Dermoscopy of cutaneous adnexal tumours: a systematic review of the literature.

Cutaneous adnexal tumours (ATs) encompass a variegated group of hamartomas and benign or malignant tumours, originating from the hair follicle, sebaceous, eccrine or apocrine glands that may simulate other cutaneous neoplasms. This study aims to provide a comprehensive overview of the spectrum of clinical and dermoscopic features of ATs, to better define these lesions and assist in the differential diagnosis. We performed a two-step systematic search of the literature in PubMed, Embase and Cochrane Library databases from inception until 4 September 2020. In the first step, we aimed to define histological variants of ATs with descriptions of dermoscopic criteria. The second step included a search for the name of each previously identified AT variants in the same databases adding 'AND (epilum* or dermosc* or dermatosc*)'. All study types in English language reporting dermoscopic images of ATs were included. Collisions between ATs and other inflammatory or neoplastic skin lesions were excluded, with the exception of collisions with a sebaceous nevus. The protocol of this study was prospectively registered in PROSPERO (CRD42021244677). In total, 206 articles met our inclusion criteria, encompassing 372 ATs in 365 patients. Most ATs were apocrine-eccrine (n = 217, 58.3%, n = 173 benign) with a prevalence of poromas (n = 82), followed by follicular ATs (n = 88, 23.7%, n = 83 benign) and sebaceous ATs (n = 67, 18.0%, n = 49 benign). Most patients had a single AT lesion (320, 86.0%), while 42 (11.3%) had multiple ATs. A syndrome causing multiple ATs was identified in 15 patients. Histopathological analysis revealed 82% benign (n = 305) and 18.0% malignant (n = 67). ATs were classified according to their ability to mimic four groups of more common skin tumours: basal cell carcinoma, squamous cell carcinoma, melanocytic lesions and benign cutaneous lesions. Moreover, we have highlighted the ability of malignant variants of ATs to simulate benign skin lesions. This systematic review offers a comprehensive overview of the common clinical and dermoscopic features of follicular, sebaceous and apocrine-eccrine ATs and details possible differential dermoscopic features.

Alopecia neoplastica as a sign of visceral malignancies: a systematic review.

Alopecia neoplastica (AN) from visceral tumours is a rare form of cutaneous metastasis in which internal malignancies spread to the scalp. The diagnosis of AN may be very challenging, especially when its onset precedes the diagnosis of the primary tumour. We aimed to improve the knowledge on AN, highlighting that in case of scarring localized alopecia, a differential diagnosis with metastasis should always be considered. We performed a systematic review to describe the main demographic and clinical features associated with AN from visceral malignancies; a survival analysis was also performed. In 118 reports, accounting for 123 patients, we found that women were more affected by AN than men (53.7% vs. 46.3%). The most frequent site of the primary tumour was the gastrointestinal tract (24.4%), followed by breast (17.9%), kidney (8.1%), lung (7.3%), thyroid (7.3%), uterus (6.5%), central nervous system (6.5%), liver (3.3%) and other anatomic areas for 18.7% of cases. Furthermore, in more than half of the cases (66.1%), AN lesions were single and were mainly diagnosed after the primary visceral tumour (71.5%). Finally, survival analysis highlighted a lower progression-free survival in men; while, no significant differences in overall survival were reported among genders. In conclusion, metastatic skin disease should always be taken into consideration when dealing with patients with localized scarring alopecia.

Cross-elicitation responses to 2-methoxymethyl-p-phenylenediamine in p-phenylenediamine highly allergic volunteers using allergy alert test: the Italian experience.

