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.
 

STUMP vs. Melanoma

presented by DJ Elpern and Jag Bhawan

Abstract:  20 yo woman with two month hx of an aggressive melanocytic neoplasm.

HPI:  This otherwise healthy 20 yo college student noticed a papule on her right upper back two months before her dermatology visit.  It rubbed on her bra and that is how she found it.

O/E:  There was a 5 mm diameter brownish papule on the right upper back.  It had no play of color, no asymmetry, and the border was fairly sharp.  Dermoscopically it was not diagnositic of benign melanocytic neoplasm or melanoma.  It was definitely and "outlier" lesion and was rapidly growing.  She had an excisional biopsy the next week.

Clinical Photo:.

Pathology:  
Excisional biopsy revealed an atypical cellular lesion with a nesting pattern, especially in the upper part of the lesion ( Fig 1,2).  The deeper part showed infiltration of reticular dermis (Fig 3 ) as well as arrector pili ( Fig 4 )by islands of atypical cells. The cells were epithelioid, pleomorphic, large with atypical nuclei and several mitoses including in deep dermis ( Fig 5). Focal pigmentation was seen. There was mild epidermal hyperplasia, but no confluence or pagetoid spread of atypical melanocytes was observed. No radial growth phase was noted. These findings were interpreted as malignant melanoma with a depth of 3.5 mm.  Lack of inflammatory response, confluence and pagetoid spread raised the possibility of melanocytic tumors of uncertain malignant potential ( MEL-TUMP ).
Two of the 5 sentinel lymph nodes were positive with atypical nests of melanocytes confirmed by MART-1 within the parenchyma of the lymph nodes. 

Figures 1 - 5 in sequential order (Courtesy of Jag Bhawan)

                                                                   

                                                                         

                                                        

Surgery.  A wide-local excision and SLN biopsy was done and this showed the tumor was completely excised by the first excision and two of 5 nodes were positive.  She will have a PET scan in a few days.


Questions for Panel:
1. Is this a MELTUMP -- Melanocytic tumor of uncertain malignant potential -- also called STUMP Spitzoid tumor of uncertain malignant potential?
2. Does this impart a better prognosis than if this is a MM?  Is thickness less important for these lesions as a prognostic tool?
3. Do MELTUMPs have a different biological behaviors than MMs?
4. If PET scan is negative, would you recommend ELND?
5.  Should her care be managed at this point by an oncologist with an interest in chemotherapy.  Role for interferon?  Can this be cured by chemotherapy or is pharmacologic therapy just palliative?
6.  Many more questions.  Your thoughts are most welcome.

References:
1.  Balch CM, et al.  Multivariate Analysis of Prognostic Factors Among 2,313 Patients With Stage III Melanoma: Comparison of Nodal Micrometastases Versus Macrometastases.  J Clin Oncol. 2010 Apr 5.
Abstract  PURPOSE: To determine the survival rates and independent predictors of survival using a contemporary international cohort of patients with stage III melanoma. PATIENTS AND METHODS: Complete clinicopathologic and follow-up data were available for 2,313 patients with stage III disease in an updated and expanded American Joint Committee on Cancer (AJCC) melanoma staging database. Kaplan-Meier and Cox multivariate survival analyses were performed. RESULTS: Among all 2,313 patients with stage III disease, 81% had micrometastases, and 19% had clinically detectable macrometastases. The 5-year overall survival was 63%; it was 67% for patients with nodal micrometastases, and it was 43% for those with nodal macrometastases (P < .001). Tremendous heterogeneity in survival was observed, particularly in the microscopically detected nodal metastasis subset (from 23% to 87% for 5-year survival). Multivariate analysis demonstrated that in patients with nodal micrometastases, number of tumor-containing lymph nodes, primary tumor thickness, patient age, ulceration, and anatomic site of the primary independently predicted survival (all P < .01). When added to the model, primary tumor mitotic rate was the second-most powerful predictor of survival after the number of tumor-containing nodes. In contrast, for patients with nodal macrometastases, the number of tumor-containing nodes, primary ulceration, and patient age independently predicted survival (P < .01). CONCLUSION: In this multi-institutional analysis, we demonstrated remarkable heterogeneity of prognosis among patients with stage III melanoma, especially among those with nodal micrometastases. These results should be incorporated into the design and interpretation of future clinical trials involving patients with stage III melanoma.

