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.

Read More

Parastomal Ulceration

Presented by Amanda Oakley, Hamilton, NZ

The patient is a 44 year old man with parastomal ulceration over the last 12 months.
He had a stable stoma for 12 years, following colostomy to remove rectal tumour (carcinoid) and is healthy otherwise with no bowel problems and no skin disease elsewhere.

Last year he received IV antibiotics for peristomal cellulitis, with complete recovery. A month or so later the peristomal skin began ulcerating. Partial healing is followed by skin lifting off at bag changes alternate days, leaving painful ulceration. Meticulous hygiene resulted in no improvement with different devices or topical steroid for one month, applied as beclomethasone nasal spray. Now trying clobetasol solution on appliance, allowed to dry before fitting to skin. He is on no medications.

Swab: group G streptococcus on two swabs - no deep fungi or mycobacteria.
Blood screen: all normal - no sign carcinoid or other disease

Clinical Photos (July '09 (top) and February '09 bottom):


Histology: paucicellular; subepidermal clefting. Not diagnostic.

Dr. Oakley's Comments: Most stomal rashes are dealt with by stoma nurses and a dermatologist's opinion is rarely sought; so we don't see many of them. I see irritant dermatitis from time to time and it responds to topical steroids. He has no risk factors for pyoderma gangrenosum, and the histology is not typical of that.

Questions: Could group G streptococcus do this? There is no cellulitis or abscess formation and I have prescribed antibiotics without improvement.
Any suggestions re: diagnosis and treatment will be gratefully received! I am hoping some dermatologists have greater exposure to stomal disease and I can benefit from their experience.

Reference:
Yeo H, Abir F, Longo WE. Management of parastomal ulcers.
World J Gastroenterol. 2006 28;12(20):3133-7.
Management of surgically placed ostomies is an important aspect of any general surgical or colon and rectal surgery practice. Complications with surgically placed ostomies are common and their causes are multifactorial. Parastomal ulceration, although rare, is a particularly difficult management problem. We conducted a literature search using MD Consult, Science Direct, OVID, Medline, and Cochrane Databases to review the causes and management options of parastomal ulceration. Both the etiology and treatments are varied. Different physicians and ostomy specialists have used a large array of methods to manage parastomal ulcers; these including local wound care; steroid creams; systemic steroids; and, when conservative measures fail, surgery. Most patients with parastomal ulcers who do not have associated IBD or peristomal pyoderma gangrenosum (PPG) often respond quickly to local wound care and conservative management. Patients with PPG, IBD, or other systemic causes of their ulceration need both systemic and local care and are more likely to need long term treatment and possibly surgical revision of the ostomy. The treatment is complicated, but improved with the help of ostomy specialists. Note: This reference is available as free full text from the publisher.

Read More

Localized Bullae

This healthy 83 year-old man has had a one year history of bullae and vesicles on the medial aspect of the left knee. They come and go. He is on no new medications and does not take any drugss on an as necessary or intermittent basis. His medications consist of valsartan (Diovan), simvastatin and omeprazole (Prilosec).

O/E: A collection of vesicles and bullae left knee.

Clinical Photo:


Lab and Path:
Bacterial Culture 8/14/o9 Negative
Biopsies were done by two other dermatologists (will try to get results). Apparently, no firm diagnosis was made.

Path Report: 9/30/08 Perivascular and interstitial dermatitis with mixed cell infiltrate including eosiniphils. Dermal hypersensitivity reaction. "These findings may be seen in the prebullous lesions of pemphigoid." DIF was negative.

Diagnosis:
Consider localized bullous pemphigoid.

Discussion: This is an 82 yo man with a one year history of a localized bullous disorder. The initial pathology showed an inflammatory process with eosinophils. The bullous process has stayed localized to the left knee. It seems likely that this is localized bullous pemphigoid. There is a "pretibial variant" and this may be related. Apparently, many of these patients have negative direct immunofluorescence (See ref. below)
Plan to treat initially with clobetasol ointment and if does well follow with tactolimus ointment 0.1%.

Questions: What do you think? What else is in your differential diagnosis? Therapeutic options?

