for dermatologists to be recognized as the most suitable specialists to diagnose and treat skin diseases. A prompt referral is probably more cost-effective than any other measure, reducing patient morbidity and, in some instances, it can also be life-saving. Another issue that merits consideration is the reimbursement of selected, non-medicated pharmaceuticals, that are medically indicated for some patients with serious dermatological disorders.

Review and expert opinion on prevention and treatment of infliximab-related infusion reactions.

Infliximab (Remicade; Schering-Plough, Kenilworth, NJ, U.S.A.) is a chimeric monoclonal antibody that acts as a tumour necrosis factor-alpha inhibitor. Infliximab is registered for the treatment of rheumatoid arthritis, psoriatic arthritis, Crohn disease, ulcerative colitis, ankylosing spondylitis and plaque-type psoriasis. Like other foreign protein-derived agents, infliximab may lead to infusion reactions during and after infusion. Infusion reactions occur in 3-22% of patients with psoriasis treated with infliximab. Most of these reactions are mild or moderate and only few are severe. Nevertheless, they may lead to discontinuation of treatment. As infliximab for psoriasis is prescribed as a last resort and is in most cases very effective, discontinuation of treatment is undesirable. With proper care and prevention of the infusion reactions the need to discontinue treatment with infliximab can be diminished. The objective of this article is to present a guideline for the management of infliximab-related infusion reactions, based on the best available evidence. This guideline can be used in patients with psoriasis as well as in dermatology patients receiving infliximab for off-label indications such as hidradenitis suppurativa or pyoderma gangrenosum.

Long-term efficacy of a single course of infliximab in hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa is a chronic inflammatory skin disease characterized by abscess formation, predominantly in the axillae and groins. The disease is difficult to treat and has a severe impact on quality of life. Recently, several case reports have been published describing successful treatment of hidradenitis suppurativa with infliximab and other tumour necrosis factor alpha inhibitors. OBJECTIVES: To evaluate the long-term efficacy of a single course of infliximab. METHODS: Ten patients with severe, recalcitrant hidradenitis were treated with infliximab (three infusions of 5 mg kg(-1) at weeks 0, 2 and 6) and followed up for at least 1 year. The disease activity was measured using laboratory parameters and a recently developed acne score. The patients rated the efficacy of infliximab on a 10-point scale at regular intervals. Quality of life was measured before and after treatment using the Dermatology Quality of Life Index (DQLI). RESULTS: All patients improved within 2-6 weeks. The average acne score diminished from 164+/-50 (mean+/-SD) before treatment to 89+/-49 after 1 year (P=0.002). The mean CRP (C-reactive protein) was reduced from 31.7 mg mL(-1) to 5.5 mg mL(-1) after 1 month (P=0.015). Patients judged the efficacy with a score of 7.9. The mean DQLI was reduced from 18.4+/-7.9 before treatment to 9.3+/-9.1 after 1 year (P=0.007). In three patients long-lasting improvement was observed, with no recurrence of lesions in a 2-year follow-up period. The other patients showed recurrence of lesions after 8.5 months (range 4.3-13.4 months). CONCLUSIONS: Infliximab is an effective treatment in severe hidradenitis suppurativa, leading to reduction of symptoms for a prolonged period.

Acquired palmoplantar keratoderma and immunobullous disease associated with antibodies to desmocollin 3.

We present a case of immunobullous disease with an impressive acquired palmoplantar keratoderma (PPK) and unique antigenicity. The palms of the patient showed hyperkeratotic ridges with a tripe pattern that decreased with the amelioration of the immunobullous condition. The histopathology of perilesional skin (blister) demonstrated eosinophilic spongiosis and suprabasal blistering as in pemphigus vulgaris. In palmar skin, acantholysis, intraepidermal pustules, papillomatosis and marked hyperkeratosis were observed. Direct and indirect immunofluorescence displayed intraepidermal intercellular IgG staining as well as linear IgG staining along the epidermal basement membrane zone. Immunochemical assays revealed IgG antibodies to the desmosomal protein desmocollin 3 and to the hemidesmosomal proteins BP230 and LAD-1. Affinity-purified antidesmocollin 3 serum IgG bound to monkey oesophagus in the typical pemphigus pattern. Desmocollins are transmembrane proteins of the desmosome. Desmosome diseases may cause hereditary PPK. In our patient with acquired PPK, we hypothesize that the antibodies to desmocollin 3 were, apart from their role in eliciting the pemphigus-like blistering disease, also implicated in the pathogenesis of the PPK.

