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.

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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.

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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.

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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.

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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.

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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.

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