Summary: Background. Allergic contact dermatitis after exposure to p-phenylenediamine (PPD)-containing hair dye products is a common and important clinical problem. Because there is a high rate of cross-elicitation of allergic contact dermatitis to other important hair dye products (such as p-toluene diamine [PTD] and other aminophenol hair dyes) in PPD allergic patients, safer alternative dyes with excellent hair coloring options are needed. We studied 2-methoxy methyl-PPD (Me-PPD), a chemical derivative of PPD for tolerance versus cross-elicitation in a cohort of eight PPD-allergic volunteers. Objective. To study tolerance to Me-PPD in a PPD highly allergic Italian cohort. Methods. Eight volunteers with a history of contact dermatitis to hair dyes or other PPD-containing chemicals and positive patch tests to 1% PPD in petrolatum, were recruited to study their immediate and delayed skin reactivity to PPD, vehicle control and 2-methoxy-methyl-PPD (Me-PPD), using the allergy alert test (simulating hair dyeing conditions) on volar forearm skin. This is a short-contact open patch test. Results. All eight volunteers reacted to PPD allergy alert test (100%); none reacted to vehicle (0%), and seven of eight reacted to Me-PPD allergy alert test (88%). However, in those seven volunteers who exhibited cross-elicitation to Me-PPD, their aggregate skin test reactivity to Me-PPD was significantly less than that of PPD (figure 3, p minore 0.0062, highly significant, paired two-tailed, students t test). Conclusions. Me-PPD may offer a safer alternative for PPD-allergic patients with an absent or reduced elicitation response in the allergy alert test simulating hair dye use conditions. Even patients with strong patch test reactions, with appropriate selection by allergy alert test and counselling, may be able to tolerate hair dyeing with Me-PPD containing products.

Dialysis-associated pseudoporphyria successfully treated with vitamin D. Report of two cases.

Pseudoporphyria refers to a rare bullous dermatosis characterized by the clinical and histological features of porfiria cutanea tarda without abnormalities in porphyrin metabolism. The pathogenesis is heterogeneous and several exogenous factors may promote the bullous lesion formation, including medications, end stage renal disease, dialysis and tanning beds. Regarding treatment of this condition, in literature different therapy have been reported, such as glutathione and his precursor N-acetylcysteine, which presents anti-oxidant properties; however even more toxic drugs, such as chloroquine, are used. Moreover, in patients with drug-induced PP discontinuation of the offending agent, if possible, is a crucial aspect of the clinical management. We report two cases of dialysis patients presenting blisters on extremities, which healed with the avoidance of UV exposure and oral Vitamin D supplementation. Interestingly Vitamin D despite the lack of antioxidant properties led to a completely resolution of PP in both our patients within 30 days. A possible explanation of this finding is that Vitamin D, playing a key role in the regulation of serum Ca2+, can modulated cadherin-cadherin interactions and led to healing of pseudoporphyria bullous lesions. Finally we highlight the prominent role of UV-exposure in PP elicitation thus a good photoprotection is essential for all patients with pseudoporphyria.

An incomplete form of childhood Behçet's disease treated with infliximab.

Behçet's disease (BD) is a multi-systemic vasculitis characterized by the possible presence of cutaneous, ocular, articular and neurological manifestations. In this report, we examine the case of a fifteen-year old boy with an incomplete form of juvenile Behçet's disease which began with joint involvement and developed into a complete form only after several years. The patient showed a rapid response to anti-TNF-alpha (infliximab) with an improvement of mucocutaneous lesions (oral and genital ulcers, pseudofolliculitis) and arthritis.

Granuloma annularis revealing Wegener's granulomatosis.

Skin manifestations are often associated with systemic autoimmune diseases (SAD). Some SAD, such as systemic lupus erythematosus, psoriatic arthritis and scleroderma display pathognomonic dermatological features, whereas other systemic diseases such as sarcoidosis, vasculitis and rheumatoid arthritis can present with non-specific skin manifestations that range from erythema nodosum to necrotic lesions. Here we report the case of a 25-year-old man with uveitis, polyarthrirtis, pulmonary involvement, nephrotic syndrome, cutaneous granuloma and pneumonia by E. coli.

