54 yo man with necrotizing vasculitis

Abstract: A 54 year old man presents with long-standing rosacea and a few week history of mildly pruritic papules on his thighs.

HPI: The patient is otherwise well and has been treated with doxycycline for greater than five years for severe rosacea. Recently, it has not been effective. He presented for alternative therapy; and at the time of the visit he mentioned an a pruritic papular eruption of his thighs for two to three weeks. He has had recent onset hypertension and was started on HCTZ about a month ago.

O/E: Erythematous papules and small nodules on face. There are scattered three - four mm papules erythematous papules on the medial thighs. The remainder of the examination is unremarkable.

Clinical Photos:

Subtle Lesions on Thighs

Pathology: Necrotizing vasculitisof deep dermal artery. (Photomicrographs courtesy of Marjan Mirzabeiji, M.D., Boston University Department of Dermatology, Dermatopathology Section)

Lab: CBC normal, Chemistries normal, BUN/Cr normal, ANA 1:1280 Homogenous, ANCA panel negative

Diagnosis: Cutaneous Polyarteritis Nodosa (drug-induced) or microscopic polyangiitis. Doxycycline or HCTZ may be putative.

Discussion: This is an "interesting" case. A man walks in with rosacea and winds up with necrotizing vasculitis. He has some protein in his urine and a positive ANA. There's an old saying: It is often more important to treat the patient who has the disease than the disease the patient has. This may be a case in point.

Questions: What is your diagnosis and what more would you do?

Reference:
Rogalski C, Sticherling M. Panarteritis cutanea benigna--an entity limited to the skin or cutaneous presentation of a systemic necrotizing vasculitis? Report of seven cases and review of the literature. Int J Dermatol. 2007 Aug;46(8):817-21
Abstract: In 1931 Lindberg described a limited and benign subcutaneous form of panarteritis nodosa, which, in contrast to systemic panarteritis, only affects the skin. The terms panarteritis nodosa cutanea benigna, cutaneous polyarteritis nodosa, apoplexia cutanea Freund as well as livedo with nodules are used synonymously for this vasculitis which predominantly affects women in the fifth decade of life. Cutaneous lesions characteristically comprise painful subcutaneous nodules or vasculitis racemosa at the lower extremities. The cutaneous panarteritis may be regarded as its own entity or an isolated skin manifestation within systemic panarteritis nodosa. Full Abstract.

Cutting

Once or twice a month, I see patients with distinctive scars, mostly, but not always, confined to their arms. A recent patient prompted this post. The photo and text are presented with her approval as she feels her story needs to be told.

The patient is a 66 year old insulin dependent diabetic who presented with a dermatitis of the abdomen. It had non-specific features and was KOH negative. Most likely a xerotic eczema or an irritant contact dermatitis. Linear scars were noted on her arms and I questioned her about this. She had been the object of sexual abuse for many years as an adolescent and cutting was her outlet.

Cutting is a form of self-injury (aka nonsuicidal self-injury - NSSI). In my, albeit limited, experience, most cutters are or have been victims of abuse, often sexual abuse. These patients usually are receptive to discussion about their cutting and its causes. Viewing these lesions may be an opportunity to show compassion and understanding for these patients.

Cutting, almost always, is a sign of "Adverse Childhood Experiences." These, ACEs have negative impacts on one's health as an adult and are well described in a New Yorker article, "The Poverty Clinic."

For more on cutting:
Wikipedia
Why People Hurt Themselves
The Poverty Clinic by Paul Tough. There is a full text pdf online which I can not link here.

If you encounter a person with acute cutting, Brief Therapy Heals Trauma in Children, by Jane Brody, is worth reading.

Recurrent BCC with Perineural Invasion

The patient is a 56 yo woman who had micrographic surgery for a BCC on the tip of the nose in August of 2008. The initial typing could not be done b/c the specimen was a superficial shave and deeper component could not be appreciated.

She presented in March 2012 with a subtle area of hypopigmentation at the site of the tumor. Because of the firmness of the nasal tip, induration could not be appreciated. The patient was worried that this might be a recurrence.

Clinical Photo:

A 3 mm punch biopsy showed "infiltrating BCC with perineural invasion (PNI)."

Photomicrographs courtesy of Dr. Jag Bhawan. Please click on Picasa for more images.
Teaching point: The initial shave bx was not adequate to type the lesion and this was also not commented on by Mohs surgeon. Complex BCCs of the nasal tip pose special problems. Dr. highlight some of these.