[Loss of cranial-nerve function caused by early syphilitic meningitis: the comeback of a pre-war syndrome]. / Hersenzenuwuitval door vroege syfilitische meningitis: terugkeer van een vooroorlogs ziektebeeld.

Two men aged 39 and 38 who had had unprotected insertive and receptive anal sexual contact with men are presented: one had paralysis of the right half of his face and the other man had erythematous macules on the palms of his hands and the soles of his feet as well as partial alopecia, earache and progressive loss of hearing in his left ear. The latter one was also HIV-seropositive and on antiretroviral medication. Syphilitic meningitis was diagnosed in both men. The 2 patients recovered after being treated with intravenous benzyl penicillin. Syphilitic meningitis is a complication seen during the early stages of a syphilis infection. Since the introduction of penicillin it has become a rare disease. Early diagnosis is of importance since syphilitic meningitis has irreversible sequelae.

Causes, investigation and treatment of leg ulceration.

Chronic ulceration of the lower leg is a frequent condition, with a prevalence of 3-5% in the population over 65 years of age. The incidence of ulceration is rising as a result of the ageing population and increased risk factors for atherosclerotic occlusion such as smoking, obesity and diabetes. Ulcers can be defined as wounds with a 'full thickness depth' and a 'slow healing tendency'. In general, the slow healing tendency is not simply explained by depth and size, but caused by an underlying pathogenetic factor that needs to be removed to induce healing. The main causes are venous valve insufficiency, lower extremity arterial disease and diabetes. Less frequent conditions are infection, vasculitis, skin malignancies and ulcerating skin diseases such as pyoderma gangrenosum. But even rarer conditions exist, such as the recently discovered combination of vasculitis and hypercoagulability. For a proper treatment of patients with leg ulcers, it is important to be aware of the large differential diagnosis of leg ulceration.

Acquired arteriovenous malformation induced by pressure: a case report.

Arteriovenous malformations may be congenital or acquired. In the latter case, usually a traumatic injury to the arteries precedes the arteriovenous anastomoses. Two elderly patients presented with large, purple-colored verrucous tumors on the buttocks. Both patients were obese and immobile, and reported repeated bleeding from the lesions after minor trauma. The tumors were soft and could be emptied by applying pressure. Doppler examination revealed arterial pulsations over the lesions. Both cases were diagnosed as pressure-induced arteriovenous malformations. The lesions are assumed to have been caused by tissue damage in the deep subcutis induced by decubitus.

Natural course of physical and chronic urticaria and angioedema in 220 patients.

BACKGROUND: Information about spontaneous remission of chronic urticaria is limited. OBJECTIVE: To investigate the natural course of urticaria, we followed up 220 adults in a prospective study. METHODS: Patients were followed up for 1 to 3 years to evaluate interventions, to detect latent causes, and to study the natural course of urticaria. The diagnosis was made by detailed history-taking as well as laboratory and provocation tests. RESULTS: Thirty-five percent of all patients were free of symptoms after 1 year. In 28.9% of patients, symptoms had decreased. Spontaneous remission occurred in 47.4% of the patients in whom no cause of their urticaria and/or angioedema could be identified and in only 16.4% of the patients with physical urticaria. A cause could be identified in 53.1% of the patients. Thirty-six percent of the patients had idiopathic urticaria. Chronic idiopathic urticaria combined with physical urticaria occurred in 10.9%. CONCLUSION: In general, the prognosis for spontaneous remission is reasonable, with the exception of the subgroup (33.2%) with physical urticaria.

