Treatment of Sweet's syndrome in pregnancy.

Pregnancy-associated Sweet's syndrome is a rare occurrence (2%), with good prognosis, spontaneous resolution after delivery, and not increased infant morbidity and mortality. However, differential diagnosis is not easy for physician not familiar with skin lesions. Systemic involvement, even though unusual, might occur in nearly every organ of the body, including pericardium, myocardium, and placenta, as well as one report of early fetal miscarriage, questioning the possibility of risks underestimation. We present two further cases, one occurred in a 31-year-old woman at 26 weeks of gestation and the other on a 26-year-old woman at 24 weeks of gestation, primigravidae. Both presented with tender papules and nodules on their face and upper body parts. Laboratory examinations and skin biopsy histology were pathognomonic. Monitoring of general maternal and fetal conditions showed no signs of sufferance, but the decision to accelerate skin symptoms release, being time to delivery quite distant, challenge the treatment options. There are no recommended treatments for Sweet syndrome and the choice is very limited during pregnancy. A short course of oral steroids was very effective, with lesions healing in few days, no relapses or fetal complications. When pregnant patients exhibit fever, neutrophilia, arthralgia or myalgia, and tender erythematous plaques or nodules, Sweet syndrome should be considered. The trained dermatologist is in the leading position to address the differential diagnosis, reassure the patient, and avoid complications, even if they are rare.

Successful ustekinumab treatment of noninfectious uveitis and concomitant severe psoriatic arthritis and plaque psoriasis.

We report the first successful treatment of noninfectious uveitis with ustekinumab in a patient with severe concomitant psoriasis and psoriatic arthritis who failed to respond to conventional immune suppressants and with contraindications to tumor necrosis factor alpha inhibitors.

Melanoma occurrence under long-term etanercept treatment for psoriasis: a case report.

Melanoma occurrence during treatment with anti-tumor necrosis factor is considered an incidental event, although very recent studies suggest a risk. Etanercept is a fusion protein that binds the tumor necrosis factor receptor and is included among TNF inhibitors, approved for the treatment of several autoimmune diseases, such as psoriasis.We described a 79-year-old man with psoriasis, being treated with etanercept, who presented with a new brown to black macule on his right shoulder; this was immediately surgically excised. Histology showed a superficial spreading melanoma, 1.2 mm Breslow thickness, one mitosis/hpf, with no vascular or neural invasion (stage T2b). Sentinel lymph node biopsy was negative. There were no apparent melanoma risk factors: normal total nevus count, photo type IV, no childhood sunburns, no family history of melanoma, and no previous immune suppressive drugs and/or phototherapies. Etanercept 50 mg/week had been administered continuously for 5 years before the melanoma occurrence. After etanercept withdrawal his psoriasis slowly, but progressively relapsed.

Leg ulcer in Werner syndrome (adult progeria): a case report.

Werner syndrome (WS; MIM#277700) or adult progeria, is a rare disease, associated with mutations of a single gene (RECQL2 or WRN), located on chromosome 8 (8p12). It codes a DNA-helicase, whose defects cause genomic instability. The highest incidences are reported in Japan and Sardinia (Italy). On this major island of the Mediterranean Basin, the WS cases have been observed in the northern areas. The authors describe the apparently first case reported in southern Sardinia, a 51-year-old woman, who was born in and resides in the province of Cagliari. She presented with a 9-year history of an intractable leg ulcer and other characteristic symptoms, including "bird-like" face, high-pitched voice, premature greying, short stature, abdominal obesity in contrast with thin body type, scleroderma-like legs, decreased muscle mass, diabetes, atherosclerosis, and premature menopause. A specialized genetic Institute of Research (IRCCS-IDI, Rome) confirmed the clinical diagnosis. There is no cure or specific treatment and patients must be periodically screened for an increased risk of cardiovascular and cerebrovascular disease and malignancies. Among the many findings, leg ulcers significantly affect the patient's quality of life. This problem may send the patient to the dermatologist, who finally suspects the diagnosis. Poor response to medical treatment may require aggressive repeated surgery, with poor or temporary results.

Tinea faciei due to microsporum canis in children: a survey of 46 cases in the District of Cagliari (Italy).

