Esther Roé

Therapy of paraneoplastic pemphigus with Rituximab: a case report and review of literature.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease with poor prognosis when associated with malignant neoplasm. We report the case of a patient with PNP associated with a CD20+ non‐Hodgkin follicular lymphoma who was treated with Rituximab plus corticosteroids and short courses of cyclosporin. One and a half years after Rituximab therapy, oral ulcerations had cleared and oral methylprednisolone was slowly tapered down without further recurrences. In the course of the disease, the patient developed sepsis due to Listeria monocytogenes and viral infections by human herpes virus 1 and 3. At the end‐stage of the disease she developed a cutaneous infection from Mycobacterium chelonae. The patient died 2 years and 7 months after the onset of PNP. Rituximab may be useful for PNP therapy, but further studies are necessary to confirm this hypothesis.

Onset of flexural psoriasis during infliximab treatment for Crohn's disease

disease. The patient has now been followed up for 12 months and remains clinically free of disease. These are the first reported cases of Bowen’s treated successfully with diclofenac HA gel. The histological and clinical clearance of Bowen’s disease in our patients after 80–90 days of topical application of diclofenac HA gel indicates that this therapy may be of help in this condition. One patient experienced moderate inflammation at the application site that necessitated early discontinuation, without loss of efficacy. Adverse effects of this preparation have been reported in 51–72% of patients, with local irritation and dryness being the most commonly reported. The mechanism of action of diclofenac HA gel is as yet unknown. However, diclofenac has been shown to inhibit murine angiogenesis induced by chronic granulomatous reaction and to exhibit an antitumour effect in murine colon-26 growth. It is postulated that this antitumour mode of action may be related to the inhibition of the cycloxygenase pathway that is mediated by nonsteroidal anti-inflammatory drugs, specifically to reduction of prostaglandin E2 synthesis via substance P. The good clinical response to this therapy in our two cases suggest that diclofenac HA gel may be a useful addition to the array of pharmacological treatments available for Bowen’s disease. However, further studies to ascertain optimum dosage regimens and long-term followup are recommended.

Imatinib-associated lichenoid eruption : acitretin treatment allows maintained antineoplastic effect

the nail plate. It is thought to be due to a defect in keratinization with persistence of keratohyaline granules and air trapping within the nail. Our patient presents with true transverse leuconychia of her thumbnails and fingernails which resolved spontaneously during the third trimester of both her pregnancies. The leuconychia decreased in intensity from the thumb to the little finger on both hands and was also associated with transverse yellow bands, most prominent on her thumbnails. Neither phenomenon is readily explained. The menstrual cycle has previously been reported as a cause of transverse leuconychia. The onset of our patient’s leuconychia with the commencement of her menses and its ‘disappearance’ and subsequent ‘reappearance’ during and following each pregnancy also implies a hormonal influence. The exact aetiology of her leuconychia remains unknown. No similar cases have been reported to date.

Bortezomib‐associated cutaneous vasculitis

Bortezomib (Velcade , Ortho Biotech, Issy-les-Moulineaux, France) is the first of a new class of drugs known as proteasome inhibitors mainly used in haematological malignancies such as multiple myeloma refractory to prior treatment and non-Hodgkin’s lymphoma (mantle cell lymphoma). Several cutaneous adverse effects of bortezomib have been reported but are poorly characterized. We report two cases of cutaneous vasculitis, which appears to be a rather typical manifestation in bortezomib-treated patients. Patient 1: A 58-year-old man was diagnosed with stage IIIA IgA kappa multiple myeloma. His disease was refractory to polychemotherapy and progressed despite autologous peripheral blood stem cell transplantation. He was treated with bortezomib at a dose of 1Æ3 mg m on days 1, 4, 7 and 10 of every 21 days. On day 4 of the first cycle, the patient suddenly developed asymptomatic erythemato-purpuric papules and plaques on the neck and upper trunk (Fig. 1). A skin biopsy specimen showed slight epidermal hyperplasia with focal spongiosis, occasional keratinocyte necrosis and vacuolar change of the basal layer. Within the dermis there was a moderately dense inflammatory infiltrate composed of lymphocytes, neutrophils with a certain degree of leucocytoclasis, a small number of eosinophils and many mononucleated epithelioid histiocytes that tended to concentrate around capillaries in the dermal superficial plexus, consistent with granulomatous vasculitis (Fig. 2). Endothelial cells showed tumefaction and there were free extravasated erythrocytes in the superficial dermis. A 1-week course of prednisone 30 mg daily was prescribed and the lesions healed in a few days leaving residual hyperpigmentation. On day 4 of the second treatment cycle, the rash recurred and responded to the same treatment. A protective dose of 20 mg prednisone was administered before each following bortezomib cycle and the lesions did not reappear. Patient 2: A 61-year-old woman was diagnosed with stage III-B IgG lambda multiple myeloma. She presented with disease progression despite polychemotherapy, interferon treatment and peripheral blood stem cell transplantation. She started treatment with bortezomib. Immediately after the second dose of the second cycle she developed erythematopurpuric papules and plaques on the neck, back and arms. A skin biopsy showed occasional vacuolar change of the basal layer with occasional keratinocyte necrosis and dyskeratosis. A perivascular lymphomononuclear infiltrate with some neutrophils and epithelioid cells and a certain degree of leucocytoclasis, endothelial cell tumefaction and free extravasated erythrocytes were observed in the superficial dermis, consistent with an early vasculitis lesion. She was treated with prednisone 30 mg daily, tapered over 10 days. The lesions healed completely in a few days. Rashes have been reported in 8–18% of patients in clinical trials of bortezomib; reports of cutaneous manifestations such Fig 1. Erythemato-purpuric papules and plaques symmetrically distributed on the neck, upper trunk and back of patient 1. Fig 2. A skin biopsy specimen from patient 1 showed a moderately dense mixed inflammatory infiltrate composed of lymphocytes, neutrophils with a certain degree of leucocytoclasis, some eosinophils and many mononucleated histiocytes that tended to form granulomas around capillaries in the dermal superficial plexus. Endothelial cells frequently showed tumefaction and there were free extravasated erythrocytes in the dermis.

