Oral involvement in autoimmune bullous diseases

The oral mucosa is frequently involved by autoimmune bullous diseases and often this is the first site of manifestation. In this site the lesions are very similar, making the clinical diagnosis difficult; therefore, the definition of the immunohistopathologic characteristics of each one becomes essential for a differential diagnosis. The authors review the clinical-pathological and therapeutic aspect of these oral injuries in order to help in the diagnosis, treatment and prognosis of the oral conditions of those diseases.     

Sensitive skin: a complex syndrome

Epidemiologic studies indicate that ever larger numbers of people report having sensitive skin, for which a European prevalence of 50% is estimated. Sensitive skin is characterized by hyperreactivity, with manifestations varying in relation to many factors. The pathogenesis of this disorder is poorly understood, although studies point to a biophysical mechanism. Objective diagnosis of sensitive skin is difficult, as information comes mainly from the patient's report of symptoms in the absence of effective, strongly predictive tests because of great interindividual variability in skin sensitivity. Substances that trigger a reaction in hypersensitive skin also vary greatly. The impact of this syndrome on quality of life is considerable and patients often present psychiatric symptoms; therefore, dermatologists should explore this possibility when taking a patient's history. Patient cooperation and physician persistence are both essential for treating sensitive skin.     

Photodermatoses during childhood

Actinic dermatitis and phototoxic and idiopathic photodermatoses occur relatively often during childhood. Photodermatoses stemming from enzyme defects and genetic disorders are much less common, while photoallergic and chronic actinic dermatitis have not been dealt with to date. A specific sensitivity of a child's skin to light is often the first manifest clinical symptom of a photodermatosis, the key is to ensure early diagnosis. However, it is often difficult to reach a diagnosis, as the lesions are barely perceptible or have even healed by the time the patient is examined. This is why photoprovocation tests are so important in diagnosing photodermatoses, particularly during childhood. In addition to early diagnosis of these illnesses and offering the appropriate treatment, it is also crucial that the parents be well informed and aware of what symptoms to look for so that they can protect their children from overexposure to UV light and thereby avoid later actinic injuries that can become as serious as malignancies.     

The histopathology of alopecia areata in vertical and horizontal sections

Alopecia areata (AA) is a relatively common disease affecting 1.7% of Americans by the age of 50 years. The diagnosis is usually made on clinical grounds. In some cases the diagnosis is elusive and biopsies are necessary. In other cases biopsies are useful from a prognostic point of view to determine whether there are enough follicles left for possible future regrowth. In view of the active research being conducted into AA, biopsies provide valuable material for further investigation. The diagnosis of AA is improved by the use of horizontal sections in addition to or instead of vertical sections of scalp biopsies. The histopathologic features favoring the diagnosis of AA include peribulbar and intrabulbar mononuclear infiltrates, degenerative changes in the hair matrix, decreased numbers of terminal anagen follicles, increased numbers of terminal catagen and telogen follicles, an increased number of follicular stelae, an increased number of miniaturized vellus hair follicles, and pigment incontinence of hair bulbs and follicular stelae. Follicular counts with horizontal sections are particularly helpful in making the diagnosis of AA when the biopsy has been taken between acute episodes and the characteristic peribulbar inflammatory infiltrate is absent.     

Epidermal multinucleated keratinocytes: a histopathologic clue to dermatitis artefacta

Dermatitis artefacta is a psycho‐cutaneous disorder characterized by self‐inflicted cutaneous injuries, often in association with an underlying psychiatric disorder or as a response to external stressors. Cutaneous lesions suggestive of dermatitis artefacta are dependent on the means of injury and thus may be morphologically variable, but typically have geometric shapes, spare hard‐to‐reach anatomic areas, and are present in variable stages of evolution at any specific time. Although a dermatologist may be suspicious of dermatitis artefacta in a given patient, making a definitive diagnosis is extremely challenging. Patients often clinically evade questioning and deny creating skin lesions, and histopathologic evaluation of lesional biopsies usually reveals non‐specific epidermal and dermal changes and inflammation. Thus, identification of clues that lend support to a diagnosis of dermatitis artefacta would be welcomed by both clinicians and pathologists. Here we present a case of dermatitis artefacta with a unique, yet previously reported, histopathological finding of multinucleated keratinocytes within the epidermis. Although probably uncommon and dependent on the etiology of cutaneous injury, we believe this finding is important for dermatopathologists to be aware of as a potential diagnostic clue when evaluating biopsies in patients suspected to have dermatitis artefacta.     