Dermatophytoses are frequent in children, but involvement of the facial skin has peculiar aspects that should be considered a separate entity: tinea faciei. Microsporum canis infection in tinea faciei has not been widely documented. To review cases of tinea faciei due to M. canis in children diagnosed at the Dermatology Clinic, University of Cagliari. Between 1990 and 2009, all children with dermatophyte infections of the facial skin were recruited for the study after parental consent. Diagnosis was made through direct microscopic and cultural examination. Age, sex, clinical form, illness duration, identified dermatophyte, source of infection, and treatment were recorded. Forty-six cases of tinea faciei due to M. canis in children aged 11 months to 15 years (29 male/17 female) were diagnosed. In 42 (91.3%) children, the illness was the result of contact with pets, and 4 (8.7%) cases resulted from contact with children affected by tinea capitis due to M. canis. Clinical manifestations were typical ringworm in 34 (74%) patients, whereas in 12 (26%) cases, atypical forms mimicking atopic dermatitis, impetigo, lupus erythematosus, and periorificial dermatitis were observed. In 18 (39%) cases, involvement of the vellus hair follicle was documented as ectothrix invasion. Topical or systemic antifungal therapy was effective in all patients. Tinea faciei shows a complex spectrum of differential diagnosis and age-related variations with respect to other superficial dermatophytosis. M. canis is the main organism responsible in children residing in Cagliari, capitol city of Sardinia, Italy. Close collaboration with veterinary and educational programs within infant communities are required for adequate prevention.

D-penicillamine elastosis perforans serpiginosa: description of two cases and review of the literature.

Long term D-penicillamine (DPA) therapy to treat Wilson disease can induce elastosis perforans serpiginosa (EPS), a very rare degenerative skin disease characterized by a transepidermal elimination of elastic fiber aggregates. The iatrogenous disease depends on DPA capacity to chelate copper and cause its depletion. Lysyl-oxidase is a copper dependent enzyme crucial to the dermal elastic fiber cross-linking, which is strongly affected by DPA copper depletion. Direct binding of the drug to collagen precursors also affects elastic fiber assemblage and maturation. The abnormal elastin accumulates into the middle dermis and produces a characteristic bramble brush or "lumpy-bumpy" appearance. In this way it acts as a foreign body and is progressively extruded through the epidermis. Clinically, the disease presents with multiple firm keratotic papules and nodules arranged in annular plaques over the neck, axillae, antecubital fossae, and forearms. The rarity of the disease frequently causes misdiagnoses and the process continues unabated causing concerns about systemic elastopathy.

Primary cutaneous cryptococcosis in an immunocompetent host.

We report a case of primary cutaneous cryptococcosis in an immunocompetent host. Several nodules, isolated or sometimes joint to form plaques, affected the right arm. The arm was paralytic and hypoplastic, and a history of numerous abrasions picking firewood up preceded the onset of the eruption. Histology on skin biopsy documented a dermal infiltrate constituted of histiocytes, lymphocytes, fibroblasts and rare giant cells. Numerous rounded periodic acid-Schiff (PAS) bodies were also present. Cryptococcus neoformans var. neoformans grew upon culture. Complete blood, biochemical and instrumental examinations resulted in findings within normal range. Treatment with itraconazole 200 mg daily for 4 months led to complete recovery. During a 2-year follow-up, the patient did not present any relapse or dissemination to other organs.

Effects of TNFα inhibitors in patients with psoriasis and metabolic syndrome: a preliminary study.

BACKGROUND: Psoriasis is an inflammatory disease, that is increasingly being considered as a systemic disorder. Among associated comorbidities, metabolic syndrome plays an important role. The effects of biological therapies on metabolic syndrome is controversial. METHODS: Thirty-one psoriatic patients with metabolic syndrome, eligible to treatment with anti-TNFα agents, were enrolled. Metabolic parameters were measured during 4 subsequent visits, one every 40 to 60 days. PASI, BSA and DLQI assessed the severity of psoriasis and the impact on quality of life. RESULTS: We include 31 patients, 18 treated with etanercept and 13 with adalimumab. Metabolic parameters evaluated at V4 in both groups showed different trends in the blood glucose values: a slight decrease in adalimumab group, an increase in etanercept group, with an almost significant comparison test (P=0.073). Similarly, the lipid profile revealed an opposing trend, with an increase in triglycerides in adalimumab patients, and a decrease in the other group, without statistically significant differences. No statistically significant difference was recorded in HDL cholesterol. An improvement in systolic and diastolic pressure was appreciated in both groups, although not significantly. The waist circumference slightly decreased in both groups. PASI 75 score was reached in 60% of the patients. In addition, BSA and DLQI improved. CONCLUSIONS: Our study showed a slight improvement of metabolic parameters, at times with a trend toward significance. Additional long-term studies and a larger number of patients are needed to more clearly define the association between psoriasis and cardiovascular disease and understand the effect of biological therapies on metabolic parameters.

