Ear Keloids and Imiquimod

We presented this patient around a year ago (she is patient # 2). The woman, now 19 years old, presented in March of 2008 for a keloidal scar in the left triangular fossa. On 12/18/08 based on suggestions and a report in MEDLINE, the lesion was shave excised and a week after surgery, imiquimod was applied nightly for six weeks. She is now one month out after stopping imiquimod. At this point, she looks very good. We will have to see if this is a long term solution.

Reference:
1. Berman B, Kaufman J. Pilot study of the effect of postoperative imiquimod 5% cream on the recurrence rate of excised keloids. J Am Acad Dermatol. 2002 Oct;47(4 Suppl):S209-11.
New adjunctive treatments are needed to reduce the high recurrence rates (50%) of excised keloids. Interferon alfa injections have been shown to decrease the size of stable keloids. This study examined the effects of postoperative imiquimod 5% cream on the recurrence of 13 keloids excised surgically from 12 patients.Starting on the night of surgery, imiquimod 5% cream was applied for 8 weeks. Patients were examined at weeks 4, 8, 16, and 24 for local erythema, edema,
erosions, pigment alteration, and/or recurrence of keloids. Of the 11 keloids evaluated at 24 weeks, none (0%) recurred. Incidences of hyperpigmentation were 63.6%. Two cases of mild irritation and superficial erosion cleared withtemporary discontinuation of imiquimod. Both patients completed the 8 weeks of topical therapy and the final 24-week assessment. At 24 weeks, the recurrence rate of excised keloids treated with postoperative imiquimod 5% cream was lower than recurrence rates previously reported in the literature.

Nodules in Search of a Diagnosis

Presented by

J. Erin Reid, M.D. Dermatology Resident &
Stephen P. Stone, M.D. Professor of Dermatology
Southern Illinois School of Medicine

Abstract: 70 yo man with a five year history of exophytic nodules on the lower extremities.

HPI: A 70 year old white male presented with a five year history of exophytic nodules on the lower extremities. They were increasing in number. A few had been removed by shave excision, and the areas that were treated did not regrow.
Over the past few years he had numerous nodules measuring up to 4 cm in diameter. A few of them were excoriated and crusted. There was no lymphadenopathy. He also had extensive areas of erythema and scale on his forearms, upper arms, and thighs.
He had been in the Navy over 50 years ago and served in Japan. He also went to Bangkok and Hong Kong 20 years ago. No significant past medical history

O/E: On the pre-tibial area the patient has multiple nodular lesions, as well as some erythematous and hypopigmented scars where previous lesions have been removed by shave excision. The lesions range from 1 cm to 3-4 cm in diameter.

Photos:





Pathology: Many biopsies have been performed. In May, 2006, a biopsy showed “superficial perivascular dermatitis of the mixed type, with eosinophilic spongiosis and pustules consistent with an allergic etiology”.
In February, 2007, biopsies of the right anterior and lateral leg showed “marked epidermal hyperplasia, spongiosis, and mixed intraepidermal and superficial dermal inflammatory cell infiltrate”. There was no evidence of malignancy or infection at that time, but there was evidence of chronic venous stasis change.
In January, 2009, we excised another nodule. This was read as “marked epidermal hyperplasia with acute and chronic inflammation” and was negative for fungal, bacterial and acid fast bacilli stains. There is also no evidence of malignancy or carcinoma. Cultures for fungus, anaerobes and AFP were all negative. Flow cytometry was negative.

Diagnosis: What is your differential diagnosis?
Questions: What further information would you want? What additional studies? How would you treat this man?

References will be added when available.

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.

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.

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."

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.

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.

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.

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.

Scalp Folliculitis in a Patient on Chemotherapy

HPI: This 55 yo woman has had a folliculitis of her scalp for the past 2 - 3 weeks. She is receiving taxol and carboplatinum every three weeks for ovarian cancer and has had two infusions thus far. This eruption began after the second infusion.

O/E: Alopecia secondary to chemotherapy. Scattered over the scalp are erythematous papules and pustules. There are no other lesions other than on the scalp.

Clinical Photos:




Lab: Bacterial culture obtained.

Pathology: Can consider biopsy

Diagnosis: Folliculitis. Probably related to Taxol.

Discussion: A Medline search found one reference to Taxol and folliculitis. This was a case report of two men with folliculitis of the bearded areas and chests after Taxol infusions. Folliculitis is also reported in women on Taxol, but there is no literature available on the subject.

