Ecthyma gangrenosum is a type of skin lesion characterized by vesicles or

blister A blister is a small pocket of body fluid ( lymph, serum, plasma, blood, or pus) within the upper layers of the skin, usually caused by forceful rubbing ( friction), burning, freezing, chemical exposure or infection. Most blisters are filled ...

s which rapidly evolve into

pustules A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of thi ...

and

necrotic Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dige ...

ulcer An ulcer is a discontinuity or break in a bodily membrane that impedes normal function of the affected organ. According to Robbins's pathology, "ulcer is the breach of the continuity of skin, epithelium or mucous membrane caused by sloughing o ...

s with undermined tender

erythema Erythema (from the Greek , meaning red) is redness of the skin or mucous membranes, caused by hyperemia (increased blood flow) in superficial capillaries. It occurs with any skin injury, infection, or inflammation. Examples of erythema not asso ...

tous border. "

Ecthyma Ecthyma( ec·thy·ma , \ ek-ˈthī-mə )is a variation of impetigo Impetigo is a bacterial infection that involves the superficial skin. The most common presentation is yellowish crusts on the face, arms, or legs. Less commonly there may ...

" means a pus forming infection of the skin with an ulcer, "gangrenosum" refers to the accompanying gangrene or necrosis. It is classically associated with ''

Pseudomonas aeruginosa ''Pseudomonas aeruginosa'' is a common encapsulated, gram-negative, aerobic– facultatively anaerobic, rod-shaped bacterium that can cause disease in plants and animals, including humans. A species of considerable medical importance, ''P. a ...

'' bacteremia, but it is not

pathognomonic Pathognomonic (rare synonym ''pathognomic'') is a term, often used in medicine, that means "characteristic for a particular disease". A pathognomonic sign is a particular sign whose presence means that a particular disease is present beyond any doub ...

. ''Pseudomonas aeruginosa'' is a gram negative, aerobic bacillus. 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 immunocompromise (e.g.

malignancy Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

HIV The human immunodeficiency viruses (HIV) are two species of ''Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the immune ...

). Although most cases are due to ''Pseudomonas aeruginosa'' infection, there are recent reports of this skin lesion in association with other microorganisms, such as ''

Escherichia coli ''Escherichia coli'' (),Wells, J. C. (2000) Longman Pronunciation Dictionary. Harlow ngland Pearson Education Ltd. also known as ''E. coli'' (), is a Gram-negative, facultative anaerobic, rod-shaped, coliform bacterium of the genus '' Esc ...

'', ''

Citrobacter freundii ''Citrobacter freundii'' is a species of facultative anaerobic Gram-negative bacteria of the family Enterobacteriaceae which currently consists of 13 recognized species. These bacteria have a rod shape with a typical length of 1–5 μm. Most ''C ...

'', ''

Klebsiella pneumoniae ''Klebsiella pneumoniae'' is a Gram-negative, non-motile, encapsulated, lactose- fermenting, facultative anaerobic, rod-shaped bacterium. It appears as a mucoid lactose fermenter on MacConkey agar. Although found in the normal flora of the mo ...

'', various other ''Pseudomonas'' species, and ''

Morganella morganii ''Morganella morganii'' is a species of Gram-negative bacteria.eMedicineMorganella infections/ref> It has a commensal relationship within the intestinal tracts of humans, mammals, and reptiles as normal flora. Although ''M. morganii'' has a wide ...

''.

Signs and symptoms

The primary skin lesion usually starts with a

macule A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this s ...

that is painless, round and erythematous. Then, it develops into a pustule, and then a

bulla Bulla (Latin, 'bubble') may refer to: Science and medicine * Bulla (dermatology), a bulla * Bulla, a focal lung pneumatosis, an air pocket in the lung * Auditory bulla, a hollow bony structure on the skull enclosing the ear * Ethmoid bulla, pa ...

with central hemorrhagic focus. The bulla progresses into an ulcer which extends laterally. Finally it becomes a gangrenous ulcer with a central black

eschar An eschar (; Greek: ''ἐσχάρᾱ'', ''eskhara''; Latin: ''eschara'') is a slough or piece of dead tissue that is cast off from the surface of the skin, particularly after a burn injury, but also seen in gangrene, ulcer, fungal infections, ...

surrounded by an erythematous halo. The lesions may be single or multiple. They are most commonly seen in

perineum The perineum in humans is the space between the anus and scrotum in the male, or between the anus and the vulva in the female. The perineum is the region of the body between the pubic symphysis (pubic arch) and the coccyx (tail bone), includi ...

and under arm pit. However, they can occur in any part of the body.

Mechanism

The organism enters directly through the breakdown of mechanical defense barriers such as

mucosa A mucous membrane or mucosa is a membrane that lines various cavities in the body of an organism and covers the surface of internal organs. It consists of one or more layers of epithelial cells overlying a layer of loose connective tissue. It i ...

or skin. Conditions which lead to the development of an immunocompromised state make the patient more susceptible to ecthyma gangrenosum and

sepsis Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is follo ...

