Anna Peroni

Drug-induced morphea: Report of a case induced by balicatib and review of the literature

Drug-induced scleroderma has been rarely reported, mostly with the features of diffuse scleroderma or acrosclerosis, and exceptionally with the characteristics of morphea. We report the case of an adult white woman, enrolled in a double-blind, placebo-controlled, multicentric trial evaluating the efficacy and safety of the cathepsin K inhibitor balicatib for osteoporosis. Typical morphea lesions developed on the patients trunk 9 months after the beginning of therapy. Lesions completely resolved after drug withdrawal and a single brief course of systemic steroids. No recurrence occurred in a 2-year follow-up. Fifteen cases of drug-induced morphea could be retrieved from the literature. Drug withdrawal determined complete remission in only a few patients. Different drug classes have been implicated. Some of these, including balicatib, alter directly connective tissue metabolism.

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria Part I. Cutaneous diseases

UNLABELLED Acute urticaria is self-limiting, and a cause can be identified in many patients. Chronic urticaria is a long lasting disease, and patients are commonly examined for an autoimmune origin and for associated diseases. Although the diagnosis of urticaria is straightforward in most patients, it may pose some difficulties at times and it may require a careful differential diagnosis with a number of conditions. Urticarial syndromes comprise both cutaneous and systemic disorders. Part I of this two-part series focuses on the clinical and histologic features that characterize common urticaria and on the cutaneous diseases that may manifest with urticarial lesions and must be considered in the differential diagnosis. LEARNING OBJECTIVES After completing the learning activity, participants should be able to distinguish between the typical wheals of urticaria and urticarial lesions suggesting other diagnoses and to assess patients with urticarial lesions in order to exclude or confirm other cutaneous diseases.

Allergic contact dermatitis in children with and without atopic dermatitis.

BackgroundPrevalence and causes of allergic contact dermatitis (ACD) in children vary with time and geographical area. ObjectiveThis study aimed to determine the relevant allergens causing ACD in children and the relation between ACD and atopic dermatitis (AD). MethodsA cohort study on 349 children (0–15 years old) patch tested over a 7-year period was conducted. ResultsPatch test results were positive for at least 1 allergen in 69.3% of patients and were relevant in 69.8%. The highest sensitization rate (76.7%) was observed in children who are 0 to 5 years old (n = 86, 64% females), followed by the group of 6- to 10-year olds (70%, n = 157, 47.8% females), whereas 62.3% of 11- to 15-year-old children (n = 106, 59.4%) were sensitized. The most frequent allergens were nickel (16.3%), cobalt (6.9%), Kathon CG (5.4%), potassium dichromate (5.1%), fragrance mix (4.3%), and neomycin (4.3%). Body areas mostly affected were upper limbs and hands (31%). Approximately one third of children also had AD. Allergic contact dermatitis was more widespread in children with AD. Patch tests resulted positive in 55.3% (50% relevant) of AD compared with 76.9% (77.5% relevant) of the children without AD. Sensitizers were similar to children without AD. ConclusionsVery young children showed a high rate of relevant positive patch test reactions to common haptens. Allergic contact dermatitis may easily coexist with AD.

Balneotherapy for chronic plaque psoriasis at Comano spa in Trentino, Italy

ABSTRACT:  Thermal therapy is used worldwide in the treatment of psoriasis but few controlled studies have evaluated its efficacy and safety. We studied the efficacy and safety of balneotherapy compared to photobalneotherapy performed at Comano spa in Trentino, Italy, in chronic plaque psoriasis in a prospective, nonrandomized, open study. Three hundred adult patients with mild to severe chronic plaque psoriasis were assigned to either balneotherapy or photobalneotherapy with daily narrow‐band ultraviolet B for a mean period of 1 or 2 weeks, reflecting the times that most patients can dedicate to thermal therapy. Patients were evaluated at baseline and end of treatment for psoriasis area and severity index (PASI) and body surface area; self‐administered PASI (SAPASI) and Skindex‐29 were evaluated at the same times, and also at 4 months by a mailed questionnaire. One‐week balneotherapy or photobalneotherapy resulted in a significant reduction in PASI score (11.54% ± 2.76 and 12.76% ± 3.79, respectively; mean ± standard deviation; p < 0.001). Two‐week therapy induced a greater response with photobalneotherapy than with balneotherapy alone, with PASI reduction of 19.8% ± 24.5 and 13.5% ± 23.1 (p < 0.005), respectively. These results were confirmed by SAPASI and Skindex‐29 evaluation. The therapy was well tolerated. Skin improvement was mostly lost after 4 months. Short‐term balneotherapy and photobalneotherapy could thus be offered to patients willing to temporarily discontinue pharmacologic therapy or as adjuvant therapy.

Continuing medical educationUrticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria: Part I. Cutaneous diseases

UNLABELLED Acute urticaria is self-limiting, and a cause can be identified in many patients. Chronic urticaria is a long lasting disease, and patients are commonly examined for an autoimmune origin and for associated diseases. Although the diagnosis of urticaria is straightforward in most patients, it may pose some difficulties at times and it may require a careful differential diagnosis with a number of conditions. Urticarial syndromes comprise both cutaneous and systemic disorders. Part I of this two-part series focuses on the clinical and histologic features that characterize common urticaria and on the cutaneous diseases that may manifest with urticarial lesions and must be considered in the differential diagnosis. LEARNING OBJECTIVES After completing the learning activity, participants should be able to distinguish between the typical wheals of urticaria and urticarial lesions suggesting other diagnoses and to assess patients with urticarial lesions in order to exclude or confirm other cutaneous diseases.

