| Pyoderma gangrenosum |
|
Pyoderma gangrenosum on the leg of a patient with Crohn's disease.
|
| Classification and external resources |
| Specialty |
dermatology |
| ICD-10 |
L88 |
| ICD-9-CM |
686.01 |
| DiseasesDB |
11064 |
| eMedicine |
article/1123821 |
| MeSH |
D017511 |
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.[1]
Contents
- 1 Types
- 2 Causes
- 3 Associations
- 4 Treatment
- 5 See also
- 6 References
- 7 External links
Types
There are two main types of pyoderma gangrenosum:[1]
- 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:[2]
- Peristomal pyoderma gangrenosum is 15% of all cases of pyoderma
- Bullous pyoderma gangrenosum
- Pustular pyoderma gangrenosum[3]
- Vegetative pyoderma gangrenosum[4]
Causes
Though the etiology is not well understood, the disease is thought to be due to immune system dysfunction, and particularly improper functioning of neutrophils. In support of an immune etiology, a variety of immune mediators such as IL-8, IL-1β, IL-6, interferon (IFN)-γ, G-CSF, TNF, matrix metallopaptidase (MMP)-9, MMP-10, and Elafin have all been reported to be elevated in patients with pyoderma gangrenosum.[5]
Also in support of an immune etiology is the finding that at least half of all pyoderma gangrenous patients suffer from immune-mediated diseases.[1] For instance, ulcerative colitis, rheumatoid arthritis, and multiple myeloma (MM) have all been associated with pyoderma gangrenosum. It can also be part of a syndromes such as PAPA syndrome.
One hallmark of pyoderma gangrenosum is pathergy, which is the appearance of new lesions at sites of trauma. [6]
Associations
The following are conditions commonly associated with pyoderma gangrenosum:[7]
- Inflammatory bowel disease:
- Ulcerative colitis
- Crohn's Disease
- Arthritides:
- Rheumatoid arthritis
- Seronegative arthritis
- Hematological disease:
- Myelocytic leukemia[8]
- Hairy cell leukemia
- Myelofibrosis
- Myeloid metaplasia
- Monoclonal gammopathy
- Autoinflammatory Disease:
- Pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome)
Also granulomatosis with polyangiitis[9]
Treatment
First-line therapy for disseminated or localized instances of pyoderma gangrenosum is systemic treatment by corticosteroids and cyclosporine. Topical application of clobetasol, mupirocin, and gentamicin alternated with tacrolimus can be effective.
Papules that begin as small "spouts" can be treated with Dakins Solution to prevent infection and entire wounds cluster also benefit from this disinfectant. Wet to dry applications of Dakins can defeat spread of interior infection. Heavy drainage can be offset with Coban dressings. Grafting is not recommended due to tissue necrosis.
If ineffective, alternative therapeutic procedures include systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; thalidomide; infliximab; or plasmapheresis.[10]
There is currently a phase III trial for the use of the IL-1B modulating agent gevokizumab in treating the ulcers of pyoderma gangrenosum. [11][12]
See also
- Superficial granulomatous pyoderma
- List of cutaneous conditions
- Brown recluse spider bite
References
- ^ a b c Jackson, J Mark; Callen, Jeffrey P (April 23, 2012). Elston, Dirk M, ed. "Pyoderma Gangrenosum". Emedicine.
- ^ Brooklyn, T.; Dunnill, G; Probert, C (2006). "Diagnosis and treatment of pyoderma gangrenosum". BMJ 333 (7560): 181–4. doi:10.1136/bmj.333.7560.181. PMC 1513476. PMID 16858047.
- ^ Shankar, S.; Sterling, J. C.; Rytina, E. (2003). "Pustular pyoderma gangrenosum". Clinical and Experimental Dermatology 28 (6): 600–3. doi:10.1046/j.1365-2230.2003.01418.x. PMID 14616824.
- ^ Langan, Sinead M.; Powell, Frank C. (2005). "Vegetative pyoderma gangrenosum: A report of two new cases and a review of the literature". International Journal of Dermatology 44 (8): 623–9. doi:10.1111/j.1365-4632.2005.02591.x. PMID 16101860.
- ^ Patel F, Fitzmaurice S, Duong C, He Y, Fergus J, Raychaudhuri SP, Garcia MS, Maverakis E (2015). "Effective strategies for the management of pyoderma gangrenosum: a comprehensive review.". Acta Derm Venereol 95 (5): 525–31. doi:10.2340/00015555-2008. PMID 25387526.
- ^ Rashid, RM (2008). "Seat belt pyoderma gangrenosum: Minor pressure as a causative factor". Journal of the European Academy of Dermatology and Venereology 22 (10): 1273–4. doi:10.1111/j.1468-3083.2008.02626.x. PMID 18837131.
- ^ Brooklyn, Trevor; Giles Dunnill; Chris Probert (2006). "Diagnosis and treatment of pyoderma gangrenosum". British Medical Journal 333: 181–184. doi:10.1136/bmj.333.7560.181. PMC 1513476. PMID 16858047. Retrieved 31 December 2013.
- ^ Tendas, Andrea; Niscola P, Barbati R, Abruzzese E, Cuppelli L, Giovannini M, Scaramucci L, Fratoni S, Ales M, Neri B, Morino L, Dentamaro T, De Fabritiis P. (May 2011). "Tattoo related pyoderma/ectyma gangrenous as presenting feature of relapsed acute myeloid leukaemia: an exceptionally rare observation.". Injury 42 (5): 546–7. doi:10.1016/j.injury.2010.08.014.
- ^ Ponniah, I.; Shaheen, Ahmed; Shankar, K.A.; Kumaran, M.G. (2005). "Wegener's granulomatosis: The current understanding". Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology 100 (3): 265–70. doi:10.1016/j.tripleo.2005.04.018. PMID 16122651.