Lichen striatus: clinical and epidemiological review of 23 cases

Lichen striatus (LS) is an asymptomatic self-limited skin disease of unknown etiology which was first described by Senear and Caro in 1941 [6]. Although LS is a frequent dermatosis among children, especially in girls between 5 and 15 years of age, there are few reviews in the literature. LS is characterized by erythematous or brownish papules with a flattened surface that are frequently scaly in appearance and occasionally display vesicles. The lesions are usually solitary and unilateral and have a linear distribution following Blaschko’s lines, usually on the extremities. Atypical forms with multiple and bilateral lesions have been described. Onset is usually sudden, with the disease progressing over days or weeks and slowly decreasing spontaneously until the papules resolve within 6–24 months, leaving a transitory residual hypopigmentation, especially in patients with a dark complexion. The inflammatory phase is not always detected, and hypopigmentation may be the first manifestation. The higher incidence during spring and summer, along with the existence of familiar clustering, suggest that viral infections could be an elicitation factor. Other possible precipitating factors may include cutaneous injury, trauma, hypersensitivity, or other as yet unspecified factors. We retrospectively reviewed the pediatric cases handled by the Department of Dermatology, Hospital de la Santa Creu I Sant Pau, in Barcelona between 1987 and 2004 using the photographic files of the Department as a selection criterion. Only patients with available clinical history were included. Diagnosis was based on clinical findings.

Successful treatment of oral erosive lichen planus with mycophenolate mofetil

Editor Mycophenolate mofetil is a new immunosuppressive drug that inhibits the proliferation of lymphocytes. It is an ester of mycophenolic acid that blocks the purine nucleotide synthesis by inhibition of inosine monophosphate. It can be useful in several dermatological conditions either as monotherapy, or in combination with other immunosuppressive agents in order to decrease its dosage and therefore avoid its adverse effects or to diminish their incidence. A 67-year-old woman presented with whitish streaks in a fern-like pattern, erosions, and ulcers on the tongue and oral mucosa, of 7 months’ evolution (fig. 1). The pain was severe and caused mental and physical symptoms that reduced her quality of life. In the previous 3 months she had lost 6 kg in weight due to swallowing difficulties. A biopsy of the oral mucosa showed a band-like submucosal lymphomononuclear infiltrate with lichenoid damage of the basal layer and necrotic keratinocytes, consistent with oral erosive lichen planus. Treatment with clobetasol propionate 0.05% and tacrolimus ointment 0.1% was given without improvement. Treatment with oral ciclosporin 300 mg/day (4 mg/ kg/day) was started; the lesions improved slightly and some alleviation of pain was noted. After 2 months, gum hypertrophy became noticeable and high serum creatinine levels 185 μmol/L were detected. Cyclosporin dose was reduced to 150 mg/day with reappearance of the pain and ulcerations, so a therapeutic trial was started adding mycophenolate mofetil 2000 mg/day to ciclosporin 150 mg/day. With this combination an excellent response was attained in less than a month, and serum creatinine levels decreased to 132 μmol/L. Cyclosporine dose was then tapered (50 mg every 15 days) and eventually withdrawn, keeping the mycophenolate mofetil dose stable. An adequate clinical response was achieved and serum creatinine levels returned to 97 μmol/L. After 2 months’ treatment with mycophenolate mofetil 2000 mg/day as monotherapy the dose was decreased to 1500 mg/day for two additional months, during which time the only apparent lesions in the oral mucosa were whitish streaks without erosions. Twelve months later she continues to be asymptomatic with 100 mg/day of mycophenolate mofetil (fig. 2). Oral erosive lichen planus is a very rare form of oral lichen planus. It is a disabling condition and is usually refractory to treatment. It is characterized by painful erosions and whitish streaks in a lacy or fern-like pattern on the tongue and oral mucosa. Multiple treatments have been tried with variable results, including corticosteroids, oral steroids, topical and systemic cyclosporin, topical and systemic retinoids, antimalarials, dapsone, PUVA therapy, laser, thalidomide and topical tacrolimus 0.1%. Nousari et al. reported a patient with cutaneous blistering and hypertrophic lichen planus that was resistant to several treatments, but in 6 weeks responded successfully to mycophenolate mofetil 1500 mg twice daily in combination with prednisone 40 mg/day. Frieling et al. reported three patients with disseminated erosive lichen planus who were treated with mycophenolate mofetil; two recovered completely and the third showed a dramatic improvement. A clinical improvement has also been described in patients with lichen planopilaris after 8 weeks