Two cases of primary endonasal leishmaniasis in Sardinia (Italy).

Leishmaniasis is an endemic protozoan infection in Sardinia, one of the major islands of the Mediterranean Basin. We report two cases of endonasal primary Leishmaniasis, which is a very rare event in adult men who are immunocompetent, born in, and residents of Sardinia. The diagnosis was confirmed by the presence of intra and extracellular Leishmania amastigotes in the histological smear. Isoenzymatic characterization identified Leishmania infantum zymodeme MON-111 in both cases. Laboratory and instrumental investigations excluded visceral involvement. Treatment with meglumine antimoniate (Glucantim) intralesional administration, 1 ml weekly for 4-5 weeks, led to complete resolution. The unusual location is likely a reflection an uncommon site of inoculation of the protozoa, transmitted by flying vectors. The patients were a shepherd and a farmer, respectively, both professions at high risk of infection because of their habits of sleeping outdoors under trees or in country cottages during spring and summer and exposed to sand fly bites. Although mucosal involvement and infection by Leishmania infantum, a potential cause of visceral leishmaniasis, the Sardinian patients experienced a benign disease course considering muco-cutaneous forms described in the New World. Differential diagnosis and early detection are necessary in order to start effective treatment and prevent more serious complications.

Bullous skin eruption in an HIV patient during antiretroviral drugs therapy.

Dermo-epidermal blistering is an uncommon presentation of adverse drug reactions. Several drugs are associated to such eruptions, but review of current knowledge does not list antiretroviral drugs. A 37-year-old Caucasian HIV-positive woman presented with a 6-week history of diffuse annular blistering affecting the trunk and limbs. Lesions appeared both on erythematous and normal-appearing skin. The patient was in treatment with antiretroviral (lamivudine + didanosine + nelfinavir) for 2 years. A history of previous adverse reactions to betalactams, nonsteroidal anti-inflammatory drugs, and a nevirapine-induced hepatitis was also referred. Histopathology showed a dermo-epidermal blister; direct immunofluorescence was positive for IgG, C3c at the basement membrane zone; enzyme-linked immunosorbent assay was positive for BP180 antigen. Oral prednisone 1 mg/kg daily for 20 days led to poor improvement. Discontinuation of the antiretrovirals was followed by a rapid healing. Blisters reappeared at first re-introduction essay 1 month later. Awareness of iatrogenic dermo-epidermal blistering is necessary to suspect the diagnosis and avoid long-term immunosuppressant treatment. Complete spontaneous recovery after withdrawal of the responsible drug and relapse at rechallenge are the main criteria for the diagnosis. Factors related to the state of the HIV infection, and/or immunodeficiency may have contributed in precipitating the reaction in the present authors' case.

Multiple cutaneous abscesses revealing disseminated nocardiosis in a patient with chronic rheumatoid arthritis.

Nocardiosis is a challenging infection that is difficult to diagnose and treat. Delayed recognition and prolonged illness are major concerns. We report the case of a 65-year-old man who underwent treatment with corticosteroids for rheumatoid arthritis and then presented with multiple abscesses on the right leg. A complete assessment performed by the dermatologist led to the diagnosis of disseminated nocardiosis with involvement of the lungs and central nervous system. The patient's history revealed development of pulmonary disease approximately 1 year prior to the appearance of the skin lesions, which was not recognized by several specialists. Long-term antibiotic therapy was effective in treating the skin and central nervous system lesions, while the chest radiologic profile improved more slowly.

Dermatophyte infections mimicking other skin diseases: a 154-person case survey of tinea atypica in the district of Cagliari (Italy).

BACKGROUND: Although usually simple, the diagnosis of dermatophyte infection is sometimes neglected. An observational study has been realized to evaluate the role of corticosteroid exposure (tinea incognito) and of other primary characteristics of the dermatophytosis that from onset mimic other diseases and mislead an unexperienced physician. MATERIALS AND METHODS: Between 1990 and 2009, all cases of atypical dermatophytosis mimicking other skin diseases were collected from the more general number of dermatophyte infections diagnosed at the Dermatology Department of Cagliari University, Italy. RESULTS: One-hundred and fifty-four cases (71 male/83 female, 2-81 years old) were studied, with a median of 7 cases/year. The most observed clinical forms were those mimicking impetigo, eczematous dermatitis, lupus erythematosus, polymorphous light eruption, psoriasis, and rosacea. The identified dermatophytes were: Microsporum canis (70 cases), Trichophyton rubrum (43 cases), Trichophyton mentagrophytes var. mentagrophytes (29 cases), Trichophyton mentagrophytes var. interdigitale (six cases), Microsporum gypseum (three cases), Epidermophyton floccosum (two cases), and Trichophyton verrucosum (one case). Diagnostic difficulties are discussed, with special attention to the origin of the pathomorphosis. CONCLUSIONS: In our experience, clinical atypia is not a mere consequence of corticosteroid therapy but present at the very onset of the illness, due to the variable dermatophyte invasive capacity, the site of invasion, physiological individual, and/or acquired condition, such as excessive washing or sun exposure. Therefore, we suggest using the term "tinea atypica" rather than "tinea incognito" to include all forms of dermatophytosis that do not present the classic features for both primary and secondary pathomorphosis.

