Presentation
We present a rare case of a 4-year-old boy with newly diagnosed Henöch-Schonlein purpura (HSP) affecting the scrotum and penis. The patient presented to the emergency department with palpable purpura symmetrically distributed over the lower limbs. [ncbi.nlm.nih.gov]
It is the presenting sign in 50 percent of patients. 12 Some patients present with predominantly petechial lesions, some present with mainly purpuric lesions, and others present with a mixture of lesion types. [aafp.org]
She denied the use of any new medications but did report resuming use of pilocarpine 3 months prior to presentation and ursodiol 2 months prior to presentation. She had previously taken both of these medications without adverse effects. [mdedge.com]
Entire Body System
- Intermittent Fever
The family reported the boy had been ill 10 days prior to presentation with intermittent fevers, nausea, emesis, and diarrhea, the latter of which had been bloody on at least one occasion. [mdedge.com]
bone pain Leukemia Intermittent fever, musculoskeletal symptoms Systemic lupus erythematosus Lethargy, polyuria, polydipsia, failure to thrive Uremia Purpura, but otherwise Healthy Idiopathic thrombocytopenic Purpura Drug use Alkylating agents Thrombocytopenia [aafp.org]
- Poor Growth
View/Print Table TABLE 2 Physical Findings and Possible Etiologies of Purpura Physical findings Possible etiology General findings Poor growth Chronic disorder Fever Infection Hypertension Chronic renal failure, renal vasculitis Characteristics of purpura [aafp.org]
Gastrointestinal
- Epigastric Mass
mass T: Petechiae or Purplish or Reddish C: Little or None P: Wiry and choppy or Wiry, small and smooth or Thready and choppy Treatment Principle Invigorate the Blood Remove Stasis Alleviate jaundice Calm the Liver Herb Formulas Points Clinical Manifestations [americandragon.com]
- Abdominal Fullness
fullness and pain Anorexia Rough, dry and scaly skin May be localized abdominal pain and tenderness Amenorrhea A dull, dark appearance of the eyes Tidal fever Maybe dry stools Maybe black, tarry stools Maybe an epigastric mass T: Petechiae or Purplish [americandragon.com]
Skin
- Eruptions
Price1 first reported a "punctate purpuric eruption" occurring in a patient receiving intramuscular penicillin, and noted that the eruption appeared for two days preceding the onset of urticaria, arthralgia, fever, and leukocytosis. [annals.org]
[…] petechial hemorrhages (___cm), petechial hemorrhages (physical finding), petechiae (diagnosis), petechiae, Rash petechial, petechial rash, petechial hemorrhage, [D]Petechiae (situation), Petechia, Petechial hemorrhage, Petechial haemorrhage, Petechial eruption [fpnotebook.com]
The clinical picture is classically a cutaneous purpuric eruption of the legs and buttocks and infrequently the upper torso and extremities. [mdedge.com]
Representative Case Studies Case 1 A 56 year old man with prior history of hypertension and adult onset diabetes mellitus presented with a skin eruption over the lower extremities of several weeks duration (appearance of the eruption is shown in Figures [clinicalmolecularallergy.biomedcentral.com]
- Palmar Erythema
[…] tenderness, subcutaneous edema, scrotal swelling Henoch-Schönlein purpura Hemarthrosis Hemophilia Butterfly rash, pallor, arthritis, lymphadenopathy Systemic lupus erythematosus Lymphadenopathy Infection, drugs, malignancy Jaundice, spider angioma, palmar [aafp.org]
Workup
Careful examination of skin including the bottom of the feet can help to direct the diagnostic workup for children with abdominal pain. [ncbi.nlm.nih.gov]
Based on the pathophysiology that is suspected, a differential diagnosis can be generated and an appropriate workup can be initiated. [link.springer.com]
A laboratory workup, including a complete blood cell count and differential, prothrombin time, partial thromboplastin time, comprehensive metabolic panel, creatinine kinase test, urinalysis, and chest radiograph, was normal, but his erythrocyte sedimentation [mdedge.com]
See Workup for more detail. Management The therapy of choice for TTP is plasma exchange with fresh frozen plasma. [emedicine.medscape.com]
Previous workup by her primary care provider included complete blood count (CBC), complete metabolic panel (CMP), and imaging of affected sites, all of which were unremarkable. [psychiatrictimes.com]
Microbiology
- Escherichia Coli O157:H7
