Keratosis lichenoides chronica: Case-based review of treatment options.

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.

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.

Allergic contact dermatitis in children with and without atopic dermatitis.

BACKGROUND: Prevalence and causes of allergic contact dermatitis (ACD) in children vary with time and geographical area. OBJECTIVE: This study aimed to determine the relevant allergens causing ACD in children and the relation between ACD and atopic dermatitis (AD). METHODS: A cohort study on 349 children (0-15 years old) patch tested over a 7-year period was conducted. RESULTS: Patch 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. CONCLUSIONS: Very young children showed a high rate of relevant positive patch test reactions to common haptens. Allergic contact dermatitis may easily coexist with AD.

Cutaneous manifestations of Churg-Strauss syndrome: report of two cases and review of the literature.

Churg-Strauss syndrome (CSS) is a small-vessel vasculitis characterized by severe asthma, lung/tissues infiltrates, extravascular necrotizing granulomas, and eosinophilia. Cutaneous involvement is common but may not be highly suggestive. Two typical cases of CSS with cutaneous involvement are herein reported, both females, 37 and 54 years old, presenting with lower limbs palpable purpura and urticarial lesions of the neck, respectively. A comprehensive review of the literature showed that cutaneous manifestations occurred in 40-81% of CSS patients and were the presenting sign in 14% of the patients. Moreover, a total of 68 cases of CSS with a detailed description of the cutaneous lesions have been published. In the majority of these patients, skin lesions allowed for the histopathological diagnosis of CSS. The most common clinical features were papulo-nodules with the histological picture of extravascular Churg-Strauss granuloma followed by purpuric and/or necrotic lesions in the lower limbs corresponding to small-vessel vasculitis with eosinophils. Less common lesions included urticarial lesions and livedo reticularis. Therefore, a high index of suspicion on skin lesions and the proper lesion selection for histological examination may be very important for early diagnosis of CSS. Clinical-pathological correlation is essential, as both clinical and histological features are not pathognomonic.

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.

Urticarial lesions: if not urticaria, what else? The differential diagnosis of urticaria: part II. Systemic diseases.

UNLABELLED: There are a number of systemic disorders that can manifest with urticarial skin lesions, including urticarial vasculitis, connective tissue diseases, hematologic diseases, and autoinflammatory syndromes. All of these conditions may enter into the differential diagnosis of ordinary urticaria. In contrast to urticaria, urticarial syndromes may manifest with skin lesions other than wheals, such as papules, necrosis, vesicles, and hemorrhages. Lesions may have a bilateral and symmetrical distribution; individual lesions have a long duration, and their resolution frequently leaves marks, such as hyperpigmentation or bruising. Moreover, systemic symptoms, such as fever, asthenia, and arthralgia, may be present. The most important differential diagnosis in this group is urticarial vasculitis, which is a small-vessel vasculitis with predominant cutaneous involvement. Systemic involvement in urticarial vasculitis affects multiple organs (mainly joints, the lungs, and the kidneys) and is more frequent and more severe in patients with hypocomplementemia. Clinicopathologic correlation is essential to establishing a correct diagnosis. LEARNING OBJECTIVES: After completing the learning activity, participants should be able to distinguish urticarial lesions suggesting diagnoses other than common urticaria; assess patients with urticarial lesions, and suspect systemic diseases presenting with urticarial skin lesions.

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

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.

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 patient's 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.