2. Soonh SJ etc.  Predicting Survival Outcome of Localized Melanoma: An Electronic Prediction Tool Based on the AJCC Melanoma Database.  Ann Surg Oncol. 2010 Apr 9. [Epub ahead of print]
Abstract:
BACKGROUND: We sought to develop a reliable and reproducible statistical model to predict the survival outcome of patients with localized melanoma. METHODS: A total of 25,734 patients with localized melanoma from the 2008 American Joint Committee on Cancer (AJCC) Melanoma Database were used for the model development and validation. The predictive model was developed from the model development data set (n = 14,760) contributed by nine major institutions and study groups and was validated on an independent model validation data set (n = 10,974) consisting of patients from a separate melanoma center. Multivariate analyses based on the Cox model were performed for the model development, and the concordance correlation coefficients were calculated to assess the adequacy of the predictive model. RESULTS: Patient characteristics in both data sets were virtually identical, and tumor thickness was the single most important prognostic factor. Other key prognostic factors identified by stratified analyses included ulceration, lesion site, and patient age. Direct comparisons of the predicted 5- and 10-year survival rates calculated from the predictive model and the observed Kaplan-Meier 5- and 10-year survival rates estimated from the validation data set yielded high concordance correlation coefficients of 0.90 and 0.93, respectively. A Web-based electronic prediction tool was also developed ( http://www.melanomaprognosis.org/ ). CONCLUSIONS: This is the first predictive model for localized melanoma that was developed based on a very large data set and was successfully validated on an independent data set. The high concordance correlation coefficients demonstrated the accuracy of the predicted model. This predictive model provides a clinically useful tool for making treatment decisions, for assessing patient risk, and for planning and analyzing clinical trials.

Nail Dystrophy in an Eight Year-Old Girl

Introduction:  In the past, we published a case of localized acrodermatitis continua.  The father of a child with this diagnosis in the U.S. came across our post on VGRD and asked our advice for his daughter.  Your opinions may help with the diagnosis and management of this child.  One can only imagine how this disorder impacts on a young child. Perhaps, one of us has had a favorable outcome with a similar patient.


History:  Please help with an opinion on our eight year-old daughter who has had an acral dermatitis for the past 5 years.   The swelling started at the cuticle and slowly moved back towards the first knuckle over the years and was associated with itching. Initially it was diagnosed as insect bites.  About a year ago her fingers became more swollen and a doctor made a clinical diagnosis of fungus (no tests were done).  She was treated first with vinegar soaks, then triamcinalone cream then Grifulvin 125mg/tsp.  None was effective and we then saw a new dermatologist who referred us to a pediatric dermatologist who she made a diagnosis: Acrodermatitis Continua of Hallopeau.  She did a fungal culture which grew out a soil contaminant that was not felt to be significant.   Our daughter is presently on clobetasol ointment.  The nail looks a bit better but not the skin.  Treatment discussions so far have included Thalidomide, Psoralen plus UVA or UVB, Acitretin, Methotrexate and others.  We know that these medications can have serious side-effects and that this disease can be resistant to treatment.  Our daughter has a lot of finger pain and can't pick up thing with her fingers.  She is only a child and we'd appreciate your thoughts.

Clinical Photos:

Questions:
1) Are there alternative diagnoses?
2) What therapies have you had success within similar cases?
3)  Any further work-up?

Case for Diagnosis

Abstract:  11 y.o. girl with 6 month history of facial eruption
HPI:  This almost 12 yo girl has had a recurring facial eruption for ~ 6 months. In her mother's words: "At first it looked like hives. It was itchy and stung. Each day the rash changed in appearance and lasted almost 3-4 weeks. It traveled behind her ears and neck, then on to her hands and arms and finally to her chest and back. The pediatrician put her on oral steroids which did not seem to help at all.  We went to 2 dermatologists, 3 pediatricians, and an allergist/immunologist. Their opinions varied from poison ivy, to a virus, to having absolutely no idea. The only thing that seemed to work was  hydroxyzine.
The second occurrence happened in February 2010. I gave the hydroxyzine immediately and the symptoms began to disappear within 24 hours.
The next occurrence happened on March 7, 2010. She has had 3 doses of the hydroxyzine and the rash seems to be almost gone.
The patient is on no other medication and has no known allergies. We have racked our brains about everything she eats and all the products we use at home but cannot come up with any rhyme or reason.
Our pediatrician wonders if it is related to the sun......She was outside for recess yesterday and it was the first nice sunny day we have had."