- ^ Reichrath, Jörg; Bens, Guido; Bonowitz, Anette; Tilgen, Wolfgang (2005). "Treatment recommendations for pyoderma gangrenosum: An evidence-based review of the literature based on more than 350 patients". Journal of the American Academy of Dermatology 53 (2): 273–83. doi:10.1016/j.jaad.2004.10.006. PMID 16021123.
- ^ https://clinicaltrials.gov/ct2/show/NCT02315417?term=gevokizumab+pyoderma+gangrenosum&rank=3
- ^ https://clinicaltrials.gov/ct2/show/NCT02326740?term=gevokizumab+pyoderma+gangrenosum&rank=2
External links
|
Wikimedia Commons has media related to Pyoderma gangrenosum. |
- DermNet reactions/pyoderma-gangrenosum
- The Study of Treatments for Pyoderma Gangrenosum
- A case of pyoderma gangrenosum successfully treated with intravenous immune globulin
|
Neutrophilic and eosinophilic dermatoses (L98.2-3)
|
|
| Eosinophilic dermatosis |
| With vasculitis |
- Eosinophilic vasculitis
- Churg–Strauss syndrome
|
|
| Without vasculitis |
- Arthropod assault
- Eosinophilic cellulitis
- Hypereosinophilic syndrome
- Papuloerythroderma of Ofuji
- Granuloma faciale
- Eosinophilic folliculitis
|
|
| Ungrouped |
- Angiolymphoid hyperplasia with eosinophilia/Kimura's disease
- Annular erythema of infancy
- Eosinophilic fasciitis
- Eosinophilic granuloma
- Eosinophilic ulcer of the oral mucosa
- Erythema toxicum neonatorum
- Incontinentia pigmenti
- Itchy red bump disease
- Juvenile xanthogranuloma
- Pachydermatous eosinophilic dermatitis
- Papular eruption of blacks
- Pruritic papular eruption of HIV disease
|
|
|
| Reactive neutrophilic dermatoses |
| Epidermis |
- Keratoderma blennorrhagicum
- Subcorneal pustular dermatosis
|
|
| Dermis |
| without vasculitis: |
- Sweet's syndrome
- Pyoderma gangrenosum
- Bowel-associated dermatosis–arthritis syndrome
|
|
| with vasculitis: |
- Neutrophilic dermatosis of the dorsal hands
|
|
|
| Ungrouped |
- Acute erythema nodosum
- Marshall syndrome
- Neutrophilic eccrine hidradenitis
- Pyogenic arthritis–pyoderma gangrenosum–acne syndrome
- Rheumatoid neutrophilic dermatitis
- Superficial granulomatous pyoderma
- Sweet's syndrome-like dermatosis
- Vesicopustular dermatosis
|
|
|
|
Index of skin
|
|
| Description |
- Anatomy
- Physiology
- Development
|
|
| Disease |
- Infections
- Vesiculobullous
- Dermatitis and eczema
- Papulosquamous
- Urticaria and erythema
- Radiation-related
- Pigmentation
- Mucinoses
- Keratosis, ulcer, atrophy, and necrobiosis
- Vasculitis
- Fat
- Neutrophilic and eosinophilic
- Congenital
- Neoplasms and cancer
- nevi and melanomas
- epidermis
- dermis
- Symptoms and signs
- Terminology
|
|
| Treatment |
- Procedures
- Drugs
- antibiotics
- disinfectants
- emollients and protectives
- itch
- psoriasis
- other
- Wound and ulcer
|
|
Index of cells from bone marrow
|
|
| Description |
- Immune system
- Cells
- Physiology
- coagulation
- proteins
- granule contents
- colony-stimulating
- heme and porphyrin
|
|
| Disease |
- Red blood cell
- Monocyte and granulocyte
- Neoplasms and cancer
- Histiocytosis
- Symptoms and signs
- Blood tests
|
|
| Treatment |
- Transfusion
- Drugs
- thrombosis
- bleeding
- other
|
|
|
|
Paraneoplastic syndromes
|
|
| Endocrine |
- Hypercalcaemia
- SIADH
- Zollinger–Ellison syndrome
- Cushing's syndrome
|
|
| Hematological |
- Granulocytosis
- Multicentric reticulohistiocytosis
|
|
| Neurological |
- Paraneoplastic cerebellar degeneration
- Encephalomyelitis
- Limbic encephalitis
- Opsoclonus
- Polymyositis
- Transverse myelitis
- Lambert–Eaton myasthenic syndrome
- Anti-NMDA receptor encephalitis
|
|
| Musculoskeletal |
- Dermatomyositis
- Hypertrophic osteopathy
|
|
| Mucocutaneous |
| reactive erythema |
- Erythema gyratum repens
- Necrolytic migratory erythema
|
|
| papulosquamous |
- Acanthosis nigricans
- Ichthyosis acquisita
- Acrokeratosis paraneoplastica of Bazex
- Extramammary Paget's disease
- Florid cutaneous papillomatosis
- Leser-Trélat sign
- Pityriasis rotunda
- Tripe palms
|
|
| Other |
- Febrile neutrophilic dermatosis
- Pyoderma gangrenosum
- Paraneoplastic pemphigus
|
|
|
|
Index of neoplasms and cancer
|
|
| Description |
- Tumor suppressing and oncogenes
- Tumor markers
- Carcinogen
|
|
| Disease |
- Neoplasms and cancer
- Symptoms and signs
- Paraneoplastic
|
|
| Treatment |
- Radiotherapy
- Drugs
- Immunotherapy
- intracellular chemotherapeutics
- extracellular chemotherapeutics
- adjuvant detoxification
|
|
|