. In case of sepsis, the bacteria reaches the skin via the bloodstream. Defective

humoral Humoral immunity is the aspect of immunity that is mediated by macromolecules - including secreted antibodies, complement proteins, and certain antimicrobial peptides - located in extracellular fluids. Humoral immunity is named so because it invo ...

cellular immunity Cell-mediated immunity or cellular immunity is an immune response that does not involve antibodies. Rather, cell-mediated immunity is the activation of phagocytes, antigen-specific cytotoxic T-lymphocytes, and the release of various cytokines ...

increases risk, as the organism is not cleared from the bloodstream as usual. The main mechanism of the organism that is causing the typical skin lesions is the invasion of the organism into the arteries and veins in the

dermis The dermis or corium is a layer of skin between the epidermis (with which it makes up the cutis) and subcutaneous tissues, that primarily consists of dense irregular connective tissue and cushions the body from stress and strain. It is divided ...

and subcutaneous tissues of the skin. This

perivascular Mural cells are the vascular smooth muscle cells (vSMCs), and pericytes, of the microcirculation. Both types are in close contact with the endothelial cells lining the capillaries, and are important for vascular development and stability. Mural cel ...

invasion leads to nodular formation, ulceration, vasculitis and necrosis due to impaired blood supply. Perivascular involvement is achieved by direct entry of bacteria through the skin or

hematogenous Bloodstream infections (BSIs), which include bacteremias when the infections are bacterial and fungemias when the infections are fungal, are infections present in the blood. Blood is normally a sterile environment, so the detection of microb ...

spreading in case of sepsis.

Diagnosis

Diagnosis is made by clinical

observation Observation is the active acquisition of information from a primary source. In living beings, observation employs the senses. In science, observation can also involve the perception and recording of data via the use of scientific instruments. The ...

and the following tests. (1)

Gram stain In microbiology and bacteriology, Gram stain (Gram staining or Gram's method), is a method of staining used to classify bacterial species into two large groups: gram-positive bacteria and gram-negative bacteria. The name comes from the Danish b ...

of the fluid from pustules or bullae, and tissue swab. (2) Blood culture (3) Urine culture (4) Skin biopsy (5) Tissue culture

Magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wave ...

can be done in case of ecthyma gangrenosum of

plantar Standard anatomical terms of location are used to unambiguously describe the anatomy of animals, including humans. The terms, typically derived from Latin or Greek roots, describe something in its standard anatomical position. This position prov ...

foot to differentiate from

necrotizing fasciitis Necrotizing fasciitis (NF), also known as flesh-eating disease, is a bacterial infection that results in the death of parts of the body's soft tissue. It is a severe disease of sudden onset that spreads rapidly. Symptoms usually include red or p ...

Prevention

The main organism associated with ecthyma gangrenosum is ''Pseudomonas aeruginosa''. However, multi-bacterial cases are reported as well. Prevention measures include practicing proper hygiene, educating the immunocompromised patients for awareness to avoid possible conditions and seek timely medical treatment.

Treatments

Treatments involve antibiotics that cover for ''Pseudomonas aeruginosa''.

antipseudomonal penicillins The extended-spectrum penicillins are a group of antibiotics that have the widest antibacterial spectrum of all penicillins. Some sources identify them with antipseudomonal penicillins, others consider these types to be distinct. This group includ ...

aminoglycoside Aminoglycoside is a medicinal and bacteriologic category of traditional Gram-negative antibacterial medications that inhibit protein synthesis and contain as a portion of the molecule an amino-modified glycoside (sugar). The term can also refer ...

s, fluoroquinolones, third generation

cephalosporin The cephalosporins (sg. ) are a class of β-lactam antibiotics originally derived from the fungus ''Acremonium'', which was previously known as ''Cephalosporium''. Together with cephamycins, they constitute a subgroup of β-lactam antibiotics ...

s or ceftriaxone aztreonam can be given. Usually, the antibiotics are changed according to the culture and sensitivity result. In patients with very low white blood cell counts,

granulocyte-macrophage colony-stimulating factor Granulocyte-macrophage colony-stimulating factor (GM-CSF), also known as colony-stimulating factor 2 (CSF2), is a monomeric glycoprotein secreted by macrophages, T cells, mast cells, natural killer cells, endothelial cells and fibroblasts that ...

may be given. Depending on the causal agents, antivirals or antifungals can be added. Surgery will be needed if there is extensive necrosis not responding to medical treatments.

Recent research

A recent retrospective study of all cases of ecthyma gangrenosum from 2004 to 2010 in a university hospital in Mexico shows that

neutropenia Neutropenia is an abnormally low concentration of neutrophils (a type of white blood cell) in the blood. Neutrophils make up the majority of circulating white blood cells and serve as the primary defense against infections by destroying bacteri ...

in immunocompromised patients is the most common risk factor for ecthyma gangrenosum.

References

External links

{{Bacterial cutaneous infections Bacterium-related cutaneous conditions