Questions to Mohs surgeons: How would you approach this woman who is concerned about cosmetic appearance of nose after second Mohs procedure? Is it likely that after almost four years of insidious growth this tumor may pose special problems for closure and necessitate plastic surgical reconstruction?

View Dr. Michael Albom's Comments on this patient.

References:
1. Leibovitch I, et. al, Basal cell carcinoma treated with Mohs surgery in Australia III. Perineural invasion. J Am Acad Dermatol 2005 Sep;53(3):458-63.
Abstract Conclusion: PNI is an uncommon feature of BCC. When present, PNI is associated with larger, more aggressive tumors, and the risk of 5-year recurrence is higher. This emphasizes the importance of tumor excision with margin control and long-term patient monitoring.

2. Geist DE et. al. Perineural invasion of cutaneous squamous cell carcinoma and basal cell carcinoma: raising awareness and optimizing management. Dermatol Surg: 2008 Dec;34(12):1642-51. Division of Dermatology, Department of Medicine, University of Massachusetts Medical School, Worcester, Massachusetts 01605, USA. david.geist@umassmemorial.org

ABSTRACT: BACKGROUND: Perineural invasion (PNI) by cutaneous squamous cell carcinoma (CSCC) and basal cell carcinoma (BCC) is an infrequent but not rare complication of traditionally low-morbidity skin cancers that can lead to catastrophic sequelae; 2.5% to 14% of CSCC and approximately 3% of BCC exhibit PNI. Tumors with PNI tend to be larger, have greater subclinical extension, have a higher rate of recurrence, and have a greater risk of metastases. Tumors with PNI may result in major neurologic deficits.

OBJECTIVE: To review current recommendations for the management of PNI and to evaluate a treatment strategy involving excision using Mohs micrographic surgery (MMS) followed by adjunctive radiotherapy.

MATERIALS AND METHODS:Cases of PNI treated with MMS and radiotherapy were reviewed for recurrence, disease-free follow-up, and adverse events.

RESULTS:Twelve patients with incidental PNI treated with MMS and adjunctive radiotherapy are presented. After 3 to 32 months of follow-up, there had been no recurrences. Adverse events from radiotherapy were minor and self-limited.

CONCLUSIONS: The use of adjunctive radiotherapy in these patients remains controversial. When managing superficial skin tumors with PNI, a multidisciplinary team including a cutaneous surgeon and a radiation oncologist familiar with PNI is recommended.

A 61 year-old disabled mason was seen for evaluation of bugs which had been burrowing into his skin for the past six months. He was an anxious-appearing man with fresh and resolving excoriations on his arms, legs and torso. His medications included lisinopril, oxycodone 60 mg three times a day, oxycodone 15 mg as needed for breakthrough pain, diazepam, montelukast, and various vitamins. Elaborately wrapped samples of the insects were presented for examination, at one point during the office visit, he noticed a dark spot on his right knee which “had just crawled out from under the skin.” A dermoscopic picture of that spot shows that this artifact is comprised of fibers from clothing. On viewing this image, the patient still felt this was insect parts, but he also alluded to his online readings about Morgellon’s disease and speculated that the fibers may have come from his skin.

Dermoscopy is a hitherto unreported aid for examining the artifacts that patients with delusions of parasitosis present to their physicians. A dermatoscope can be quickly attached to a digital camera and the nature of the specimens can be verified. Unfortunately, it is difficult to dissuade these patients of their delusions. Patients with delusions of parasitosis often doctor-shop when their clinicians do not accept their theories of infestation. Medications such as opioids and cocaine can occasionally precipitate formications (the feeling of bugs crawling on his skin) and this man’s high doses of oxycodone may well be related to his fixed ideas.

Reference:
Smith MJ, Thirthalli J, Abdallah AB, Murray RM, Cottler LB. Prevalence of psychotic symptoms in substance users: a comparison across substances. Compr Psychiatry. 2009 May-Jun;50(3):245-50. Epub 2008 Sep 23. Full Text.

Case 2 March 20, 2012
The patient is a 56 year old carpenter with a ten month history of matter being extruded from his groin and scrotum. He feels this is coming from his skin but does not specify the nature of the artefacts. He is very guarded and suspicious of me. He states he does not belong in a skin clinic, but that is where his internist sent him. For the past few months he has been treated with a variety of antifungal creams. He had a bovine aortic valve replacement done a year ago. He presented specimens on a paper towel in a zip-lock baggie. A dermoscopic picture was taken (see below) and when he was told that the material looked like it could have come from the fibers of his blue jeans, he got defensive and left the office a few moments later saying he'd get other opinions. There is one report of Delusions of Parasitosis after cardiac surgery in the literature. What is the risk/benefit relationship of the antipsychotics used to treat this disorder? He is, after all at this time, able to work. It's remarkable how similar this dermoscopic image is to the previous one. In another culture, shamanism might help these people.

