External Male Genitalia in Henoch–Schönlein Syndrome: A Systematic Review
Abstract
:1. Introduction
2. Materials and Methods
2.1. Data Source
2.2. Eligibility Criteria and Data Extraction
2.3. Completeness of Reporting—Analysis
3. Results
3.1. Search Results
3.2. General Data
3.2.1. General Data
3.2.2. Penile Involvement
3.2.3. Scrotal Involvement
4. Discussion
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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|
All Cases | Isolated Penile Involvement | Isolated Scrotal Involvement | Penoscrotal Involvement | p Value | |
---|---|---|---|---|---|
N | 114 | 18 | 77 | 19 | |
Age | |||||
years | 5.8 [4.0–8.0] | 4.9 [3.5–7.5] | 6.0 [4.0–8.0] | 5.0 [4.0–5.8] | 0.0586 |
≤18 years, N | 104 | 16 | 70 | 19 | 0.3600 |
Precursors | 0.5214 | ||||
Infection | 41 | 9 | 21 | 11 | |
Upper respiratory infection, N | 33 | 6 | 17 | 10 | |
Further infections, N | 8 | 3 | 4 | 1 | |
Vaccine, N | 1 | 0 | 1 | 0 | |
Hymenoptera sting, N | 1 | 0 | 1 | 0 | |
Time relationship | 0.2144 | ||||
Skin before genitalia by ≥3 days *, N | 55 | 10 | 36 | 9 | |
Skin and genitalia concomitant, N | 41 | 4 | 29 | 8 | |
Genitalia before skin by ≥3 days **, N | 10 | 0 | 9 | 1 | |
Information not available, N | 8 | 4 | 3 | 1 | |
CAAR-grading of organ involvement | |||||
Cutaneous involvement | 0.8483 | ||||
Mild, N | 73 | 11 | 49 | 13 | |
Moderate, N | 26 | 5 | 18 | 3 | |
Severe, N | 11 | 2 | 6 | 3 | |
Information not available, N | 4 | 0 | 4 | 0 | |
Abdominal involvement | <0.03 ◆ | ||||
None, N | 49 | 13 | 26 | 10 | |
Mild, N | 24 | 5 | 14 | 5 | |
Moderate, N | 20 | 0 | 17 | 3 | |
Severe, N | 19 | 0 | 18 | 1 | |
Information not available, N | 2 | 0 | 2 | 0 | |
Articular involvement | 0.1647 | ||||
None, N | 45 | 5 | 33 | 7 | |
Mild, N | 52 | 10 | 36 | 6 | |
Moderate, N | 10 | 2 | 3 | 5 | |
Severe, N | 5 | 1 | 3 | 1 | |
Information not available, N | 2 | 0 | 2 | 0 | |
Kidney involvement | 0.4818 | ||||
None, N | 73 | 14 | 48 | 11 | |
Mild, N | 23 | 2 | 16 | 5 | |
Moderate, N | 14 | 2 | 9 | 3 | |
Severe, N | 2 | 0 | 2 | 0 | |
Information not available, N | 2 | 0 | 2 | 0 |
Penile Manifestations | N (%) |
---|---|
Swelling, N | 26 (70%) |
Erythema, N | 23 (62%) |
Purpuric rash, N | 20 (54%) |
Pain, N | 15 (40%) |
Transient micturition disorders, N | 2 (5%) |
Priapism, N | 2 (5%) |
Laterality | |||||
---|---|---|---|---|---|
Total N = 213 | Left N = 82 | Right N = 38 | Bilateral N = 83 | Not Specified N = 10 | |
Scrotal skin inflammation, N | 83 | 22 | 10 | 51 | 0 |
Epididymitis, N | 49 | 26 | 6 | 12 | 5 |
Testicular involvement, N | 39 | 15 | 10 | 14 | 0 |
Orchitis, N | 30 | 10 | 7 | 13 | 0 |
Ischemic damage, N | 9 | 5 | 3 | 1 | 0 |
Without torsion, N | 5 | 3 | 1 | 1 | 0 |
With torsion, N | 4 | 2 | 2 | 0 | 0 |
Spermatic cord involvement, N | 14 | 7 | 5 | 0 | 2 |
Funiculitis, N | 13 | 6 | 5 | 0 | 2 |
Spermatic vein thrombosis, N | 1 | 1 | 0 | 0 | 0 |
Hydatid torsion, N | 3 | 3 | 0 | 0 | 0 |
Poorly defined intrascrotal inflammation, N | 13 | 4 | 3 | 3 | 3 |
Hydrocele, N | 12 | 5 | 4 | 3 | 0 |
Imaging Studies | ||||
---|---|---|---|---|
History—Examination | Ultrasound | Color Doppler | Differential Diagnosis | |