Reason Presented: I discussed her findings with her oncologist who said he sees this picture frequently. It's peculair that there are no case reports. Folliculitis can be bacterial, sterile, fungal or even eosinophilic pustular folliculitis. A biopsy might help. In the absence of guidelines, I started the patient on doxycycline 100 mg. bid. If anyone has seen and treated a similar patient, I would appreciate your insights and recommendations.

Pyoderma Gangrenosum

Abstract: 46 yo man with 1.5 year history of leg ulcers
History: The patient, a disabled 46 yo Cambodian man, has a four year history of poorly controlled ulcerative colitis. He has had painful leg ulcers for the past two years. These begin with pustules or vesicles by history. At present he is taking 1200 mg of Asacol t.i.d. and prednisone 30 mg. per day. In addition to the prednisone he has used potent topical steroids for his ulcers and has been treated at a wound care clinic.
Social History: The patient emigrated from Cambodia 25 years ago. He is married with three children and was employed until he became disabled 2 years ago from colitis and leg ulcers. His English is limited and I had no Cambodian translator.
O/E: There are two ulcers with raised overhanging borders on the left medial malleolus. In addition, there is post-inflammatory hyperpigmentation and proximal scarring secondary to previous ulcerations. The patient has Cushingoid facies.
Clinical Photos:



Lab: N/A
Histopath: N/A
Diagnosis: Pyoderma gangrenosum (P.g.)
Discussion: There is no effective therapeutic protocol for P.g. He has been treated with high dose prednisone for months and his P.g. is only poorly controlled. Super-potent topical steroids have been used without improvement. It seems to us that tacrolimus ointment should be tried because there are many reports of its efficacy with P.g. and it is a more benign therapy than oral cysclsporin or mycophenolate mofetil. Colectomy may be a more permanent solution, but the patient and his gastroenterologists are not ready for that.
Questions: Your suggestions are welcome.
References:
1. eMedicine.com: P.G.

2. Reichrath J, Bens G, Bonowitz A, Tilgen W. Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol. 2005 Aug;53(2):273-83.

Dermatology Clinic, The Saarland University Hospital, Homburg/Saar, Germany. hajrei@uniklinik-saarland.de

Because the incidence of pyoderma gangrenosum (PG) is low, no prospective randomized controlled trials and only a few studies with case numbers of more than 15 patients have been published. To date no guidelines for treatment of PG have been established far. The aim of the study was to provide an evidence-based review of the literature and an evaluation of recommendations for PG treatment. We performed an electronic search using the PubMed database and the term "pyoderma- gangrenosum." Literature published in the English language during the past two decades was reviewed. All relevant studies that could be obtained regardless of the study design were evaluated for grades of recommendation and levels of evidence. Data on patient characteristics including severity of the disease, localization of lesions, associated diseases, and treatment procedures were abstracted and evaluated for therapeutic outcome. We conclude that therapeutic efficacy of systemic treatment with corticosteroids and cyclosporine is best documented in the literature for disseminated as well as for localized disease and should be considered first-line therapy. In cases that do not respond to this treatment, we recommend alternative therapeutic procedures (eg, systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; infliximab; or plasmapheresis), considering additional factors including associated diseases.

Alopecia in a Child

This 11 yo boy has had this alopecic area since infancy. He has been with adoptive parents since he was a baby and his mother says this has been here since coming to live with her. At first, I thought this was a nevus sebaceous, but the scalp looks normal here with none of the raised "pebbly" surface seen in this disorders in older children.



My working diagnosis here is "Congenital Triangular Alopecia."

References: (supplied by Brian Maurer)
1. Elmer KB, George RM. Congenital triangular alopecia: a case report and review. Cutis. 2002 Apr;69(4):255-6.
Congenital triangular alopecia is a nonscarring loss of hair mass on the scalp's temporal regions. The area of hair diminution commonly is described as triangular or lancet shaped. Although previously considered congenital, this condition usually is noticed after 2 years of age and, more recently, is thought to be acquired. We propose that this entity be renamed triangular alopecia. Because this condition involves normal rather than inflamed skin, it does not respond to topical or intralesional steroids. It is important to make the correct diagnosis to avoid unnecessary and potentially harmful interventions. We present the case of a 10-year-old boy with triangular alopecia.

