Progressive nodular histiocytosis with normal karyotypic analysis.

Non-Langerhans cell histiocytoses (NLH) comprise a spectrum of diseases that includes sinus histiocytosis with massive lymphadenopathy, hemophagocytic lymphohistiocytosis, xanthogranuloma, and reticulohistiocytoma. Progressive nodular histiocytosis (PNH) is a rare NLH that microscopically mimics juvenile xanthogranuloma but presents with disseminated persistent and progressive papulonodules in adults. Herein, we describe a case of PNH presenting as diffuse, progressively enlarging papules, nodules, and pedunculated tumors in a 38-year-old male. The diagnosis is supported microscopically by the morphologic and immunohistochemical findings. Whereas conventional cytogenetic analysis of Langerhans cell histiocytosis and juvenile xanthogranuloma has previously been described, there are no reports of the karyotype of PNH. In our patient, conventional cytogenetic analysis of the tumor revealed a normal karyotype. Although these results may represent the overgrowth of normal stromal cells rather than lesional cells, we believe this to be an important finding, indicating karyotypic analysis will not allow for distinction between PCH and other NLH or Langerhans cell histiocytoses.

Treatment of hidradenitis suppurativa by photodynamic therapy with aminolevulinic acid: preliminary results.

BACKGROUND: The current standard of care for hidradenitis suppurativa (HS) includes antibiotics (oral/topical), retinoids (oral/topical) and intralesional steroids and is unsatisfactory. Photodynamic therapy (PDT) with 20% 5-aminolevulinic acid (ALA) has been used ?off label? to treat acne vulgaris and may hold promise as a therapy for HS. This open-label, non-blinded study investigated the efficacy and safety of ALA PDT for the treatment of HS using two blue light sources and intense pulsed light (IPL) for photoactivation. METHODS: Twelve subjects with active HS enrolled to undergo ALA PDT once weekly for four weeks with follow-up visits 4, 8, and 12 or more weeks later. Nine subjects completed the study through the week 8 follow-up visit. Lesions were counted at each treatment visit at week 4, week 8 and at the final week. RESULTS: Mean lesion counts were 11.25 at baseline, 6.5 at 4 weeks (50.8% reduction), and 7.5 at 8 weeks (29.9% reduction). Mean Global Severity Scores were 2.2 at baseline, 1.5 at 4 weeks, and 1.8 at 8 weeks. Mean DLQI scores were 17.3 at baseline, 13.1 at 4 weeks (27.2% improvement), 14.00 at 8 weeks (19.3% improvement) and 14.0 (19.3% improvement) at the final week (16-62 weeks). Three subjects (25%) had complete clearance and no active lesions 4 weeks after the final treatment. Treatments were more tolerable for subjects treated with blue light than with IPL. CONCLUSION: ALA PDT may be a safe and effective treatment of hidradenitis suppurativa.

A review of photodynamic therapy (PDT) for the treatment of acne vulgaris.

BACKGROUND: Photodynamic therapy (PDT) is increasing in dermatology. Antibiotic resistance and the challenges of isotretinoin therapy have led to investigation of PDT in the treatment of acne vulgaris. OBJECTIVE: To review the results of clinical trials and case series with respect to light source, topical photosensitizing agent, adverse events, efficacy and skin type. METHODS: A non-critical review is presented of a PubMed search for studies examining PDT in the treatment of acne vulgaris. RESULTS: The authors found 21 clinical trials and case series of various designs. Eight studies employed a split-face design comparing photosensitizer to placebo, no treatment or another photosensitizer. Two trials used three test spots and one control spot per patient. Three studies utilized control subjects receiving no photosensitizer with or without light therapy. All 21 studies reported a reduction in inflammatory lesions and/or a significant improvement in acne. The light sources utilized included blue light, pulsed-dye laser (PDL), intense pulsed light (IPL) and red light. Studies comparing the use of PDT to light therapy alone demonstrated greater improvement in treatment groups pretreated with a photosensitizer. CONCLUSION: All studies reported reduction in inflammatory lesions or significant improvement in acne. Several studies confirm a light source combined with photosensitizer is superior to light alone. Adverse reactions including photosensitivity, pustular eruptions, and crusting varied among photosensitizers and light sources. PDT appears to be a useful therapeutic option for acne patients who are recalcitrant to standard treatments and poor candidates for systemic retinoids. Further studies are still needed before a consensus protocol can be established. Additional investigations are needed to establish optimal incubation time, activating light source and frequency of treatment.

Combining systemic retinoids with biologic agents for moderate to severe psoriasis.

