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.

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.

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

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

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.

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.

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.

Neuropathy Dermatitis

Presented by Professor Khalifa Sharqie
Chief of Dermatology
University of Baghdad, Iraq

Abstract:  Sixty year-old woman with neuropathy dermatitis at the right side of incision scar following right knee-joint replacement.

History: This 60 year-old woman had a right knee joint replacement on 7 Jan 2010. About three months post-op, she noticed non-itchy rash on the right side of the incision scar which has gradually enlarged in size. No other important medical history.

O/E:  I saw the patient on 9 June 2010 with the slightly scaly erythematous rash forming a plaque on the front of the right knee joint, on the right side of the incisional scar only.   It does not cross to other side.  The rash is completely anesthetic as confirmed by neurological assessment, while there is normal sensation on the left side of the scar.

Clinical Photo:

Diagnosis: Neuropathy dermatitis

Comment and question: This is similar to post-bypass dermatitis along one side of the saphenous vein harvesting scar on the front of the leg at the site of sensory neuropaphy. This rash also does not cross to the other side.  The present case seems to confirm that many skin diseases might follow the course of neuropathy like vitiligo and dermatitis.

I would like to ask my colleagues about any similar observations simulating the present case and await their fruitful comments.

References:
1. Sharquie KE. Post-Bypass dermatitis.  October 10, 2009 VGRD Blog

2. Logue EJ 3rd, Drez D Jr. Dermatitis complicating saphenous nerve injury after arthroscopic debridement of a medial meniscal cyst.  Arthroscopy. 1996 Apr;12(2):228-31
Abstract:  We report the case of a patient who developed hypesthesia in the distribution of the saphenous nerve after an arthroscopic debridement of a medial meniscal cyst. Dermatitis developed in the area of the hypesthesia 3 months later, Both complications responded to symptomatic treatment. A review of the literature confirms the unusual nature of these complications.

3. Satku K, Fong PH, Kumar VP, Lee YS. Dermatitis complicating operatively induced anesthetic regions around the knee. A report of four cases. J Bone Joint Surg Am. 1993 Jan;75(1):116-8.  No Abstract available

4. Mathias CG.  Post-traumatic eczema.  Dermatol Clin. 1988 Jan;6(1):35-42.
Abstract:
Thirteen cases of eczema that followed acute cutaneous trauma were observed. On the basis of the present case series, the following conclusions may be drawn: 1. Cutaneous trauma may precipitate eczema. 2. The trauma is sufficient to cause obvious tissue damage accompanied by an inflammatory or regenerative response. 3. Eczema usually begins within a few weeks of acute injury at the site of the cutaneous trauma. 4. Eczema may occur as an isolated idiopathic reaction or as an isomorphic reaction either preceding or following the appearance of an endogenous eczematous condition in nontraumatized skin. 5. Individual lesions of post-traumatic eczema may persist or recur for long periods of time. 6. The occurrence of post-traumatic eczema following occupational injury has important medicolegal implications.

.

35 yo woman with short history of urticarial vasculitis

Abstract: 35 yo woman with three day history of an atypical urticarial eruption

HPI: This 35 yo woman developed an urticarial eruption 8 - 10 days after starting amoxicillin for a dental infection.  At first the lesions blanched with pressure but over the last few days before her office visit the some of the lesions looked hemorrhagic.  She had mild arthralgias but no fever or malaise.

O/E: There was a wide-spread eruption mostly on legs and arms.  On her thighs the lesions appeared hemorrhagic.  The torso, head and neck were mostly spared.

Clinical Photos:

Pathology:  Two 4 mm punch biopsies were obtained from the thighs.  There was a superficial and mid dermal mixed inflammatory infiltrate composed mostly of neutrophils and eosinophils with a few lymphocytes.  The pathology was read as leucocytoclastic vasculitis vs. urticarial vasculitis.
Photomicrographs are 10x, 20x, 40x and courtesy of Dr. Jag Bhawan

Lab:  CBC nl; Chem panel nl; UA nl

Diagnosis:  Most consistent with Drug-Induced Urticarial Vasculitis (UV).