The syndrome is most often associated with infection by verocytotoxin-producing Escherichia coli O157:H7. [aafp.org]
Treatment
When treatment is necessary, it is not due to the rash itself. Treatment is needed for causes such as leukemia, or effects including kidney failure. [medicalnewstoday.com]
Prognosis
What is the prognosis for patients with HSP? The prognosis (outlook) for patients with HSP is generally excellent. Nearly all patients have no long-term problems. The kidney is the most serious organ involved when it is affected. [medicinenet.com]
Click here for example image of HSP Links: aetiology pathogenesis clinical features treatment and prognosis complications urticaria [gpnotebook.co.uk]
The prognosis of Henoch Schoenlein nephritis. Clin Nephrol. 1978 Mar; 9 (3):87–90. [ PubMed ] [ Google Scholar ] Meadow SR, Glasgow EF, White RH, Moncrieff MW, Cameron JS, Ogg CS. Schönlein-Henoch nephritis. [ncbi.nlm.nih.gov]
Prognosis Gastrointestinal or pulmonary bleeding can be life-threatening. The long term prognosis depends on the extent of the renal involvement. [orpha.net]
Etiology
Both thrombocytopenia and purpura have been shown to have a complex etiologic pattern with multiple potential etiologic factors. [ashpublications.org]
Regarding the etiology, prodromal viral illness has been described as an etiology of HSP. Though there is a higher occurrence of HSP in the late fall and spring, some patients are seen throughout the year. [journals.lww.com]
The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes. [ncbi.nlm.nih.gov]
PLATELET DYSFUNCTION Congenital Etiologies. [aafp.org]
Epidemiology
Epidemiology Sex: ♂ > ♀ More common in children 90% of cases < 10 years old Peak incidence: 6 years References:[1][2] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
Here, we will review the epidemiology, clinical and molecular characteristics of HSP, as well as abnormalities of Th cell subsets in this disorder. [ncbi.nlm.nih.gov]
The epidemiology, pathophysiology, clinical features, differential diagnosis and management of purpura and bruising due to different causes are discussed. The full text of this article hosted at iucr.org is unavailable due to technical difficulties. [onlinelibrary.wiley.com]
George at the Department of Biostatistics and Epidemiology, Hudson College of Public Health, University of Oklahoma Health Sciences Center, 801 NE 13th St., Oklahoma City, OK 73104, or at [email protected]. [nejm.org]
Epidemiology of Henoch-Schonlein purpura. Cleve Clin J Med. 2002;69(Suppl 2):SII87-SII89. EBSCO Medical Review Board Michael J. Fucci, DO, FACC 201805 20180710 [winchesterhospital.org]
Pathophysiology
Henoch-Schonlein purpura Summary Epidemiology Etiology Pathophysiology Clinical features History Manifestations Diagnostics Laboratory tests Imaging Biopsy Differential diagnoses Purpura Arthritis/Arthralgia Renal disease Treatment Mild disease Severe [amboss.com]
The epidemiology, pathophysiology, clinical features, differential diagnosis and management of purpura and bruising due to different causes are discussed. The full text of this article hosted at iucr.org is unavailable due to technical difficulties. [onlinelibrary.wiley.com]
Each of these morphologies is associated with a specific underlying pathophysiology. [link.springer.com]
Pathophysiology The pathophysiology of Henoch-Schonlein purpura is not fully understood; however, IgA plays a significant role. [ncbi.nlm.nih.gov]
ALL) Acute myeloid leukemia (AML) Background Epidemiology Incidence (Audemard-Verger, 2015) Children – 3-26/100,000 Adults – 0.1-1.8/100,000 Age – typically diagnosed in children 3-10 years Majority are diagnosed in children >5 years Sex – M>F, 1.5:1 Pathophysiology [arupconsult.com]
Prevention
Abstract Immunoglobulin G is used to both prevent infection in primary immunodeficiency diseases (PIDs) and prevent bleeding in immune thrombocytopenic purpura. [ncbi.nlm.nih.gov]
However, they have not been shown to reduce the extent or severity of renal involvement, nor did they prevent the development of renal damage. [news-medical.net]
Can Henoch-Schönlein purpura be prevented or avoided? Doctors do not know how to prevent HSP. The disease is more common in boys than in girls. Children 2 to 11 years of age are more likely to be affected, but adults can get it too. [familydoctor.org]
Eight studies evaluated therapy to prevent persistent kidney disease in HSP. [cochrane.org]