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There are two main types of pyoderma gangrenosum:
- the 'typical' ulcerative form, which occurs in the legs
- an 'atypical' form that is more superficial and occurs in the hands and other parts of the body
Other variations are:
- Peristomal pyoderma gangrenosum comprises 15% of all cases of pyoderma
- Bullous pyoderma gangrenosum
- Pustular pyoderma gangrenosum
- pyoderma gangrenosum
Pyoderma gangrenosum is a condition that causes tissue to become necrotic, causing deep ulcers that usually occur on the legs. When they occur, they can lead to chronic wounds. Ulcers usually initially look like small bug bites or papules, and they progress to larger ulcers. Though the wounds rarely lead to death, they can cause pain and scarring.
The disease was identified in 1930. It affects approximately 1 person in 100,000 in the population. Though it can affect people of any age, it mostly affects people in their 40s and 50s.
PAPA syndrome usually begins with arthritis at a young age, with the skin changes more prominent from the time of puberty.
The arthritis is the predominant feature, noted by its juvenile onset and destructive course. Individuals often recall episodes of arthritis precipitated by a traumatic event. With repeated episodes the joints become damaged with multiple joint replacements required. Hopefully, with improved treatment options, the damage will be limited in new cases.
Pyoderma gangrenosum is variably expressed, which means that it is not always present in all individuals with the disease. It presents as poorly healing ulcers with undermined edges. Pathergy is an important feature (this term refers to the tendency of ulcers to arise at points of injury). There are reports of lesions developing at the site of a joint replacement wound, central venous line and intravenous drip insertion.
Acne affects most individuals with PAPA syndrome but to a variable degree. It is usually of a severe nodulocystic type which if untreated results in scarring.
Blastomycosis-like pyoderma (also known as "Pyoderma vegetans") is a cutaneous condition characterized by large verrucous plaques with elevated borders and multiple pustules.
Pyoderma means any skin disease that is pyogenic (has pus). These include superficial bacterial infections such as impetigo, impetigo contagiosa, ecthyma, folliculitis, Bockhart's impetigo, furuncle, carbuncle, tropical ulcer, etc. Autoimmune conditions include pyoderma gangrenosum. Pyoderma affects more than 111 million children worldwide, making it one of the three most common skin disorders in children along with scabies and tinea.
The primary skin lesion usually starts with a macule that is painless, round and erythematous. Then, it develops into a pustule, and then a bulla with central hemorrhagic focus. The bullae progresses into an ulcer which extends laterally. Finally it becomes a gangrenous ulcer with central black eschar surrounded by erythematous halo.
The lesion may be single or multiple. They are most commonly seen in perineum and under arm pit. However, it can occur in any part of the body.
Acute, tender, erythematous plaques, nodes, pseudovesicles and, occasionally, blisters with an annular or arciform pattern occur on the head, neck, legs, and arms, particularly the back of the hands and fingers. The trunk is rarely involved. Fever (50%); arthralgia or arthritis (62%); eye involvement, most frequently conjunctivitis or iridocyclitis (38%); and oral aphthae (13%) are associated features.
PAPA syndrome is an acronym for pyogenic arthritis, pyoderma gangrenosum and acne. It is a rare genetic disorder characterised by its effects on skin and joints.
Ecthyma gangrenosum is a type of skin lesion characterized by vesicles or blisters which rapidly evolve into pustules and necrotic ulcers with undermined tender erythematous border. "Ecthyma" means a pus forming infection of the skin with an ulcer, "gangrenosum" means the gangrene or necrosis. It is the pathognomonic of "Pseudomonas aeruginosa" bacteremia. "Pseudomonas aeruginosa" is a gram negative, aerobic, coccobacillus bacterium.
This type of skin lesion was first described in association with "Pseudomonas aeruginosa" by L. Barker in 1897. It was given the name "ecthyma gangrenosum" by Hitschmann and Kreibich.
It mostly occurs in patients with underlying immunocompromised conditions (e.g. Malignancy). Although most cases are found in "Pseudomonas aeruginosa" infection, there are recent reports of this skin lesion associated with other microorganisms, such as "Escherichia coli, Citrobacter freundii, Klebsiella pneumonia", various other Pseudomonas species, and "Morganella morganii."
