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Background: Alopecia areata (AA) is an organ-specific autoimmune disease that affects the hair follicles of the scalp and the rest of the body causing hair loss. Due to the unpredictable course of AA and the different degrees of severity of hair loss, only a few well-designed clinical studies with a low number of patients are available. Also, there is no specific cure, but topical and systemic anti-inflammatory and immune system suppressant drugs are used for treatment. The need to create a global registry of AA, comparable and reproducible in all countries, has recently emerged. An Italian multicentric electronic registry is proposed as a model to facilitate and guide the recording of epidemiological and clinical data and to monitor the introduction of new therapies in patients with AA., Methods: The aim of this study was to evaluate the epidemiological data of patients with AA by collecting detailed information on the course of the disease, associated diseases, concomitant and previous events, and the clinical response to traditional treatments. Estimate the impact on the quality of life of patients., Results: The creation of the National Register of AA has proven to be a valid tool for recording, with a standardized approach, epidemiological data, the trend of AA, response to therapies and quality of life., Conclusions: AA is confirmed as a difficult hair disease to manage due to its unpredictable course and, in most cases, its chronic-relapsing course, capable of having a significant impact on the quality of life of patients.

Background: There is no universally-accepted classification of Spitzoid tumors. This makes it difficult to assign a correct diagnosis and select a treatment that minimizes the risk of overestimating, or worse, underestimating, the malignant potential of these tumors. The aim of this study was to describe the clinical-pathological and epidemiological features of Spitzoid tumors, as well as to assess mortality in these patients., Methods: This retrospective cohort study looked at data on Spitzoid tumors excised in 1999-2012 at the Dermatologic Clinic of the Turin University Hospital. Spitzoid melanoma specific survival curves were generated with the Kaplan-Meier method and compared using the log-rank test., Results: In this time period, 1663 lesion were described at the pathologic report as Spitzoid. 262 (15.75%) were Spitz nevi, 307 (18.46%) Reed nevi, 827 (49.73%), 810 (48.71%) Spitzoid dysplastic nevi, 17(1.02%) atypical Spitzoid tumors, and 267 (16.06%) Spitzoid melanomas. Median follow-up time was 9 years. Out of the entire cohort only 24 patients died from melanoma. All of them received a diagnosis of Spitzoid melanoma. None of the patients with a diagnosis of not melanoma Spitz tumor died for melanoma during the follow-up., Conclusions: In the large majority of the cases, Spitz tumor should be considered as benign lesion and excised only if melanoma features are seen. The used clinical pathological classification avoid misdiagnoses, inappropriate treatment and the risk of death for melanoma.

The term "invisible dermatoses" is often used in two different contexts: 1) dermatoses which are invisible to the clinician, i.e., diseases with no significant clinical features; 2) dermatoses which are invisible to the pathologist. These latter comprise a wide spectrum of entities which present with definite and obvious clinical features, but subtle or hidden histological features and are therefore "invisible" histologically. Diagnosis of such diseases represents a great challenge to both the dermatologist and dermatopathologist. The list of such diseases, originally comprising only few entities, has been gradually expanded over decades and now is a large one. This review focuses on the recently expanded spectrum of such diseases, and offers to the dermatopathologist a strategy for their diagnosis, based mainly on proper awareness, recognition of subtle features, special stains, special investigations such as immunofluorescence and histochemistry and proper clinicopathological correlation.

