Diseases Don't Read Textbooks

Abstact: 5 yo girl with enlarging plaque on back.

HPI: The patient is a 5 year old girl seen on February 27, 2009 with a 10 day history of an enlarging plaque on the left back. She had a similar, but less dramatic lesion in April 2008 which was treated with cefuroxime for two weeks. Her family lives in a wooded area and her mother had Lyme Disease last year. The patient feels well, may have had some mild arthralgias according to her mother. No neurological symptoms. She is allergic to penicillin, amocicillin and sulfonamides.

O/E: 17 x 12 cm plaque left back. 2 x 2 cm plaque right arm. These lesions are somewhat urticarial in appearance. The center of the larger lesion is paler than the periphery.

Photos:


Lab: Lyme titers pending

Diagnosis: Presumptive Lyme Disease. She was started on cefuroxime by her pediatrician.
Questions:
1) What else would you consider in the differential diagnosis
2) Can one have ECM more than once? This child had something similar 10 months ago.
3) Presuming this is Lyme -- how long shoud she be treated?

Reasons Presented: Lyme Disease is unusual in the winter. Can one have "primary lesions" with a reoccurence? In a young patient where tetracyclines are contraindicated with a proven allergy to penicillins, what is the best third line drug and how long to administer.

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Interesting Follow-up: Paronychia in a Child

In October 2007, we presented the case of an eight year old girl with chronic paronychial inflammation located on the left index finger (Paronychia in a Child). She had no other dermatoses. The patient is adopted so we have no family history. We assumed this was some kind of localized psoriasis or acrodermatitis continua. Clobetasol ointment was prescribed which she has used since. (Photo above from 10/2007)

The patient was seen in follow-up recently. The paronycial inflammation had subsided but the finger tip was still abnormal, especially on the palmar surface and there is now hypopigmentation and atrophy distal to the area of inflammation. This latter is likely secondary to the clobetasol. Her topical therapy was switched to calcipotriene cream (the ointment is no longer available in the US.)

Photos:






Questions:
1) What do you think the diagnosis is?
2) Side-effects on the fingers from super-potent topical corticosteroids are rarely reported. One suspects that they are not that unusual. When does the treatment get worse than the disease? (I should have been more diligent in follow-up)
3) Who thinks that these preparations can cause bone changes?
Your comments will be appreciated.

References:
1. Deffer TA, Goette DK.. Distal phalangeal atrophy secondary to topical steroid therapy. Arch Dermatol. 1987 May;123(5):571-2.

2. Tosti A, Fanti PA, Morelli R, Bardazzi F. Psoriasiform acral dermatitis. Report of three cases. Acta Derm Venereol. 1992;72(3):206-7.
Department of Dermatology, University of Bologna, Italy.
The authors report 3 patients affected by psoriasiform acral dermatitis, a distinctive clinical entity characterized by a chronic dermatitis of the terminal phalanges, associated with marked shortening of the nail beds of the affected fingers. The skin biopsy showed in all cases the pathological features of a subacute spongiotic dermatitis. X-ray examination of affected fingers showed no bone or soft tissue changes. Differential diagnosis of psoriasiform acral dermatitis included psoriasis, atopic or contact dermatitis and corticosteroid-induced distal phalangeal atrophy.

3. Brill TJ, Elshorst-Schmidt T, Valesky EM, Kaufmann R, Thaçi D. Successful treatment of acrodermatitis continua of Hallopeau with sequential combination of calcipotriol and tacrolimus ointments. Dermatology. 2005;211(4):351-5.
Department of Dermatology, J.W. Goethe University, Frankfurt, Germany.
Acrodermatitis continua of Hallopeau (ACH) is a rare type of pustular psoriasis affecting the digits. We report on a 43-year-old female patient who had been suffering from ACH for more than 20 years. Despite the fact that the disease was localized on one finger during the whole period, several topical and systemic treatments resulted in only temporary or partial improvement of the lesion. Although the monotherapies with calcipotriol and tacrolimus ointments gave no satisfying results in the long-term management of the disease, the combination of both agents led to a continuous improvement of the patient's skin condition. Copyright 2005 S. Karger AG, Basel.