Dermal organization in scleroderma: the fast Fourier transform and the laser scatter method objectify fibrosis in nonlesional as well as lesional skin.

Scleroderma, a chronic, progressive disorder, is characterized by dermal fibrosis with collagen bundles orientated parallel to the epidermis. Simple objective parameters to evaluate disease progression and therapies are needed. We describe two methods, the laser scatter method and the fast Fourier transform (FFT), to measure collagen bundle orientation and spacing. Lesional sclerodermic skin (LS), nonlesional sclerodermic skin (nonLS), and control skin (CS) sections were evaluated for orientation ratio using the laser scatter method. The FFT was used to calculate orientation ratio, variation, and spacing of collagen bundles. Parameters were correlated with local and mean skin score measurements, on a scale of 0 (normal) to 3 (severely sclerotic). With both the laser scatter method and the FFT, orientation ratios of LS (respectively, 2.16 +/- 0.33 and 1.83 +/- 0.62) were significantly higher than CS (respectively, 1.70 +/- 0.35 and 1.38 +/- 0.15). NonLS orientation ratios (respectively, 1.92 +/- 0.15 and 1.48 +/- 0.44) were between LS and CS ratios. Orientation variation and bundle spacing of LS (respectively, 57.3 +/- 19.4 and 15.7 +/- 5.6 microm) were significantly reduced compared to CS (respectively, 73.8 +/- 15.0 and 18.9 +/- 1.9 microm). NonLS orientation ratios (respectively, 57.2 +/- 29.0 and 15.6 +/- 6.1 microm) were similar to LS. Bundles in LS are more parallel, show less variation in orientation, and are more densely packed than in CS. There was a linear correlation between mean skin score and orientation ratio. Local skin score was not linearly correlated to orientation ratio. Our findings suggest that nonLS dermis without clinical sclerosis already shows fibrotic characteristics. Both techniques were easy to use and suitable for objectifying dermal fibrosis in scleroderma lesions. FFT is more accurate and reproducible than the laser scatter method and allows simultaneous pathological evaluation of the location of the analyzed tissue sections. Future studies will need to focus on the correlation between clinical disease severity and collagen bundle characteristics.

Treatment of patients with systemic sclerosis with extracorporeal photochemotherapy (photopheresis).

BACKGROUND: Effective treatment modalities for systemic sclerosis, a life-threatening and disabling disease, are still lacking. Possible efficacy of photopheresis has been reported in several studies. Because of the complexity of the treatment, placebo-controlled trials are difficult to perform. OBJECTIVE: We investigated the effect of photopheresis on clinical parameters (skin score and internal organ functions), immunologic parameters, and quality of life. METHODS: Nineteen patients with progressive systemic sclerosis of less than 5 years' duration were randomized into 2 groups. One group (group A) received photopheresis for 1 year, the other group (group B) received no treatment at all. After 1 year the groups switched (crossover design). Photopheresis was performed on 2 consecutive days every 4 weeks; the psoralens were administered parenterally. The main outcome parameter was the skin score after 1 year of treatment compared with that of the control group. RESULTS: The average skin score improved with 5.4% (standard error [SE], 20. 8%) in group A and deteriorated with 4.5% (SE, 13.8%) in group B (not significant; P =.71). Before crossover, the average increase in skin score was 5.3% (means of entire group). No change was observed in other clinical parameters. Approximately 1 year after crossover, the skin score reversed to what would have been expected with an average increase of 5.3% per year. There was also no effect on immunologic parameters. Quality of life did not change during treatment. CONCLUSION: We were not able to show that photopheresis, performed as described above, is an effective treatment in systemic sclerosis. The difference in average skin score was statistically and clinically insignificant. Despite the small sample size, we concluded that the magnitude of the observed changes is too small to justify photopheresis as a regular treatment.