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 Behcet'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

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

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. 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. 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. 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 an 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

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 carbamazepin

The diagnosis of androgenetic alopecia in children: Considerations of pathophysiological plausibility

Androgenetic alopecia (AGA), one of the most common causes of hair loss in men and women, is an infrequent cause of alopecia in children. In AGA, patients generally start noticing hair thinning after the onset of puberty due to progressive miniaturisation of the hair follicle which leads to vellus transformation of terminal hair. However, the occurrence of prepubertal AGA has rarely been reported in the literature. The pathophysiology of AGA is tightly linked to androgen hormones; prepubertal children do not usually produce significant amounts of adrenal or gonadal androgens. When it does occur, an underlying abnormality should be suspected. Secondary causes of AGA must be excluded when evaluating a patient before the appearance of puberty. Premature puberty, polycystic ovarian syndrome and other causes of hyperandrogenism can present with hair loss in an androgenetic pattern. This article reviews the normal physiology of androgen hormones and their role in the pathophysiology of childhood AGA

Are dissecting cellulitis and hidradenitis suppurativa different diseases

Although dissecting cellulitis (DC) and hidradenitis suppurativa (HS) are classified separately, they share many clinical, dermatoscopic, pathogenetic, and histologic aspects, as well as therapeutic options. The association between DC, HS, and acne conglobata represents the follicular occlusion triad or follicular occlusion tetrad, which may include a pilonidal sinus. DC, also known as “folliculitis et perifolliculitis capitis abscendes et suffoidens,” is classified as a secondary cicatricial and neutrophilic alopecia. It occurs with perifolliculitis of the scalp, dermal abscesses, sinus tract development, and secondary scarring alopecia. HS, sometimes known as acne inversa, is a chronic relapsing inflammatory disease afflicting apocrine gland–rich areas of the body with painful nodules and abscesses, sinus tracts, and scarring. Given the overlap between the clinical features and the pathogenesis of DC and HS, it would be more appropriate to consider these conditions as two different localizations of the same disease rather than two different pathologies, being a follicular occlusion disease occurring on the scalp and on the apocrine gland–rich areas of the body

Associations between alopecia areata and multiple sclerosis: a report of two cases and review of the literature

Alopecia areata (AA) is an immune‐mediated inflammatory disorder that targets anagen phase hair follicles (HFs), leading to nonscarring hair loss.1 Several autoimmune diseases are commonly associated with AA. Herein we describe two cases (Table 1) of patients affected by AA, who have developed after several years multiple sclerosis (MS), which is one of the most frequent autoimmune diseases of the central nervous system

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