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Pustule

MedGen UID:
488804
Concept ID:
C0241157
Finding
Synonym: Pustules
SNOMED CT: Pustule (271760008); Pussey spot (271760008); Pustule (47002008)
 
HPO: HP:0200039

Definition

A small elevation of the skin containing cloudy or purulent material usually consisting of necrotic inflammatory cells. [from HPO]

Conditions with this feature

Acrodermatitis continua suppurativa of Hallopeau
MedGen UID:
581114
Concept ID:
C0392439
Disease or Syndrome
A rare, genetic, chronic, recurrent, slowly progressive, epidermal disease characterized by small, sterile, pustular eruptions, involving the nails and surrounding skin of the fingers and/or toes, which coalesce and burst, leaving erythematous, atrophic skin where new pustules develop. Onychodystrophy is frequently associated and anonychia and osteolysis are reported in severe cases. Local expansion (to involve the hands, forearms and/or feet) and involvement of mucosal surfaces (e.g. conjunctiva, tongue, urethra) may be observed.
Sterile multifocal osteomyelitis with periostitis and pustulosis
MedGen UID:
411230
Concept ID:
C2748507
Disease or Syndrome
Chronic recurrent multifocal osteomyelitis-2 with periostitis and pustulosis (CRMO2) is an autosomal recessive multisystemic autoinflammatory disorder characterized by onset of symptoms in early infancy. Affected individuals present with joint swelling and pain, pustular rash, oral mucosal lesions, and fetal distress. The disorder progresses in severity to generalized severe pustulosis or ichthyosiform lesions and diffuse bone lesions. Radiographic studies show widening of the anterior rib ends, periosteal elevation along multiple long bones, multifocal osteolytic lesions, heterotopic ossification, and metaphyseal erosions of the long bones. Laboratory studies show elevation of inflammatory markers. The disorder results from unopposed activation of the IL1 inflammatory signaling pathway. Treatment with the interleukin-1 receptor antagonist anakinra may result in clinical improvement (Aksentijevich et al., 2009). For a discussion of genetic heterogeneity of CRMO, see 609628.
Inflammatory skin and bowel disease, neonatal, 1
MedGen UID:
482131
Concept ID:
C3280501
Disease or Syndrome
Any neonatal inflammatory skin and bowel disease in which the cause of the disease is a mutation in the ADAM17 gene.
STING-associated vasculopathy with onset in infancy
MedGen UID:
863159
Concept ID:
C4014722
Disease or Syndrome
STING-associated vasculopathy with onset in infancy is an autoinflammatory vasculopathy causing severe skin lesions, particularly affecting the face, ears, nose, and digits, and resulting in ulceration, eschar formation, necrosis, and, in some cases, amputation. Many patients have interstitial lung disease. Tissue biopsy and laboratory findings show a hyperinflammatory state, with evidence of increased beta-interferon (IFNB1; 147640) signaling (summary by Liu et al., 2014).
Inflammatory skin and bowel disease, neonatal, 2
MedGen UID:
863567
Concept ID:
C4015130
Disease or Syndrome
Neonatal nephrocutaneous inflammatory syndrome (NNCIS) is an autosomal recessive disorder characterized by intrauterine growth retardation and premature birth, fragile infection-prone skin, and nephromegaly with tubular dysfunction. Some patients have chronic diarrhea, and necrotizing enterocolitis with intestinal perforation has been observed. Other features include facial dysmorphisms and cardiac anomalies. Most patients require ventilatory and circulatory support at birth, exhibit failure to thrive, experience recurrent infections with sepsis as a common complication, and die within 6 months (Mazurova et al., 2020; Labbouz et al., 2023). Reviews Takeichi and Akiyama (2021) reviewed published reports of patients with mutation in the EGFR gene, whose features included intrauterine growth restriction; thin, translucent, and fragile skin (14 of 15 cases); skin desquamation (10 of 17 cases); ichthyosis (9 of 17 cases); recurrent skin infections and sepsis (9 of 12 cases); nephromegaly (10 of 16 cases); and congenital heart defects (7 of 17 cases). Other observed features included erythroderma, tubulopathy, necrotizing enterocolitis/intestinal perforation, cryptorchidism, hyperimmunoglobulin E, and dentinogenesis imperfecta. Almost all children died within 2.5 years after birth. The authors suggested that EGFR-associated systemic inflammatory diseases should be considered a part of the clinical spectrum of 'autoinflammatory keratinization diseases' (AiKDs).
Immunodeficiency 51
MedGen UID:
934770
Concept ID:
C4310803
Disease or Syndrome
Immunodeficiency-51 (IMD51) is an autosomal recessive primary immune deficiency that is usually characterized by onset of chronic mucocutaneous candidiasis in the first years of life. Most patients also show recurrent Staphylococcal skin infections, and may show increased susceptibility to chronic bacterial respiratory infections. Patient cells show a lack of cellular responses to stimulation with certain IL17 isoforms, including IL17A (603149), IL17F (606496), IL17A/F, and IL17E (IL25; 605658) (summary by Levy et al., 2016).
Immunodeficiency 66
MedGen UID:
1717128
Concept ID:
C5394265
Disease or Syndrome
Immunodeficiency-66 (IMD66) is an autosomal recessive primary immune disorder caused by defective immune cell migration and chemotaxis resulting from defects in cytoskeletal actin dynamics. Neutrophils are primarily affected, although there may be defects in dendritic cells and T and B cells. The phenotype is characterized by onset of recurrent bacterial infections in infancy. Laboratory studies show normal levels of myeloid and lymphoid cells, but there may be mild thrombocytopenia (summary by Record et al., 2015).