Clinical Photo

Lab: Consider obtaining parvovirus B19-specific antibodies if this has not been done.  CBC was done a few months back.  This and an ANA panel will be obtained.

Questions:  What are your thoughts as to possible diagnoses?  The erythema of the cheeks suggests Erythema infectiosum, but this is almost never recurrent.

Diagnosis: This child's case was presented for ideas.  She was not seen and her parents have had problems getting an appointment with a pediatric dermatologist.  Based on the history and photograph I would consider an atypical erythema infectiosum, urticaria, a collagen vascular disease.

References: 
Musiani M, et. al. Recurrent erythema in patients with long-term parvovirus B19 infection. Clin Infect Dis. 2005 Jun 15;40(12):e117-9. Epub 2005 May 11.
Department of Clinical and Experimental Medicine, University of Bologna, Bologna, Italy. monica.musiani@unibo.it
We describe 3 patients with long-term parvovirus B19 infection (defined as detectable parvovirus B19 DNA load for >6 months after the onset of symptoms), which we monitored by serial testing for parvovirus B19 load and the presence of parvovirus B19-specific antibodies in blood. The patients showed recurrent erythema at intervals of several months.

Note:  Informed consent to present this patient's history and photograph was obtained from her parents.

An Orphan Patient

Abstract:  44 yo man with a 10 year history of a progressive and disabling dermatitis if the feet.
HPI:  This 44 yo professional was first seen 10 years ago with a dermatitis of both feet and nails.  KOH prep from toe nails was positive for hyphae and he was treated with 3 months of Lamisil p.o.  Nails and feet improved at that time.  He was next seen in 2004 with dermatitis of both feet located on plantar areas which was predominantly hyperkeratotic with areas of excoriation.  He had developed a cellulitis of the right leg which required hospitalization.  KOH from affected aeas was negative in 2004.  Treated with betamethasone diproprionate 0.05% ointment and wet compresses and was "80%" improved in two weeks.  At that time a diagnosis of "keratoderma" and possible "dyshidrosis" was considered.  The process recurred and he asked his PCP to place him on prednisone which was done and seemed to help for a while.  From 2004 - 2010 he saw a number of other dermatologists and podiatrists both locally and at a large university center where a number of other therapies were tried, including Castelanni's paint.  None worked for very long and he was seen back at my office in March 2010.  The patient is at the end of his wits with this.  It dominates his life and is the cause of pain which interferes with his ability to stand at work.

O/E:  March 1, 2010:  Symmetrical hyperkeratosis of the plantar aspects of both feet with areas of excoriation.  Nails look normal.  Palms normal.  KOH prep from plantar dermatosis is negative for hyphae and a fungal culture was plated.

Photos March 2010:

Diagnosis:  Is this keratoderma, tylosis or an unusual contact dermatitis? Could this have begun with tinea pedis nine years ago or was than an incidental finding?

Plan:  Patch testing needs to be considered to r/o occult contact.  I doubt biopsy will help.  Will start therapy with Salex Cream (6% salycilic acid) as we await fungal culture.

Questions:  Does anyone have strong feelings about a diagnosis here?  If so, what therapy should be tried? 

Reference:
1. Shelley WB, Shelley ED.  The orphan patient. N Engl J Med. 1988 Mar 10;318(10):646. In this important letter to the NEJM, the Shelleys define the orphan as an individual “with a unique, inchoate, baffling and often disabling disease and yet clearly not discernable in the medical literature.”  While the patient described here is not strictly an "orphan patient" his 10 year unsuccessful quest for control or cure, puts him in that unfortunate category.  Your help will be appreciated.

2. Brian Maurer sent us an important review of "Shoe Dermatitis" by Robert Adams which appeared in California Medicine in 1972.  It is still valuable.

Digital Cameras for Clinical Photography

All one needs for teledermatology is the ability to take good quality digital photos. A fine and timely review of inexpensive digital cameras appeared in the NY Times on February 4, 2010. If you are in the market for a new camera for your office, David Pogue's 2010 Review of Digital Cameras will be helpful. The Canon Power Shot, Fuji FinePix or Nikon Coolpix described in the article seem appropriate, but I'd suspect any of the cameras reviewed will serve the average physician's needs. Be sure to see the multimedia and video attachments to this article.

Henry or I will be happy to answer specific questions.