Delusional infestation: a prototype of psychodermatological disease

Delusional infestation (DI) is a disorder in which patients express a firm, unwavering belief that they are infested with some type of organism but otherwise have a typical grasp on reality with relatively normal cognitive functioning. Although classified as a somatic delusional disorder, DI requires special consideration due to its complicated clinical presentation, requiring attention to several possible explanations for the symptoms the patients describe. The purpose of the current review is to first summarize the clinical background and features of the diagnosis then explore treatment options. DI is a rare disorder though has reported cases dating back to the 19th century and spanning across the globe. Patients often experience the disorder as secondary to a medical condition, including substance use/withdrawal. However, there have also been many reported cases of primary DI, occurring in the absence of any other psychiatric or medical disorder. Clinically, DI is a diagnosis of exclusion, where the physician must rule out other medical conditions, including genuine dermatological disorders or infestations, or contributions from medications or substances. Patients with the disorder more commonly present to nonpsychiatric healthcare providers, making it essential for all clinicians to be able to identify the disease. Treatment can include either first or second generation antipsychotics, but it is important to proceed tactfully in discourse with the patient, being careful to address patients in a straightforward manner without reinforcing or questioning the delusion and focusing conversation on what can be done for the symptoms. Future research can continue to evaluate pathophysiology underlying primary DI, which historically has been an under-studied topic.     

The relationship of psychopathology and hyperhidrosis

BACKGROUND: Hyperhidrosis is a common yet poorly understood disease that is often exacerbated by emotional stress. While a psychiatric explanation of causality is frequently offered, there is little evidence to support or reject the view that the condition is primarily an anxiety-based disorder. OBJECTIVES: To quantify objectively the degree of psychopathology in patients with hyperhidrosis. METHODS: Forty-two patients diagnosed as having hyperhidrosis were examined prior to endoscopic sympathectomies. All patients took the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) and State-Trait Anxiety Inventory (STAI) before surgery. Results were compared with established norms. RESULTS: The group scored well within established norms on both psychometric measures. On scales measuring anxiety, depression and conversion phenomena, 88% of the MMPI-2 profiles lacked elevations, and 86% of the patients lacked elevations on the STAI State and Trait Anxiety scales. Personality variables were not associated with postsurgical outcome. CONCLUSIONS: Most individuals suffering from essential hyperhidrosis lack overt psychopathology. While some patients subjectively describe symptoms of anxiety, mild depression and social isolation, these complaints appear often to be in reaction to or superimposed upon an organic disease process and not the primary cause of their condition.     

Dermatitis artefacta unter dem Bild einer Lichtdermatose

Dermatitis artefacta is a form of a self-injury due to psychiatric disorders or internal conflicts. Delayed diagnosis often leads to unnecessary treatments. A 17-year old girl was referred with a putative photodermatosis presenting with erosions on an erythematous base on the face and forearms. The unusual rapid onset of new lesions following phototesting and in particular a reaction induced after a simulated light exposure substantiated the diagnosis of dermatitis artefacta. Faced with the diagnosed the patient admitted she had induced the lesions and was referred for psychiatric care.     

Picking apart the picker: a clinician's guide for management of the patient presenting with excoriations

Management of patients who "pick" at their skin is often difficult. Etiologies and maintaining factors can be unclear. Significant psychiatric overlay is often present, and the clinician is left with an overwhelming differential diagnosis and a poorly focused treatment plan that can result in suboptimal clinical outcomes. The purpose of this paper is to provide a conceptual framework for the evaluation and treatment of patients presenting with excoriations. Ten diagnostic categories are examined, and specific suggestions for treatment are offered.     

Chronic Pruritus in the Absence of Skin Disease: Pathophysiology,Diagnosis and Treatment

Chronic pruritus arises not only from dermatoses, but also, in up to half of cases, from extracutaneous origins. A multitude of systemic, neurological, psychiatric, and somatoform conditions are associated with pruritus in the absence of skin disease. Moreover, pruritus is a frequently observed side effect of many drugs. It is therefore difficult for physicians to make a correct diagnosis. Chronic pruritus patients frequently present to the dermatologist with skin lesions secondary to a long-lasting scratching behavior, such as lichenification and prurigo nodularis. A structured clinical history and physical examination are essential in order to evaluate the pruritus, along with systematic, medical history-adapted laboratory and radiological tests carried out according to the differential diagnosis. For therapeutic reasons, a symptomatic therapy should be promptly initiated parallel to the diagnostic procedures. Once the underlying factor(s) leading to the pruritus are identified, a targeted therapy should be implemented. Importantly, the treatment of accompanying disorders such as sleep disturbances or mental symptoms should be taken into consideration. Even after successful treatment of the underlying cause, pruritus may persist, likely due to chronicity processes including peripheral and central sensitization or impaired inhibition at spinal level. A vast arsenal of topical and systemic agents targeting these pathophysiological mechanisms has been used to deter further chronicity. The therapeutic options currently available are, however, still insufficient for many patients. Thus, future studies aiming to unveil the complex mechanisms underlying chronic pruritus and develop new therapeutic agents are urgently needed.     

