Search form

Topics:

ubmslateCL-logo-ubm

CL Mobile Menu

Puzzling Purpuras: Tale of an Unusual Bruise

Puzzling Purpuras: Tale of an Unusual Bruise


  • Puzzling Purpuras: A Tale of a Bruiser...
  • Your pediatric hematology colleague asks you to see a young boy with concerning bruising that may or may not be a malignancy or bleeding disorder.
  • The 7-year-old boy's "rash" is of 3 months' duration. Screening CBC was normal. Malignancy can not yet be ruled out.
  • Past medical history, family history, and review of systems are all non-contributory. The child is also completely asymptomatic.
  • Rash began as darkened areas around right ankle that slowly enlarged over 2-3 months.
  • Pertinent findings: Reddish-brown macular lesion over area of distal right fibula and ankle. Non-palpable petechiae on periphery of lesion.
  • Results of laboratory tests are all normal: CBC, platelets, bleeding time, PT/PTT, urinalysis.
  • The macular area appears as a reddish-brown "bruise" and is non-palpable.
  • Note the pin-head sized petechial lesions at the periphery of the involved area.
  • Possible diagnoses? Not a malignancy; not classic Henoch-Schonlein purpura; not fixed drug eruption; bruising is persistent...
  • Differential diagnosis: Schamberg disease. pigmented purpuric lichenoid disorder; purpura annularis telangiectodes (Majocchi disease); lichen aureus; atypical Henoch-Schönlein purpura; eczematoid-like purpura.
  • Schamberg disease is the answer.
  • Schamberg disease: one of the pigmented purpuras; all are considered capillaritis; hemosiderin-laden macrophages seen on histology.
  • Schamberg disease: benign, chronic, can occur at any age; 5 times more common in males; lesions are red-brown punctate ("cayenne pepper spots).
  • Schamberg disease: Occasionally pruritic; tends to be persistent with flare-ups.
  • The pigmented purpuras: Schamberg disease, pigmented purpuric lichenoid disorder (PPLD) of Gougerot and Blum; purpura annularis telangiectodes (Majocchi’s disease); lichen aureus; eczematid-like purpura of Doucas and Kapetankis (itching purpura).
  • Not all that looks like purpura IS purpura; detailed history/physical and appropriate labs plus basic understanding of the pigmented purpuras may help you ease family's anxiety.

An otherwise healthy 7-year-old boy has an odd "rash" on one ankle. The pediatric hematologist has ruled out a number of malignancies and bleeding disorders. The parents are very anxious to find out what's wrong. And now they bring him to you.

In these slides, follow Dr Jonathan Schneider's typically cautious, consistently thorough process to puzzle out the cause of the unusual bruise.

References: 

Recommended reading

►Tristani-Firouzi P, Meadows KP, Vanderhooft S. Pigmented purpuric eruptions of childhood:  a series of cases and a review of the literature.  Pediatr Dermatol. 2001;18:299-304

►Magro CM, Schaefer JT, Crowson AN, Li J, Morrison C. Pigmented purpuric dermatosis: classification by phenotypic and molecular profiles. Am J Clin Pathol. 2007;128:218-229.

►Zvulunov A, Avinoach I, Hatskelzon L, et al. Pigmented purpuric dermatosis (Schamberg's purpura) in an infant.  Dermatol Online J. 1999;5:2.

►Nagata K, Danno K, Tanaka S, Unilateral Schamberg disease in a 14-year-old Japanese boy. J Dermatol. 1999;26:348-351.

►Lasocki AL, Kelly RI. Narrowband UVB therapy as an effective treatment for Schamberg's disease. Australas J Dermatol. 2008;49:16-8 http://onlinelibrary.wiley.com/doi/10.1111/j.1440-0960.2007.00411.x/abstract

►Aiba S, Tagami H. Immunohistologic studies in Schamberg's disease. Evidence for cellular immune reaction in lesional skin. Arch Dermatol. 1988;124:1058–62. doi: 10.1001/archderm.124.7.1058.

►Newton RC, Raimer SS. Pigmented purpuric eruptions. Dermatol Clin. 1985;3:165-9. http://www.ncbi.nlm.nih.gov/pubmed/4092379

►Abeck D, Gross GE, Kuwert C, et al. Acetaminophen-induced progressive pigmentary purpura (Schamberg's disease).  J Am Acad Dermatol, 1992;27:123-4

►Baselga E, Drolet BA, Esterly NB. Purpura in infants and children.  J Am Acad Dermatol. 997;37:673-705 http://www.ncbi.nlm.nih.gov/pubmed/?term=J+Am+Acade+Dermatol.+997%3B37%3A673-705

Comments

I have seen this in adults but never in kids so thanks for the expansion of my knowledge base!

George @

Include a little more information about shamberg disease

Randall @

Very helpful

L.w.ch. @

Add new comment

 
Loading comments...

By clicking Accept, you agree to become a member of the UBM Medica Community.