ULE?

Abstract: 4 yo girl with 3 week hx of papular eruption left axilla
HPI: This otherwise healthy four year old girl has had a mildly pruritic papular eruption which began in the left axilla with a dermatitic appearance and spread centrifugally where it appeared more papular. There was no antecedent illness. The eruption was first seen on 12/23/2010, cleared after a few days and they reoccurred. The child is not bothered by it. No animals at home and no other family members similarly affected.
O/E: Discrete erythematous papules in the left axilla and surrounding areas. The individual lesions are 3 - 5 mm in diameter. The right axilla and thoracic area are clear. There is left axcillary adenopathy
Clinical Photos: 12/31/2010
Left Axilla

Right Axilla
Lab: none

Diagnosis: Unilateral Laterothoracic Exanthem.

DDx: The lesions look like bites. Against that is the lack of symptoms, the unilateral location, and no likely cause of bites. Contact dermatitis seems unlikely.

Discussion: This is an unusual entity but the child seems well otherwise and I have recommended no treatment for present. I have not seen ULE before (to my knowledge); yet this seems the likely diagnosis. Unfortunately, the pictures in the textbooks are not very good. I suspect, that like Gianotti-Crosti, ULE may be caused by a number of viruses and that it will be difficult to come up with an etiology. The clinical appearance seems to be protean, but the distribution aids in making a diagnosis.

Reference:
1. Emedicine.com has a good chapter, however, they use the name "Asymmetric Periflexural Exanthem of Childhood."

2. McCuaig CC, et. al. Unilateral laterothoracic exanthem. A clinicopathologic study of forty-eight patients. J Am Acad Dermatol. 1996 Jun;34(6):979-84.
Department of Pediatrics, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
Abstract
BACKGROUND: Four years ago, we began seeing young children with an unusual, predominantly unilateral, morbilliform and eczematous, self-limited cutaneous eruption. It appeared to correspond to unilateral laterothoracic exanthem (ULE) reported from France and to an eruption described as "a new papular erythema of childhood" in the United States.
OBJECTIVE: We conducted a prospective study of ULE to define its clinical evolution, pathology, and therapy. In addition, we performed epidemiologic and microbiologic investigations in an attempt to determine the cause of ULE.
METHOD: We studied 48 children with ULE. In some patients, blood, urine, stool, as well as skin biopsy specimens were analyzed.
RESULTS: ULE is a morbilliform, eczematous eruption that often begins close to the axilla and spreads to become bilateral, although it usually retains a unilateral predominance. Patients' mean age at onset is 24.3 months, with a female predominance (2:1) and mean duration of 5 weeks, followed by spontaneous resolution that may or may not be improved with topical corticosteroids. It is characterized by a unique eccrine lymphocytic infiltration. Although signs of infection were reported by most patients, no one infectious agent was identified. No significant epidemiologic factor was found.
CONCLUSION: ULE, in young children, is a self-limited morbilliform and scarlatiniform eruption that may represent a specific skin reaction to one or more infectious agents.

Follow-up Note: The patient's symptoms continued to wax and wane and she was seen by a pediatric dermatologist. At that time, there was just one lesion in the left axilla ( see picture) and a diagnosis of psoriasis was made. There were no other stigmata for psoriasis. If this is the case, the initial picture did not suggest that. Since the sole lesion present now is a plaque in the left axilla, if this turns out difficult to control with topical steroids, consideration to using tacrolimus should be given.