See: W.B. Shelley,E.Dorinda Shelley Delusions of parasitosis associated with coronary bypass surgery. British Journal of Dermatology v. 118, p. 309-10, February 1988

Central Centrifugal Cicatricial Alopecia (CCCA)

Abstract: 46 year old Ghanaian woman with scarring alopecia

HPI: The patient is a 46 yo woman from Ghana with 3 - 4 mo history of progressive alopecia. She has lived in the U.S. for ten years, takes no medications p.o. and has used hot combs only infrequently in the past.

The Examination shows patchy areas of complete hair loss on frontal, parietal and vertex areas of the scalp.
Clinical Photos:


Pathology: (Photomicrographs courtesy of Marjan Mirzabeigi, M.D. Department of Dermatopathology, Boston University School of Medicine.)









These show: Marked decrease in the number of follicular units which have been replaced with extensive fibrosis.

Diagnosis: Central Centrifugal Cicatricial Alopecia (CCCA)

Discussion: Dr. Lynn Goldberg, Boston University Department of Dermatology: "The patchy alopecia in the vertex is consistent with CCCA. If the frontal loss is contiguous it could be CCCA, although these patients often have coexistent traction. My first line of therapy is a topical steroid. Most patients will experience stabilization. I reserve ILK and doxycycline for those patients with persistent symptoms and loss, or for those patients who also have pustules, which, in my experience, is infrequent. Some physicians will start with 6 months of topical and intralesional steroids and doxy. There are no controlled trials!

Reference:
Gathers RC, Lim HW. Central centrifugal cicatricial alopecia: past, present, and future. J Am Acad Dermatol. 2009 Apr;60(4):660-8.
Abstract: Clinical scarring alopecia in African American women has been recognized for years. The classification of this unique form of alopecia dates back to Lopresti, who first described the entity called "hot comb alopecia." More recently, the term "central centrifugal cicatricial alopecia" has been adopted to describe a progressive vertex-centered alopecia most common in women of African descent. While this form of hair loss is widely recognized, and may even be on the rise, the causes of central centrifugal cicatricial alopecia are a constant source of debate and remain to be elucidated. This review outlines the descriptive evolution of central centrifugal cicatricial alopecia and the historical controversies ascribed to its pathoetiology; it also examines African hair structure and discusses how hair structure along with common physical and chemical implements utilized by individuals with African hair type may play a causal role in the development of central centrifugal cicatricial alopecia.

Segmental Pityriasis Rosea

Abstract: 32 yo woman with seven week history of a dermatitis on l. chest and breast.

HPI: Her dermatitis began with a single erythematous plaque on the left breast. Within one to two weeks, it spread to the present extent (almost all was on the upper chest and breast). She had used a topical imidazole cream without relief. No new medications.

O/E: There are discrete and confluent scaly erythematous papules and small plaques on left chest and breast. A few similar but more subtle lesions are present on right chest and abdomen.

Lab: KOH prep was negative.

Clinical Pictures: Taken on initial visit and after two weeks.

February 14, 2012

February 27, 2012

Impression: This is probably segmental pityriasis rosea.

Course: She was given clobetasol 0.05% ointment with instructions to use for no more than 7 - 10 days. After three days the lesions were gone and she stopped using the medicine. (Her insurance allows only certain topical corticosteroids, otherwise I would have prescribed a weaker one)

Questions: Do you accept this diagnosis? Any alternative suggestions?

References:
1. Zawar V, Godse K. Segmental lesions in pityriasis rosea: a rare presentation. Skinmed. 2011 Nov-Dec;9(6):382-4.
Skin Diseases Centre, Shreeram Sankul, Opp Hotel Panchavati, Vakilwadi, Nashik, Maharashtra State, India. vijayzawar@yahoo.com

based on a comment by Dr. Zawar, we found the following:
2. Ahmed I, Charles-Holmes R. Localized pityriasis rosea. Clin Exp Dermatol. 2000 Nov;25(8):624-6.
Abstract: Pityriasis rosea is a relatively common skin disorder. In its typical form it is easily recognizable; however, atypical forms can pose diagnostic problems. We report a 44-year-old woman with an acute onset of a localized eruption on her left breast. The morphology of the rash and the time course were typical of pityriasis rosea. Localized pityriasis rosea is an unusual variant, which has been described previously.

