Puzzling Purpura

Abstract: 11 yo with three week history of localized purpura

HPI: This is a healthy, stable 11 year old who has had two episodes of purpura on the upper arms. Mildly pruritic. Her pediatrician reported the family to social services. She's on no meds.

O/E: There are purpuric bruises on both upper arms. The remainder of the cutaneous examination if unremarkable.

Laboratory: All hematologic studies are normal

Pathology: A biopsy was performed. Results pending

Impression: Puzzling Purpura. Her pediatricians were concerned about child-abuse and referred her to social services. Their report found no evidence of this. It is likely that this is due to some kind of intentional or unintentional trauma. See a similar case "Diagnostic Challenge" presented by Dr. Amanda Oakley in 2007. I am also considering Gardner-Diamond syndrome (whatever that really is). Further reading raises the question of a purpuric contact dermatitis from azo and other clothing dyes.

Question: What are your thoughts?
One Week Follow-up:

Note: Cleared completely after one week. This argues for factitial disease (as our readers' felt)









Reference:
1. Rasmussen JE. Puzzling purpuras in children and young adults. J Am Acad Dermatol. 1982 Jan;6(1):67-72.
2. Meeder R, Bannister S. Gardner-Diamond syndrome: Difficulties in the management of patients with unexplained medical symptoms. Paediatr Child Health. 2006 Sep;11(7):416-9. Available full text.

Painful Brusing in a 29 yo Woman

Presented by Hamish Dunwoodie, MBBS
Moncton, New Brunswick, Canada

Abstract: 29 yo woman with one week history of painful bruising on thighs

HPI: The patient is an otherwise healthy 29 yo woman with a one week history of painful bruises on her thighs. Five years ago she had leucocytoclastic vasculitis of her lower legs and very mild proteinuria. A renal consult felt she probably had mild IgA nephropathy. This has cleared. Her only medication is paroxetine, which she has been on for three months. She denies any trauma. The patient is a single mother of two children (11 and 3 years old) and lives alone with her kids. She was in school recently but is now on disability for "seizures" (although she is on no antiepileptic medications at present). She has been assaulted by a boyfriend in the past, but denies trauma this time.

O/E: There is purpura of the lateral thighs bilaterally. No evidence of LCV any longer. The remainder of the cutaneous examination is unremarkable.

Clinical Photo:


Lab: CBC, Chemistries, Urine Analysis all normal save for trace + rbcs. No proteinuria any longer.

Diagnosis: This is most likely traumatic purpura in a young woman who is reluctant todivulge an accurate history. Gardner Diamond Syndrome (autoerythrocyte sensitization syndrome, psychogenic purpura) was considered as well.

Questions: What are your thoughts?

References: (Full Text Online)
1. Gardner-Diamond syndrome: Difficulties in the management of patients with unexplained medical symptoms. Meeder R, Bannister S. Paediatr Child Health. 2006 Sep;11(7):416-9.

2. Gardner-Diamond Syndrome: bruising feeling. Bostwick JM, Imig MW. Mayo Clin Proc. 2008 May;83(5):572. (This is a short article)

Raccoon Purpura

I received this email from an otherwise healthy 23 yo woman who I saw a month ago for an unrelated problem: "I was wondering if you might have any insight to another skin problem I am having. After receiving some terrible news, I have popped a number of blood vessels around my eyes and face to the the point of having dark purple bruises around and on my eyes. I do not know what to do. I look like a victim of abuse and would like to heal my face as soon as possible."

Discussion: One can see eyelid purpura and petechiae with a number of pathologic processes (amyloidosis, coagulopathy) but also after valsalva maneuver, violent vomiting, coughing. I suspect the latter and need more information from the patient. Any thoughts?

Reference: Anesth Analg. 2007 Dec;105(6):1561-3, table of contents.
Periorbital ecchymoses during general anesthesia in a patient with primary amyloidosis: a harbinger for bleeding? Available Free Full Text Online
Weingarten TN, Hall BA, Richardson BF, Hofer RE, Sprung J.
Source
Department of Anesthesiology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Abstract: Primary amyloidosis is a result of proliferation of a population of plasma cells that leads to an increased secretion of monoclonal immunoglobulins (amyloid). Amyloid protein infiltrates increase capillary fragility. Such capillaries can burst, even after minor stress, resulting in periorbital hemorrhage. We describe a 64-yr-old man with primary amyloidosis who underwent general anesthesia. His eyes were gently closed with tape. Upon removal of the tape bilateral periorbital purpura was noted. All coagulation studies were normal. The periorbital hemorrhage was attributed to amyloidosis-induced capillary fragility.

Annals of Puzzling Purpuras

Percussion Purpura -- Taiko Purpura
Submitted by Trudi Shim, San Antonio, Texas

History: The patient is a 63 year-old Japanese-Hawaiian woman who plays Taiko drums as a hobby. She has a past history of porphyria cutanea tarda (PCT) from estrogens which is now quiescent. A few years back, she developed tendonitis of her wrists and received cortisone injections near each wrist from two different physicians. When she developed a hypopigmented patch of skin on her right forearm she attributed it to the cortisone injection. The patch was characterized by an large, flat, smooth area of hypopigmentation, and occasionally some round, bright red spots appeared in the hypopigmented area. They would disappear after a week or two. She consulted a dermatologist a year ago because she worried that this might be contagious. He diagnosed tinea, but did no KOH prep and she has been faithfully applying clotrimazole ever since, but without relief. The dermatologist also raised the spectre of Hansen's Disease which worried the patient, a retired public health nurse, greatly. She does not have any known skin problems elsewhere on her body at this time.

On Examination: The patch is now predominately reddish in color, with sharp irregular borders. The skin is fragile in the sense that if she hits her arm against something, it will break and take a while to heal. - similar to what used to happen way back when she had PCT. This only occurs in the localized area of hypopigmentation.

Clinical Photos:
Patient and Taiko




Discussion: This case presentation contains some interesting points. The patient is a late middle-aged Japanese-Hawaiian woman who has lived in the sub-tropics for her entire life. Thus, she has a fair amount of solar elastosis. The intra-articular corticosteroid most likely caused more localized atrophy and the trauma the arm experienced doing heavy drumming was all she needed to cause purpura. The diagnosis of tinea without a KOH prep showed a certain cavalier approach on the part of her practitioner; and the suggestion of the possibility of Hansen's Disease should not have been made unless a biopsy followed. Leprosy in economically comfortable people born in Hawaii is very unusual. Most cases seen in the Hawaiian Islands today are in immigrants from the Philippines, Oceania and Southeast Asia. Purpura is not a usual finding, but rather a hypopigmented anesthetic plaque (tuberculoid leprosy). Lepromatous leprosy would often appear more dramatically. The patient's PCT is likely not related, but she should have regular liver function studies and ferritin levels looked at. In the absence of other findings urinary porphyrins may not be necessary. "Percussion Purpura" while likely not super rare, has not been reported before.

Comments: Your thoughts and questions will be most welcome.