References:
Tacrolimus ointment is effective for psoriasis on the face and intertriginous areas in pediatric patients.
Brune A, Miller DW, Lin P, Cotrim-Russi D, Paller AS. Pediatr Dermatol. 2007 Jan-Feb;24(1):76-80.
Abstract
Children with psoriasis often have involvement of the face and intertriginous areas. While corticosteroids have been the mainstay of treatment for plaque-type psoriasis, the face and intertriginous areas are more sensitive to local effects of topical steroid use such as cutaneous atrophy. Topical tacrolimus has shown promise in adult patients as an alternative antiinflammatory without the cutaneous side effects of steroids. Eleven patients between 6 and 15 years of age with facial or inverse psoriasis were evaluated in a 6-month, single-center, open-label trial. Clinical evaluations were made at baseline and days 30, 90, and 180. Severity was assessed using the physician's global assessment of improvement relative to baseline, a 6-point rating scale for signs of disease (erythema, infiltration, desquamation), and an overall severity score. Within the first 30 days of treatment, every patient had cleared or achieved excellent improvement with the use of tacrolimus ointment. Statistically significant improvement was achieved in each sign of disease and the overall severity score. The only adverse event reported in 6 months of observation was significant pruritus in one patient. We therefore conclude that tacrolimus ointment is an effective treatment for psoriasis on the face or intertriginous areas in children.

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Dermatomal Eruption

Abstract: 39 yo man with two month history of dermatomal eruption.
HPI: This 39 yo man developed a dermatomal vesicular eruption 2 months ago. He was seen by his GP and treated with valcyclovir and it cleared somewhat but not completely. The eruption continues to evolve. He complains of pain and pruritus. Feels well otherwise. No underlying diseases known of.
O/E: There is a dermatomal process extending from T-10 to L 2 on the right side. The lesions are scaly patches. There are no vesicles. The lesions do not cross the mid-line. Area biopsied today is only a couple of days old, by history.
Photos:





























Diagnosis: Atypical Herpes Zoster. H.z. progressing over a two month period (especially after valcyclovir) is quite unusual in a healthy, immunocompetent person.

Plan: I did a biopsy and checked his chemistries and CBC. I have seen patients with HIV/AIDS and a patient with angioimmunoblastic lymphadenopathy with atypical HSV and HZ; but never a patient like this. Perhaps, this is something I am not thinking about. I will post a photomic when path is reported and lab results. For the time being, I prescribed acyclovir 800 mg 5 times a day.

Addendum: Fran Storrs felt this was an eczematous process, possibly a contact dermatitis. The pathology showed no multinucleated giant cells and had features of a "dermatitis." So, was this a dermatitis secondary to H.Z., an atypical contact dermatitis, or factitial (the patient did ask for pain meds when first seen, which were not given)? He is now being treated with a topical corticosteroid now and we'll see how he does. When seen for suture removal sight days after biopsy, the eruption looked a bit better, was less symptomatic and had not spread beyond the dermatomes first involved.

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The Power of BLINCK

Presented by Yoon Cohen MS IV, University of New England, Biddeford, Maine and David Elpern MD, Williamstown, Massachusetts

Abstract: 68 yo woman with 4-6 months history of an atypical melanocytic lesion.

HPI: This healthy 68 yo woman with type II skin presented to the clinic with 4-6 months history of an atypical melanocytic lesion on the left knee. She had noticed an increase in size and change in color and was concerned about these changes in the lesion.

O/E: There was a 7 mm in a diameter asymmetrical brownish macule on the left knee. The lesion showed an irregular border with varied colors.

Clinical photographs:

Dermoscopic images:

Microscopic images:

Dermatopathology report:

The specimen exhibits a proliferation of moderate to severely atypical melanocytes distribubted in irregular nests, as well as singly at and above the dermal epidermal junction, with pagetoid spread to the granular layer and near confluence over at least three rete ridges. These findings support the histologic diagnosis of melanoma-in-situ.

Our appreciation to Dr. Deon Wolpowitz, MD from Boston Univeresity, Dermatopathology, for providing these photomicrographs for the case.

4X

10X

20X

20X

Diagnosis:

Malignant melanoma in situ

Discussion:

The BLINCK approach:

We followed the BLINCK checklist, introduced by Dr. Peter Bourne, a founder of the Skin Cancer College of Australia and New Zealand (SCCANZ). The score for the lesion was added up to 4 by criteria as following.