Discussion:  While UV is recognized to present as a cutaneous drug eruption, MEDLINE has no reports of UV from amoxicillin.  In this otherwise healthy woman, this seems to be the best diagnosis.  She was treated with prednisone 20 mg b.i.d. and at one week her skin lesions had completely resolved.  The dose was dropped to 20 mg per day for the second week and then she will stop.  We are aware of cases of presumably drug-induced UV which can last for weeks to months and be associated with hypocomplementemia and positive ANA and antihistone antibodies. Since this woman did well and her process resolved quickly more specialized tests were not done.

Questions:
I don't feel any further work-up is indicated at this point.  If she stays clear the case is probably closed.  If she continues to have UV-like lesions once prednisone is discontinued, a more in-depth work-up will be initiated.  Does anyone feel we should be more aggressive?

Reference:
1. eMedicine.com  Urticarial Vasculitis
2. There are no reports of UV from amoxicillin and only one with ampicillin but it is very vague.

Note:  I will ask the patient to add her comments.

64 Year-old Man with dysesthesia and alopecia

Abstract: 64 yo man with abnormal sensation and localized alopecia on the left thigh.

History:  This 64 yo business man has experienced dysesthesia on the lateral aspect of his left thigh for the past few years.  He has noticed alopecia at the site of his symptoms.  His health is good and he takes no medications by mouth.  For the past 30 years, he has done business in Indonesia and spends two to three months a year there.  There is a history of lower back pain, but no diabetes and no history of trauma.
O/E:  On the left lateral thigh there is a side, 7 cm in diameter area of mild lichenification and alopecia.  The findings are subtle but real.  I am not sure how convincing the photos are.
Clinical Photos:

IMPRESSION:  This is most likely merlagia paresthetica.      

Questions:
The findings are subtle.  Do you accept this diagnosis?  Would a referral to a neurologist be appropriate?  Should the patient just be reassured?

References:
1. Nabavi DG, et. al.. [Meralgia paresthetica. A rare differential diagnosis of circumscribed alopecia]  Dtsch Med Wochenschr. 1996 Jun 21;121(25-26):834-8. ([Article in German])
Klinik und Poliklinik für Neurologie, Universität Münster.
Abstract
HISTORY AND CLINICAL FINDINGS: Two patients with circumscribed alopecia on the lateral aspect of the thigh underwent a neurological investigation after medical and dermatological examinations had failed to establish the cause. Patient 1 also had neuralgia of the genitofemoral nerve after osteotomy of the iliac crest; patient 2 had insulin-dependent diabetes mellitus. Within the affected part of the skin both patients had sensory dysfunctions over the area of distribution of the cutaneous lateral femoral nerve. Patient 2 additionally had sensory dysfunctions in other areas of innervation. INVESTIGATIONS: Neurogram and recordings of sensory evoked potentials revealed decreased amplitudes on the affected side, establishing the diagnosis of meralgia paresthetica. TREATMENT AND COURSE: The painful neuropathy was successfully treated in both patients with carbamazepine (patient 1: 1.600 mg daily; patient 2: 900 mg daily). CONCLUSION: Circumscribed alopecia can be caused by peripheral nerve lesions. It should be considered in the differential diagnosis, particularly as the cause can be easily established.