Reference:

Kurzhals G, et. al. [Localized cicatricial bullous
pemphigoid of the Brunsting-Perry type] Hautartz.
1993 Feb;44(2):110-3 [Article in German]
Localized cicatricial pemphigoid of the Brunsting-
Perry type is a very rare bullous condition, which
has so far been reported in 51 cases. It is
characterized by scarring blisters confined to the
head, scalp and neck.  Diagnosis can be difficult
because of the discrete skin lesions, often repeatedly
false-negative direct immunofluorescence, and the
absence of circulating antibodies. We report on a
87-year-old male patient with the typical clinical
feature of a cicatricial pemphigoid of the Brunsting-
Perry type and give a reviewof the 51 cases published
in the world literature.

Follow-up Photo (08/21/09):  The patient was seen after a week for a second visit.  No
treatment had been rendered and the bullae had resolved.  One wonders
what triggers the bullae?  He's a tennis player, so could it be minor
trauma, UVL?

Read More

Tan Papules in a Child

Abstract: Eight year old boy with six month hx of tan macules

HPI: This otherwise healthy boy has had a six-month history of asymptomatic tan macules on his torso. Takes no meds by mouth and is asymptomatic.

O/E: Six - eight smooth surfaced tan macules measuring from 5 mm to 15 mm in diameter on torso. After lesions are rubbed they urticate mildly and are faint pink. Dermographia is negative.

Photos:




























Pathology:

Diagnosis:

Read More

Photodermatitis in a Teenage Girl

Abstract: 8 yr history of photodermatitis in a 17 yo girl

HPI: This 17 yo student has an 8 year history of a summer eruption. She has a pruritic eruption of her hands and distal forearms starting in spring and lasting till late fall. In winter her skin is perfectly normal. She has used triamcinalone 0.1% ointment without relief and similarly has not been helped with sunscreens.

O/E: Mild lichenification and a fine micro-papular eruption on the dorsum of the hands. A few serum crusts. No vesicles or h/o vesicles.

Photos (7/16/09)






Note no facial lesions


Diagnosis: Atypical Photodermatitis in a teenaged girl. Consider PCT, Hydroa variant, photoactivated atopoic dermatitis (nothing fits perfectly at this time)

Plan: 24 hour urine for porphyrins. Broad spectrum sunscreen for hands and clobetasol ointment after a 20 minute soak. Not sure biopsy will be helpful, but will do at next visit. PCT would be unusual but the porphyrias need to be ruled out.

Comments and suggestions?

Read More

Recurrent Toxic Erythema vx. Cellulitis

H.P.I. The patient is a 70 yo woman who has had ~ 10 episodes of a cellulitis-like picture of her legs over the past ten years. She has never had fever or constitutional signs. The process can affect one or both lower extremities. I saw her in 2001 for this and then she was referred by the emergency room yesterday with the same picture. It began with a fall two days ago. She hit her hip -- was in pain and took ibuprofen before this episode. She said she does not recall taking this before other episodes.

O/E: Patchy, fiery erythema of left lower extremity. Very mild erythems of right lower leg (mostly around the ankle).

Clinical Photos:





Labs: Biopsy obtained and CBC ordered

Diagnosis: Is this an atypical cellulitis, or toxic erythema secondary to and NSAID?

Discussion: If the patient remains afebrile and wbc is normal, I'd be inclined to just watch and wait.

Read More

Unusual Pigmentation of Legs

The patient is a healthy 51 yo woman with a 5 - 6 year history of asymptomatic progressive hyperpigmentation of the legs. She is in good general health and takes no medications by mouth. The process started on the calves and has spread proximally to the knees. She has rosacea in addition. She thinks her father has a similar problem.

O/E: Both legs from just above the ankles to the knees show punctate hyperpigmentation. The skin here has a slightly pebbly feel. Other than erythematous papules on both cheeks, the remainder of the cutaneous exam is normal. (There is no sclerodactyly, telangiectasas or sclerotic changes).

Photos:
Affected skin


Digital Zoom


Normal Skin (adjacent)


Dermoscopic Image


Lab: Biopsies of affected and normal skin were taken.

Diagnosis: Punctate Hyperpigmentation of the Legs. This does not look like the "salt and pepper" picture of scleroderma. Could this be an unusual genodermatosis?

Plan: Present to VGRD. Perhaps get serologies for scleroderma.

References: Nothing helpful found on PubMed.

Read More