1. B. The lesion was not clearly benign at our first initial evaluation

2. L. The lesion appeared to be lonely without any other similar melanocytic lesion near by

3. I. The lesion appeared to be irregular outline and color on our dermoscopic exam

4. N & C. The patient was nervous about the change in color in past 4-6 months

5. K. The lesion exhibited known clues when viewed with a dermatoscope. See "Chaos and Clues" reference below.
BLINCK Score = 4

According to the BLINCK approach, a lesion should be biopsied if the BLINCK score is 2 or more out of a possible 4. Therefore, the we excised this lesion and sent for a pathologic evaluation.

According to Dr. Bourne, the BLINCK approach is presented as a simple method to assist the clinician with the decision of whether to biopsy a skin lesion or not. The use of this algorithm will improve the pickup rate of potentially serious skin cancers as well as reduce the number of unnecessary benign lesion excisions. BLINCK may be especially helpful to clinicians who have only basic or intermediate dermoscopy skills but who are regularly called upon to assess skin lesions in their practices.

Questions:

1. Would you consider using the BLINCK approach at your practice?

2. If you already have adapted the BLINCK approach, how have your experiences been?

References:

1. McColl I. BLINCK. http://idsblinck.blogspot.com/2009/11/blinck.html. Updated November 19, 2009. Accessed September 7, 2010

2. Rosendahl C, Kittler H, Cameron A, et al. CHAOS & CLUES - The Algorithm. http://www.chaosandclues.blogspot.com. Updated November 19, 2009. Accessed September 7, 2010

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Palmoplantar Erythrodysesthesia Syndrome

Presented by Yoon Cohen, MS IV
University of New England College of Osteopathic Medicine

Abstract: 45 yo woman with a metastatic breast cancer and a painful hand/foot dermatitis.

HPI: This is a 45 yo woman who was diagnosed with a metastatic breast cancer a few years ago. She has developed "atopic dermatitis" like symptoms on her hands and feet since starting capecitabine. She has completed 5 cycles of Xeloda (capecitabine) and has had to reduce the dose because of this condition. The lesions started as dry edematous, erythematous areas on palms and soles with a tingling sensation.

O/E: The examination reveals general areas of desquamation and hyperlinearity with mild erythema on both palms. This is best shown on the first photograph. 

Clinical photographs:

Diagnosis: Palmoplantar erythrodysesthesia syndrome, hand-foot syndrome, chemotherapy-induced acral erythema

Discussion:

Chemotherapy-induced acral erythema or palmoplantar erythrodysesthesia syndrome is a well-defined reaction to some of the chemotherapeutic agents such as methotrexate, cytarabine, doxorubicin, fluorouracil, cytosine arabinoside, and bleomycin. This reaction is characterized by symmetric, well-demarcated, painful erythema of the palms and soles, which may progress to desquamation or blisters. It appears to be dose dependent, and is likely a direct toxic effect of the drug. Tingling on the palms and soles is followed in a few days by painful, symmetric, well-defined swelling and erythema [4].

Questions:

1. Please feel free to share your experiences with treatment options.

References:

1. Marini A, Hengge UR. Hand-foot syndrome with capecitabine therapy. Hautarzt. 2007 June; 58(6):532-6

Abstract: A 72-year-old patient with esophageal carcinoma developed a severe hand-foot syndrome during second-line therapy with the oral fluoropyrimidine capecitabine. We also summarize the current knowledge with regard to the hand-foot syndrome and distinguish it from palmoplantar erythrodysesthesia.

2. Degen A, Alter M, Satzger I, et al. The hand-foot-syndrome associated with medical tumor therapy- classification and management. J Dtsch Dermatol Ges. 2010 Sep; 8(9):652-61

Abstract: The hand-foot-syndrome (HFS, palmoplantar erythrodysesthesia, chemotherapy-associated acral erythema) is characterized by painful predominantly palmo-plantar lesions. The association with different chemotherapeutic agents has been known for over 20 years. More recently, HFS has been reported in association with regimens using targeted agents, in particular the multikinase inhibitors (MKI) sorafenib and sunitinib. The HFS associated with MKI has a different distribution and clinical appearance than the traditional disorder. In this review, similarities and differences between chemotherapy- and MKI-associated HFS are discussed and current recommendations for their prophylaxis and management are summarized.