Dermatitis artefacta in a patient affected by impulse control disorder: case report

Dermatitis artefacta is a disease characterized by self-inflicted skin lesions in fully aware patients. Mechanical and chemical devices are most commonly used to produce such injuries. Several psychological disorders like depression, obsessive compulsive disorders, hysteria, etc. are associated with this kind of disease. Most of the patients are young females aged between 15 and 30, but the diagnosis of dermatitis artefacta may even be made in pediatric patients or elderly people. Because of its rarity and the polymorphism of lesions, dermatitis artefacta is often a challenge for the clinicians. More difficulties might be due to the lack of cooperation in these patients, who usually refuse the dialogue with doctors and deny their primary role in damaging their skin. We present a case of an elderly woman who showed a peculiar pattern of deep excoriating lesions disseminated on the upper part of her body, with an evident state of depression. Diagnostic and therapeutic procedure, that is often long lasting and difficult in such cases, was made by teamwork of dermatologists, psychiatrists and psychologists, leading to steady control of impulses and full remission of cutaneous symptoms.     

Onychotillomania: clinicopathologic correlations

Onychotillomania represents an unusual dermatosis affecting the nail unit. The term refers to ‘neurotic picking at a nail until it is permanently altered’. It can be difficult for dermatologists to diagnose, as typically patients will deny the self‐destructive behavior and the clinical features may mimic other inflammatory conditions affecting the nail unit. Aside from the difficulty in establishing the diagnosis clinically, the histopathologic features of onychotillomania are nonspecific. With this limited knowledge, dermatopathologists could have difficulty in correlating the clinical and histopathologic features and determining the correct diagnosis. Establishing the diagnosis is critical, as onychotillomania is sometimes associated with major depression and obsessive‐compulsive disorder, and can be a clue to uncovering these debilitating psychiatric disorders. Here we present two cases of onychotillomania and emphasize the clinical‐pathological confrontation necessary to secure the diagnosis.     

Ulcers in congenital arteriovenous fistula of the lower extremities

Ulcers of the lower legs caused by arteriovenous fistulae show, on one hand, that diagnoses are often made carelessly and, on the other, that the treatment initiated is often inadequate. In both cases reported, there were several small arteriovenous fistulae in the lower legs, although no symptoms were observed for a long time. The ulcers, which then recurred chronically, resulted from very minor injuries. Their characteristics remained unnoticed for many years. Surgical intervention to remove these fistulae is not always simple because they cannot all be removed at once. Lower leg ulcers caused by arteriovenous shunts are an example of how difficult it can be to prove the cause of such lower leg ulcers.     

Cutaneous manifestations of intestinal helminthic infections

Several intestinal helminths may involve the skin. In cutaneous larva migrans and enterobiasis this is often the only organ involved, whereas hookworm and trichinosis are characterized by multisystem disease. The clinical spectrum of strongyloidiasis may range from a low-grade chronic dermatitis to a fulminant, often fatal, hyperinfection syndrome in the immunocompromised host. In the absence of recent breakthroughs in diagnostic testing, a meticulous history and knowledge of the life cycles and the often characteristic skin manifestations of these parasites remain essential to prompt diagnosis and effective treatment.     

Autoerythrocyte sensitization syndrome with vertigo and hemilateral auditory impairment

Autoerythrocyte sensitization syndrome (AES) is characterized by recurrent, painful purpura or ecchymosis. Testing for the reappearance of lesions after injection of the patient's own erythrocytes is usually useful for the diagnosis of AES, but the significance of this test is still controversial. As the lesions often appear in patients with psychiatric disorders, mental factors such as depression and stress are considered to be involved in the occurrence and exacerbation of AES. We report a 28-year-old woman who presented recurrent episodes of painful purpura with vertigo and hemilateral auditory impairment after difficulties at her workplace. After the diagnosis of AES, she was referred for psychiatric counselling, after which the symptoms disappeared. These findings suggest that treatment for psychological disorders is important in patients with AES.     

