Amytrophic (?) Dermatomyositis

Presented by Henry Foong
Ipoh, Malaysia

HPI: A 82 yr old housewife presented with 6 month history of skin eruptions on her face, neck and hands. They were aggravated with exposure to sun. She was diagnosed with carcinoma of the stomach in 2007 and completed chemotherapy about 3 months ago. While on chemotherapy she noticed weakness in getting up and putting on her shirt. She has improved since then. She has a history of diabetes mellitus, and IHD for more than 10 years.

O/E: Pertinent findings: bilateral and symmetrical erythematous macules and plaques on the dorsum of the PIP, DIO and MCP jpints. They have a violaceous hue and were non tender. Erythematous discoloration was noted
bilaterally and symmetrically around the eyes, nose and forehead.

Photos:


Lab:
TWBC 5,500
CK 41 U/l (<201) style="font-weight: bold;">Clinical Diagnosis: Dermatomyositis in Ca stomach
Could this be amytrophic dermatomyositis?
ANA + 1:320 Speckled and homogeneous

Questions: How would you proceed? Would you do a skin biopsy?
Would you treat her? Oral prednisolone or plaquenil alone?

Reference: (from eMedicine.com) Dr. Jeff Callen

Dermatomyositis sine myositis, also known as amyopathic dermatomyositis, is diagnosed in patients with typical cutaneous disease in whom no evidence of muscle weakness exists and in whom serum muscle enzyme levels are repeatedly normal for a 2-year period in the absence of disease-modifying therapies such as corticosteroids, immunosuppressive agents, or both. When studied, some patients with amyopathic dermatomyositis have abnormal ultrasound, MRI or magnetic resonance spectroscopy, or muscle biopsy findings. These patients have muscle involvement, and their condition may be better classified as hypomyopathic dermatomyositis. Patients with these variations may also reflect an underlying malignancy, and some develop severe pulmonary disease, particularly persons from Asian countries.
Patients exist in whom myositis resolves following therapy but whose skin disease remains as an active, important feature of the disease. These patients are not classified as having amyopathic dermatomyositis, despite the fact that, at this point in time, the skin is the major and often only manifestation of the disease. Sontheimer has suggested the term postmyopathic dermatomyositis for these patients.

Therapy for the cutaneous disease is often difficult. Patients who present primarily with skin disease (amyopathic dermatomyositis) and those in whom the muscle component is controlled but who still have significant skin disease exist. The first-line of therapy is recognizing that the patient is photosensitive and advising the patient to avoid sun exposure and to use sun protective measures, including broad-spectrum sunscreens. Hydroxychloroquine and chloroquine have been beneficial in small open-label case studies. Methotrexate is also useful. Mycophenolate mofetil has been reported to be useful. IvIg not only benefited the muscle but also cleared the skin lesions in the patients in whom it was used. Rituximab has been used for skin disease, but the results are mixed. Efalizumab has been used and may have some benefit. Efalizumab (Raptiva), a drug indicated for psoriasis, is being withdrawn from the US market and will no longer be available after June 8, 2009, because of potential risk for progressive multifocal leukoencephalopathy (PML). PML is a rapidly progressive infection of the central nervous system caused by the JC virus that leads to death or severe disability. Demyelination associated with PML is a result from the JC virus infection. JC virus belongs to the genus Polyomavirus of the Papovaviridae.

Read More

Lyme Tick

This 60 yo man came in for a skin cancer check. He had a basal cell removed from his forehead two years ago. The exam was negative until I checked his leg and noted a funny looking lesion. On closer examination, I realized it was a tick. It's head was embedded in the patient's leg. I removed the tick and gave the patient 200 mg. of doxycycline. This latter might be unnecessary, but a study published in the NEJM gives support to this fairly benign prophylaxis.

Reference:
Prophylaxis with single-dose doxycycline for the prevention of Lyme disease after
an Ixodes scapularis tick bite.

Nadelman RB, wt. al. Tick Bite Study Group.

Department of Medicine, New York Medical College, Valhalla 10595, USA.