3. Janusch M, Fischer M, Marsch WCh, et al. The hand-foot syndrome - a frequent secondary manifestation in antineoplastic chemotherapy. Eur J Dermatol. 2006 Sep-Oct; 16(5): 494-9

Abstract: The hand-foot syndrome (HFS) (palmoplantar erythrodysesthesia) designates acute, painful erythemas of the palms and soles of the feet caused by antineoplastic chemotherapies. The most frequent trigger substances are 5-fluoruracil and its derivates. At maximum severity, the HFS is bullous to erosive or ulcerous in character. The pathogenesis has not yet been clarified. Histologically, the HFS is characterized by a toxic keratinocyte reaction. Furthermore, there is sub-basal edema with a tendency to bullae, dilated blood and lymph capillaries and usually only mild perivascular lymphocytic infiltration. Early recognition and delineation from other differential diagnoses is prerequisite to targeted management of the disease. Depending on the severity, HFS requires dose reduction, interruption or switch in the antineoplastic chemotherapy. (You can access to the article at http://www.john-libbey-eurotext.fr/e-docs/00/04/26/D7/vers_alt/VersionPDF.pdf)

4. Habif TP. Clinical Dermatology. 5th edition. USA: Elsevier Science 2010

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Keratolysis exfoliativa

HPI: The patient is a 27 yo medical assistant who was seen on August 27, 2010 with a six day history of slightly pruritic scaling of the palms.  She is well otherwise and has had no recent illnesses.

O/E: The examination reveals discrete areas of desquamation on both palms.  No vesicles. Soles are normal and remainder of cutaneous exam is unremarkable.

Photos:

Diagnosis: The clinical picture is consistent with Keratolysis exfoliativa.  Strangely, PubMed has only three references to this relatively common disorder, and only one is helpful (see below).  Most dermatologists are familiar with this entity.  There's a brief description on DermNet.

A throat culture taken to r/o post-streptococcal desquamation of the palms was negative.

Refererence:
Recurrent focal palmar peeling.
Lee YC, Rycroft RJ, White IR, McFadden JP.
Australas J Dermatol. 1996 Aug;37(3):143-4.
St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom.
Abstract
Recurrent focal palmar peeling, previously known as keratolysis exfoliativa, is an idiopathic condition characterized by chronic palmar and occasionally plantar peeling. It can be exacerbated by environmental factors, and may be misdiagnosed as chronic contact dermatitis. Accurate diagnosis is from the history and examination. It is supported by a negative patch test result. Three cases of recurrent focal palmar peeling are presented, of which two were misdiagnosed as chronic dermatitis. Although there are few references on recurrent focal palmar peeling, it is likely to be a common condition that rarely presents to dermatologists because it is largely asymptomatic. A correct diagnosis is essential due to the social, occupational and legal implications if misdiagnosed.

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Atypical Lymphocytic Infiltrate

Presented by:
Ron Yaar, Boston University Skin Path and D.J. Elpern


Abstract:  50 yo man with few month history of an asymptomatic nodule on the scalp.

HPI: This 50 yo man has had a slowly enlarging tumor of the mid parietal scalp for three to four months.  No other similar lesions.  He has Type II diabetes and hypertension.  Meds: furosemide, metformin, Diovan, atenolol.


O/E: 15/6/2010  6-7 mm papule w/o diagnostic features mid-parietal
         15/7/2010 (reevaluation) 8 mm firm pink papule.  Difficult to see because covered with hair.

Clinical Photo: 

Pathology:

Image HE – 20x -  Dense, pandermal lymphocytic infiltrate.  Focal crush cell artifact at edges.
Image HE – 100x – A mixed population of cells.  A clear Grenz zone is present.
Image HE – 400x – Smaller lymphocytes mixed with highly pleomorphic cells.
Photos courtesy of Ron Yaar, M.D.