BACKGROUND: Moderate to severe psoriasis, which is defined as psoriasis affecting more than 20% of the body surface area, often requires a combination of therapies to achieve remission. Although numerous data exist regarding the use of acitretin and biologic agent therapy alone for psoriasis, little is known about the efficacy, safety, and tolerability of acitretin combined with biologic agents. METHODS: Fifteen patients with psoriasis treated with concomitant acitretin and a biologic agent were identified, and their charts were reviewed for response to therapy, additional therapy necessary for disease management, side-effects, and laboratory abnormalities whilst on combination therapy. The Institutional Review Board did not require approval for this chart review. RESULTS: Twenty-nine per cent of patients showed clearance of psoriasis, 43% of patients showed an improvement of 90%, 14% showed an improvement of 75%, and 7.1% showed no change. During treatment with acitretin and biologic agent, five patients required no adjunctive treatment. Three patients were able to stop narrow-band ultraviolet-B (UV-B) therapy after an average of 2.33 months of combination therapy. Only one patient continued to require phototherapy (UV-B) in addition to the biologic agent. Three patients developed squamous cell carcinoma (SCC) whilst on combination therapy, but all patients had a previous history of SCC. One patient developed non-Hodgkin's lymphoma after 3 years of etanercept and acitretin, and the etanercept was discontinued. CONCLUSIONS: Acitretin combined with biologic agents offers a promising method of managing refractory psoriasis. More research is needed to determine the long-term safety and efficacy of this combination.

Successful treatment with injected hyaluronic acid in a patient with lip asymmetry after surgical correction of cleft lip.

BACKGROUND: Surgical repair of cleft lip, while correcting deformity and dysfunction, may leave residual cosmetic imperfections. The resultant asymmetry and low volume of the upper lip can be addressed surgically and via less invasive methods. OBJECTIVE: We present the first reported use of injectable hyaluronic acid to correct the characteristic lip asymmetry and poor volume after surgical repair of a cleft lip. METHODS AND MATERIALS: Using injectable hyaluronic acid, we treated the patient's upper lip to restore symmetry and achieve an augmented volume. RESULTS: We obtained a symmetric correction and aesthetically pleasing volume augmentation in the affected lip. These results lasted approximately 4 months. CONCLUSION: Using a temporary, alloplastic, injectable soft tissue filler such as hyaluronic acid satisfactorily achieves correction of asymmetry and low volume in a surgically repaired cleft lip. For patients who have endured multiple corrective surgeries, this is a novel and less invasive way to improve their cosmetic concerns.

Common and alternate oral antibiotic therapies for acne vulgaris: a review.

Acne vulgaris is an extremely common disorder affecting many adolescents and adults throughout their lifetimes. The pathogenesis of acne is multifactorial and is thought to involve excess sebum, follicular hyperkeratinization, bacterial colonization, and inflammation. Many therapeutic options exist for treating acne, including topical benzoyl peroxide, topical and oral antibiotics, topical and oral retinoids, and oral contraceptives. Oral antibiotics have been a mainstay in the treatment of acne for decades and function by exerting an antibacterial effect by reducing the follicular colonization of Propionibacterium acnes. Systemic antibiotics also have anti-inflammatory and immunomodulatory properties. This article reviews the English language literature on the efficacy of various systemic antibiotics for treating acne vulgaris, including second-line and less historically used medications. We discuss the tetracyclines, including subantimicrobial dose doxycycline, macrolides (notably azithromycin), trimethoprim-sulfamethoxazole, cephalosporins, and fluoroquinolones as treatment options for acne vulgaris.

Primary cutaneous B-cell lymphoma.

Primary cutaneous B-cell lymphomas include extranodal marginal zone B-cell lymphoma, follicular lymphoma, large B-cell lymphoma, and, rarely, mantle cell lymphoma. Our purpose in conducting this review was to determine the clinical and behavioral characteristics of primary cutaneous B-cell lymphomas, their relationship to infectious triggers, and therapeutic response. We conducted a retrospective chart review of 23 adult patients presenting to the dermatology clinic at M. D. Anderson Cancer Center with primary cutaneous B-cell lymphoma between January 1999 and May 2003. Primary cutaneous B-cell lymphomas generally present on the head and neck, with the trunk and extremities afflicted to a lesser extent. Patients were found to have serologic evidence of prior infection with Borrelia burgdorferi (n = 10), Helicobacter pylori (n = 5), and Epstein-Barr virus (n = 6). Overall, treatment of primary cutaneous B-cell lymphoma should involve multiple modalities; however, specific treatment aimed at concurrent or suspected infection, particularly B burgdorferi, is a helpful adjunct and may achieve complete remission in a small subset of patients.

Practical considerations in future psoriasis therapies.

Exciting new therapies are becoming available that allow dermatologists and patients safe and effective alternatives to traditional psoriasis therapy. Because these new biologic drugs are parenterally administered, practical aspects of their integration into clinical practice must be addressed. This article offers guidelines for incorporating subcutaneous,intramuscular, and intravenous injectables into dermatology offices. Several tiers of psoriasis care are outlined to encourage individual physicians to choose the optimum level of service compatible with their individual practices.