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.

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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

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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.

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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.

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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!!

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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.

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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

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