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Pemphigus is a group of autoimmune blistering diseases that may be classified into the following types:
Morbidity and mortality range from both extremes as the significance correlate with the underlying systemic disease.
There seems to be beneficial responses to clindamycin therapy as the lesions regress. This leads to the hypothesis that microorganisms may be playing a role in the initial stages of Kyrle disease.
A family with Kyrle disease were examined which their skin lesions were benign. However, when three of the young adult members were closely examined, they had posterior subcapsular cataracts and two of those three developed multiple tiny yellow-brown anterior stromal corneal opacities. In order to determine if there is any correlation between Kyrle disease and the ocular observations, more cases of Kyrle disease are to be analyzed.
All in all, since Kyrle disease is relatively rare, more cases need to be studied and analyzed in order to understand the underlying pathogenesis and to improve the management of the disease.
Pemphigus defines a group of autoimmune interepithelial blistering diseases that are characterized by loss of normal cell-cell adhesion (acantholysis), and by the presence of pathogenic (predominantly IgG) autoantibodies reacting against epithelial adhesion molecules. Pemphigus is further divided in two major subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). However, several other disorders such as IgA pemphigus, IgE pemphigus, pemphigus herpetiformis, drug induced pemphigus, Senear Usher syndrome and endemic pemphigus foliaceus exist;recognized by a dermatologist from the appearance and distribution of the skin lesions. It is also commonly diagnosed by specialists practicing otolaryngology- head and neck surgery, periodontists, oral and maxillofacial surgeons and eye doctors, as lesions can affect the eyes and mucous membrane of the oral cavity. Intraorally it resembles the more common diseases lichen planus and mucous membrane pemphigoid. Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a dermatopathologist or oral pathologist. The skin biopsy is taken from the edge of a blister, prepared for histopathology and examined with a microscope. The pathologist looks for an intraepidermal vesicle caused by the breaking apart of epidermal cells (acantholysis). Thus, the superficial (upper) portion of the epidermis sloughs off, leaving the bottom layer of cells on the "floor" of the blister. This bottom layer of cells is said to have a "tombstone appearance".
Definitive diagnosis also requires the demonstration of anti-desmoglein autoantibodies by direct immunofluorescence on the skin biopsy. These antibodies appear as IgG deposits along the desmosomes between epidermal cells, a pattern reminiscent of chicken wire. Anti-desmoglein antibodies can also be detected in a blood sample using the ELISA technique.
Cutaneous perforating disorders include the following:
- Acquired perforating dermatosis (Acquired perforating collagenosis)
- Kyrle disease
- Perforating folliculitis
Acquired perforating dermatosis (also known as "Acquired perforating collagenosis") is clinically and histopathologically similar to perforating folliculitis, also associated with chronic kidney failure, with or without hemodialysis or peritoneal dialysis, and/or diabetes mellitus, but not identical to Kyrle disease.
Verrucous perforating collagenoma is a very rare skin disorder which presents with papules with a transepidermal elimination of collagen.
Studies show a moderate neutrophilia (less than 50%), elevated ESR (greater than 30 mm/h) (90%), and a slight increase in alkaline phosphatase (83%). Skin biopsy shows a papillary and mid-dermal mixed infiltrate of polymorphonuclear leukocytes with nuclear fragmentation and histiocytic cells. The infiltrate is predominantly perivascular with endothelial-cell swelling in some vessels, but vasculitic changes (blood clots; deposition of fibrin, complement, or immunoglobulins within the vessel walls; red blood cell extravasation;inflammatory infiltration of vascular walls) are absent in early lesions.
Perivasculitis occurs secondarily, because of cytokines released by the lesional neutrophils. True transmural vasculitis is not an expected finding histopathologically in SS.
There is no cure for this condition. Treatment is generally lifelong and takes the form of bathing and soaking in mineral oils and washing with antibiotic shampoos to try to alleviate symptoms and slow the condition's progression. Antiseptic and antibiotic shampoos (chlorhexidine or benzoyl peroxide) are used to manage further secondary bacterial infection. For some breeds, cyclosporine or corticosteroids and immunosuppressant drugs may be effective, and it is postulated, through some studies, that large doses of vitamin A given orally may result in some improvement.
It has been suggested that the more aggressively one applies the topical methods of treatment, the less aggressively one needs to employ the immunosuppressant therapy. The suggestion is that this phenomenon may be due to a feedback whereby secondary infection, when not aggressively treated with topical therapy, increases and contributes to further sebaceous gland inflammation.