BACKGROUND: It is unclear whether antimicrobial treatment after an Ixodes scapularis tick bite will prevent Lyme disease. METHODS: In an area of New York where Lyme disease is hyperendemic we conducted a randomized, double-blind, placebo-controlled trial of treatment with a single 200-mg dose of doxycycline in 482 subjects who had removed attached I. scapularis ticks from their bodies within the previous 72 hours. At base line, three weeks, and six weeks, subjects were interviewed and examined, and serum antibody tests were performed, along with blood cultures for Borrelia burgdorferi. Entomologists confirmed the species of the ticks and classified them according to sex, stage, and degree of engorgement. RESULTS: Erythema migrans developed at the site of the tick bite in a significantly smaller proportion of the subjects in the doxycycline group than of those in the placebo group (1 of 235 subjects [0.4 percent] vs. 8 of 247 subjects [3.2 percent], P<0.04). p="0.02)" p="0.02)." style="font-weight: bold;">CONCLUSIONS: A single 200-mg dose of doxycycline given within 72 hours after an I. scapularis tick bite can prevent the development of Lyme disease.

Read More

Abdominal Sinuses in a 23 yo Man

Three month history of draining sinuses lower abdomen.

HPI: This college student developed abdominal pain and bloody diarrhea in August of 2008. A diagnosis of ulcerative colitis was made and a number of theapies were tried (including prednisone and Remicaid). All were ineffective and he had a subtotal colectomy and ileostomy performed in December of 2008. Subsequent to that he continues to have some pain in the rectal stump and is scheduled for a J-pouch procedure in a few weeks. Three months ago, he developed painful draining tracts in the lower abdomen.

O/E: The patient is a healthy-appearing 23 yo man. The cutaneous findings are 5 - 10 mm in diameter sinus tracts with sero-sanguinous drainage. There are four active lesions at this time. The remainder of the cutaneous exam is negative.

Photos: (June 9, 2009)


Lab and Path: Nil

Diagnosis: Could this be an extra-intestinal manifestation of inflammatory bowel disease? This is more common with Crohn's disease than U.C. My working diagnosis is sinus tracts or abdomino-cutaneous fistulae. The patient was referred for the question of pyoderma gangrenosum. If this is P.g., it is a very atypical case.

Questions:
Has anyone seen and treated a similar patient?
He is scheduled to have a resection of the rectal stump with a re-anastamosis of small bowel to the rectum allowing closure of his ileostomy (I am not sure of exact procedure). Perhaps this will help. Your thoughts are appreciated.

Read More

Trichoepithelioma

A Dermatologic Vignette.

The patient is a 78 yo woman with a few year history of an enlarging lesion on the nasal tip. Excellent health.

Eight mm in diameter pearly papule nasal tip. Clinically and dermoscopically BCC.



Pathology shows this to be a trichoeoithelioma:






Question: Observe, refer to Mohs, radiate?
Would surgery be deforming in this site? vs. Is radiotherapy overkill?

Reference: There are very few references which address this type of lesion, unless we consider this to be like a BCC. But here is one.

Aygun C, Blum JE. Trichoepithelioma 100 years later: a case report supporting the use of radiotherapy. Dermatology. 1993;187(3):209-12.
Trichoepitheliomas are rare skin tumors which can cause significant cosmetic and functional impairment when they occur in the head and neck area. Multiple methods of treatment including plastic surgery, dermabrasion, cryosurgery and laser surgery have been reported in the literature. A 32-year-old male with multiple coalesced lesions in his ear canals was treated with radiation therapy after he failed more conventional methods of treatment. He is free of tumor in the radiated area 17.5 months after treatment with significant functional improvement. Various aspects of this tumor were reviewed. More data are still needed to define the proper place for radiation in the management of this disease.

Remember Dorinda and Walter Shelley's poem:
Who knows
Who's nose
Needs Mohs

Read More

Dissecting Cellulitis of the Scalp

Abstract: 18 yo man with three year history of cystic lesions scalp, axillae, chin

HPI: This 18 year-old man has had dissecting cellulitis of the scalp for three years. He has been treated with doxycycline 100 mg b.i.d. and excisions of cysts and sinuses by a plastic surgeon. He presented in May of 2009 for another opinion. He has had a few cysts of the axillae and chin. The patient has observed that his scalp is worse after wearing a helmet for football.

O/E: The patient is a healthy, moderately obese African-American teenager. He has painful cysts, nodules and draining sinuses mostly on the occipital portion of the scalp and around the vertex. He has a hypertrophic scar at the site of an excision in the occipital region. He has a few hyperpigmented nodules in the axillae and some small acne cysts on his chin in the bearded area.