SS can be classified based upon the clinical setting in which it occurs: classical or idiopathic SS, malignancy-associated SS, and drug-induced SS.
The first symptom is a sudden onset of swelling of the face, which develops within two days into papules and pustues on the lips, nose, and around the eyes. These pustules release a purulent discharge, causing a crust to form on the skin. There is also lymphadenopathy (swelling of lymph nodes) in the main lymph nodes of the head. The feet, body, prepuce or perianal area may be affected. Pustular otitis externa can occur, with the pinnae (ear flaps) becoming thickened by edema. Affected areas are painful but not itchy. Approximately half of affected puppies have lethargy and depression. Less common symptoms include pyrexia, anorexia, and joint pain, caused by sterile suppurative arthritis.
Progressive vaccinia (also known as "Vaccinia gangrenosum," and "Vaccinia necrosum") is a rare cutaneous condition caused by the vaccinia virus, characterized by painless, but progressive, necrosis and ulceration.
Grover's disease often starts quite suddenly. There are intensely itchy spots on the central back, mid chest and occasionally elsewhere. Frequently, it follows sweating or some unexpected heat stress.
The itchy eruption lasts an average of 10–12 months. It is characterized by papules and papulovesicles with excoriations occurring on the chest, back, lower sternum, arms, and thighs. The papules are most commonly found on the mid chest.
Sometimes the features of Grover's are found in people who do not itch or have a conspicuous rash. Most of the people with Grover's who visit a dermatologist, however, itch a lot.
Pseudomonal pyoderma is a cutaneous condition, a superficial infection of the skin with "P. aeruginosa".
Nearly all people with Behçet's disease present with some form of painful ulcerations inside the mouth. They are a form of aphthous ulcers or non-scarring oral lesions. The oral lesions are similar to those found in inflammatory bowel disease and can be relapsing. Painful genital ulcerations usually develop around the anus, vulva, or scrotum and cause scarring in 75 percent of the patients. Additionally, patients may present with erythema nodosum, cutaneous pustular vasculitis, and lesions similar to pyoderma gangrenosum.
Four rosacea subtypes exist, and a patient may have more than one subtype:
1. Erythematotelangiectatic rosacea exhibits permanent redness (erythema) with a tendency to flush and blush easily. It is also common to have small, widened blood vessels visible near the surface of the skin (telangiectasias) and possibly intense burning, stinging, or itching. People with this type often have sensitive skin. Skin can also become very dry and flaky. In addition to the face, signs can also appear on the ears, neck, chest, upper back, and scalp.
2. Papulopustular rosacea presents with some permanent redness with red bumps (papules); some pus-filled pustules can last 1–4 days or longer. This subtype is often confused with acne.
3. Phymatous rosacea is most commonly associated with rhinophyma, an enlargement of the nose. Signs include thickening skin, irregular surface nodularities, and enlargement. Phymatous rosacea can also affect the chin (gnathophyma), forehead (metophyma), cheeks, eyelids (blepharophyma), and ears (otophyma). Telangiectasias may be present.
4. In ocular rosacea, affected eyes and eyelids may appear red due to telangiectasias and inflammation, and may feel dry, irritated, or gritty. Other symptoms include foreign body sensations, itching, burning, stinging, and sensitivity to light. Eyes can become more susceptible to infection. About half of the people with subtypes 1–3 also have eye symptoms. Blurry vision and vision loss can occur if the cornea is affected.
Signs include facial redness, small and superficial dilated blood vessels on facial skin, papules, pustules, and swelling.
Grover's disease (GD) is a polymorphic, pruritic, papulovesicular dermatosis characterized histologically by acantholysis with or without dyskeratosis. Once confirmed, most cases of Grover's disease last six to twelve months, which is why it was originally called "transient". However it may last much longer. Nevertheless, it is not to be confused with relapsing linear acantholytic dermatosis.
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.