CD3 – Numerous T lymphocytes present.  Higher mag shows that most are smaller cells.
CD20 – Numerous B lymphocytes present.  Higher mag shows many of them correspond to the larger, pleormorphic cells.

H & E  20x

H & E 100x

H & E 400 x

CD 3  40x

CD 3 400x

CD 20  40x

CD 20  400x

Lab:  At this point we don't have any lab results. Will check on his latest CBC.

Diagnosis:  Solitary Atypical Lymphoid Infiltrate.  Benign or Malignant?

Questions:  Have you seen a similar case?  Could this be a reaction to a bite?  How would you approach this?

References:
1. Talpur R, Duvic M.  Atypical lymphoid infiltration occurring at the site of a healed varicella zoster infection.  Clin Lymphoma. 2003 Mar;3(4):253-6.
Abstract:  Herpes zoster infection has been associated with a number of cutaneous reactions. The authors report the first case of a patient with an atypical epidermotropic lymphoid infiltrate that arose within skin previously affected by herpes varicella zoster. The differential diagnosis of such lesions and review of literature on previous cutaneous infiltrates occurring at sites of zoster infection are discussed.

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Facial Excoriations in a 22 yo woman

Presented by Dr. Euan Coig
The Pass, Manitoba


Abstract:  Twenty-two yo homemaker with three to four year history of excoriations on face, arms and chest.

HPI:  This woman's chief complaint was "itching and pimples."  She grew up in a dysfunctional family ~ 100 miles from where she now lives .  Her father was an alcoholic who was physically and verbally abusive to her mother, her younger sister and herself.  She denies sexual abuse.  She was diagnosed with ADHD at age eight and has been treated for that since then with Ritalin.  The also suffers from migraines.  The patient went to college for two years and was studying sociology but ran out of money and dropped out.  She is now married with an 18 month old child and her husband is deployed in Afghanistan with the Canadian forces.

O/E:  The patient is an obese somewhat unkempt young woman. She has excoriations on her face, arms and chest.  Many (mostly atrophic) scars on arms and chest.  Back spared.  Many of the excoriations have serous crusts.

Photographs:

Diagnosis:  Excoriations in a young woman.  This is more serious than acne excoriee.  These type of lesions are self-inflicted but the patient is often not aware of doing this or will deny having done so. Many of these patients (who are almost always woman) have a history or abusive childhoods (physical and/or sexual).  This is a form of "self-harm" behavior.  These patients often fall into a no-man's zone between dermatology and psychiatry and prove difficult to treat.

Questions:  How would you approach a similar patient?

Special Comments:  Here are in-depth comments from two experts in this area, Drs. Anna Luise Kirkengen and Caroline Koblenzer. 

References:
1. Shenefelt PD. Using hypnosis to facilitate resolution of psychogenic excoriations in acne excoriée.  Am J Clin Hypn. 2004 Jan;46(3):239-45. pshenefe@hsc.usf.edu
Abstract:  Hypnotic suggestion successfully alleviated the behavioral picking aspect of acne excoriée des juenes filles in a pregnant woman who had been picking at the acne lesions on her face for 15 years. Acne excoriée is a subset of psychogenic or neurotic excoriation. Conventional topical antibiotic treatment was used to treat the acne. Compared with other treatments for uncomplicated acne excoriée, hypnosis is relatively brief and cost-effective and is non-toxic in pregnancy.

