Massimino Negosanti

Treatment of refractory pemphigus vulgaris with anti‐CD20 monoclonal antibody (rituximab): Five cases

Background: Pemphigus vulgaris is an autoimmune disease characterized by blisters and widespread erosions, involving skin and mucous membranes, caused by autoantibodies to desmoglein 1 and 3. This pathology is associated with increased morbidity and mortality if untreated. The treatment of pemphigus vulgaris requires multiple immunosuppressive agents, but often it is particularly resistant. Objective: To evaluate the efficacy and safety of rituximab therapy in refractory pemphigus vulgaris. Methods: Five patients diagnosed as having pemphigus vulgaris were treated with anti‐CD20 monoclonal antibody (rituximab). Each patient was treated with rituximab intravenously at a dosage of 375 mg per square metre of body surface area once weekly for 4 weeks. Results: All the patients presented clinical resolution. No adverse effects were observed. It is important to observe the clinical evolution in the future, but our experience is still limited to a short lifetime and follow‐up. Conclusion: In our experience rituximab has been an effective and safe treatment for refractory pemphigus vulgaris.

Acne inversa treated with infliximab: different outcomes in 2 patients.

dition usually presents with painful, inflamed lesions in the apocrine-gland-bearing areas of the body, most commonly the axillary, inguinal and anogenital areas. The disease usually occurs after puberty and before the age of 40 years, suggesting a hormonal influence on the pathogenesis of the disease. Occlusion of the apocrine duct by a keratinous plug and defects of the follicular epithelium have usually been considered the aetiology of acne inversa. Contributing factors include friction from axillary adiposity, sweat, heat, stress, tight clothing and genetic and hormonal components (1). Acne inversa can be treated with antibiotics, retinoids, corticosteroids, cyclosporine, incision and drainage, local wound care, local excision, radiation and laser therapy. Although there are a wide range of therapies suggested for the treatment of acne inversa, the disease is often resistant and the psychological impact on the patient can be great (2), encompassing social, personal and occupational challenges. We describe here 2 case reports of patients affected by acne inversa resistant to traditional therapies, who were treated with infliximab.

Adapting a Vacuum Assisted Closure dressing to challenging wounds: negative pressure treatment for perineal necrotizing fasciitis with rectal prolapse in a newborn affected by acute myeloid leukaemia.

We report the case of a newborn with acute myeloid leukaemia, who developed perineal necrotizing fasciitis due to Pseudomonas Aeruginosa, after twenty days of life. Following surgical debridement, she was effectively treated with topical negative pressure therapy (V.A.C.(R) device) with silver foam dressings, this achieved complete closure in thirteen days. Negative pressure therapy should be considered when conventional wound care fails to achieve complete wound closure, even in neonates.

A long history of widespread asymptomatic giant plaques

A 79-year-old man was referred to our department with a long history of asymptomatic plaques disseminated all over his body, including the head, arms, legs, and upper trunk. Despite the prominence of the cutaneous lesions, the patient himself had never been worried and now sought medical attention only due to his family’s concerns. He denied systemic symptoms such as fever, arthralgia, or fatigue. Comorbidities included type 2 diabetes, hypertension, mild kidney failure, and hepatic steatosis. Laboratory tests showed elevated serum glucose and glycated hemoglobin levels (157 mg/dL and 62 mmol/mol, respectively). Serum calcium and angiotensin-converting enzyme (ACE) levels as well as other routine laboratory parameters were within normal limits. Skin scrapings and swabs were negative for fungi. A routine chest A long history of widespread asymptomatic giant plaques Case for Diagnosis

Keratosis lichenoides chronica with an atypical clinical presentation and variable histopathological features.

A 44-year-old, otherwise healthy, Moroccan man was referred to us because of rapid onset of erythematous, scaly, raised, slightly pruritic plaques on the trunk, extremities (Figure 1a–c), face, and scalp (Figure 1d). He showed neither nail changes nor mucosal (oral or genital) involvement. His medical history was unremarkable, and he denied any drug intake. A complete blood cell count, liver and kidney function tests, thyroid parameters, and urinalysis were within normal limits. Syphilis screening, hepatitis B, C, and HIV serology, as well as a QuantiFERON-TB test were negative. A chest X-ray and abdominal ultrasound were normal. Multiple skin biopsies were performed. Those taken from the trunk and extremities showed extensive epidermal hyperparakeratosis, psoriasis-like acanthosis, hypogranulosis (Figure 2a–c) as well as neutrophilic microabscesses (Figure 2c). Other findings included necrotic keratinocytes surrounded by lymphocytes (satellite cell necrosis) and a band-like inflammatory infiltrate associated with melanophages (Figure 2d). Periodic acid-Schiff (PAS) staining was negative. Immunocytochemistry showed a reactive inflammatory infiltrate (the makeup of the lymphoid population was as follows: CD3+/–, CD4–/+, CD8+/–, CD20 rare; “+/–” signified 50–75 % of the population; “–/+”, 25–50 %; and “rare”, 10–25 %). Based on these findings, lymphoma was ruled out. Direct immunofluorescence was negative. By contrast, the biopsies taken from the face showed focal hyperparakeratosis, irregular acanthosis, vasodilation, edema (Figure 3a, b), as well as a dermal – predominantly lymphocytic – inflammatory infiltrate (Figure 3b, c) with admixed melanophages (Figure 3b) and “satellite cell necrosis” (Figure 3d). Except for hyperparakeratosis, these findings (in particular the inflammatory infiltrate with melanophages, and the necrotic keratinocytes) were more suggestive of lupus erythematosus. Antinuclear antibodies, anti-extractable nuclear antigen antibodies, anti-DNA antibodies, antiphospholipid antibodies, antineutrophil cytoplasmic antibodies, rheumatoid factor, as well as complement component 3 and 4 were all negative. Therapy with systemic corticosteroids resulted in a good response, however, there was a rebound following treatment discontinuation. Subsequently, many different therapeutic approaches were attempted, including cyclosporine, methotrexate, acitretin, PUVA therapy, topical calcipotriol, and topical corticosteroids. However, they all proved to be ineffective. Despite their variability, the histopathological features showed a certain degree of repetitiveness, which is characteristic of our eventual diagnosis: keratosis lichenoides chronica (KLC). Systemic corticosteroids were the only effective treatment.

Keratosis lichenoides chronica mit untypischer klinischer Präsentation und variblen histopathologischen Merkmalen

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