Periocular Dermatitis in a Child

Melanie Austin, a pediatrician asks:
"This is a 4 y/o with 10 day history of this rash around only her right eye. No eye discharge. The rash is not bothersome - mother describes at times it looks worse. H/o eczema, keratosis pilaris
I don't recognize the rash and I didn't want to start and topical steroids due to proximity to eye. Mom denies any make up or topical treatments at home...
What are your thought?
Thanks!!

Dialysis Patient with Atypical Ulcers

Abstract: 77 y.o.woman on dialysis with skin ulcers for over a year

HPI: The patient is a 77 yo woman with diabetes and ESRD on hemodialysis for a number of years. She had a diagonsis of porphyria cutanea tarda over four years ago, but no lab studies are available and it's unclear if this was a clinical or laboratory diagnosis. For over a year she has had painful skin ulcers which are located on the abdomen, scalp, breast and hands. These do not heal with good wound care. Her medications include Lantus insulin, sevelamer, cinacalcet (used to treat secondary hyperpartahyroidism), midodrine, risedronate, pravastatin, omeprazole, fenofibrate, famotadine.

O/E: This is a chronically ill-appearing woman. She has a periungual ulceration on her right ring finger. There are ulcers with escars on the abdomen, buttock, sccalp and extremities. The ulcers vary from one to 4 cm in diameter.

Clinical photos (presented with patient's permission)




Photo above is L. buttock and to right is abdomen

Labs; Although a diagnosis of PCT was made four years ago, the only reference to porphyrin levels is that they were "low." We do not have parathyroid hormone or Ca++ levels. These should be available from her nephrologists.

Pathology: Bx. 11/20/11 Read by Dan Carter, M.D.
Histologic changes consistent with "acquired perforating dermatosis of dialysis."

Diagnosis: Atypical Skin Ulcers in a Renal Transplant Patient. The etiology of her ulcers is unclear at this time.

Discussion: The patient is anemic with iron deficiency and her nephrologists are reluctant to treat with i.v. iron because of the past diagnosis of PCT. The current lesions do not look like classic PCT. Nor do they look like perforating dermatosis. They are also quite atypical for calciphylaxis.

Questions: What are your thoughts? Serum porphyrin levels could be done prior to and after iron infusion. Perhaps a deeper biopsy. Has she been checked for hyperparathyroidism? Has anyone seen a case like this?

References: I could find no helpful references for this case.

Update from her nephrologist: After this posting, this woman had a GI bleed and developed very painful decubitus ulcers. Last Friday I spoke with her while she was on dialysis. Her quality of life has been poor and no reasonable expectation that things were going to improve for her. She and I agreed that dialysis was no longer allowing her to have the acceptable quality of life she has had on dialysis for over 12 years. She terminated her dialysis treatment and passed away about 48 hours later.

Isotretinoin: Rare Side-Effects

Isotretinoin (Acutane, RoAcutane, Sotret, Claravis, Amnesteen) is a true wonder drug that has revolutionized the treatment of severe cystinc acne since its introduction in 1980. Over twenty million people have used it. There are some rare idiosyncratic reactions. We welcome comments here from patients, their families and practitioners.

The index case is a is an outgoing 16 yo girl with severe cystic acne with scarring. She was started on isotretinoin 30 mg per day (0.5 mg/kg per day) after routine lab tests were normal. She was on no other medications and had declined oral contraceptives since she is abstinent.

After two weeks, she reported the onset of vivid nightmares. These involved her being in contact with Nazis who were persecuting Christians. (Her step-father is a minister, and she had been studying the Holocaust at school). There is one reference to dreams on isotretinoin, but no reports of nightmares with the drug in the literature. I elected to continue the medication at the same dose and the nightmares resolved.

At week seven of isotretinoin therapy, she started to experience auditory hallucinations. These occurred at school and at home. The voices at home were telling her to kill herself. She developed a mild nosebleed which is a common isotretinoin side-effect the night before an emergency visit to my office, and reported that the voices told her she might as well "finish the job." Scared, she told her parents.

The drug was stopped and the hallucinations disappeared. At her request, she was rechallenged with 10 mg per day. Within a few days, the auditory hallucinations recurred and isotretinoin was discontinued with resolution of symptoms.

Physicians often tend to discount such reports from patients if the reaction is not documented in our medical literature; however patients need to be heard. This case was recently reported.

How many other patients may have had similar symptoms? Is this a coincidence, or a true neuropsychiatric drug-effect?