2. Harney D, Patijn J.   Meralgia paresthetica: diagnosis and management strategies. Pain Med. 2007 Nov-Dec;8(8):669-77.
Department of Anesthesiology and Pain Management, University Hospital Maastricht, Maastricht, The Netherlands. dharney@hotmail.com
Abstract
Meralgia paresthetica (MP), coined from the Greek words meros (thigh and algos), meaning pain, is a neurological disorder characterized by a localized area of paresthesia and numbness on the anterolateral aspect of the thigh. The incidence of MP is more common than often reported in the literature. The etiology of MP includes mechanical factors such as obesity, pregnancy, and other conditions associated with increased intrabdominal pressure, surgery of the spine, and pelvic osteotomy. A coherent history and pertinent physical examination is essential in making the diagnosis; however, red flags such as tumor and lumbar disk herniations must be recognized and appropriately treated. While the diagnosis of MP is essentially a clinical diagnosis, sensory nerve conduction velocity studies are a useful adjunctive diagnostic tool. The management of MP includes treating the underlying cause (if any) and conservative management. Surgery should only be adopted when all nonoperative therapies have failed to manage the condition in an effective manner.

Facial erythema Secondary to Topical Corticosteroids

Abstract:  37 yo man with marked facial erythema who has been using hydrocortisone valerate 0.2% cream (HC valerate) for 20 years.

HPI:  HC valerate was prescribed for a facial eruption when the patient was a teenager.  He's been using it ever since.  Over time, he has developed marked painful facial erythema.

O/E:  There is fiery erythema over the malar eminences, periorbital areas and portions of forehead.  Three weeks after stopping the HC valerate, using cool compresses b.i.d. and minocycline 100 mg b.i.d. the process persists.

Clinical Photo: May 20, 2010 (three weeks after stopping HC valerate

Diagnosis:  Red Face Syndrome.  Facial addiction to topical corticosteroid.

Questions:  Other than abstinence and cold compresses, are there any other treatments you have had success with?  What about topical tacrolimus ointment?

Reference:  The most helpful reference I have found is:
Papaport MJ, Rapaport V. Eyelid dermatitis to red face syndrome to cure: clinical experience in 100 cases.  J Am Acad Dermatol. 1999 Sep;41(3 Pt 1):435-42.
Abstract:
A retrospective review of all eyelid dermatitis patients seen over an 18-year period revealed a large subgroup of patients who had, as the basis for their ongoing problem, an addiction to the use of topical or systemic corticosteroids. This group of 100 patients often sought many consultations with various physicians. Unrelenting eyelid or facial dermatitis often resulted in the use of increasing amounts of corticosteroids for longer periods of time. Soon the skin became addicted. Once the work-up ruled out other causes, the remedy for the problem was absolute total cessation of corticosteroid usage. This article describes the typical history of the problem, the evaluation of these patients, and the distinctive pattern of flaring erythema that ensued when the corticosteroids were ceased. We stress the absolute necessity of total cessation of corticosteroid use as the only treatment for corticosteroid addiction. We also demonstrate that no additional therapy or further consultations were necessary once remission was obtained after topical corticosteroid abuse was halted.

This may be worth a trial:
Goldman D. Tacrolimus ointment for the treatment of steroid-induced rosacea: a preliminary report.  J Am Acad Dermatol. 2001 Jun;44(6):995-8
BACKGROUND: Excessive topical corticosteroid application to facial areas commonly leads to steroid-induced rosacea. This may be a recalcitrant problem that requires months of antibiotic and anti-inflammatory therapy before it resolves. OBJECTIVE: The purpose of this article is to review the use of tacrolimus ointment, a macrolide anti-inflammatory ointment for the treatment of 3 patients with steroid-induced rosacea. METHODS: Three patients with steroid-induced rosacea applied tacrolimus ointment, 0.075% twice daily for 7 to 10 days. Patients were also instructed to avoid topical corticosteroid use and other rosacea-aggravating substances including caffeine, spicy foods, alcohol, hot fluids, and fluoride. Patients were observed for tenderness, erythema, and relief of pruritus. RESULTS: Pruritus, tenderness, and erythema were resolved in all 3 patients after 7 to 10 consecutive days' use of tacrolimus 0.075% ointment in conjunction with avoidance of topical steroids, caffeine, spicy food, alcohol, hot fluids, and fluoride. CONCLUSION: This preliminary study demonstrates that tacrolimus 0.075% ointment may be effective for patients with steroid-induced rosacea, when combined with avoidance of topical steroid use, as well as avoidance of other agents known to aggravate rosacea (caffeine, spicy foods, alcohol, hot fluids, and fluoride).