Basal cell carcinoma: differences according to anatomic location and clinical-pathological subtypes.

AIM: Basal cell carcinoma (BCC) is the most common skin malignant neoplasm in humans. Its localization and its clinical-pathological aspects are fundamental for the treatment and the outcome of these tumors. We wanted to verify if different clinical-pathological subtypes of BCC may be present with different frequencies on single skin areas. METHODS: Three hundred six patients affected by BCC seen in Sant'Andrea Hospital, U.O.C. Dermatology, NESMOS Department, Faculty of Medicine, University of Rome "Sapienza", from January 2008 to December 2010, were retrospectively included in this study. Findings from all patients were tabulated and analyzed to characterize the clinical-pathological aspects of BCC according to their anatomic localization. We considered the following clinical subtypes of BCC, nodular, superficial, sclerodermiform, pearly and ulcerative. RESULTS: One hundred ninety-seven out of 306 patients (64.4%) were localized on the head, 6 (1.9%) on the neck, 73 patients (23.9%) on the trunk, 2 (0.6%) on the perineum, 4 (1.3%) on upper limbs and 24 (7.9%) on legs. On the head BCC were mostly nodular (44.7%). On the trunk they were mostly superficial (34.3%). BCC on legs were ulcerative in all the 24 patients. CONCLUSION: Our data confirm that BCC may have different clinical-pathological aspects on single skin areas. Interestingly in our casistic BCC on the legs were present in an uncommon high percentage. They presented as ulcerative lesions and this fact leads to conclude that in every patient presenting a chronic ulcer on the leg with difficulty to be cured a biopsy is mandatory to put in evidence the possible presence of BCC and consequently to perform the correct surgical treatment to obtain a complete response for the patient.

Multiple skin ulcers due to Serratia marcescens in a immunocompetent patient.

Serratia marcescens is a species of gram negative bacillus, classified as a member of the Enterobacteriaceae, mainly involved in opportunistic infections, particulary in the hospital environment. Cutaneous infections have rarely reported in literature and are predominantly observed in elderly or in immunocompromised patients. The clinical manifestations of skin infections include granulomatous lesions, necrotizing fasciitis, nodules, cellulitis, ulcers, dermal abscesses. Infections caused by S. marcescens may be difficult to treat because of resistance to a variety of antibiotics, including ampicillin and first and second generation cephalosporins. Aminoglycosides have good activity against S. marcescens, but resistant strains have also been described. We report a very intriguing case of S. marcescens infection, in an immunocompetent 18-year-old man, causing multiple rounded ulcers of varying sizes, along with few pustular lesions that both clinically and histopathologically mimic a pyoderma gangrenosum (PG). This is a non infectious neutrophilic skin disorder, characterized by painful and rapidly progressing skin ulceration. According to our experience, we would strongly recommend to perform cultures of multiple skin ulcers resembling PG, even in young healthy patients, to ensure correct diagnosis and treatment, since resistant to conventional antibiotics bacteria such as S. marcescens may be the cause of these lesions, like in the case here reported.

Pemphigus vulgaris in a patient with arthritis and uveitis: successful treatment with immunosuppressive therapy and acyclovir.

A case of pemphigus vulgaris in a 41-year-old man with undifferentiated arthritis and uveitis is described. Histology of labial mucosa showed acantholytic, necrotic, and multinucleated giant keratinocytes having some nuclear inclusions suggestive of a virus infection. Specific serological tests revealed IgG positivity for HSV-1, CMV, and EBV, while real-time polymerase chain reaction assay from a biopsy of the mucosal lesion showed the presence of HSV-1/2 DNA. Treatment with prednisone, methotrexate, and acyclovir induced the complete remission of mucosal and joint symptoms, which then relapsed after interruption of antiviral therapy or immunosuppressive therapy. Therefore, a combined treatment with low doses of prednisone, methotrexate, and acyclovir was restarted and during 18 months of follow-up no recurrence was registered. Correlations between pemphigus and the herpes virus infection and also between autoimmune arthritis and herpetic agents have been well documented, but the exact role of the herpes virus in these disorders still needs further discussion. Our case strongly suggests that when autoimmune disorders do not respond to immunosuppressive agents, a viral infection should be suspected, researched, and treated.