Have you had an unusual side-effect with isotretinoin? The VGRD Blog may be used as a vehicle to collect singular side-effects to medications, in this case isotretinoin. All responses will be anonymous. We would like to establish a database for idiosyncratic isotretinoin effects that may help other patients and their care givers.

Reference: The Voice of Isotretinoin

25th Hot Spots Program

For More Information see Hot Spots Blog
Special Tuition for all VGRD Members

Erosive Diaper Dermatitis

Here is a tough one:

Abstract: Thirteen yo boy with 6 month history of erosive diaper dermatitis.
HPI: The patient is a 13 yo boy with Down's Syndrome. As an infant he had Hirschprung's disease corrected by surgery. Subsequently, he has had problems with bowel movements and at this time is still incontinent of stool. He needs diapers to prevent soiling. Six months ago, he was diagnosed with Crohn's Disease. Since his colonoscopy, he has developed a painful and recalcitrant diaper dermatitis. See mother's note below*.

O/E: There is an erosive papular dermatitis of the perirectal area. Penis and scrotum not involved.

Clinical Photo:

(the artifact in the photo is a Maalox and Aquaphor mixture)

Diagnosis: Erosive papular dermatitis of perirectal area, most likely "Jacquet's Diaper Dermatitis."

Comment and Questions: The patient was difficult to examine and was very disruptive. Frustrating as the office visit was, I marveled at the patience of his mother for whom he is a full-time job. My experience with erosive diaper dermatitis is limited and this boy's mother told me her son has been seen by three pediatricians and a gastroenterologist and his problem persists. I suppose the Hirschprung's has set up a situation where he is incontinent of stool and his Down's makes management of that even more difficult. Ted Rosen described a similar patient (see references) and the management was fairly simple. I will get Ted's opinion. Please let me know your thoughts.

* Mother's note: Hi Doctor, Thank-you for today's appointment. I'm grateful for your taking the time to research this. My partner and I were at our wits end watching J. suffer with this. I forgot to mention one other product that I use on him is Caldesene powder. This has worked well but only if it is timed just right. Meaning, if we were lucky after applying it, Jarod would stay dry /continent for a long period of time, like through the entire night, and the powder would dry it out and it would begin healing. He has an average of 4 to 5 bowel movements a day, sometimes more sometimes less and his stool is the consistency of toothpaste most of the time. Is there any other info. I could share with you that would be helpful in figuring a treatment plan ?
Thank-you again for your help. I really appreciate it. Sincerely, W.

References:

1. Van L, Harting M, Rosen T. Jacquet erosive diaper dermatitis: a complication of adult urinary incontinence. Cutis. 2008 Jul;82(1):72-4.

Abstract: Jacquet erosive diaper dermatitis is typically described as a severe irritant dermatitis of the perianal region. However, Jacquet erosive diaper dermatitis, perianal pseudoverrucous papules and nodules, and granuloma gluteale infantum/ adultorum have been regarded as discrete entities or all part of the same clinical spectrum, representing the result of chronic, severe, irritant contact dermatitis. We present a case of Jacquet erosive diaper dermatitis and a discussion of the clinical spectrum of diseases to which it belongs.

2. Clinical Presentation and Treatment of Diaper Dermatitis (Full Text)

Atypical Diaper Dermatitis

Abstract: 9 week old infant with recalcitrant diaper dermatitis.

HPI: This child has had a dermatitis which began in the napkin area at ~ 1 month of age. He has been treated with topical Nystatin, clotrimazole cream, Aquaphor and Maalox. No response. New lesions have appeared around umbilicus and neck. His paternal grandfather may have psoriasis.

O/E: There is a sharply demarcated erythematous dermatitis in the pubic, perineal and perirectal area. The umbilicus is involved and there are a few patches in the neck folds. The child is otherwise healthy in appearance.

Clinical Photos:



Diagnosis: I am suspicious of psoriasis or a psoriasiform diaper dermatitis here. This is so well-demarcated and the umbilical lesion may be a clue. I have started him on triamcinalone 0.1% ointment after bath and will reevaluate in a week.

Questions: What alternative diagnoses would you suggest? What may I be missing? Would biopsy be helpful?

References:

Photo after 7 days of TAC 0.1% ointment:

Abstract: 80 yo man with scalp erosions following micrographic surgery.

HPI: The patient is an otherwise healthy 80 yo man who underwent Mohs surgery on November 16, 2011 for a basal cell carcinoma of the mid-parietal area of the scalp. The large defect needed a complex closure. Within a few days there was some evidence of inflammation and a wound culture grew out staph aureus sensitive to methicillin but resistant to penicillin, clincamycin and erythromycin. He was treated with cephalexin and seemed to do well, but presented on January 19, 2012 with thick crusts along a portion of the scar (unfortunately not photographed). He feels well otherwise.