Adverse cutaneous reactions to selective cyclooxygenase 2 inhibitors: experience of an Italian drug-surveillance center.

BACKGROUND: Selective cyclooxygenase (COX) 2 nonsteroidal anti-inflammatory drugs (NSAIDs) have been associated with a general lower incidence of side effects compared with nonselective NSAIDs. Postmarketing information has highlighted the need to reassess the risk evaluation for specific organs, including the skin. OBJECTIVE: A prospective databank to record all cases of adverse cutaneous reactions associated with the use of COX inhibitors was conducted at the Centre for Drug Surveillance of the Dermatology Department of Cagliari University. MATERIAL AND METHODS: An intensive surveillance program from November 2000 to October 2004, adopting the World Health Organization Collaborating Centre for Drug Monitoring causality assessment criteria and algorithm. RESULTS: Seventeen cases, 4 male and 13 female, were studied. None had previously presented any drug intolerance or allergy. Clinical manifestations were mainly maculopapular exanthema followed by urticaria-angioedema. A severe case of leukocytoclastic vasculitis was also observed. Responsible drugs were celecoxib (13 cases; 76%), rofecoxib (3 cases; 18%), and etoricoxib (1 case; 6%). All cases recovered with drug withdrawal. Causality was probable for all eruptions, except for the fixed drug eruption, for which causality was certain. DISCUSSION: Although most cases were associated with celecoxib, the observation of severe eruptions owing to rofecoxib and etoricoxib in this prospective study is consistent with a class effect of COX inhibitors on the skin, which merits further studies to explain the fine underlying mechanisms.

Tinea capitis caused by Microsporum canis treated with terbinafine.

Tinea capitis is a disease found throughout the world. It frequently affects children and only rarely adults, usually post-menopausal women. Numerous dermatophytes of the genus Microsporum and Tricophyton can cause tinea capitis and griseofulvin is still today the treatment of choice. To study the effectiveness and tolerability of terbinafine treatment in tinea capitis caused by Microsporum canis we treated 26 patients - 22 children and four women - for a period of 12 weeks. Dosage adopted was 62.5 mg day(-1) in patients weighing less than 20 kg, 125 mg day(-1) in those weighing between 20 and 40 kg, and 250 mg day(-1) in patients weighing more than 40 kg. Clinical and mycological healing was achieved in 22 patients (84.6%), tolerability was excellent and in no cases were side effects or abnormal results in blood chemistry tests observed.

Case report. Pityriasis versicolor mimicking Pityriasis rotunda.

Pityriasis versicolor is a common dermatomycosis, occurring throughout the world, characterized by irregular, slightly scaly patches, varying in color from red/light brown to white. Pityriasis rotunda, on the other hand, is an uncommon disease, reported in specific ethnic groups, and characterized by perfectly round or oval patches of varying color, with a scaly surface. The histologic pattern is that of ichthyosis vulgaris. We report here the case of a male patient, aged 31, from Sardinia (Italy), affected by Pityriasis versicolor mimicking Pityriasis rotunda. Mycological examination allowed us to formulate the correct diagnosis, and ensuing treatment with antifungal drugs was entirely successful. The authors, while pointing out the rarity of this case, stress the possibility that Pityriasis versicolor mimics Pityriasis rotunda and vice-versa, especially in those countries in which the two diseases are endemic. More widespread recourse to microscopic examination can help avoid the risk of mistaken diagnosis and consequent incorrect treatment.

Kerion Celsi in a newborn due to Microsporum canis.

The present study deals with a case of a 40-day-old girl with kerion Celsi caused by Microsporum canis. The source of the infection were the parents who presented tinea corporis caused by M. canis. Systemic treatment was carried out with terbinafine and complete recovery was achieved. Tinea capitis is unusual in children during their first year of life and its evolution towards kerion is very rare in newborns.