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Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients., Competing Interests: None

Competing Interests: None

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., (© 2018 Wiley Periodicals, Inc.)

Psoriasis is a chronic complex multisystem, inflammatory, skin disorder that causes vasodilatation and hyperproliferation of keratinocytes, whose clinical expression includes a thickened, erythematous skin, often covered with silver gray scales. Psoriasis is a unique disease where both autoimmune and autoinflammatory responses coexist and the balance between the two components is essential in determining its clinical and histopathological presentation. Adaptive immune responses prevail in chronic plaque psoriasis while innate and autoinflammatory responses predominate in pustular psoriasis. The histopathology of psoriasis is easily recognizable when the disease involves the typical sites such as the extensor surfaces. Although a biopsy is rarely required in case of classic psoriasis, in atypical and controversial conditions, histopathological examination remains the main diagnostic tool that can help in differentiating psoriasis from other dermatoses. In this review, we will discuss the histopathological pictures of the different clinical variants of psoriasis giving some clues to drive the correct diagnosis when the clinical aspects are not enough indicative of the disease.

Cutaneous mucinoses are a group of conditions characterized by increased amounts of acid mucin in the dermis. They can be generalized or localized and occur isolated or in the setting of systemic diseases. Obesity-associated lymphedematous mucinosis is a distinct variant of mucinosis occurring in obese patients without any thyroid dysfunction. So far, only few cases of this rare condition have been reported in the English literature. Here, we describe two new cases and discuss some histological differences with the pretibial myxedema.

Bullous morphoea is a rare variant of localized scleroderma whose pathogenesis has been widely discussed. We retrospectively reviewed the records of all histopathologically confirmed cases of morphoea followed from 2005 to 2015 at the Dermatology Clinic and Pathology Institute of the University of Cagliari, Sardinia, Italy. Among 137 patients with morphoea, 2 cases of the bullous variant were identified, which were successfully treated with methotrexate. Thus, the bullous form comprised 1.4% of all cases of morphoea, which is much lower than the 7.5% previously reported. In one of the cases, histopathological examination revealed a peculiar 'stretching' pattern of basal keratinocytes attached to the epidermal roof of the bulla, together with increased lymphatic vessels, which were either collapsed or dilated, stressing the role of lymphatics and possibly of excessive skin trauma and friction in the development of bullous lesions., (© 2017 British Association of Dermatologists.)

As clinical skills improve and innovative diagnostic techniques become available in the field of dermatology and dermatopathology, new types or additional variants of malignant skin tumors are described. This article reviews the current nomenclature, clinico-pathological features, differential diagnosis, prognostic and therapeutic implications of some new dermato(patho)logical rare emerging skin tumors, including epithelial tumors (squamous cell carcinoma with mucinous metaplasia), adnexal tumors (endocrine mucin-producing sweat gland carcinoma), soft tissue tumors of vascular differentiation (pseudolymphomatous cutaneous angiosarcoma, pseudomyogenic hemangioendothelioma), hematopoietic tumors (blastic plasmacytoid dendritic cell neoplasm) and mixed epithelial/melanocytic tumor (squamomelanocytic tumor).

Background: Granulomatous reaction is a well-known complication following soft filler procedures. However, the diagnosis of filler-induced granulomas may be challenging because of the occasional reluctance of patients to report the previously performed aesthetic procedure., Objective: To describe a new clinical situation in which some patients, in the quest for physical perfection, become addicted to multiple sequential cosmetic injections, increasing the risk of adverse reactions., Methods: We describe three women who developed diffuse facial nodular tumefaction after multiple procedures of filler injections into their face that occurred at different times in the previous years., Results: Histopathology showed a granulomatous reaction including different combined substances that were identified with different types of micro-implants in the same biopsy., Conclusions: Excessive demand of multiple cosmetic injections may increase the frequency of skin granulomatous reactions and can be included in the spectrum of similar addictive dysmorphophobic behaviours. Histopathology is the best mean to achieve the diagnosis., (© 2014 European Academy of Dermatology and Venereology.)

Diffuse dermal angiomatosis is a form of cutaneous reactive angiomatosis characterized clinically by painful erythematous or violaceous lesions with ulcers that may mimic cutaneous vasculitis/vasculopathy. Histologically it shows a benign, diffuse proliferation of endothelial cells with tiny blood vessels in the papillary and reticular dermis. Herein, we report four patients with diffuse dermal angiomatosis in the setting of calciphylaxis and monoclonal gammopathy and review the cases previously published in the literature. Comorbidities and management will also be discussed.

Background: Allopurinol is extensively prescribed for conditions associated with urate excess, despite being responsible for severe cutaneous adverse drug reactions (ADR)., Objective: A cross-sectional survey of allopurinol cases observed at the main Dermatology Department with inpatients facilities in southern Sardinia. (approx 560,836 inhabitants)., Material and Methods: Data collection of all consecutive patients referred for ADR between 2001 and 2010. Causality assessment followed the WHO Collaborating Centre for Drug Monitoring criteria; illness severity score was adopted for toxic epidermal necrolysis (SCORTEN)., Results: Allopurinol was the culprit drug in 84 of 780 cutaneous ADR cases (10.7%; 8.4 cases/year). Mean age was 74 years, 58% of the patients were female, 95% of patients required hospitalization. Clinical forms were maculo-papular eruptions (34 cases), Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (31 cases), vasculitis (six cases), Drug Rash Eosinophilia and Systemic Symptoms (DRESS) (three cases), Acute Generalized Exanthematous Pustolosis (AGEP) (three cases), Pityriasis rosea-like eruption (three cases), lichenoid dermatitis (two cases), fixed drug eruption (one case), erythroderma (one case). The indication for allopurinol prescription was asymptomatic hyper-uricemia in 95% of the patients. Twelve patients were under allopurinol dosage adjustment according to creatinine clearance. Final causality assessment was definite for 12% of the cases and probable for the remaining 88%. Full recovery was achieved in 88% of subjects; ten SJS/TEN patients died (12% overall mortality; 32% mortality of the SJS/TEN cases)., Conclusion: Considering the populations size of Southern Sardinia, is plausible that 1.5/100,000 Sardinian will be affected by allopurinol related ADR per year. Advanced age, and inappropriate allopurinol prescription were the main conditions affecting morbidity and mortality., (© 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology.)