O/E: 1/19/2012. There were thick honey-colored crusts in a linear distribution over ~ 1/2 of the "S" closure. The crusts were lifted off with a number 15 blade and the base was covered with creamy pus which was cultured and cleansed. The base was glistening granulation tissue, in some areas eroded in others raised.

Clinical Photo after very gentle debridement

Culture Report: Pending

Diagnosis: Erosions secondary to subacute infection. Role of subcuticular sutures may be key. Possible erosive pustular dermatosis of the scalp secondary to inadequately treated infected Mohs wound.

Plan: At this time will wait for culture report and then treat with an appropriate antibiotic. I will debride the hypergranulation tissue and consider using a topical steroid as recommended for erosive pustular dermatosis of the scalp.

Your Comments will be appreciated.



2/22/12 Healed after Keflex 500 mg b.i.d. x 2 weeks and H2O2 cleansing

Puzzling Purpura

Abstract: 11 yo with three week history of localized purpura

HPI: This is a healthy, stable 11 year old who has had two episodes of purpura on the upper arms. Mildly pruritic. Her pediatrician reported the family to social services. She's on no meds.

O/E: There are purpuric bruises on both upper arms. The remainder of the cutaneous examination if unremarkable.

Laboratory: All hematologic studies are normal

Pathology: A biopsy was performed. Results pending

Impression: Puzzling Purpura. Her pediatricians were concerned about child-abuse and referred her to social services. Their report found no evidence of this. It is likely that this is due to some kind of intentional or unintentional trauma. See a similar case "Diagnostic Challenge" presented by Dr. Amanda Oakley in 2007. I am also considering Gardner-Diamond syndrome (whatever that really is). Further reading raises the question of a purpuric contact dermatitis from azo and other clothing dyes.

Question: What are your thoughts?
One Week Follow-up:

Note: Cleared completely after one week. This argues for factitial disease (as our readers' felt)









Reference:
1. Rasmussen JE. Puzzling purpuras in children and young adults. J Am Acad Dermatol. 1982 Jan;6(1):67-72.
2. Meeder R, Bannister S. Gardner-Diamond syndrome: Difficulties in the management of patients with unexplained medical symptoms. Paediatr Child Health. 2006 Sep;11(7):416-9. Available full text.

Keratosis Follicularis

Presented by DJ Elpern
Photomicrographs by Jag Bhawan

Abstract: 10 yo girl with 4 month history of a dermatosis on the neck

HPI: The patient is a pleasant 10 year old who presents for evaluation of a symmetrical papular eruption on the sides of the neck which has been present for about 4-5 months. She has been treated with a number of different topicals by her pediatrician without relief. The patient lives with a grandmother and there is no pertinent family history.

O/E: The examination show somewhat rough, 1 - 2 mm in diameter, keratotic micropapules on the lower folds of the neck. The remainder of the cutaneous examination is unremarkable.

Clinical Photos:

Biopsy: Focal acantholysis, multiple dyskeratotic cells, corps ronds and grains consistent with Darier's disease.

10 x and 20 x

40 x

Diagnosis: Keratosis follicularis (Darier's disease). It's unusual to see keratosis follicularis when it first appears. This is a sweet 10 year old and it's sad to contemplate what this may turn into. After "reviewing the literature" I decided to try pimecrolimus cream as there have been some reports of success.

Questions: How would you treat this child? Have you seen forme-frustes of keratosis follicularis?

References:

1. Good Overview: Darier's Disease eMedicine

2. Pérez-Carmona L, et. al. Successful treatment of Darier's disease with topical pimecrolimus. Eur J Dermatol. 2011 Mar-Apr;21(2):301-2.

3. (supplied by Yoon Cohen) Rubegni P, Poggiali S, Sbano P, Risulo M, Fimiani M. A case of Darier's disease successfully treated with topical tacrolimus. J Eur Acad Dermatol Venereol. 2006 Jan;20(1):84-7.
Abstract: Tacrolimus is a macrolide that inhibits T-cell activation. The most extensive experience with topical tacrolimus has been in treating atopic dermatitis but it has been used in various skin diseases, including Hailey-Hailey disease, with encouraging results. We report a case of extensive Darier's disease successfully treated with topical tacrolimus, after suspension of oral isotretrinoin due to major depression.