Scrotal skin inflammation | Erythema (sometimes with purpuric lesions), warmth and swelling (painless or only mildly painful) of the scrotal sac (usually bilateral) | Thickening and swelling of the scrotal sac, small bilateral hydroceles, normal testes | Hypervascularity of the scrotal sac (fountain sign) | Acute idiopathic scrotal edema |
Orchitis (sometimes associated with epididymitis) | Testicular pain, swelling and warmth, often associated with swelling and erythema of the scrotal sac (usually unilateral) | Increased testicular volume with focal or diffuse hypoechogenicity, often mild-moderate thickening of the scrotal wall, hydrocele (anechoic or with debris), funiculitis | Increased testicular (and, often, epidydimal) blood flow | Reperfusion after intermittent torsion |
Epididymitis (mostly associated with orchitis) | Pain posterior to the testicle, often associated with swelling and erythema of the scrotal sac (usually bilateral) | Increased epididymal volume with heterogeneous echogenicity, often mild-moderate thickening of the scrotal wall, hydrocele (anechoic or with debris), funiculitis | Increased epidydimal (and, often, testicular) blood flow | Reperfusion after intermittent torsion |
Primary vascular testicular damage | Acute or subacute unilateral testicular pain, testicle usually tender and swollen | Increased testicular volume, focal or diffuse hypoechogenicity, absent twisting of the spermatic cord | Focal or diffuse decrease or absence of testicular blood flow | Focal post-traumatic infarction (due to increased pressure resulting in venous obstruction) |
Testicular torsion | Abrupt onset of severe unilateral testicular or scrotal pain—testicle usually tender, swollen, and slightly elevated because of shortening of the cord from twisting—often nausea and vomiting | Twisting of the spermatic cord (whirlpool sign), redundant spermatic cord in the scrotal sac, testis rotated with increased volume, heterogeneous hyperechogenicity, sometimes hydrocele (anechoic or with debris) and mild-moderate thickening of the scrotal wall. | Diffuse decrease or absence of testicular blood flow (pulsed Doppler: absence or reduced first venous and then arterial blood flow) |
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Montorfani-Janett, V.M.L.; Montorfani, G.E.; Lavagno, C.; Gualco, G.; Bianchetti, M.G.; Milani, G.P.; Lava, S.A.G.; Cristallo Lacalamita, M. External Male Genitalia in Henoch–Schönlein Syndrome: A Systematic Review. Children 2022, 9, 1154. https://doi.org/10.3390/children9081154
Montorfani-Janett VML, Montorfani GE, Lavagno C, Gualco G, Bianchetti MG, Milani GP, Lava SAG, Cristallo Lacalamita M. External Male Genitalia in Henoch–Schönlein Syndrome: A Systematic Review. Children. 2022; 9(8):1154. https://doi.org/10.3390/children9081154
Chicago/Turabian StyleMontorfani-Janett, Valentina M. L., Gabriele E. Montorfani, Camilla Lavagno, Gianluca Gualco, Mario G. Bianchetti, Gregorio P. Milani, Sebastiano A. G. Lava, and Marirosa Cristallo Lacalamita. 2022. "External Male Genitalia in Henoch–Schönlein Syndrome: A Systematic Review" Children 9, no. 8: 1154. https://doi.org/10.3390/children9081154
APA StyleMontorfani-Janett, V. M. L., Montorfani, G. E., Lavagno, C., Gualco, G., Bianchetti, M. G., Milani, G. P., Lava, S. A. G., & Cristallo Lacalamita, M. (2022). External Male Genitalia in Henoch–Schönlein Syndrome: A Systematic Review. Children, 9(8), 1154. https://doi.org/10.3390/children9081154