Professional guidelines

PubMed

Hadavand MA, Kaffenberger B, Cartron AM, Trinidad JCL
J Am Acad Dermatol 2022 Sep;87(3):632-639. Epub 2020 Sep 11 doi: 10.1016/j.jaad.2020.09.024. PMID: 32926975
Maverakis E, Ma C, Shinkai K, Fiorentino D, Callen JP, Wollina U, Marzano AV, Wallach D, Kim K, Schadt C, Ormerod A, Fung MA, Steel A, Patel F, Qin R, Craig F, Williams HC, Powell F, Merleev A, Cheng MY
JAMA Dermatol 2018 Apr 1;154(4):461-466. doi: 10.1001/jamadermatol.2017.5980. PMID: 29450466
Steele RB, Nugent WH, Braswell SF, Frisch S, Ferrell J, Ortega-Loayza AG
Br J Dermatol 2016 Jan;174(1):77-87. Epub 2015 Dec 14 doi: 10.1111/bjd.14230. PMID: 26474193

Recent clinical studies

Etiology

Maturu VN, Prasad VP, Biradar M, Narahari NK
J Bronchology Interv Pulmonol 2023 Oct 1;30(4):354-362. doi: 10.1097/LBR.0000000000000887. PMID: 35968962
Marrakchi S, Puig L
Am J Clin Dermatol 2022 Jan;23(Suppl 1):13-19. Epub 2022 Jan 21 doi: 10.1007/s40257-021-00655-y. PMID: 35061228Free PMC Article
Metzger W, Mordmueller BG
Cochrane Database Syst Rev 2007 Jul 18;2007(3):CD004913. doi: 10.1002/14651858.CD004913.pub2. PMID: 17636779Free PMC Article
Proença NG
Int J Dermatol 2006 Apr;45(4):389-93. doi: 10.1111/j.1365-4632.2006.02473.x. PMID: 16650164
Iizuka H, Takahashi H, Ishida-Yamamoto A
Arch Dermatol Res 2003 Apr;295 Suppl 1:S55-9. Epub 2003 Jan 25 doi: 10.1007/s00403-002-0372-5. PMID: 12677433

Diagnosis

Prinz JC, Choon SE, Griffiths CEM, Merola JF, Morita A, Ashcroft DM, Viguier M
J Eur Acad Dermatol Venereol 2023 Feb;37(2):256-273. Epub 2022 Dec 3 doi: 10.1111/jdv.18720. PMID: 36331364
Heath CR, Usatine RP
J Fam Pract 2021 Sep;70(7):356. doi: 10.12788/jfp.0271. PMID: 34818170
Iranmanesh B, Khalili M, Amiri R, Zartab H, Aflatoonian M
Dermatol Ther 2021 Jan;34(1):e14578. Epub 2020 Dec 13 doi: 10.1111/dth.14578. PMID: 33236823Free PMC Article
Maverakis E, Ma C, Shinkai K, Fiorentino D, Callen JP, Wollina U, Marzano AV, Wallach D, Kim K, Schadt C, Ormerod A, Fung MA, Steel A, Patel F, Qin R, Craig F, Williams HC, Powell F, Merleev A, Cheng MY
JAMA Dermatol 2018 Apr 1;154(4):461-466. doi: 10.1001/jamadermatol.2017.5980. PMID: 29450466
Saigal R, Singh Y, Mittal M, Kansal A, Maharia HR
J Assoc Physicians India 2010 Jun;58:378-83. PMID: 21125780