Facial flush in a pregnant woman

Presented by Henry Foong

Ipoh, Malaysia

A 37 year old restaurant waitress had these rashes on the face for several years, but worse recently since her pregnancy. She is G2P1 at the end of her first trimester. The rash was described as itching, burning. She had seen a dermatologist in Japan and was diagnosed as rosacea. There was no fever or polyarthralgia. Family history was insignificant. Drug history nil.

She feels very uncomfortable. Examination was unremarkable except facial flushing with for bilateral and symmetrical erythematous papules on both cheeks with a mild involvement of the bridge of nose. There was no comedones. Her scalp was normal.
What do you think of the diagnosis? Do you think this is rosacea? What other differentials would you consider - lupus erythematosus, seborrheic dermatitis? How would you manage her remembering that she 3 months pregnant? Would you use topical metrondazole?

Neurotic Excoriations

Abstract: 37 yo woman with few year history of excoriations

HPI: The patient is a disabled 37 yo woman who has suffered with painful sores on face, arms, buttocks, upper back for a few years. She was a trainer of race horses till a few years ago. She has a history of alcoholism. A few years back, she was diagnosed with alcoholic hepatitis and hemochromatosis (she gets regular phlebotomies). There is a history of sexual abuse starting at age 14 or 15 which continued for ten years. Thereafter she was in a physically abusive relationship. She is on a Fentanyl patch.

O/E: Fresh and healing excoriations on face, upper back, left earlobe, buttocks.


Diagnosis: Neurotic Excotiations (NE).

Discussion: In my experience, most women who excoriate their faces and bodies in this way have experienced sexual or physical abuse. This is similar to "cutting behavior." NE may be a minor varient of cutting. Adverse Childhood Experiences can manifest themselves in this kind of self-destructive behavior. Her alcoholism may be another expression.

Treatment: These patients are very diffuclt to reach. I started with clobetasol ointment and mupirocin ointment -- these are sometime helpful although one has to be careful not to use for too long on the face. Psychotrophic medications may be helpful. Cognitive behavioral therapy can help but is rarely available for poor patients. The ones who need it the most are those least likely to find a therapist who will help. Patients with NE like this woman are very needy. It can take months to reach them.

Painful Red Scrotum

Over the past twenty years, we have seen a few patients a year with scrotal burning and/or redness (erythema). Some of these individuals had used topical steroids for prolonged periods, some only for a few weeks. I don't recall if any had not used steroid creams. The condition is called scrotodynia, scrotopyrosis, and red scrotum syndrome. The medical literature gives few clues to its etiology, except that topical steroids can play a significant role in some (or many) of these patients. There is a condition called "vulvodynia" which is similar in some ways. This post tells one patient's story and is a call for information from physicians, other care givers and, importantly, from individuals who suffer with this disorder. It is anonymous. Hopefully as practitioners and patients collaborate we will reach some clarity and start to help those who suffer. If you are a patient making a comment please give your age, occupation and any other information you may consider pertinent.

Patient's History: (November 2011)
I am a health 46-year-old man in the technology field who has suffered with a burning scrotum for past two months. I had knee surgery in May of 2011 which sidelined me from physical activity until September of this year. Upon resuming a workout regiment (primarily of basketball and running) I developed what was diagnosed as a fungal in my groin (tinea cruris - commonly referred to as “jock itch”), specifically in the creases of my thighs. The red scrotum seemed to appear along with the fungal issue, but being unfamiliar with tinea cruris (it was my first time with the condition) I assumed that the red scrotum was part of the same problem. My first attempt at resolving the issue came with a visit to a dermatologist (who I happened to be seeing for a minor skin condition on my hands). It was a “by-the-way can you prescribe something for this rash I have” which first turned our/my attention to the red scrotum.

Initially the dermatologist prescribed Hydrocortisone ointment USP 2.5% for the redness/inflamation and Ketoconazole cream 2% for the fungal issue. The instructions were to first apply the Hydrocortisone to the inflamed area (the creases of my legs were rather red with a fungal rash) for one week to reduce the inflammation. Then apply the Ketoconazole for one week and return for evaluation. I applied the Hydrocortisone to the creases of my thighs and to my inflamed scrotum. The redness in the creases of my thighs subsided marginally but there was no change to the red scrotum. I then applied the Ketoconazole for one week and did see relief of the jock itch. Upon my return to the dermatologist I reported that the fungal treatment was working but there was no change in my scrotum. It was here where I first heard the term “Red Scrotum Syndrome” as a possible diagnosis. I was then prescribed Triamcinalone Acetonide ointment USP 0.1% (a topical steroid) and instructed to apply it to the scrotum for one more week, twice daily, (which I did) and report back. After one week of applying the Triamcinalone ointment to my scrotum there was no change in my condition. I was told by the dermatologist that she was out of ideas and to report to my primary care physician for further treatment.