Clinical Photos:


Lab: Nil
Path: Nil

Diagnosis: Dissecting Cellulitis of the Scalp in the setting of Follicular Triad Syndrome. An older name for the scalp process is the hard to remember "Perifolliculitis Capitis Abscedens et Suffodiens

Treatment: To date, only doxycycline 100 mg b.i.d. and frequent excisions by a plastic surgeon. I injected some active lesions with triamcinalone acetonide 10 mg/cc and am considering following the rifampicin and isotretinoin protocol reported in the reference below.

Reason Presented: For therapeutic suggestions

References:
1. Georgala S, et al. Dissecting cellulitis of the scalp treated with rifampicin and isotretinoin: case reports. Cutis. 2008 Sep;82(3):195-8.

Dissecting cellulitis of the scalp, or perifolliculitis capitis abscedens et suffodiens, is an uncommon chronic suppurative disease of the scalp manifested by follicular and perifollicular inflammatory nodules that suppurate and undermine, forming intercommunicating sinuses, and leading to scarring alopecia. Treatment generally fails to obtain a permanently successful result; thus, many therapeutic options have been proposed. We report 4 cases of dissecting cellulitis of the scalp successfully treated with oral rifampicin and oral isotretinoin. To our knowledge, this is the first report of oral rifampicin used concomitantly with oral isotretinoin in this disease entity. We also present a brief review of the literature on the topic.

2. Dissecting Cellulitis of the Scalp Emedicine.com chapter

Read More

Teledermatology Rules: Vasculitis

Abstract: 2o yo man with one week history of palpable purpura.
HPI: This 20 yo college student was started on isotretinoin for severe cystic acne a month before he developed a rash on his legs. He also had an upper respiratory infection two weeks before the eruption began. He is away at school (a two hour drive). His mother called the office and spoke to my secretary. Busy week. When I heard that he had a rash, I relayed the message that it was probably the common dermatitis we see with patients on isotretinoin and if worried to send me a photo. Two days later, this photo was sent:


The patient was then emailed and asked to come in the next day. Labs were ordered done before the visit.

O/E: Palpable purpura both L.E. Right ankle swollen and tender. Patient limping.

Lab: CBC normal, UA normal. Pending Labs: Throat culture, ANA, ASOT. (Hep C, Stool for OB, not ordered)

Path: Biopsy performed. Not back

Diagnosis: Leucocytoclastic vasculitis. Etiology: The URI, isotretinoin, idiopathic

Plan: Rest for a few days. No specific therapy at this time except stopping the isotretinoin. If he improves uneventfully without evidence of GI or renal involvement will offer a re-challenge with isotretinoin.

Discussion: A few cases of LCV have been reported with isotretinoin. This patient has severe cystic acne with scarring and it would be a shame to withhold drug if it were not putative for the LCV. I admit I did not pay proper attention to the first telephone call. This illustrates the power of teledermatology which can be almost standard in a few years as cell phone cameras become better and people know how to use them more adroitly.

Questions: What are your thoughts and suggestions?

Read More

Ear Keloids and Imiquimod

We presented this patient around a year ago (she is patient # 2). The woman, now 19 years old, presented in March of 2008 for a keloidal scar in the left triangular fossa. On 12/18/08 based on suggestions and a report in MEDLINE, the lesion was shave excised and a week after surgery, imiquimod was applied nightly for six weeks. She is now one month out after stopping imiquimod. At this point, she looks very good. We will have to see if this is a long term solution.

Reference:
1. Berman B, Kaufman J. Pilot study of the effect of postoperative imiquimod 5% cream on the recurrence rate of excised keloids. J Am Acad Dermatol. 2002 Oct;47(4 Suppl):S209-11.
New adjunctive treatments are needed to reduce the high recurrence rates (50%) of excised keloids. Interferon alfa injections have been shown to decrease the size of stable keloids. This study examined the effects of postoperative imiquimod 5% cream on the recurrence of 13 keloids excised surgically from 12 patients.Starting on the night of surgery, imiquimod 5% cream was applied for 8 weeks. Patients were examined at weeks 4, 8, 16, and 24 for local erythema, edema,
erosions, pigment alteration, and/or recurrence of keloids. Of the 11 keloids evaluated at 24 weeks, none (0%) recurred. Incidences of hyperpigmentation were 63.6%. Two cases of mild irritation and superficial erosion cleared withtemporary discontinuation of imiquimod. Both patients completed the 8 weeks of topical therapy and the final 24-week assessment. At 24 weeks, the recurrence rate of excised keloids treated with postoperative imiquimod 5% cream was lower than recurrence rates previously reported in the literature.

Read More