14 yo boy with a genodermatosis

Presented by:
Drs Israa Al Shawi, FICMS,  & Ali Al Hilaly, DVD
Al_Hashmia Hospital
Babylon,Iraq

Abstract:  14 to boy with photosensitivity and verrucous skin lesions.

HPI:  This 14 yo boy's story started when he was two months old. He developed many bullae on scalp and extremities which healed spontaneously leaving a thickened skin.  Over the years, he also developed many dark brown lesions in a generalized distribution.   He has two sisters (age three and five) and two young cousins (male and female) with the same features.  His parents are cousins and they are unaffected. There are a sister and brother who are normal

O/E:  Large numbers of  brown-black  verrucous  papules  and  plaques distributed  all over the body. These lesions  resemble seborrheic keratosis. He has erythema of sun-exposed areas and thick brown scales of scalp and sides of the face with alopecia.  His body hair is coarse and is reported to sometimes improve spontaneously.

Clinical Photos:

Lab:  None available at present

Pathology: One verrucous papule showed hyperkeratosis, acanthosis, papillomatosis and intranuclear inclusions consistent with verruca vulgaris.

Diagnosis: Not clear

Questions: This  appears to be an autosomal recessive disorder.
If we consider it as Epidermodysplasia verruciformis, what is the explanation for scarring alopecia, photosensitivity and hypertrichosis?
Could this be a patient with Rothmund-Thompson Syndrome?
What are your opinions about the diagnosis?
What further studies can be done to establish the diagnosis, or can this be made clinically?
If genetic testing is indicated, what tests should be done and what kind of samples would they need?

References:
Rothmund-Thomson Syndrome [Internet].
Wang LL, Plon SE.
In: Pagon RA, Bird TC, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-.
1999 Oct 06 [updated 2009 Apr 07].

Excerpt

Disease characteristics. Rothmund-Thomson syndrome (RTS) is characterized by poikiloderma; sparse hair, eyelashes, and/or eyebrows/lashes; small stature; skeletal and dental abnormalities; cataracts; and an increased risk for cancer, especially osteosarcoma. The skin is typically normal at birth; the rash of RTS develops between age three and six months as erythema, swelling, and blistering on the face and subsequently spreads to the buttocks and extremities. The rash evolves over months to years into the chronic pattern of reticulated hypo- and hyperpigmentation, punctate atrophy, and telangiectases, collectively known as poikiloderma. Hyperkeratotic lesions occur in approximately one-third of individuals. Skeletal abnormalities include dysplasias, absent or malformed bones (such as absent radii), osteopenia, and delayed bone formation. Diagnosis/testing. The diagnosis of RTS is established by clinical findings — in particular, the characteristic rash. Routine cytogenetic studies of lymphocytes or skin fibroblasts may reveal mosaic abnormalities of chromosome 8, such as trisomy 8, partial 8q duplication, and tetrasomy 8q, which have been seen in individuals with RTS but are not diagnostic. Skin biopsy may show poikilodermatous changes, which are nonspecific but consistent with RTS. RECQL4 is the only gene associated with RTS to date; although evidence suggests genetic heterogeneity, no other locus for RTS has been identified. Molecular testing of RECQL4 is clinically available. Management. Treatment of manifestations: pulsed dye laser to treat the telangiectatic component of the rash; surgical removal of cataracts; and standard treatment for cancer. Prevention of secondary complications: use of sunscreens with both UVA and UVB protection to prevent skin cancer. Surveillance: annual physical and eye examination, monitoring of skin for lesions with unusual color or texture, screening for osteosarcoma. Agents/circumstances to avoid: excessive sun exposure. Genetic counseling. RTS is inherited in an autosomal recessive manner. At conception, each sib of an affected individual has a 25% chance of being affected, a 50% chance of being an asymptomatic carrier, and a 25% chance of being unaffected and not a carrier. Once an at-risk sib is known to be unaffected, the risk of his/her being a carrier is 2/3. Carrier testing for at-risk relatives and prenatal testing for pregnancies at increased risk are possible if the disease-causing mutations in the family are known.