2. Congenital Triangular Alopecia occurring in sisters. Full Text. Original in Portugese

3. García-Hernández MJ, Rodríguez-Pichardo A, Camacho F. Congenital triangular alopecia (Brauer nevus). Pediatr Dermatol. 1995 Dec;12(4):301-3.Department of Medical-Surgical Dermatology and Venereology, Virgen Macarena University Hospital, Seville, Spain.
Abstract: Congenital triangular alopecia is manifested at 3 to 5 years of age by unilateral or, less frequently, bilateral patches of alopecia in the frontotemporal region. At this age the differential diagnosis is important, particularly as regards alopecia areata. Only about 47 cases have been reported, probably because the lesion is benign and nonprogressive. In 6200 patients seen in index visits, we found 7 with triangular alopecia, a frequency of 0.11%. We believe that males do not require treatment because of the later development of androgenic alopecia, but in women, surgical treatment is successful.2. Tosti A. Congenital triangular alopecia. Report of fourteen cases.

4. Tosti A. Congenital triangular alopecia. Report of fourteen cases. J Am Acad Dermatol. 1987 May;16(5 Pt 1):991-3.
Abstract: Fourteen patients affected by congenital triangular alopecia are presented. The clinical and histologic features of this condition are discussed. I suggest that the condition is considerably more common than hitherto has been thought.

Acanthosis Nigricans in a Child

Abstract: 11 yo girl with three year history of acanthosis nigricans

History: This is a healthy 11 y.o. girl. Her mother noticed gradual darkening of skin in neck folds, axillae and groin around three years ago. The child is Chinese. Has not had her first menstrual cycle yet, although has some breast development. She is mildly overweight (not obese). Fitzpatrick Skin Type IV - V. No hirsuitism.
O/E: Velvety hyperpigmentation of skin folds. There are a few skin tags in axillae. Some perioral darkening
Clinical Photo:

Lab: Insulin Level 43 (nl 3 - 28)
Hgb A1C normal, Serum Testosterone Level 75 (normal < style="font-weight: bold;">Histopathology: N/A
Diagnosis or DDx: Acanthosis Nigricans
This child may have AN associated with obesity or a syndromic AN associated with insulin resistance. Too early to say if PCOS is related. We do not have any pediatric endocrinologists in our area, but I feel that she should travel to see one. Dr. Susan Ratzan has kindly given us some guidelines (see below)
Questions: How would you approach this patient and initiate an appropriate work-up? (See Dr. Ratzan's comments below) Is this high insulin level significant?
Reason(s) Presented: To Discuss implications of this diagnosis and work-up.
References:
1. eMedicine.com

2. Hermanns-Lê T, Scheen A, Piérard GE. Acanthosis nigricans associated with insulin resistance : pathophysiology and management. Am J Clin Dermatol. 2004;5(3):199-203.
Departments of Dermatopathology, University of Liège, Liège, Belgium.

The association of acanthosis nigricans, skin tags, diabetes mellitus due to insulin resistance, and obesity in adolescents and young adults represents a well defined syndrome. Hyperandrogenism may also be present. The endocrine origin of this condition is beyond doubt. Insulin and insulin-like growth factor-1, and their receptors on keratinocytes are obviously involved in the complex regulations leading to the peculiar epidermal hyperplasia. This condition is unrelated to other types of acanthosis nigricans, including the congenital and the paraneoplastic types.Control of obesity contributes largely to reverse the whole process, essentially by reducing both insulin resistance and compensatory hyperinsulinemia. Several drugs including metformin, octreotide, retinoids and topical colecalciferol (vitamin D(3)) analogs are also beneficial in clearing acanthosis nigricans.

Comments by Susan Ratzan, M.D. (pediatric endocrinologist):
As far as I am concerned AN is the cutaneous manifestation of hyperinsulinism or insulin resistance. For starters I would get a very good family history for type 2 diabetes, PCOS, hirsutism, infertility, irregular menses, and obesity. You described her very politely as "chunky" but what is her BMI? The way I would document her degree of insulin resistance/carbohydrate tolerance is by doing a 2 hr oral glucose tolerance test with samples at 0, 30, 60, 90 and 120 minutes for BOTH insulin and glucose. At the 0 sample, since she will be fasting, I would also get cholesterol, LDLdirect, triglycerides and HDL(many of these children have the dyslipidemia associated with metabolic syndrome which is elevated TG and low HDL). The best treatment for insulin resistance, but the most difficult to achieve, is a healthier lifestyle, lots of fresh fruits and veggies, healthy oils, fat free milk, no fast food, no soda or other sugar sweetened beverages and juice limited to 4-6 oz/day. We also recommend limiting carbs to 5-6 servings(and they need to be taught what a serving is)/day. If only I could live like this!! Exercise needs to be worked up to an hour a day by turning off the TV and the video games/internet. If the child has glucose intolerance or severe insulin resistance, we use metformin even in children as young as 11, but nothing works as well as lifestyle change.