Intertrigo refers to a type of inflammatory rash (dermatitis) of the superficial skin that occurs within a person's body folds. These areas are more susceptible to irritation and subsequent infection due to factors that promote skin breakdown such as moisture, friction, and exposure to bodily secretions such as sweat, urine or feces. Areas of the body which are more likely to be affected by intertrigo include the inframammary fold, intergluteal cleft, armpits, and spaces between the fingers or toes. Skin affected by intertrigo is more prone to infection than intact skin.
The term "intertrigo" commonly refers to a secondary infection with bacteria (such as "Corynebacterium minutissimum"), fungi (such as "Candida albicans"), or viruses. A frequent manifestation is candidal intertrigo.
Intertrigo occurs more often in warm and humid conditions. Generally, intertrigo is more common in people with a weakened immune system including children, the elderly, and immunocompromised people. The condition is also more common in people who experience urinary incontinence and decreased ability to move.
Juvenile cellulitis, also known as puppy strangles or juvenile pyoderma, is an uncommon disease of dogs. Symptoms include dermatitis, lethargy, depression and lameness. When puppies are first presented with what appears to be staphylococcal pyoderma, juvenile cellulitis, a relatively rare condition, may not be considered.
Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20 percent of cases. Ocular involvement can be in the form of posterior uveitis, anterior uveitis, or retinal vasculitis. Anterior uveitis presents with painful eyes, conjuctival redness, hypopyon, and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters. A rare form of ocular (eye) involvement in this syndrome is retinal vasculitis which presents with painless decrease of vision with the possibility of floaters or visual field defects.
Optic nerve involvement in Behçet's disease is rare, typically presenting as progressive optic atrophy and visual loss. However, cases of acute optic neuropathy (specifically anterior ischemic optic neuropathy) have also been reported to occur. Optic nerve atrophy has been identified as the most common cause of visual impairment. Behçet's disease may result in primary or secondary optic nerve involvement. Papilledema as a result of dural sinus thrombosis and atrophy resulting from retinal disease, have been characterized as secondary causes of optic nerve atrophy in Behçet's disease.
Signs and symptoms of acute optic neuropathy include painless loss of vision which may affect either one or both eyes, reduced visual acuity, reduced color vision, relative afferent pupillary defect, central scotoma, swollen optic disc, macular edema, or retrobulbar pain. When these symptoms occur with concurrent mucocutaneous ulcerations, they raise suspicion of acute optic neuropathy in Behçet's Disease. Progressive optic atrophy may result in decreased visual acuity or color vision. Intracranial hypertension with papilledema may be present.
Intertrigo can be diagnosed clinically by a medical professional after taking a thorough history and performing a detailed physical examination. Many other skin conditions can mimic intertrigo's appearance including erythrasmascabies, pyoderma, atopic dermatitis, candidiasis, and seborrheic dermatitis, and fungal infections of the superficial skin caused by "Tinea versicolor" or "Tinea corporis".
Signs and symptoms due to the cryoglobulins of type I disease reflect the hyperviscosity and deposition of cryoglobulins within the blood vessels which reduce or stop blood perfusion to tissues. These events occur particularly in cases where blood cryoglobulin levels of monoclonal IgM are high in patients with IgM MGUS, smoldering Waldenström's macroglobulinemia, or Waldenström's macroglobulinemia and in uncommon cases where the levels of monoclonal IgA, IgG, free κ light chains, or free λ light chains are extremely high in patients with non-IgM MGUS, non-IgM smoldering multiple myeloma, or multiple myeloma. The interruption of blood flow to neurological tissues can cause symptoms of confusion, headache, hearing loss, and peripheral neuropathy. Interruption of blood flow to other tissues in type I disease can cause cutaneous manifestations of purpura, acrocyanosis, necrosis, ulcers, and livedo reticularis; spontaneous nose bleeds, joint pain, membranoproliferative glomerulonephritis; and cardiovascular disturbances such as shortness of breath, hypoxemia, and congestive heart failure.
The signs and symptoms in the increasingly rare cases of cryoglobulinemic disease that cannot be attributed to an underlying disease generally resemble those of patients suffering Type II and III (i.e. mixed) cryoglobulinemic disease.