Sepsis is a potentially life-threatening complication of an infection where cutaneous lesions often represent one of the early signs. A myriad of microorganisms including bacteria, fungi, yeasts, viruses, protozoas, helminths and algae can be implicated. A broad spectrum of clinical and histopathologic findings can be observed in the skin and the common denominator is a thrombotic vasculopathy. The pathogenesis of cutaneous septic vasculitis (SV)/vasculopathy is complex and includes five main mechanisms: disseminated intravascular coagulation, direct invasion and occlusion of blood vessel walls by microorganisms, hypersensitivity reaction with immune complex deposition into blood vessel walls, embolism from a distant infectious site and vascular effects of toxins. Herein we describe the clinicopathologic findings of some selected cases of SV recently observed in our hospital, including purpura fulminans, necrotizing fasciitis, cutaneous meningococcemia, malignant syphilis and disseminated alternaria infection. Histopathologically, a wide spectrum of histopathologic changes was observed in skin specimens from the various entities, involving the intensity and composition of the inflammatory infiltrate, the degree of vascular changes and the presence of microorganisms, that ranged from a predominant not inflammatory, thrombotic-occlusive vasculopathy in purpura fulminans to leukocytoclastic vasculitis like changes in cutaneous meningococcemia to a dermal angiomatosis-like pattern in disseminated Alternaria infection. The different pathologic presentations may be related to the microorganism involved, the main pathogenetic mechanism that induced the vascular injury and the individual immunologic burden. Early skin biopsy for histopathologic examination and microbiologic culture is a cornerstone in the diagnosis of life-threatening diseases that present with cutaneous septic vasculitis. Ancillary techniques, such as immunohistochemistry and polymerase chain reaction are additional novel and helpful tools to identify pathogens, leading to definite diagnosis in cases with challenging or ambiguous clinical and/or pathologic findings.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by an heterogeneous group of severe dermatologic manifestations and systemic involvement, due to several groups of medicaments. A series of 9 consecutive cases, observed from 2008 to 2013 in the Department of Dermatology, University of Pavia, is reported, all satisfying the clinical, hematological and systemic diagnostic criteria of DRESS. Clinically, 4 out of 9 patients had an urticarial and papular eruption, 2 an erythema-multiforme-like (EM-like) pattern, 2 erythroderma and 1 had an erythematous and macular reaction. Aim of the study was to describe the histopathologic features of DRESS and to trace a possible correlation between the four clinical recognized types of the syndrome and the histopathological patterns. Predominantly, a superficial perivascular lymphocytic infiltrate, extravasation of erythrocytes, and focal interface changes characterized DRESS cases. Less frequently, histopathology revealed the presence of necrotic keratinocytes; surprisingly, only in 2 cases the presence of rare dermal eosinophils was detected, even if all the patients had significant peripheral eosinophilia. A histopathological diagnosis of DRESS seems per se, according to our data, not feasible, since the main histopathological changes (interface changes, superficial perivascular dermatitis, focal spongiosis, lichenoid infiltrate, rare presence of necrotic keratinocytes) can be interpreted generically as a drug induced dermatitis. The above mentioned histopathological changes, however, when associated with clinical information on cutaneous and systemic involvement of the patient, allow the pathologist or the dermatopathologist to make a diagnosis of DRESS with a reliable margin of certainty.

Erythema multiforme (EM), Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute bullous disorders associated to different prognosis, mainly due to infections and drugs. More in particular EM in more than 90% is caused by infections (especially Herpes virus infection), while, on the other hand SJS and TEN are referable in more than 95% of cases to drugs. Distinction among these three forms is often controversal and still debated. An attempt to distinguish these forms has been possible mainly according to anamnesis, clinical presentation (morphology, involved sites, extension of lesions) and pathogenetic mechanisms, being on the contrary more difficult from an histopathological point of view. Nowadays a clear diagnosis and a distinction from other life-threatening diseases is possible with the integration of all the mentioned aspects. Moreover, this recognition should be as early as possible in order to perform a prognostic evaluation of the case and to start supportive cares and therapies as soon as possible.

Several factors hamper the clinical and histologic diagnosis of panniculitis. Clinically the patients tend to present with erythematous subcutaneous nodules with quite a monotonous appearance, without additional symptoms. Histopathologically, as the subcutaneous fat responds to a variety of insults in a limited number of forms, there are sometimes subtle pathologic differences among the conditions. Although the biopsy plays a critical role in the diagnostic process of a panniculitis, a series of prerequisites must be met in order to obtain as much information as possible from this procedure. If the biopsy is inadequate, i.e., does not include sufficient subcutaneous fat or the site of sampling site/biopsy timing is wrong, histopathologic assessment is limited and the correct diagnosis may be delayed and further sampling may be required. This article introduces the reader to the field of panniculitides under the histopathologic perspective through a brief description of the normal histology of subcutaneous fat. I also includes the definition of the types of fat necrosis, role of biopsy of panniculitis and its rules and pitfalls, up to a microscopic approach of a slide.