Rebound Growth of Infantile Hemangiomas After Propranolol Therapy.

BACKGROUND AND OBJECTIVES: Propranolol is first-line therapy for problematic infantile hemangiomas (IHs). Rebound growth after propranolol discontinuation is noted in 19% to 25% of patients. Predictive factors for rebound are not completely understood and may alter the management approach. The goal of the study was to describe a cohort of patients with IHs treated with propranolol and to identify predictors for rebound growth. METHODS: A multicenter retrospective cohort study was conducted in patients with IHs treated with propranolol. Patient demographic characteristics, IH characteristics, and specifics of propranolol therapy were obtained. Episodes of rebound growth were recorded. Patients’ responses to propranolol were evaluated through a visual analog scale. RESULTS: A total of 997 patients were enrolled. The incidence of rebound growth was 231 of 912 patients (25.3%). Mean age at initial rebound was 17.1 months. The odds of rebound among those who discontinued therapy at <9 months was 2.4 (odds ratio [OR]: 2.4; 95% confidence interval [CI]: 1.3 to 4.5; P = .004) compared with those who discontinued therapy between 12 to 15 months of life. Female gender, location on head and neck, segmental pattern, and deep or mixed skin involvement were associated with rebound on univariate analysis. With multivariate analysis, only deep IHs (OR: 3.3; 95% CI: 1.9 to 6.0; P < .001) and female gender (OR: 1.7; 95% CI: 1.1 to 2.6; P = .03) were associated. Of those with rebound growth, 83% required therapeutic modification including 62% of patients with modifications in their propranolol therapy. CONCLUSIONS: Rebound growth occurred in 25% of patients, requiring modification of systemic therapy in 15%. Predictive factors for rebound growth included age of discontinuation, deep IH component, and female gender. Patients with these predictive factors may require a prolonged course of therapy.

Anti-Tumour Necrosis Factor-Induced Visceral and Cutaneous Leishmaniasis: Case Report and Review of the Literature

Background: Leishmaniasis is a chronic protozoan disease in which organisms are found within phagolysosomes of the mononuclear phagocyte system. There are three major forms: cutaneous, mucocutaneous and visceral. We report the first case of visceral leishmaniasis with cutaneous involvement in a patient with rheumatoid arthritis treated with the anti-tumour necrosis factor (anti-TNF) adalimumab. Objective: To highlight cutaneous leishmaniasis as the first indicator of a kala-azar disease in a patient treated with anti-TNF and to review the literature on leishmaniasis in the context of anti-TNF therapy. Case Report: A 59-year-old woman presented with a crusted plaque on the right elbow 34 months after the initiation of adalimumab. A cutaneous biopsy showed intracellular amastigotes. No Leishmania parasites were observed in a bone marrow aspirate, but laboratory tests showed anaemia and impaired liver function, abdominal ultrasonography showed hepatomegaly, and ELISA serology was strongly positive for Leishmania antibodies in serum and urine. Adalimumab was withdrawn and treatment combining intralesional pentavalent antimonials and liposomal amphotericin was started. Eight weeks later, the leishmaniasis had resolved. Conclusion: A skin biopsy disclosing leishmaniasis should prompt tests to rule out visceral leishmaniasis, especially in an area such as the Mediterranean where the prevalence of latent Leishmania infection is high.

A Case of Vulvar Pyoderma Gangrenosum Associated with Collagenous Colitis

Pyoderma gangrenosum is a reactive inflammatory dermatosis which belongs to the spectrum of neutrophilic dermatoses. Due to a lack of diagnostic criteria, pyoderma gangrenosum is mainly a diagnosis of exclusion. It is rarely observed on the perineum, and vulvar involvement is even less frequent. Collagenous colitis is an idiopathic inflammatory colonic disease that is included in the microscopic colitides. The colonic mucosa and the crypt architecture are preserved but histologic alterations are found. We describe a case of collagenous colitis associated with vulvar pyoderma gangrenosum that improved spectacularly with cyclosporine 3 mg/kg/day and the twice-daily application of topical tacrolimus 0.1%.

Plasma cell balanitis of zoon treated with topical tacrolimus 0.1%: report of three cases.

284 JEADV 2007, 21, 247–289