Hypopigmentation in a Young Child

Presented by Dr. Henry Foong
Ipoh, Malaysia

History: A 6 year old boy presented with patches of hypopigmentation on the trunk and extremities since one year's of age. It was occasionally pruritic. He is otherwise well and has no history of fever or other constitutional symptoms. There was no family history of similar problem and no personal or family history of atopy.

Examination showed the skin was quite dry. Multiple irregular patches of hypopigmentation 2-4 cm diameter were distributed over the back of trunk involving both the lower back extending to the gluteal areas, the arms and anterior chest wall. Sensation was normal in the affected areas.

Photos:

Lab: KOH examination did not reveal hyphae or spores.
Pathology: Biopsy not performed as yet.

Diagnosis: ? Progressive Macular Hypopigmentation ? Pityriasis Alba

Questions: Have you seen this in a young child?? What is your diagnosis and therapeutic recommendations.

Reference:

Progressive macular hypomelanosis: an overview.
Relyveld GN, Menke HE, Westerhof W.
Am J Clin Dermatol. 2007;8(1):13-9.
The Netherlands Institute for Pigment Disorders, Academic Medical Center,
University of Amsterdam, Amsterdam, The Netherlands.

Distinctive Disorder

The patient is a seven-year old girl with a one year history of a linear band of confluent hypopigmented scaly papules. She has Fitzpatrick Skin Type IV.



The clinical diagnosis is Lichen Striatus.

Reference: You can read a good chapter about L.S. on: Emedicine.com

Questions: This does not particularly bother the child or her parents. Would you treat this? And if so with what? A topical corticosteroid? Pimecrolimus? Tacrolimus? Other?

Clam Digger's Legs

Mike LaCombe, a cardiologist from Maine, has a question for our panel.

"G.M. is a 48 y.o. clam digger from Maine. These individuals are exposed to avian schistosomiasis, and on a web search, the consequent clam-digger’s itch has been rarely associated with lymphedema.

Sorry, I have no pictures. This man has well-documented lymphedema of the lower extremities for six months.

All studies negative: CT scans of pelvis, abdomen, chest, ultrasound of leg veins, cardiac echo ruling out any cor pulmonale, and labs showing no evidence of liver disease, hypoalbuminemia.

My questions are:
1. Does anyone have any experience with this?
2. Any specific tests to confirm the diagnosis?
3. Therapy suggestions? "

There are more things in heaven and earth, Horatio, than are dreamt of in your philosophy." Always something new.

Hypopigmentation in an African Child

Katie Ratzan, a third year Dartmouth Medical School student serving as a Schweitzer Fellow at the Schweitzer Hospital in Gabon, Africa, would like help and advice.
" I would like to ask your help with a six year old girl who presented to our clinic at the Hopital Schweitzer, with her father & aunt. The child has a recent onset of hypopigmentation of the left side of her face & neck. As of six to seven weeks ago, her skin was entirely normal. This change in skin color progressed over the past six weeks. It is asymptomatic. She has had no constitutional symptoms. She was not sick during the months/weeks prior to the color change, did not take any medications prior to the skin change, did not travel, did not have an accident with any sort of chemical, does not use anything on her face (i.e. cremes, etc.). No one else around her has anything like this. No one else around her is sick. She's never had this before. She now puts some sort of indigenous healing/darkening creme on the spots on the back of her neck, which is why that is darker than the areas of her face.

By history, this started on her cheek and moved toward her nose. It stops abruptly at midline. It has since spread to her neck and scalp. It's macular/patch-like depending on the confluence of abutting lesions. There is no involvement of mucous membrance (mouth & vagina are normal). She has no trouble with vision, taste, hearing, and her neuro exam (my brief version of it which essentially only tested sensation and gross motor) was normal.