A clinical approach to the vexing problem of diagnosis of panniculitis is traced in this paper, in order to obtain from the clinical findings, history and laboratory data of the patient useful, detailed and precise information, essential to address dermatologists to a specific clinical diagnosis of panniculitis. This approach is created in the same way as when a dermatologist faces any other dermatological disease, be it inflammatory or neoplastic. A common behavior in case of panniculitis is in fact just to take an adequate biopsy and wait for the pathologist report. This is indeed a limitation both for the dermatologist and above all for the pathologist, who is in tremendous need for detailed clinical information before signing his report. The most common types of panniculitides, taking into account their main clinical diagnostic criteria, will be considered. In particular, Erythema Nodosum, Panniculitides in Sarcoidosis, Pancreatic Panniculitis, Lupus Panniculitis, Erythema Induratum/Nodular Vasculitis and Weber-Christian Panniculitis/Rothman-Makai Pannicultis will be analyzed. Every chapter will consider general criteria (epidemiology, age and gender, distribution of the lesions, laboratory findings) and specific findings (characteristics of the lesions, i.e. redness, pain, tenderness, evolution, ulceration, sites of involvement) as well as comorbidities and systemic signs and symptoms. Detailed analysis of the general criteria integrated with the specific findings will allow the clinicians to reach a clinical diagnosis with a high degree of confidence.

Aim of this work was to define the histopathological features of post-surgical panniculitis. Dermal and hypodermal changes will be analyzed in detail, to understand the cascade of events that characterize the tissue response to surgical trauma. Cutaneous re-excision specimens of cases of basal cell carcinoma, squamous cell carcinoma, and melanoma consecutively seen from January 1, 2011 to June 30, 2011 at the Department of Dermatology, University of Pavia, were included in this study. Only the cases in which the first surgical procedure included the subcutaneous fat, were considered. In addition, the time elapsed from the first surgical procedure and the re-excision had to be included in a period of time from one to three months. All the specimens were stained with hematoxylin and eosin. Thirty cutaneous re-excision specimens were studied. Histopathologic examination revealed changes of epidermis, ranging from slight atrophy to moderate hyperplasia. In two cases focal ulceration was seen, with transfollicular elimination of foreign body material. The main dermal changes observed were the: 1) scar with well defined vertical orientation along the dermal suture line; 2) rounded cicatricial areas with radial branching septa of scarring tissue; 3) foreign body granuloma formation; 4) alignment of hystiocytes at the dermo-hypodermal border; 5) traumatic neuromas. The subcutaneous fat changes included: 1) lobular panniculitis with consistent presence of foam cells; 2) striking anisocytosis with pseudocystic degeneration and necrosis of adipocytes; 3) eritrocyte extravasation, mainly at the dermo-hypodermal border; 4) deep seated phlebitis. Post-surgical panniculitis is a lobular foam cell panniculitis characterized by simultaneous dermal and hypodermal changes, expression of the multi-faceted tissue response to a surgical trauma. This type of peculiar lipophagic response puts post-surgical panniculitis into the wider chapter of lipophage tissue response seen in atherosclerosis, glomerulosclerosis and some infectious models such as Mycobacterium tuberculosis and Chlamydia pneumoniae infections. Furthermore it may be seen as a reliable and convenient model for laboratory investigation on foam cell tissue response.

A case of a 30 year-old man with numerous, pink to whitish, 1-2 mm, cystic lesions, located exclusively on the face and helices in symmetrical distribution is reported. Microscopic examination of serial sections of two biopsies disclosed dermal cystic cavities with vellus hair shafts into the lumen. The cyst wall was connected to rudimentary pilar structures. This picture is typical of eruptive vellus hair cysts (EVHC). Facial variant of this dermatosis is reported only in two cases in the literature. Histogenesis and differential diagnosis are discussed.

A 23-year-old man with a keratotic-nodular lesion localized on the neck since infancy is reported. Histologically, there was a funnel-shaped follicle with central horny material and multiple digitations of the follicular sheath epithelium with some features resembling dilated pore of Winer. In addition, there were pale dyskeratotic epithelial cells in the infundibular portion of the tumor and in the surrounding epidermis. For this unusual tumor the term of tricholemmal hamartoma is proposed.

A case of a 6-month-year old child with a yellow, macular and papular, asymptomatic, eruption involving the extremities, upper part of trunk and especially the head is reported. In the early stage histological and immunohistochemical studies were not contributory. Successively, the diagnosis of juvenile xanthogranuloma was made on the basis of the histological, immunohistochemical and ultrastructural findings. Problems of differential diagnosis are discussed.