Sensitivity of different assays for the serological diagnosis of epidermolysis bullosa acquisita: analysis of a cohort of 24 Italian patients.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disease characterized by tissue-bound and circulating autoantibodies to the dermal-epidermal junction. The autoantibody target is type VII collagen (Col VII) which is involved in dermal-epidermal adhesion. Diagnosis is made by clinical and histopathological findings, linear deposition of autoantibodies at the dermal-epidermal junction detected by direct immunofluorescence, and binding to the dermal side of salt-split skin by indirect immunofluorescence (IIF). However, the detection of specific anti-Col VII reactivity has an important confirmatory value. METHODS: The humoral immune response in EBA sera was analysed by (i) IIF on human skin, (ii) a commercial Col VII ELISA, and (iii) immunoblotting on Col VII produced by an epithelial cell line. OBJECTIVE: The aim of this study was to compare the sensitivity of different approaches for the serological diagnosis of EBA. RESULTS: The vast majority of EBA sera (79.2%) bound to the Col VII non-collagenous domains by a commercial ELISA, while a small proportion of patients (12.5%) exclusively reacted to the collagenous domain by immunoblotting. Of note, the autoantibodies reactivity to Col VII was more frequently detected by IB (91.7%) than by IIF (83.3%) and ELISA (79.2%). Interestingly, 2 out of 24 sera recognized Col VII epitopes undetectable in the native secreted protein but present in the context of extracellular matrix proteins, as assessed by immunomapping on Col VII-deficient skin. CONCLUSION: Our findings show that the use of multiple assays allows to improve diagnostic performance. An algorithm for efficient serological diagnosis of EBA is proposed.

Pharmacological management of pediatric Kerion celsi.

We report a case of severe tinea capitis, treated successfully with griseofulvin. In our opinion, the treatment of this severe dermatophytosis with griseofulvin is safe and effective. Other treatments, such as itraconazole pulsed therapy, failed, despite an initial improvement, leading to an aggressive recurrence of the lesion. We chose griseofulvin for its well-known large spectrum activity, also against uncommon species, like Microsporum Gypseum, which are responsible for the most severe cases.

Role of steroid therapy in pseudoxanthoma elasticum-like papillary dermal elastolysis.

Pseudoxanthoma Elasticum-like Papillary Dermal Elastolysis (PXE-PDE) is a peculiar idiopathic elastolytic disorder that clinically resembles Psudoxanthoma elasticum(PXE). It is histologically characterized by a total or partial loss of elastic fibers in the papillary dermis. It more often affects elderly women and is characterized by asymptomatic and symmetrical yellowish papules localized predominantly on the neck, supraclavicular regions, and flexural areas. After analyzing a series of cases and the recent literature suggesting that glucocorticoids may down-regulate the elastin gene expression and elastin mRNA, in cultured human skin fibroblasts, we think that high dose and prolonged steroid therapy may contribute to the appearance of PXE-PDE lesions.

Lichen sclerosus et atrophicus induced by carbamazepine: a case report.

We report a case of Lichen Sclerosus in a 73-year-old man who had been treated for epilepsy with carbamazepine. Lichen sclerosus et atrophicus (LSA), also called lichen sclerosus (LS), is a chronic inflammatory cutaneous condition characterized by white plaques with epidermal atrophy and scarring. To date no cases of LSA has been linked to carbamazepine, although in a few cases lichenoid eruptions but without sclero-atrophy have been described after exposure to this drug. Therefore, to our knowledge, this is the first report of a Lichen sclerosus et atrophicus induced by carbamazepine.