Traction Alopecia

Abstract: 15 yo girl with one year history of alopecia
HPI: This 15 yo African-American girl has noted progressive alopecia for the past year or so. Earlier in her life her hair was in corn-rows for one to two years. She has used "relaxers" for many years but stopped ~ a year ago. Her hair was pulled back for many years. Her mother has been applying "fish oil" to the area which they think may be helping.
O/E: There is marked thinning of the hair at the temporal and occipital areas. Much less involvement on frontal and parietal areas. No inflammation, scaling or scarring is appreciated.
Photos:







Diagnosis: This is most likely " Marginal Traction Alopecia"
Questions: What would you offer this young woman as for treatment. I told her to leave her hair natural, avoid relaxers or any tension on hair.
References:
1. eMedicine.com has a good chapter on Traction Alopecia: Here is an excerpt: "Traction alopecia is a common cause of hair loss due to pulling forces exerted on the scalp hair. This excessive tension leads to breakage in the outermost hairs. This condition is seen in children and adults, but it most commonly affects African American women. The 2 types of traction alopecia are marginal and nonmarginal. Unlike trichotillomania, a psychiatric disorder of compulsive hair pulling that leads to patchy hair loss, traction alopecia is unintentionally induced by various hairstyling practices (eg, use of braids, hair rollers, weaves, twists, locks, or "cornrows"). In the initial stages, this hair loss is reversible. With prolonged traction, alopecia can be permanent. Physicians, especially dermatologists, must recognize this condition early to prevent irreversible hair loss."

2. I would recommend renting Chris Rock's documentary "Good Hair" when it is available.

Cheilitis in a Young Woman

Abstract: 1.5 year history of cheilitis in a 26 yo woman
HPI: This is a a 23 yo esthetician who first developed cheilitis on her honeymoon in Mexico. She was using many lip balms at the time. These were discontinued and she found toothpaste without cinnamates. The process continued to flare. While initially on upper and lower lips, it is now just on the lower lip. She has read a lot on the subject and has many concerns. The patient has had a problem with anxiety since her father's death when she was 14 and sees a therapist. Cool compresses and fluocinalone 0.025% ointment control the problem fairly well; but it flares when she stops this. At one point, a KOH prep was positive for Candida (but that was when she'd been using a optical corticosteroid and this resolved quickly with ketoconazole cream).
O/E: Recently, the process is located on the lower lip. Here there is erythema, some induaation and scaling.
Clinical Photos: 1/10/1010










This photo was taken on Feb. 8, 2010 after a month off usual lip products and use of Vaseline and/or fluocinalone ointment.

Pathology: A 3 mm punch biopsy was taken from the lower lip.
This shows "confluent scale crust containing neutrophils, acanthosis with spongiosis and a dense lichenoid infiltrate. No granulomatous changes. The pathologist felt that these changes were non-diagnostic but "consistent with cheilitis glandularis."
Diagnosis: Cheilitis. In the ddx is contact, cheilitis glandularis and factitial cheilitis.
Discussion: I am not comfortable with a disgnosis of cheilitis glandularis here. Contact and factitial etiologies could still play a role. Patch testing will be done, but if negative and the problem persists consideration to having more in-depth patch testing may be given. We are also working with patient to stop licking and chewing lips.
Questions: What are your thoughts re: 00etiology here?
Addendum: A few months after this case was presented, the patient recalled that she had received a new dental retainer shortly befor the cheilitis began. Her old retainer was plastic and the new one was metal. She stopped using this new retainer and her cheilitis disappeared. Thus, a metal sensitivity (most likely nickel) was the culprit.
References:
1. Nico MM, Nakano de Melo J, Lourenço SV. Cheilitis glandularis: A clinicopathological study in 22 patients. J Am Acad Dermatol.. [Epub ahead of print]
Department of Dermatology, Medical School, São Paulo, Brazil.
BACKGROUND: Cheilitis glandularis (CG) is a condition in which thick saliva is secreted by minor labial salivary glands and adheres to a swollen lip causing discomfort to the patient. Most publications refer to single case reports or small case series. OBJECTIVE: We sought to report and to analyze clinical, pathological, and therapeutic data on 22 patients with CG seen at the department of dermatology at our university. METHOD: Retrospective data about 22 patients with CG are reviewed and presented. RESULTS: Seventeen patients were male and 5 were female. All were fair skinned, including 6 albino individuals. Several of them presented significant signs of photodamage on the lips. Surgical treatment was performed in 10 severely affected patients and consisted of a vermilionectomy followed by minor salivary gland removal. Histopathological study revealed various degrees of chronic sialadenitis and vermilion epithelial changes. Superficially invasive and in situ squamous cell carcinoma of the vermilion was detected in 3 cases. LIMITATIONS: Biopsy and surgery were not performed in all patients. CONCLUSIONS: CG is strongly related to sun sensitivity and may be more severe in albino patients. The swollen, sun-exposed lip may become more susceptible to the occurrence of squamous cell carcinoma.