Toxic epidermal necrolysis and Stevens‐Johnson syndrome at the Prague Burn Centre 1998–2008

Background Toxic epidermal necrolysis (TEN) and Stevens‐Johnson syndrome (SJS) are skin disorders characterized by extensive necrosis of the mucous membranes and the epidermis caused by an autoimmune response. Objectives  To present experience of treating TEN and SJS at the Prague Burn Centre (PBC) 1998–2008. Methods  22 patients with the diagnosis TEN or SJS were enrolled. We collected data including the extent of the skin involvement, the ratio of men : women, the average age, the mortality rate, presence of comorbidities, the length of hospital stay, the period from the first symptoms to the admission to the PBC, corticosteroids administration prior to admission. Results  82% patients had injuries larger than 30% of body surface area. The ratio of men : women was 1 : 1.5; the average age was 48.4 years; the mortality was 32%. Significant comorbidities were present in 41% of the cases. The average length of hospital stay was 12.9 days. The period from the first symptoms to the admission to the PBC was 9.6 days; corticosteroids were administered to 68% of the cases. Conclusion  Treatment strategies at the PBC are based upon a multidisciplinary approach and focus on the general principles of treating patients with extensive skin loss. Corticosteroids must be avoided, antibiotics are not indicated and intravenous immunoglobulins are not justified in the standard therapy. Early diagnosis, prompt transport to a burn center are essential for patients outcomes. The medical community should be informed about these rare but potentially life threatening diseases.     

Pressure injuries during the SARS-CoV-2 pandemic: A retrospective,case-control study

Aim of studyThe main objective of this study was to ascertain whether severe alterations in hypoxemic, inflammatory, and nutritional parameters in patients diagnosed with SARS-CoV-2 infection were associated with the occurrence and severity of developed dependency-related injuries. The secondary objective was to determine whether there were prognostic factors associated with the occurrence and severity of developed dependency-related injuries during the SARS-CoV-2 pandemic.Material and methodsA retrospective, single-centre, case-control study was conducted to compare SARS-CoV-2 patients who developed dependency-related injuries after the first 48 h after admission with a control group made up of SARS-CoV-2 patients without dependency-related injuries. The cases of the 1987 patients diagnosed with SARS-CoV-2 infection during the study period were reviewed. Data from 94 patients who developed dependency-related injuries and from 190 patients who did not develop them during hospital admission were analysed.ResultsHigh baseline dependency levels, prolonged hospital stays, and low oxygen saturation levels on arrival in emergency department triage were associated with the occurrence of dependency-related injuries among patients diagnosed with SARS-CoV-2 infection.ConclusionsSARS-CoV-2 infection can lead to complications such as dependency-related injuries. Although there are several non-modifiable variables associated with the occurrence of dependency-related injuries in these patients, it is essential to conduct further research and introduce consensus guidelines to reduce their incidence and prevalence.     

Atypical herpetic manifestations

Herpetic lesions may be readily identified by modern virological techniques. In immunocompromised patients, the herpetic lesions are often atypical and even completely different from conventional forms; in these patients, the course of herpes virus infections may be life-threatening. Therefore, early diagnosis of herpetic infections is essential in order to begin with acyclovir treatment in time.     

Cutaneous lesions of malignant histiocytosis. About five cases (author's transl)]

Five cases of malignant histiocytosis are reported. They associate specific cutaneous lesions with the other much more classical symptoms of the illness; fever, weight loss, lymphadenopathy, splenomegaly, hepatomegaly. The principal clinical and histological findings observed during the course of the illness in these patients are described. In a review of the literature concerning this rare but now well individualized illness, 34 cases of malignant histiocytosis associated with cutaneous lesions are found among which 24 were studied on a clinical and histological point of view. The principal characteristics of the cutaneous eruption are presented: clinical characteristics with the most frequent nodular aspect of the lesions, but also histological features with the relative specificity of the disposition of the infiltrate whose cytological features will not be forgotten to analize. Nevertheless, the diagnostic will often be established by the histological analysis of the lymph node biopsy or sometimes of biopsy of the other affected organs. These cutaneous lesions might also be in several cases of some prognostic interest.     

Diffuse poikiloderma with acrokeratosis, preceded by bullous lesions: Weary-Kindler's disease. Nosologic and histopathologic discussion

The authors report the case of a six and a half year old girl, with extensive poikilodermatous condition and acrokeratosis, preceded by early blistering and light sensitivity. The sequence of cutaneous symptoms suggests the diagnosis of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler; forty-two cases have already been reported in the literature and our patient would better fit into Kindler's type. The original findings under electron microscopy and direct immunofluorescence (colloid bodies, IgM deposition) that are reported herein are discussed from a pathogenic point of view.