The visit to my Primary Care Physician began with a careful review of the notes from my dermatologist coupled with a detailed description of what was happening by me. Upon examination my PCP admitted that he had never seen a case like this before. He stated that his medical references offered little help but he did find some info by doing an internet search. The research suggested a treatment of Doxycycline (an antibiotic) 100 mg, twice per day for 10 days. I promptly began taking the oral dose of Doxycycline but after 10 days again there was no change in my condition.

During my initial visit with my PCP I asked if I should stop using the Ketoconazole even though there was still remnants of the tinea cruris. The doctor said to stop all ointment treatment to the groin and instead take an oral anti-fungal medicine to kill the jock itch once and for all. Not knowing the dosage his office requested advice from another dermatologist who upon contacting prescribed Fluconazole (one pill one time). I have taken the Fluconazole and coupled with the Ketoconazole I seem to have the tinea cruris under control.

Next my PCP referred me to a urologist who, like my first Dermatologist and Primary Care Physician, admitted that he had never seen this condition before. He checked my prostate (normal) and gave me a urine test (which also came back normal). The urologist wished me luck and apologized for not being more helpful.

It is here where my luck changed as the second Dermatologist recognized the symptoms and suggested I pay him a visit. Upon examination he too diagnosed the condition as Red Scrotum Syndrome (RSS) or in some circles known as “Great Balls of Fire”. He knew of two doctors (one in Boston and one in Sweden) that have had experience with RSS. Pictures and a description were emailed to each and we await feedback. From prior cases and research the dermatologist advised me to take gabapentin (300 mg 3 times per day). Gabapentin was originally developed for the treatment of epilepsy, and currently is also used to relieve neuropathic pain. I am on day three of the medication and I do not feel any change in the condition.

Hopefully some relief is in sight as the pain is annoying. Some days are significantly worse than others. In fact on some days I continue my normal family and work routine and barely notice the RSS. On other days it’s more pronounced and sitting for any length of time at my desk is uncomfortable. Walking and sitting seem to aggravate the sensation. Having had the shingles (Herpes zoster) at the age of 44 I liken the pain to having scrotal shingles. Perhaps there is something neurological in the equation because I’ve been told that 44 years old is unusually young for shingles. A final note is that high levels of stress (mostly caused by work) occurred during my shingles and when the RSS manifested. A psychological component to the condition cannot be ruled out.

Unfortunately I’ve been told that I am what the medical field calls an “orphan” patient. That RSS exists in a medical space between Dermatology and Neurology and neither discipline is really focused on the condition. I know there are others out there who are suffering with the same pain and that have possibly found a solution to this annoying problem. Hopefully, this post is seen by others, offers helpful information and lets them know that they are not alone. I also hope that any sufferers out there who have had Red Scrotum Syndrome and discovered a remedy reply back and give us a helpful start.

References:
1. Gabapentin for Neuropathic Pain

2.  Wollina U.  Red scrotum syndrome.  J Dermatol Case Rep. 2011 Sep 21;5(3):38-41.  Red Scrotum Free Open Access

20 y.o. man with multisystem disease

Presented by Henry B.B. Foong
Foong Skin Clinic, Ipoh, Malaysia

Abstract: 20 yo man with mouth ulcers, arthralgias, skin nodules

HPI: The patient is a 20 yr old student who presented with a 3 year history of recurrent mouth ulcers, polyarthralgia (knee, ankles), fever and tender nodules over the shoulders, elbows and legs. The attacks occur about every 6 months and responded to oral prednisolone. Apparently the nodules run a predictable course - initial erythema, then tender nodule then ulcerate and then subside leaving behind post inflammatory hyperpigmentation – all over 3-4 weeks. There is no photosensitivity, alopecia or cough. There is a family history of similar illness.

O/E: Multiple erythematous tender nodules over the elbows, legs , upper shoulders and scrotum. Those on the scrotum – severe, multiple tender nodules, of which ulcerated with scab formation. Multiple tender ulcers were also noted on the inner mouth.

Clinical Photos: (taken with iPhone)


LAB: (Some pending)
TWBC 11, 700 (N 67% L 18% E 1% M 12% B1%) ESR 44 mm/hr
ANA
ANCA
Mycoplasma serology 1: 160 ( N<1:40)
LFT and renal normal
CXR

Pathology: Pending

Dignosis: Behcet’s? PAN? SLE?

Questions: What are your thoughts? Any further studies indicated?