2. Aydin E, Gokoglu O, Ozcurumez G, Aydin H. Factitious cheilitis: a case report. J Med Case Reports. 2008 Jan 29;2:29.
Baskent University Department of Otolaryngology, Ankara, Turkey. erdinca@baskent-ank.edu.tr
INTRODUCTION: Factitious cheilitis is a chronic condition characterized by crusting and ulceration that is probably secondary to chewing and sucking of the lips. Atopy, actinic damage, exfoliative cheilitis, cheilitis granulomatosa or glandularis, contact dermatitis, photosensitivity reactions and neoplasia should be considered in the differential diagnosis of crusted and ulcerated lesions of the lip. CASE PRESENTATION: We present a 56 year-old female with an ulcerated and crusted lesion on her lower lip. The biopsy showed granulation tissue and associated inflammation but no malignancy. Based on the tissue examination and through clinical evaluation the diagnosis of factitious cheilitis was rendered. CONCLUSION: Thorough clinical history, utilization of basic laboratory tests and histopathologic evaluation are required to exclude other diseases and a thoruough psychiatric evaluation and treatment is vital for successful management of these patients.

3. Torgerson RR, Davis MD, Bruce AJ, Farmer SA, Rogers RS 3rd. Contact allergy in oral disease. J Am Acad Dermatol. 2007 Aug;57(2):315-21. Epub 2007 May 25.
Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.
BACKGROUND: The role of contact allergy in oral cavity disease processes is unknown. OBJECTIVE: We sought to determine the prevalence of contact allergy to flavorings, preservatives, dental acrylates, medications, and metals in patients with oral disease. METHODS: Patients were tested with an 85-item oral antigen screening series. Data were analyzed retrospectively. RESULTS: We evaluated 331 patients with burning mouth syndrome, lichenoid tissue reaction, cheilitis, stomatitis, gingivitis, orofacial granulomatosis, perioral dermatitis, and recurrent aphthous stomatitis. Positive patch test results were identified in 148 of the 331 patients; 90 patients had two or more positive reactions. Allergens with the highest positive reaction rates were potassium dicyanoaurate, nickel sulfate, and gold sodium thiosulfate. Of the 341 positive patch test reactions, 221 were clinically relevant. LIMITATIONS: No follow-up data were available in this retrospective analysis. CONCLUSION: The positive and relevant allergic reactions to metals, fragrances, and preservatives indicated that contact allergy may affect oral disease.
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Pityrosporon Folliculitis

Abstract: 32 yo woman with 4 day history of folliculitis chest and back
HPI: This 32 year-0ld woman has had a folliculitis for 3 - 4 days. She has a history of ulcerative colitis and had been on prednisone for a few weeks and the dosage was recently increased. She is also on Apriso and Cipro.
O/E: This is a healthy-appearing woman with scores of erythematous papules admixed with a few pustules on upper chest and back. No other findings.
Clinical Photos:




















Diagnosis: Steroid acne vs. Pityrosporon folliculitis.

Biopsy: There are dilated follicles with basophilic debris and numerous PAS positive spores (no hyphae). This is P. folliculitis.













Discussion: She was treated with ketoconazole 200 mg daily for a month. Will put a f/u after she is seen back. Although it seems obvious that the prednisone played a role, there is scant support for this in the literature. We will have to see how the patient does with treatment and whether she suffers recurrences.

Reference:
Lévy A, Feuilhade de Chauvin M, Dubertret L, Morel P, Flageul B. [Malassezia folliculitis: characteristics and therapeutic response in 26 patients] Ann Dermatol Venereol. 2007 Nov;134(11):823-8.

[Article in French]

Service de Dermatologie I, Hôpital Saint-Louis, Paris.

