PEMPix is the American Academy of Pediatrics Section on Emergency Medicine’s annual visual diagnosis competition. This year, in addition to the 10 finalists Maneesha Agarwal will be presenting at the National Conference and Exhibition we will be sharing four cases online in advance of the conference. This is the second of the four cases.

This case was submitted by…

A 6-year-old previously healthy female was brought to your emergency department with extremity pain. 4 days earlier, she developed rhinorrhea, cough, and fever to Tmax 101F. Two days later, she developed a non-pruritic tender rash on her hands and feet the was uncomfortable and interfered with sleep. She was seen at an urgent care the day prior to presentation, diagnosed with a viral exanthem, and discharged with oral prednisone. However on the day of presentation, she had worsening pain and swelling in her hands and feet to the point of being unable to walk due to pain. The family denies any sore throat, abdominal pain, diarrhea, vomiting, conjunctivitis. She had a normal appetite until the day of presentation.

Physical exam

Initial vital signs: T 37.5 °C (99.5 °F), HR 140, RR 12, BP 104/67, sat 99% on room air

The patient was crying and appeared uncomfortable

Moist mucous membranes. Conjunctiva normal. Tympanic membranes are normal

Tachycardic with sinus arrhythmia.  No murmurs, rubs, or gallops

Normal pulmonary effort with lungs clear to auscultation bilaterally

Abdomen was soft, non-tender, scaphoid in appearance, without masses or fluid wave

Normal range of motion of her extremities

Skin exam notable for an erythematous and violaceous macular rash on her upper and lower extremities. The rash is exquisitely tender and associated with non-pitting edema

A. Auto-erythrocyte sensitization
B. Papular purpuric gloves and socks syndrome
C. IgA vasculitis
D. Serum sickness-like-reaction
E. Urticarial vasculitis

B. Papular purpuric gloves and socks syndrome

The patient was diagnosed with papular purpuric gloves and socks syndrome (PPGSS). This is a viral exanthem caused by Parvovirus B19. It is rare and usually seen in young adults rather than children. It presents with acute onset, rapidly progressive, symmetric swelling and erythema of the hands and feet, often with petechiae or purpura. Patients often describe a sensation of burning or stinging pain rather than itching. It may also be associated with fevers and a lacy erythematous macular rash like classic Parvovirus B19.

The diagnosis can be made clinically. Laboratory analyses are rarely helpful, but they may show anemia and thrombocytopenia in more severely affected patients. Symptoms typically resolve over 1 to 2 weeks and the treatment is supportive care. Topical/oral steroids may be prescribed but their benefit is unclear given the rarity of this entity and paucity of evidence.

This patient had labs showing a normal BMP, normal CBC, and slightly elevated inflammatory markers. She was ultimately admitted for 3 days for pain control, which entailed scheduled ketorolac and acetaminophen with oxycodone as needed. She completed two weeks of prednisone taper.

References

Paller, AS, Mancini, AJ. Chapter 16: Exanthematous Diseases of Childhood, In Paller, AS, Mancini, AJ, eds. Clinical Pediatric Dermatology 5th ed, Elsevier, 2016, 382-401.

Sridharan M, Ali U, Hook CC, et al. The Mayo Clinic Experience With Psychogenic Purpura (Gardner-Diamond Syndrome). Am J Med Sci 2019; 357:411.

Gu SL, Jorizzo JL. Urticarial vasculitis. Int J Womens Dermatol. 2021 Jan 29;7(3):290-297.

Leung AK, Chan KW. Evaluating the child with purpura. Am Fam Physician 2001; 64:419.