Barbara Horvath

Systemic therapy with immunosuppressive agents and retinoids in hidradenitis suppurativa: a systematic review.

Hidradenitis suppurativa (HS) is a difficult disease to treat. Although the pathogenesis of this inflammatory skin disease is largely unknown, the important role of the immune system has been demonstrated in both experimental and clinical studies. Clinicians are therefore increasingly prescribing systemic treatments with immunosuppressive agents, but the more traditionally used systemic retinoids, especially isotretinoin, also remain relatively common therapies. In order to provide an overview of all currently available systemic immunosuppressive agents and retinoids for the treatment of HS, a systematic search was performed using the Medline and Embase databases. All published papers concerning systemic retinoids or immunosuppressive treatments for HS in adults were included. The primary endpoints were the percentages of significant responders, moderate responders and nonresponders. Other endpoints were the relapse rate and adverse events. In total 87 papers were included, comprising 518 patients with HS who were treated with systemic retinoids, biological agents or another immunosuppressive agents, including colchicine, ciclosporin, dapsone or methotrexate. The highest response rates were observed with infliximab, adalimumab and acitretin. Overall, the quality of evidence was low and differed between the agents, making direct comparisons difficult. However, based on the amount of evidence, infliximab and adalimumab were the most effective agents. Acitretin was also effective in HS, although the quality of the evidence was low. The therapeutic effect of isotretinoin is questionable. Randomized controlled trials are needed to confirm the effectiveness of acitretin, and to identify the most effective immunosuppressive agents in HS.

Bidirectional communication between histamine and cytokines

Abstract: Histamine plays fundamental roles in numerous immune reactions. In addition to its well-characterized effects in the acute inflammatory and allergic responses, histamine also influences the expression and actions of several cytokines. The interaction between histamine and the cytokines is bidirectional, since some cytokines were found to modulate the production and release of histamine as well. Because several pharmacological agents that modulate the actions of histamine (e.g. antihistamines) are widely used in the treatment of various human diseases (allergy, peptic ulcer etc.), this complex interaction could have general medical relevance too, but the current situation appears to be rather controversial and further studies are needed to elucidate these sophisticated interactions more precisely.¶

Low-dose rituximab is effective in pemphigus

Background  Rituximab, an anti‐CD20 antibody, was shown in open series studies to be effective in treating pemphigus at a dose of 4 × 375 mg m−2 as approved for B‐cell malignancies.

The addition of rituximab to anthracycline‐based chemotherapy significantly improves outcome in ‘Western’ patients with intravascular large B‐cell lymphoma

Some case reports and a Japanese series suggest benefit from the use of rituximab in patients with intravascular large B‐cell lymphoma (IVL). Rituximab efficacy was evaluated in Western patients with IVL, comparing outcome of 10 patients treated with rituximab + chemotherapy (R‐CT) and of 20 patients treated with chemotherapy alone (CT). There were no significant differences in patients’ characteristics between the two subgroups. The addition of rituximab was associated with improved complete remission rate (90% vs. 50%; P = 0·04), event‐free survival (3‐year: 89% vs. 35%; P = 0·003) and overall survival (3‐year: 89% vs. 38%; P = 0·01). In conclusion, rituximab may substantially change the dismal prognosis of IVL.

Ustekinumab in hidradenitis suppurativa: clinical results and a search for potential biomarkers in serum

Treatment of hidradenitis suppurativa (HS) is difficult and the search for effective therapies continues.

Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides

IMPORTANCE Large case series suggest that patients with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic mycosis fungoides (MF). However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. Distinction between indolent and aggressive FMF may have important therapeutic consequences but is hampered by the inability of the current tumor-node-metastasis-blood (TNMB) staging system to classify patients with FMF in a clinically meaningful way. OBJECTIVE To differentiate between indolent and aggressive FMF using clinicopathological criteria and to define prognostic factors in patients with FMF. DESIGN, SETTING, AND PARTICIPANTS In this prospective cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry between October 1985 and May 2014 at a tertiary referral center hosting the Dutch Cutaneous Lymphoma Registry. Overall, 220 patients with FMF had been registered, but 17 patients with incomplete follow-up data or a history of classic MF were excluded. MAIN OUTCOMES AND MEASURES Main outcomes included clinical and histological characteristics, disease progression, and survival. Prognostic factors were investigated using Cox proportional hazard regression analysis. Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimens from patients presenting with plaques. RESULTS In a cohort of 147 men and 56 women (median [range] age, 59 [15-93] years), patients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to patients with only patches and/or follicular papules (10-year OS, 71% vs 80%), while the survival rate of patients with histologically advanced plaque-stage FMF was almost identical to that of patients presenting with tumors (10-year OS, 25% vs 27%). Subsequently, 3 clinical subgroups with significantly different survival data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n = 17; 5-year and 10-year OS, 23% and 2%). Age at diagnosis, large cell transformation and secondary bacterial infection were independent risk factors for disease progression and/or poor survival. CONCLUSIONS AND RELEVANCE The results of this study provide useful criteria to differentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with a favorable prognosis.

Surgical Treatment of Sinuses by Deroofing in Hidradenitis Suppurativa

Patients with mild HS (Hurley I) may benefit from topical or systemic treatment, sometimes combined with surgery (incision with drainage), but if sinuses have formed, response to topical and oral medication is poor, and surgical treatment is necessary. In patients with Hurley stage III, a combination of oral medication and more-extensive surgery (entire block resection) is often needed. Results of conventional surgical methods may be disappointing; incision and drainage have high recurrence rate, whereas block excision takes prolonged wound healing.

Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy

Background  Langerhans cell histiocytosis (LCH) in adults first presenting in the skin is rare. Guidelines for staging, treatment and follow‐up are lacking.

Surgery under general anaesthesia in severe hidradenitis suppurativa: a study of 363 primary operations in 113 patients

Treatment of hidradenitis suppurativa (HS) is a difficult undertaking, especially as there is no consensus on what surgical technique is preferred. At our centre severe HS (Hurley II/III) is operated under general anaesthesia, mostly with the STEEP procedure.

IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis.

Please cite this paper as: IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis. Experimental Dermatology 2010; 19: 648–653.