BACKGROUND: Malassezia folliculitis is most often described in patients living in hot and humid countries or in immunocompromised patients. Its frequency in France is unknown. We report 26 cases diagnosed at Saint-Louis Hospital between May 2002 and April 2004. The clinical features, the contributing factors, the results of direct mycological examination and/or histology and the efficacy of antifungal treatments were compared to the literature. PATIENTS AND METHODS: The inclusion criteria were the presence of folliculitis on the trunk confirmed by direct microscopy and/or histopathology showing abundant yeast cells in the follicles. RESULTS: Patients comprised 22 men and 4 women (M/F sex ratio: 5: 5) with a mean age of 46 years. Five patients (19%) were immunocompromised. In normal patients, the duration of folliculitis was long with a mean of 61 months. The eruption was typical, with follicular papules and superficial pustules distributed predominantly on the trunk. Itching was frequent (70%). Direct microscopy was more often positive than histology (89% vs 33%). Some sixty-five percent of the patients had been previously treated by topical or systemic antibiotics or anti-acne drugs, which was ineffective in all cases. Cure with topical ketoconazole, oral ketoconazole alone or in combination with topical ketoconazole occurred respectively in 12%, 75% and 75% of patients, but with consistent recurrence within 3 to 4 months after cessation of treatment. DISCUSSION: Malassezia folliculitis is probably misdiagnosed, as suggested by the long time between onset and diagnosis and the high frequency of non-antifungal treatments prescribed. In our study, direct mycological examination provided more effective diagnosis than histology. Treatment is difficult especially because of the high frequency of relapses. CONCLUSION: A diagnosis of Malassezia folliculitis should be considered in young adults or immunocompromised patients with an itching follicular eruption. Further therapeutic trials are needed due to the frequency of relapse.

Follicular Mucinosis?

Abstract: 27 yo woman with 6 month history of plaques face
HPI: This 27 year-old baker has asymptomatic plaques on the right cheek for six months. She has not been treated as yet.
O/E: Two ill-defined plaques on right cheek. There is a suggestion that follicular openings are dilated.
Clinical Photos:
One can compare the right and left cheeks. Dermoscopic image as well.
Normal left cheek on left, affected right cheek on right.




The image to the right is taken through a dermoscope.
I believe it shows dilated follicular openings.
I am not sure this has been reported.







Diagnosis: Possible Follicular mucinosis

Questions: Do you think this young woman should have a biopsy? Should she be observed? Treated?

Reference:
Gorpelioglu C, Sarifakioglu E, Bayrak R. A case of follicular mucinosis treated successfully with pimecrolimus. Clin Exp Dermatol. 2009 Jan;34(1):86-7.
This paper describes a 24 yo man with biopsy proven follicular mucinosis who was successfully treated with pimecrolimus.

Acneiform Eruption in an 11 year-old boy

Abstract: 11 y.o. boy with localized papular dermatitis of face

HPI: This 11 yo boy has a 2 month history of a facial eruption. Initially, his mother applied Bacitracin ointment. It was not effective and they then used a prescription "hydrocortisone" ointment for a few weeks which was similarly unhelpful. Good general health. He takes Adderal for ADHD.

O/E: There are erythematous acneiform papules around the right alar groove. Face is otherwise clear.

Clinical Photos




Labs: N/A

Clinical Diagnosis: My first thought is that this may be a variant of perioral dermatitis. Prior to puberty, P.O.D. is seen in both sexes. We see women with "perialar dermatitis" which is usually bilateral but can look like this. In the differential diagnosis one would consider Demodicois and atypical acne. Perhaps, I should have done a scraping for demodex mites.
Alternatively, this could be the onset of acne vulgaris, localized at this early stage. Perhaps, I jumped at a zebra when this is just common acne.

Therapy: The patient was given a prescription for doxycycline 100 mg b.i.d. and ketoconazole cream which I have found to be effective for perialar dermatiis. He will be reevaluated in a month.

Questions: Do you think this is periorificial dermatitis, demodicosis, or atypical acne? Your comments will be appreciated.