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R/O Subungual Melanoma

70 yo man referred for suspected subungual melanoma.

HPI: The patient is a retired engineer with a one month history of subungual pigmentation. He suffers from Waldenstrom's macroglobulinemia and peripheral neuropathy. If he had injured his toe, he would not know.

O/E: The left middle toenail shows brown-blackish subungual pigmentation. It was difficult to appreciate if this was melanin or blood both clinically or dermoscopically. Hutshinson's sign is negative.


Dermoscopy before 3 mm punch biopsy

Diagnosis: Probably subungual hematoma. Need to r/o melanoma.

Procedure: Modification of Haneke Technique.

1. Patient soaks foot in warm water for 20 - 30 minutes
2. Carefully drive a 3 mm punch through the nail with care not to cut into the nail bed.
3. Lift off the cut disk of nail and observe the nail bed.


Dermoscopy after 3 mm punch biopsy and H2O2 to defect

In this case, what appeared to be dried blood was present. The area was cleaned with hydrogen peroxide and a normal appearing nail bed was see. There was no pigment noted. Dr. Hanecke's technique utilizes a Hemocult stick to test scraping from underside of nail, however, our strips were outdated and not reliable.

Note: Dr. Eckhart Haneke pioneered this technique but is not acknowledged in the literature. Here are his comments to this case: "Thank you very much for your email and the links, which I saw for the first time. Thank you also for giving me the credit.
You are completely right that we do not even need the hemoccult test strip for the correct diagnosis, but it is very convincing and impresses the patient. And of course, it is one more proof.
Also clinically, as this is no streaky lesion a melanoma is improbable - however, a very fast growing melanoma can appear like this.
When you apply hydrogen peroxide and the pigment disappears this is due to the hemodestructive action of H2O2 on erythrocytes: hemoglobin has a pseudocatalase action splitting H202 into H2O and O. That is why hydrogen peroxide is also a very good disinfective agent and I use it to cleanse my dermatosurgery field from blood."

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Pseudocyst of the Auricle

The patient is a 20 yo college wrestler with a one week history of a painless swelling in the right ear. The area outlined is fluctuant but not inflamed.

This is an auricular pseudocyst. It is overly optimistic to think that simple drainage will ensure cure. A number of therapies have been proposed.

For an excellent (and reasonably succinct) review of this subject see eMedicine/Pseudocyst.

This patient was referred to a plastic surgeon -- I will put an addendum after I learn of the therapy. The problem is that most dermatologists do not have enough experience treating this entity. Inadequate treatment can result is a permanent deformity of the auricle.

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Magic Cure?

Abstract: 45 yo man with two year history of painful fingers
Posted by DJ Elpern
HPI: The patient is a 45 yo electrician and professional pianist who developed hyperkeratotic patches on his hands two years ago. Nothing new in exposures. After much questioning, he remembered that his mother-in-law moved in with them around that time. (not kidding). The fissures are very painful, especially when playing keyboard. He can use gloves doing electrical work. Patch testing has not been done but is planned.
O/E: Hyperkeratotic areas around thumb and middle finger tips bilaterally. Fissures are deep but clean. He has had similar areas on thenar and hypothenar eminences in past. Remainder of cutaneous exam is unremarkable.
Photos:






Diagnosis: Hyperkeratotic Hand Eczema, Psoriasis variant? Fristional Contact Dermatitis
Treatment: He has tried potent topical steroids with occlusion and with the Soak and Smear technique. Crazy glue for fissures. Intralesional triamcinalone 10 mg/cc helped the palmar keratoses. He has had one month of methotrexate 10 mg per week. Only the intralesional TAC has helped but he does not want finger tips injected at this time.
Questions:
1. What do you think the diagnosis is? The role of trauma may be key as he works with his hands as an electrician and his fingers are "traumatized" on the keyboard.
2. Do you have any magical therapeutic suggestions?
3. I have heard that X-ray treatment was used in the past. Any rational for Grenz?
4. Further work-up
Reason Presented: This man is at his wit's end with pain. He can't play the piano since every time he hits a key he has exquisite pain. I have had one or two similar patients -- they just got better over a few years seemingly not related to treatment.
Reference:
E. McMullen, D.J. Gawkrodger, Physical friction is under-recognized as an irritant that can cause or contribute to contact dermatitis. Br J Dermatol. 2006:154;154-156
Department of Dermatology, Royal Hallamshire Hospital, Sheffield U.K.
Full Text of Article
Background The role of physical friction as an irritant in the causation of contact dermatitis is under-recognized. Frictional dermatitis is defined as an eczematous process in which physical frictional trauma contributes to the induction of a dermatitis process.
Objectives To examine the clinical background of patients in whom friction was contributing to dermatitis.
Methods Over a 30-month period during which 2700 new patients were seen, frictional irritancy was identified as playing a role in the dermatosis in 31 cases: in 27 of these, case notes were evaluated for a range of parameters.
Results Physical friction was identified as causing or contributing to the dermatitis in 18 men and nine women, mean age at onset 42 years. The hands, usually the fingers of the dominant hand, were affected in all but two cases. Occupational frictional activities were found in 25 cases: commonly handling small metal components, paper, cardboard or fabric, and driving. Potential frictional activities in hobbies were noted in 12 cases. Wet work irritancy contributed in four cases (15%). Patch testing showed relevant contact allergies as cofactors in seven of 25 subjects tested (26%). Psoriasis was a cofactor in four (15%), and atopic dermatitis in 11. The study was selective, being based in a teaching hospital clinic with a special interest in contact dermatitis. Frictional irritancy is often one of several factors contributing to dermatitis.
Conclusions The contribution of friction to contact dermatitis is under-recognized probably because dermatologists do not think about the potential for physical forces to induce eczematous changes in the skin.

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Onychomadesis

The patient is a 21 yo college student who emailed me around a month ago. He was away at school at the time:
Nov. 15, 2008 Dear Dr. Elpern, I was wondering if you had any idea what this skin rash / irritation is being caused by. On my hands and feet I've got these little red dots scattered all over. They don't itch, but offer a mild pain when applying pressure. Most of them are plush (sic) with the skin, but some of them are raised up slightly. Also my taste buds are inflamed and red... but I think this is an unrelated condition. Any help you could offer would be greatly appreciated.
He wrote back on December 20, 2008: Shortly after writing you the dots seemed to go away, so I didn't bother setting up an appointment; however, although the red dots went away, I did notice that the white half circle, that are supposed to be at the bottom of the nail, seemed to become weird and displaced on both middle fingers. About two weeks went by and nothing really changed. Yesterday things got worse. Both my middle finger nails seem to be falling off at their roots. I'm not sure what's causing this, and I was wondering if you thought I should set up an appointment, or if you think that I should seek help elsewhere.

O/E: The patient was seen on December 23, 2008: At this time, he had a separation of the proximal nail fold of both middle fingers. No other abnormal findings.

Clinical Photos:




Diagnosis: Post viral onychomadesis. The illness he had was most likely Hand, Foot and Mouth Disease or a related enterovirus infection. I have never seen nail dystrophy after this, but onychomadesis has been reported at least three times after similar episodes. One report is of an outbreak in Spain. I wonder if this is not another enterovirus infection.
Question: Has anyone else seen this?
References:
1. Salazar A, et al. Onychomadesis outbreak in Valencia, Spain, June 2008. Euro Surveill. 2008 Jul 3;13(27). pii: 18917. Available Full Text
2. Bernier V, Labrèze C, Bury F, Taïeb A. Nail matrix arrest in the course of hand, foot and mouth disease. Eur J Pediatr. 2001 Nov;160(11):649-51
Onychomadesis describes complete nail shedding from the proximal portion; it is consecutive to a nail matrix arrest and can affect both fingernails and toenails. It is a rare disorder in children. Except for serious generalised diseases or inherited forms, most cases are considered to be idiopathic. Few reports in literature concern common triggering phenomena. We present four patients in whom the same benign viral condition in childhood appeared as a stressful event preceding onychomadesis. In each case, spontaneous complete healing of the nails was achieved within a few weeks. CONCLUSION: Onychomadesis and/or onycholysis is a newly recognised complication in the course of viral infections presenting clinically as hand, foot and mouth disease, and because of mild forms, is probably underestimated.
Clementz GC, Mancini AJ. Nail matrix arrest following hand-foot-mouth disease: a report of five children. Pediatr Dermatol. 2000 Jan-Feb;17(1):7-11.
Hand-foot-mouth disease (HFMD) is a contagious enteroviral infection occurring primarily in children and characterized by a vesicular palmoplantar eruption and erosive stomatitis. Nail matrix arrest has been associated with a variety of drug exposures and systemic illnesses, including infections, and may result in a variety of changes, including transverse ridging (Beau's lines) and nail shedding (onychomadesis). The association of HFMD with Beau's lines and onychomadesis has not been reported previously. Five children, ages 22 months-4 years, presented with Beau's lines and/or onychomadesis following physician-diagnosed HFMD by 3-8 weeks. Three of the five patients experienced fever with HFMD, and none had a history of nail trauma, periungual dermatitis, periungual vesicular lesions, or a significant medication intake history. All patients experienced HFMD within 4 weeks of one another, and all resided in the suburbs of the Chicago metropolitan area. In all patients the nail changes were temporary with spontaneous normal regrowth. The mechanism of the nail matrix arrest is unclear, but the timing and geographic clustering of the patients suggests an epidemic caused by the same viral strain.

Comment: It is likely that this young man's nails will regrow. However, it may take longer than in a young child. All other previous cases have been in children. It is also possible that this is a related virus and not the usual putative agent of HFAM Disease.

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Retroauricular Dermatitis

Abstract: 16 yo boy with 3-4 year history of retroauricular dermatitis
History: This 16-year-old boy was seen for evaluation of a retroauricular dermatitis that has been present for 3-4 years. He is in his usual state of health. He does not have a history of atopy. He does not wear glasses.
O/E: Honey-colored crusting in the superior retroauricular sulci bilaterally.
Clinical Photo:

click image to enlarge
Lab: Culture positive for many Staph. aureus with usual sensitivities.
Histopathology: N/A
Diagnosis or DDx: Retroauricular Dermatitis: This is felt to be a marker for atopic dermatitis or atopy. However, this boy is not atopic and the finding may not be all that specific. There is only one article has appeared on this subject (see Reference).
Treatment: The patient was given a sample tube of retapamulin ointment (Altabax) to use b.i.d. for one week. The next photo shows appearance after one week of use as monotherapy. I plan to now use fluocinalone 0.025% ointment daily for a week or two for the residual dermatitis. This may well recur. The natural history of retroauricular dermatitis is poorly defined. There is only one article in the medical literature that discusses this entity.

status post 0ne week of retapamulin ointment

Questions: Does anyone have any comments on this entity? How often do you see this? I see one or two cases a year.
Reason(s) Presented: For interest. It is curious that there are no more reports on this since it appears to be an entity.
References:
Marks MB, et. al. An unsuspected sign of cutaneous allergy. J Am Acad Dermatol. 1981 May;4(5):519-22.

An eczematous eruption in the superior retroauricular areas of the scalp and often
on the posterior aspects of the pinnas may be seen in about 30% of allergic
children. The eruption is not generally noticed because the overhanging hair covers
the affected areas. The dermatitis is seen mainly in those children afflicted with
bronchial asthma, perennial allergic rhinitis, or both. A previous history of atopic
or seborrheic dermatitis is, as a rule, not elicited.

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