Histiocytoid Sweet syndrome is infiltrated predominantly by M2-like macrophages

BACKGROUND Histiocytoid Sweet syndrome (HSS) is a rare variant of Sweet syndrome (SS). The nature of histiocytoid cells is still uncertain. OBJECTIVE We sought to offer a comprehensive overview on clinical features of HSS and further information on immunohistochemical phenotype of the infiltrate. METHODS The clinical, histologic, and immunohistochemical features of 12 of our patients with HSS and all cases retrieved through a PubMed search were analyzed. RESULTS Lesions consisted of erythematous-violaceous papules and plaques, randomly distributed mostly on the trunk and the limbs. Three patients had myelodysplastic syndrome and 1 had a monoclonal gammopathy. The infiltrate was mainly composed of CD68(+)CD163(+)myeloperoxidase(+)myeloid cell nuclear differentiation antigen(+)CD117(-)CD15(-)CD34(-), a phenotype suggestive of M2-like macrophages. A few mature neutrophils and lymphocytes were also present. Review of all HSS cases showed no sex predominance and no extracutaneous infiltrates; inconstant presence of fever and blood neutrophilia; association with hematologic or solid neoplasms (26%), autoimmune conditions (12%), and infectious diseases (10%); and good response to steroid treatment, with rare relapses or recurrences. LIMITATIONS The study includes a limited case series. The pathogenesis of the disease remains to be clarified. CONCLUSIONS HSS lesions are infiltrated mostly by M2-like macrophages. The clinical features present more similarities than differences with SS.

Heat urticaria: a revision of published cases with an update on classification and management

Heat urticaria (HU) is a rare type of physical inducible urticaria, characterized by itchy erythema and well‐demarcated weals appearing soon after heat exposure. Most cases occur in female patients aged 20–45 years. Both localized and generalized forms exist, depending on the limitation of the reaction to the skin area directly exposed to the physical stimulus or the involvement of distant sites, respectively. In most cases, HU is an immediate reaction, but delayed forms (mostly familial) have been described. HU is a long‐lasting disease with overall duration at diagnosis of approximately 2 years. In about half of cases it is associated with systemic symptoms such as weakness, wheezing, headache, flushing, nausea, vomiting, diarrhoea, tachycardia, even dyspnoea or syncope. The main differential diagnosis includes cholinergic urticaria, exercise‐induced anaphylaxis and solar urticaria. The diagnosis of HU is established by provocation testing, which is also helpful to evaluate the critical temperature threshold. The mean threshold temperature is about 44 °C. A heat desensitization programme can be an effective treatment. Nonsedating H1 antihistamines administered at licensed doses are the mainstay of symptomatic therapy in nearly 60% of patients, but full symptom relief is achieved in only a minority of them. Omalizumab has proven effective in recent case reports.

Preheated autologous serum skin test in localized heat urticaria

Localized heat urticaria (LHU) is a rare type of physical urticaria, characterized by itching and erythema and well‐demarcated weals, appearing within minutes at heat‐exposed body sites. Its pathogenesis has not yet been clarified. We report the case of a 46‐year‐old woman with a generalized form of LHU, which was induced by exposure to warm baths, and consumption of warm food and drinks. Weal reaction was obtained 10 min after application of a metal cylinder heated to 43 °C. Interestingly, only serum previously heated to 56 °C and injected intradermally for autologous serum skin test induced a weal and flare reaction, whereas serum preheated to 45 °C did not induce any reaction. Our patient did not respond to high‐dose antihistamines, and refused a heat desensitization programme. Treatment with colchicine 1 mg/day or ciclosporin A 3.5 mg/kg/day for 1 month yielded no improvement. Mild improvement was obtained with intramuscular injection of triamcinolone acetonide 40 mg every 2 weeks for 2 months.

Keratosis lichenoides chronica: Case-based review of treatment options

Abstract Keratosis lichenoides chronica (KLC) is a rare dermatological condition characterized by keratotic papules arranged in a parallel linear or reticular pattern and facial lesions resembling seborrheic dermatitis or rosacea. The clinical, histological and therapeutic information on 71 patients with KLC retrieved through a PubMed search plus one our new case were analyzed. KLC affects patients of all ages, with a modest male predominance. Pediatric cases represent about one quarter of patients. Diagnosis is usually delayed and histologically confirmed. All patients have thick, rough and scaly papules and plaques arranged in a linear or reticular pattern, on limbs (>80%) and trunk (about 60%). Face involvement is described in two-thirds of patients. Lesions are usually asymptomatic or mildly pruritic. Other manifestations, such as palmoplantar keratoderma, mucosal involvement, ocular manifestations, nail dystrophy, are reported in 20–30% of patients. Children present more frequently alopecia. No controlled trials are available. Results from small case series or single case reports show that the best treatment options are phototherapy and systemic retinoids, alone or in combination, with nearly half of patients reaching complete remission. Systemic corticosteroids as well as antibiotics and antimalarials are not effective.

Adult-onset cutaneous mastocytosis in monozygotic twins

(mean age 42 years). In our presentation of monozygotic twins, genetic influences were identical and the environmental influences were very similar, except for the intake of hydroxyurea for essential thrombocytosis by the twinwith squamous dysplasia. The patient presented is case-controlled by her monozygotic twin, with the one important difference between them being the long-term use of hydroxyurea. This therefore provides further evidence for hydroxyurea causing cutaneous squamous dysplasia.