2. Arnold LM, Auchenbach MB, McElroy SL. Psychogenic excoriation. Clinical features, proposed diagnostic criteria, epidemiology and approaches to treatment.  CNS Drugs. 2001;15(5):351-9.
Women's Health Research Program, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA. arnoldlm@email.uc.edu
Abstract:  Psychogenic excoriation (also called neurotic excoriation, acne excoriée, pathological or compulsive skin picking, and dermatotillomania) is characterised by excessive scratching or picking of normal skin or skin with minor surface irregularities. It is estimated to occur in 2% of dermatology clinic patients and is associated with functional impairment, medical complications (e.g. infection) or substantial distress. Psychogenic excoriation is not yet recognised in the DSM. We propose preliminary operational criteria for its diagnosis that take into account the heterogeneity of behaviour associated with psychogenic excoriation and allow for subtyping along a compulsivity-impulsivity spectrum. Psychiatric comorbidity in patients with psychogenic excoriation, particularly mood and anxiety disorders, is common. Patients with psychogenic excoriation frequently have comorbid disorders in the compulsivity-impulsivity spectrum, including obsessive-compulsive disorder, body dysmorphic disorder, substance use disorders, eating disorders, trichotillomania, kleptomania, compulsive buying, obsessive-compulsive personality disorder, and borderline personality disorder. There are few studies of the pharmacological treatment of patients with psychogenic excoriation. Case studies, open trials and small double-blind studies have demonstrated the efficacy of selective serotonin (5-hydroxytryptamine; 5-HT) reuptake inhibitors in psychogenic excoriation. Other pharmacological treatments that have been successful in case reports include doxepin, clomipramine, naltrexone, pimozide and olanzapine. There are no controlled trials of behavioural or psychotherapeutic treatment for psychogenic excoriation. Treatments found to be effective in case reports include a behavioural technique called 'habit reversal'; a multicomponent programme consisting of self-monitoring, recording of episodes of scratching, and procedures that produce alternative responses to scratching; and an 'eclectic' psychotherapy programme with insight-oriented and behavioural components.

3. Arnold LM, McElroy SL, et. al. Characteristics of 34 adults with psychogenic excoriation.  J Clin Psychiatry. 1998 Oct;59(10):509-14.
Biological Psychiatry Program, University of Cincinnati Medical Center, Ohio 45267-0559, USA.
Abstract: BACKGROUND: Psychogenic excoriation, characterized by excessive scratching or picking of the skin, is not yet recognized as a symptom of a distinct DSM-IV disorder. The purpose of this study was to provide data regarding the demographics, phenomenology, course of illness, associated psychiatric comorbidity, and family history of subjects with psychogenic excoriation. METHOD: Thirty-four consecutive subjects were recruited from an outpatient dermatology practice and by advertisement. Subjects completed the Structured Clinical Interview for DSM-IV augmented with impulse control disorder modules, the Yale-Brown Obsessive Compulsive Scale, and a semistructured interview for family history, demographic data, and clinical features. RESULTS: Most subjects were women who described a mean age at onset of 38 years and a chronic course. Subjects excoriated multiple sites, most frequently the face. The behavior caused substantial distress and dysfunction. All 34 subjects met criteria for at least 1 comorbid psychiatric disorder, with a mood disorder the most common. Family histories were notable for depressive disorders and psychoactive substance use disorders. Most subjects experienced both mounting tension before excoriation and relief after excoriation as in impulse control disorders. A minority of subjects excoriated skin as part of obsessive-compulsive disorder. Body dysmorphic disorder with preoccupation about the skin's appearance precipitated excoriation in about a third of subjects. CONCLUSION: Psychogenic excoriation is chronic, involves multiple sites, and is associated with a high rate of psychiatric comorbidity. The behavior associated with the excoriation is heterogeneous and spans a compulsive-impulsive spectrum. Most subjects in this sample described features of an impulse control disorder.

4. Mohammad Jafferany, M.D.  Psychodermatology: A Guide to Understanding Common Psychocutaneous Disorders. Prim Care Companion J Clin Psychiatry. 2007; 9(3): 203–213.
Abstract: More than just a cosmetic disfigurement, dermatologic disorders are associated with a variety of psychopathologic problems that can affect the patient, his or her family, and society together. Increased understanding of biopsychosocial approaches and liaison among primary care physicians, psychiatrists, and dermatologists could be very useful and highly beneficial. This article is available free Full Text.

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