Therapy

Xu JM, Wang HM, Jin HZ
Expert Rev Clin Immunol 2023 May;19(5):499-516. Epub 2023 Mar 27 doi: 10.1080/1744666X.2023.2185775. PMID: 36970858
Steele RB, Nugent WH, Braswell SF, Frisch S, Ferrell J, Ortega-Loayza AG
Br J Dermatol 2016 Jan;174(1):77-87. Epub 2015 Dec 14 doi: 10.1111/bjd.14230. PMID: 26474193
Metzger W, Mordmueller BG
Cochrane Database Syst Rev 2007 Jul 18;2007(3):CD004913. doi: 10.1002/14651858.CD004913.pub2. PMID: 17636779Free PMC Article
Sawalka SS, Phiske MM, Jerajani HR
Indian J Dermatol Venereol Leprol 2007 Mar-Apr;73(2):117-9. doi: 10.4103/0378-6323.31900. PMID: 17456921
Plewig G, Jansen T
Dermatology 1998;196(1):102-7. doi: 10.1159/000017841. PMID: 9557242

Prognosis

Xu JM, Wang HM, Jin HZ
Expert Rev Clin Immunol 2023 May;19(5):499-516. Epub 2023 Mar 27 doi: 10.1080/1744666X.2023.2185775. PMID: 36970858
Prinz JC, Choon SE, Griffiths CEM, Merola JF, Morita A, Ashcroft DM, Viguier M
J Eur Acad Dermatol Venereol 2023 Feb;37(2):256-273. Epub 2022 Dec 3 doi: 10.1111/jdv.18720. PMID: 36331364
Maturu VN, Prasad VP, Biradar M, Narahari NK
J Bronchology Interv Pulmonol 2023 Oct 1;30(4):354-362. doi: 10.1097/LBR.0000000000000887. PMID: 35968962
Saigal R, Singh Y, Mittal M, Kansal A, Maharia HR
J Assoc Physicians India 2010 Jun;58:378-83. PMID: 21125780
Proença NG
Int J Dermatol 2006 Apr;45(4):389-93. doi: 10.1111/j.1365-4632.2006.02473.x. PMID: 16650164

Clinical prediction guides

Maturu VN, Prasad VP, Biradar M, Narahari NK
J Bronchology Interv Pulmonol 2023 Oct 1;30(4):354-362. doi: 10.1097/LBR.0000000000000887. PMID: 35968962
Metzger W, Mordmueller BG
Cochrane Database Syst Rev 2007 Jul 18;2007(3):CD004913. doi: 10.1002/14651858.CD004913.pub2. PMID: 17636779Free PMC Article
Proença NG
Int J Dermatol 2006 Apr;45(4):389-93. doi: 10.1111/j.1365-4632.2006.02473.x. PMID: 16650164
Marsland AM, Chalmers RJ, Hollis S, Leonardi-Bee J, Griffiths CE
Cochrane Database Syst Rev 2006 Jan 25;2006(1):CD001433. doi: 10.1002/14651858.CD001433.pub2. PMID: 16437433Free PMC Article
Rapelanoro R, Mortureux P, Couprie B, Maleville J, Taïeb A
Arch Dermatol 1996 Feb;132(2):190-3. PMID: 8629828

Recent systematic reviews

Safa I, Ines L, Noureddine L, Meriem J, Manel N, Belhajali H, Faten Z, Zili J
Dermatol Ther 2021 Sep;34(5):e15087. Epub 2021 Aug 25 doi: 10.1111/dth.15087. PMID: 34351040
Kearns DG, Chat VS, Zang PD, Han G, Wu JJ
J Dermatolog Treat 2021 Aug;32(5):492-494. Epub 2020 Jan 15 doi: 10.1080/09546634.2019.1682502. PMID: 31697211
Saric S, Clark AK, Sivamani RK, Lio PA, Lev-Tov HA
J Altern Complement Med 2017 Dec;23(12):920-929. Epub 2017 Jun 26 doi: 10.1089/acm.2016.0398. PMID: 28650692
Metzger W, Mordmueller BG
Cochrane Database Syst Rev 2007 Jul 18;2007(3):CD004913. doi: 10.1002/14651858.CD004913.pub2. PMID: 17636779Free PMC Article
Marsland AM, Chalmers RJ, Hollis S, Leonardi-Bee J, Griffiths CE
Cochrane Database Syst Rev 2006 Jan 25;2006(1):CD001433. doi: 10.1002/14651858.CD001433.pub2. PMID: 16437433Free PMC Article

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