12 yo Boy with Chest Pain and Skin Lesions

From the Department of Medicine
People's College of Medical Sciences
Bhopal,  India

Abstract: 12 year old boy with shortness of breath, intermittent chest pains and skin lesions.

History:  This 12 year-old boy was admitted to the pediatric service with a three month history of shortness of breath.  He has been having sleepless nights and we witnessed his distress in the echo room when he developed severe chest pain ( no sweating etc) and it remarkably subsided after 5 minutes of standing up after the echo examination!  He has had skin lesions since the age of four.

O/E: We saw him in the echocardiography room. On examination he had these remarkable cutaneous lesions in the elbows, legs and perianal region and over the Achilles tendons. There were reddish-yellow nodules over the extensor aspects of the knees and elbows and discrete subcutaneous nodules over the Achilles tendons.

Clinical Photos:

Lab: Serum cholesterol 641 mg%. His echo showed a global hyopkinesia with dilated left atrium and ventricles.

Diagnosis: Familial Hypercholesterolemia with Tuberous and Tendon Xanthomas.

Questions:
1. What further diagnostic studies are needed?
2. Do you think this is the homozygous variant?
3. We have yet to find a suitable explanation for his variable chest pain that aggravates only on lying down and subsides on standing. Could it be due to a myxomatous tissue near the coronary ostia?
3. What is the evidence surrounding the efficacy of drugs and even LDL apheresis for familial hypercholesterolemia?
5. What are the chances of failure to respond to therapy and what is the long term prognosis?

References:
1. Christopher Sibley and  Neil J Stone . Familial hypercholesterolemia: a challenge of diagnosis and therapy. Cleve Clin J Med. 2006 Jan;73(1):57-64
Abstract
People with familial hypercholesterolemia (FH) have dramatically high levels of low-density lipoprotein cholesterol (LDL-C), which can lead to accelerated atherosclerosis and, if untreated, early cardiovascular death. Although the heterozygous form of FH is often unrecognized, detecting it early can enable risk reduction before premature coronary heart disease occurs.  Available Free Full Text on PubMed


2. Beigel R, Beigel Y. Homozygous familial hypercholesterolemia: long term clinical course and plasma exchange therapy for two individual patients and review of the literature.  J Clin Apher. 2009;24(6):219-24
Heart Institute, Chaim Sheba Medical Center, Tel-Hashomer, Israel. beigelr@yahoo.com
Abstract
Familial hypercholesterolemia (FH) is an autosomal dominant disease. Homozygous FH (HFH) manifests with severe hypercholesterolemia since birth (cholesterol levels >5-6 the upper normal limit), which, if untreated, leads to early onset accelerated atherosclerosis and premature coronary death, usually before the 2nd or 3rd decades of life. Various invasive procedures (iliocecal bypass, porto-caval shunt, liver transplant, and gene therapy) have been introduced for lowering low density lipoprotein (LDL) aiming at reducing atherosclerosis and improving survival of HFH patients. Of all the various methods, LDL apheresis has become the most attractive. Although its impressive effect on LDL-C reduction is well established, its long-term (of more than 10 year) effect on the atherosclerotic process and specifically cardiac end-points in HFH is hardly documented. We herewith report on the longest term lipophoresis so far reported in two HFH patients, each treated with plasma-exchange and LDL-apheresis for more than 20 years. The observations provide an opportunity to focus on various aspects regarding not only the procedure itself but also its effect on various clinical endpoints. By this description together with reviewing the literature, we discuss several issues, some of them are generalized while others are individualized, dealing with the approach of long term LDL apheresis in HFH.