In general, sebaceous adenitis is underdiagnosed in dogs. Diagnosis confirmation requires multiple punch biopsies analysed by a dermopathologist who will comment on the condition of the sebaceous glands, revealing granulomatous or pyogranulomatous inflammation surrounding the sebaceous glands or even complete destruction of sebaceous glands.
Other conditions with similar presentations include: bacterial folliculitis and demodicosis, dermatophytosis, endocrinopathy, pemphigus foliaceus, zinc responsive dermatosis, vitamin A-responsive dermatosis, ichthyosis, and nutritional deficiencies. As well as, superficial pyoderma, primary idiopathic seborrhea and other endocrine diseases.
Visual diagnosis is made by the "stuck on" appearance, horny pearls or cysts embedded in the structure. Darkly pigmented lesions can be challenging to distinguish from nodular melanomas. Furthermore, thin seborrheic keratoses on facial skin can be very difficult to differentiate from lentigo maligna even with dermatoscopy. Clinically, epidermal nevi are similar to seborrheic keratoses in appearance. Epidermal nevi are usually present at or near birth. Condylomas and warts can clinically resemble seborrheic keratoses, and dermatoscopy can be helpful. On the penis and genital skin, condylomas and seborrheic keratoses can be difficult to differentiate, even on biopsy.
To date, the gold standard in the diagnosis of seborrheic keratosis is represented by the histolopathologic analysis of a skin biopsy.
The clinical differential diagnosis includes pyoderma gangrenosum, infection, erythema multiforme, adverse drug reactions, and urticaria. Recurrences are common and affect up to one third of patients.
DPN is not a premalignant condition nor is it associated with any underlying systemic disease. DPN lesions show no tendency to regress spontaneously, and often increase in size and number as an individual ages.
Reactive perforating collagenosis is a rare, familial, nonpuritic skin disorder characterized by papules that grow in a diameter of 4 to 6mm and develop a central area of umbilication to which keratinous material is lodged. The cause of reactive perforating collagenosis is unknown.
CRMO/CNO is a diagnosis of exclusion. This means that other diseases must be ruled out before the diagnosis can be made. Generally, many tests are required, such as blood tests, x-rays, bone scans, MRI and often a bone biopsy.
Perforating granuloma annulare is a skin condition of unknown cause, usually appearing on the dorsal hands, presenting as papules with a central keratotic core.
Grover's may be suspected by its appearance, but since it has such a characteristic appearance under the microscope a shave skin or punch biopsy is often performed.
Perforating calcific elastosis (also known as "Localized acquired cutaneous pseudoxanthoma elasticum," "Perforating periumbilical calcific elastosis," and "Periumbilical perforating pseudoxanthoma elasticum") is an acquired, localized cutaneous disorder, most frequently found in obese, multiparous, middle-aged women, characterized by lax, well-circumscribed, reticulated or cobble-stoned plaques occurring in the periumbilical region with keratotic surface papules.
Sweet's syndrome-like dermatosis is a cutaneous condition associated with bowel disorders.
Pustulosis palmaris et plantaris (also known as pustulosis of palms and soles, palmoplantar pustulosis, persistent palmoplantar pustulosis, pustular psoriasis of the Barber type, and pustular psoriasis of the extremities) is a chronic recurrent pustular dermatosis (that is, a pustulosis or pustular psoriasis) localized on the palms and soles only, characterized histologically by intraepidermal pustules filled with neutrophils. It can occur as part of the SAPHO syndrome.
History and examination by a physician with characteristic signs and symptoms are sufficient in many cases in ruling out systemic causes of venous hypertension such as hypervolemia and heart failure. An ultrasound (usually a lower limbs venous ultrasonography) can detect venous obstruction or valvular incompetence as the cause, and is used for planning venous ablation procedures, but it is not necessary in suspected venous insufficiency where surgical intervention is not indicated.
Many conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment. The skin weighs an average of four kilograms, covers an area of two square meters, and is made of three distinct layers: the epidermis, dermis, and subcutaneous tissue. The two main types of human skin are: glabrous skin, the hairless skin on the palms and soles (also referred to as the "palmoplantar" surfaces), and hair-bearing skin. Within the latter type, the hairs occur in structures called pilosebaceous units, each with hair follicle, sebaceous gland, and associated arrector pili muscle. In the embryo, the epidermis, hair, and glands form from the ectoderm, which is chemically influenced by the underlying mesoderm that forms the dermis and subcutaneous tissues.