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.

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.

Pityriasis rosea is a common, acute eruption of uncertain etiology. A rash very similar to this idiopathic disease is also attributed to several drugs, and recovery, which depends on withdrawal of the responsible drug, can be delayed by its late identification. A prospective study to record all cases of adverse cutaneous reactions presenting with pityriasis rosea like manifestations was conducted at the center for drug-surveillance of the dermatology department of Cagliari University. We developed an intensive surveillance program from June 2002 to May 2005, adopting the WHO Collaborating center for Drug Monitoring causality assessment criteria and algorithm. Eight cases, six male and two female, were studied in a 3-year period. None had previously suffered from drug intolerance or allergy. Clinical manifestations were very similar to pityriasis rosea. Responsible drugs were mainly angiotensin-converting enzyme inhibitors, alone or in combination with hydrochlorothiazide, followed by one case each for hydrochlorothiazide plus sartan, allopurinol, nimesulide, acetyl salicylic acid. Recovery was obtained in all cases with drug withdrawal. Final causality assessment was probable for all eruptions. Frequency of drug pityriasis rosea-like eruptions is probably underreported. The mildness of the eruption, mimicking a very common and self-limiting disease does not prompt physicians to verify the use of medications until persistence, severity of lesions and itching require re-evaluation of the original diagnosis.

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.

Cutaneous Crohn disease refers to granulomatous skin manifestations not contiguous with gastrointestinal affected areas. It is a very rare condition, especially in children, and is easily misdiagnosed when characteristic gastrointestinal symptoms are absent. We report a 10-year-old Caucasian girl with a 6-month history of erythematous, firm tumescence of the left labium majus pudendi and moist vegetations circumscribing the anal ostium. Histologic analysis of skin biopsy specimens from both types of lesions showed a characteristic granulomatous noncaseating infiltrate throughout the dermis. Endoscopic examination and a colon biopsy specimen showed chronic granulomatous inflammation consistent with Crohn disease. Treatment with prednisolone 20 mg daily, metronidazole 250 mg three times daily, topical corticosteroids, and mupirocin ointment produced marked improvement of the vulvar edema, whereas the perianal lesion had a relapsing course. Early recognition of extra-intestinal manifestations of Crohn disease, which are extremely rare in children, may be difficult. Coexistence of contiguous and noncontiguous lesions, representing the very first signs of the disease, are further peculiarities in our patient.

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.

Background: Wells' syndrome was first described by Wells in 1971 as a recurrent granulomatous dermatitis with eosinophilia and was later named eosinophilic cellulitis. It is defined by the following criteria: (i) sudden onset of annular or circinate erythematous-edematous patches that rapidly evolve to morphea-like blue-slate-colored plaques; (ii) a histological picture usually characterized by the presence of 'flame figures'; (iii) non-constant blood hypereosinophilia., Methods: We describe the case of a 49-year-old woman who reported the sudden appearance of a few hard, pasty, oval-shaped, reddish-violet, moderately itchy, erythematous-edematous patches on both arms about 2 months before our observation., Results: The remote pathologic history showed that the woman have been submitted to quadrantectomy with lymph node dissection followed by cobalt therapy for breast cancer. Based on the pharmacological history, intake of drugs was excluded. Routine blood chemistry and instrumental tests did not show any alteration, nor did assays of the main autoantibodies, complement, circulating immunocomplexes, or tumor markers., Conclusions: Histologically the picture was compatible with the diagnosis of Eosinophilic cellulitis. Following topical corticosteroid therapy the lesion healed rapidly. It relapsed 2 months later, and was again cured with the same topical treatment. No further relapses were observed in a 1 year follow-up.

The antifungal efficacy and tolerability of 1% terbinafine cream vs. 1% bifonazole cream were assessed in a single blind randomized trial in patients with pityriasis versicolor. Terbinafine, a drug of the allylamines group, a new class of anti-mycotic agents, blocks sterol biosynthesis in the pathogen through inhibition of squalene epoxidase and consequent squalene accumulation, a primarily fungicidal process. Forty pityriasis versicolor patients, (18 M, 22 F; mean age 32.4 years; min. 16, max. 65), used 1% terbinafine cream or 1% bifonazole cream for a maximum of 4 weeks. All patients were followed-up weekly both clinically and mycologically. Clinical cures, defined as negativization of each clinical parameter, were recorded for 20 terbinafine patients (100%) and 19 bifonazole patients (95%), with routine microscopy and Wood's light tests both negative. By the 2nd week of treatment, 2 terbinafine patients were mycologically cured (10%). By the 3rd week, 14 terbinafine patients (70%) and 5 bifonazole patients (25%) were mycologically cured. The present controlled clinical trial consequently demonstrates that terbinafine is rapidly effective and well tolerated for treatment of pityriasis versicolor.