References:
1. Nguyen V, Eichenfield LF. Periorificial dermatitis in children and adolescents. Volume 55, Issue 5, Pages 781-785 (November 2006)
There has been very little evaluation of the history, morphology, or disease course of perioral/periorificial dermatitis in children.
Objective: We sought to elucidate the clinical manifestations and treatment outcomes in this condition.Methods A retrospective chart review with telephone follow-up was used to study 79 children and adolescents. Results: Patients ranged from 6 months to 18 years of age. The average duration of the rash at presentation was 8 months. Seventy-two percent had a history of topical, inhaled, or systemic steroid exposure. Seventy percent of patients had perioral involvement, 43% perinasal, and 25% periocular involvement. A perivulvar rash was reported in 1% of patients. Treatment with topical metronidazole was associated with clearing on follow-up examination.
Limitations: This is a retrospective study without case controls and is subject to interviewer and memory bias. Conclusion: Perioral dermatitis appears at all ages in childhood and adolescence and may be associated with topical corticosteroid use. It may be responsive to topical metronidazole in children and adolescents and is more appropriately termed periorificial dermatitis.

2. Hsu CK, Hsu MM, Lee JY. Demodicosis: a clinicopathological study. J Am Acad Dermatol. 2009 Mar;60(3):453-62.
Department of Dermatology, College of Medicine, University Hospital, Tainan, Taiwan.
BACKGROUND: Demodex mites are common commensal organisms of the pilosebaceous unit in human beings and have been implicated in pityriasis folliculorum, rosacea-like demodicosis, and demodicosis gravis. OBJECTIVE: We sought to describe the spectrum of clinicopathological findings and therapeutic responses of demodicosis in Taiwanese patients. METHODS: We conducted a retrospective study to review clinicopathologic findings and therapeutic responses of 34 cases of diagnosed demodicosis. RESULTS: Fifteen cases with positive results of potassium hydroxide examination, standardized skin surface biopsy specimen, and/or skin biopsy specimen, and resolution of skin lesions after anti-Demodex treatment were included for final analysis. Nineteen cases were excluded because of insufficient positive data to make a definite diagnosis. There were 4 male and 11 female patients (age 1-64 years, mean age 38.7 years). The disease was recurrent or chronic with a duration ranging from 2 months to 5 years (mean 15.7 months). The skin lesions were acne rosacea-like (n = 8), perioral dermatitis-like (n = 5), granulomatous rosacea-like (n = 1), and pityriasis folliculorum (n = 1). Skin biopsy was performed in 7 patients. Overall, the histopathology was characterized by: (1) dense perivascular and perifollicular lymphohistiocytic infiltrates, often with abundant neutrophils and occasionally with multinucleated histiocytes; (2) excessive Demodex mites in follicular infundibula; and (3) infundibular pustules containing mites or mites in perifollicular inflammatory infiltrate. The skin lesions resolved after treatment including systemic metronidazole, topical metronidazole, crotamiton, or gamma benzene hexachloride. LIMITATIONS: Small sample size and a fraction of patients without long-term follow-up are limitations. CONCLUSION: Demodicosis should be considered in the differential diagnosis of recurrent or recalcitrant rosacea-like, granulomatous rosacea-like, and perioral dermatitis-like eruptions of the face. Potassium hydroxide examination, standardized skin surface biopsy, skin biopsy, or a combination of these are essential to establish the diagnosis.

Does this ring a bell?

HPI: The patient is a 36 yo woman on home hemodialysis after a failed renal transplant for membraneous glomerulonephtritis who was referred by her rheumatologist for a "stat" skin biopsy. She sees the rheumatologist for amyloid nephropathy.

Shs is having the second episode of a painful erythematous eruption on the dorsum of the left foot. The first episode, two months ago, resolved after two weeks of Keflex and Bactrim.

Meds: Oxycontin, prednisone 20 mg daily, Keflex (x 4 days)

O/E: This is an upbeat but chronically ill woman. The pertinent findings are on the legs. There is dusky erythema on the dorsum of the left foot. In addition, there are petechaie on both lower extremities. She has a 2/6 pansystolic murmur which the rheumatologist feels is cardiac but her nephrologist feels radiates from her dialysis shunt (left arm).

Clinical Photos:




Labs: CBC: WBC 8900 normal differential. Creatinine 11.32 (normal < 1.2 mg%")
Biopsy: Performed today

Impression: Second episode of an erythematous process left foot. Although this was treated initially as "cellulitis" no real evidence of infection. This does not look infectious to me. I wonder about an embolic phenomenon. I know I have not seen this before.

Questions:
What is your presumptive diagnosis?
What more would you do at this time?
(On thinking about this patient, and the recurrence, the question of Fixed Drug Eruption arose. I will question her about intermittent prescription and OTC drug use.)

I will give F/U re: bx findings.