The epidermis is the most superficial layer of skin, a squamous epithelium with several strata: the stratum corneum, stratum lucidum, stratum granulosum, stratum spinosum, and stratum basale. Nourishment is provided to these layers by diffusion from the dermis, since the epidermis is without direct blood supply. The epidermis contains four cell types: keratinocytes, melanocytes, Langerhans cells, and Merkel cells. Of these, keratinocytes are the major component, constituting roughly 95 percent of the epidermis. This stratified squamous epithelium is maintained by cell division within the stratum basale, in which differentiating cells slowly displace outwards through the stratum spinosum to the stratum corneum, where cells are continually shed from the surface. In normal skin, the rate of production equals the rate of loss; about two weeks are needed for a cell to migrate from the basal cell layer to the top of the granular cell layer, and an additional two weeks to cross the stratum corneum.
The dermis is the layer of skin between the epidermis and subcutaneous tissue, and comprises two sections, the papillary dermis and the reticular dermis. The superficial papillary dermis with the overlying rete ridges of the epidermis, between which the two layers interact through the basement membrane zone. Structural components of the dermis are collagen, elastic fibers, and ground substance. Within these components are the pilosebaceous units, arrector pili muscles, and the eccrine and apocrine glands. The dermis contains two vascular networks that run parallel to the skin surface—one superficial and one deep plexus—which are connected by vertical communicating vessels. The function of blood vessels within the dermis is fourfold: to supply nutrition, to regulate temperature, to modulate inflammation, and to participate in wound healing.
The subcutaneous tissue is a layer of fat between the dermis and underlying fascia. This tissue may be further divided into two components, the actual fatty layer, or panniculus adiposus, and a deeper vestigial layer of muscle, the panniculus carnosus. The main cellular component of this tissue is the adipocyte, or fat cell. The structure of this tissue is composed of septal (i.e. linear strands) and lobular compartments, which differ in microscopic appearance. Functionally, the subcutaneous fat insulates the body, absorbs trauma, and serves as a reserve energy source.
Conditions of the human integumentary system constitute a broad spectrum of diseases, also known as dermatoses, as well as many nonpathologic states (like, in certain circumstances, melanonychia and racquet nails). While only a small number of skin diseases account for most visits to the physician, thousands of skin conditions have been described. Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. Therefore, most current textbooks present a classification based on location (for example, conditions of the mucous membrane), morphology (chronic blistering conditions), etiology (skin conditions resulting from physical factors), and so on. Clinically, the diagnosis of any particular skin condition is made by gathering pertinent information regarding the presenting skin lesion(s), including the location (such as arms, head, legs), symptoms (pruritus, pain), duration (acute or chronic), arrangement (solitary, generalized, annular, linear), morphology (macules, papules, vesicles), and color (red, blue, brown, black, white, yellow). Diagnosis of many conditions often also requires a skin biopsy which yields histologic information that can be correlated with the clinical presentation and any laboratory data.
Pigmented purpuric dermatosis (also known as "progressive pigmentary dermatosis," "purpura pigmentosa chronica," "pigmentary purpuric eruptions," or "progressive pigmenting purpura" or "Schamberg's disease") refers to one of the three major classes of skin conditions characterized by purpuric skin eruptions.
Pigmented purpuric dermatosis are distinguished from other purpura by size (0.3–1 cm) and are most often seen in the lower extremities. Pigmentary purpuric eruptions may present with one of several clinical patterns. There may be overlapping characteristics among pigmented purpuric dermatosis and between their signs and those of other purpuric eruptions. Examples of the pigmented purpuric dermatosis group include:
Although vascular damage may be present, it is insufficient for these conditions to be considered forms of vasculitis.
No treatment of seborrheic keratoses is necessary, except for aesthetic reasons. Since a slightly increased risk of localized infection caused by picking at the lesion has been described, if a lesion becomes itchy or irritated by clothing or jewelry, a surgical excision is generally recommended.
Small lesions can be treated with light electrocautery. Larger lesions can be treated with electrodesiccation and curettage, shave excision, or cryosurgery. When correctly performed, removal of seborrheic keratoses will not cause much visible scarring except in persons with dark skin tones.
DPN lesions are benign and no treatment generally is indicated unless lesions are cosmetically undesirable. Surgical options including curettage, cryotherapy and laser therapy are options Scarring, postoperative skin discoloration or keloid formation are potential complication. Therefore, conservative dpn treatment is advisable.