Questions from Katie:
1. Does anyone think this is anything other than vitiligo?
2. Is this segmental vitiligo, and if so what special significance does this have?
3. What therapy would be appropriate for a child like this in this setting?
4. What is known of the psychological and social implications of such hypopigmentation in a girl in this setting?

Thank you,
Katie

One More Unfortunate...

Gina Kaulukukui is a grief counselor on the island of Kauai, Hawaii. She sent me the following text along with these photographs. Your impressions may help to solve this tragic mystery.

“I would like you to review the attached photos and give me your impressions. This 21 y.o woman was found in the water at Tunnels Beach on the north shore of Kauai following a night of partying. She was reportedly in the water about 7-15 minutes when she was pulled out unresponsive. When she was brought to the ER (at least a ½ drive) it was believed that she nearly drowned. She was unresponsive and not expected to survive.

While in the ER she presented on her thighs with unusual markings. The first appeared red jagged rings starting from her bikini line to just above the knee on one side and more toward the inner thigh on the other leg. Both were oval and completely blanched in the middle. There was a slight bruised color line in the center of one of the blanched areas. There were no others marks, lesions, etc.

The next day, the blanching went away and the red ring doubled in size from about 1 inch to about 2 inches. It was very red, raised and angry looking.

Later in the day when I went to check the young lady and convinced the ICU to photograph the area as it was again changing. Where the bruising was, small water blisters began to appear on the one leg, while the markings on the other leg was beginning to disappear. When the doctor was called to look at the blistering, she diagnosed it as a thermal burns.

The attached pictures were taken few hours following her death and as you can see on one side there is no marking left (the red mark on the upper thigh is from her blood pooling and not the original location of the blanching). The other side speaks for it self.

There were never any marks or puncture wounds that would indicate a jelly fish sting. The water was calm that day. She had high levels of cocaine and alcohol in her system. I would love to know what you think. We have yet to receive the biopsy results. I appreciate your time...love and aloha Gina

Distal Onycholysis

Abstract: 76 yo retired nurse with a 1 year history of nail dystrophy.

History: This 76 yo retired registered nurse had distal onycholysis of her right thumb nail a little over a year ago. It eventually "spread" to involve all finger nails. Her medications include lorazepam, citalopram, Premarin and thyroid. All have been taken for many years. She has not used acrylic nails for more than five years. No unusual trauma, but she does use a nail file now. She was seen for around a year by a provider who was treating her with ciclopirox. The patient admits to being very anxious and plays with her nails.

O/E.: The patient is a pleasant, well-groomed woman who appears anxious and concerned. She has distal onycholysis of all finger nails. Toe nails are normal. Scant subungual debris.
Clinical Photos:




Lab: Three KOH preps negative. Fungal cultures were obtained 30 July, 2008.
Pathology: A "few" fungal elements were reported on PASD stained clipping of an affected nail

Diagnosis: Distal onycholysis. I am leaning away from onychomycosis. This would be an unusual presentation. I think this will likely be traumatic onycholysis.

Therapy: Pending culture report, I initiated therapy with 15% sulfactamide in ethanol twice daily. She was asked not to use a nail file and to clip separated portions of nails every day or so. Also, keep hands out of water as much as possible.

Questions: What are your thoughts? Any further work-up?

References:
1. eMedicine.com

Skin Cancer Observation from Baghdad

Case presentation by:
Professor Khalifa Sharquie,
Baghdad, Iraq

I have had the opportunity to see many cases of skin grafting on the face after excision of multiple skin solar keratosis and skin malignancy. Some of these have been in patients with xeroderma pigmentosa (XP). The grafts remained free of actinic disease and have stayed clear for many years, in some cases for more than 20 years. I have never observed them to develop solar damage, solar keratosis or malignancy.

Today, I am presenting one of these cases. A 65 yo man with history of marked sun damage since early life. During the course of his illness, he has developed frequent and multiple solar keratosis and squamous cell carcinoma. Positive family history was seen in his son. Excisions and graftings have been carried out for big cancers since 1982 but he has never developed any solar damage or skin malignancy in the grafts.





Questions:
1. Is it justifiable to excise the skin of such patients with multiple keratosis and malignancies, especially in patients with XP early in life as a part of preventive measures against skin malignancy especially malignant melanoma.
2. What is the mechanism behind this odd observation. Could fibroblasts of the graft share in prevention?
3. Is there any role in the use of imiquimod in these patients? (last question from DJ Elpern)