An Unusual Case of Scalp Ulceration

33 yo woman with six month history of extensive scalp ulceration

History: The patient is a 33-year-old unemployed cashier who presented for evaluation of facial erythema.  She has had insulin dependent diabetes since childhood but apparently, it was not treated for many years.  She is being seen at the Wound Care Clinic now for a large erosion of the scalp present for ~ six months. Other than insulin, her only medication is gabapentin for her scalp symptoms. 

She is married.  Lives with her husband and 18 cats.

EXAMINATION:  The examination shows a 33-year-old woman with a flat affect. She has some erythema on her cheeks and bridge of the nose.  A few, but not many, papules. 

She was wearing a  base-ball cap and when I asked her to remove it, I saw a 7 x 8 cm erosion on the scalp She says she has been picking on this for quite some time because it was itching and painful. 

The remainder of the exam was unremarkable other than showing poor dental hygiene.  

Pathology: Scalp: There is dense and deep dermal fibrosis with superficial fibrin deposition, and a mild to moderate superficial and deep perivascular lymphoplasmacytic infiltrate with occasional neutrophils and focal multinucleated giant cells.  These changes are consistent with chronic and impetiginized ulcer but are not specific for its etiology that could include previous traumatization (although the depth of the dermal fibrosis is unusual for a simple excoriation) or an infectious etiology.
Biopsy of face confirmed findings of rosacea.  No evidence of demodex or collagen vascular disease.

Lab:  CBC, Complete Chem profile normal.  ANA negative.  KOH prep from face failed to reveal demodex.

IMPRESSION:  This is a complex problem.  Her facial erythema will be treated with doxycycline.  Her scalp excoriation is worrisome. (A similar patient was described by Atul Gawande in the New Yorker a number of years ago (see reference).  I wonder if the 18 cats at home are significant.

Questions: Any thoughts you have will be appreciated.  It will be great if we can help this unfortunate woman.

References: 

1. Gawande A.  The Itch: Its mysterious power may be a clue to a new theory about brains and bodies.  New Yorker.  6/30/2008.  Full Free Text Online.

2. Dr. Sharquie from Baghdad sent us a recent article of his: The Major Psychodermatological Disorders in Iraq.  

3.  Cat’s Curse: A Case of Misdiagnosed Kerion

MB Mazlim, L Muthupalaniappen

Malays Fam Physician. 2012; 7(2-3): 35–38.  Free Full Text.

Abstract:  Kerion is an inflammatory type of tinea capitis which can be mistaken for bacterial infection or folliculitis as both conditions display similar clinical features. It occurs most frequently in prepubescent children and rarely in adults. We report a 26-year-old woman who presented with multiple tender inflammed nodules on her scalp. Her condition was misdiagnosed as bacterial abscess and treated with multiple courses of antibiotics without improvement. Later, her condition was re-diagnosed as kerion based on clinical appearance, history of contact with infected animal and Wood’s lamp examination. symptoms and lesions resolved completely with systemic antifungal treatment leaving residual scarring alopecia. The delay in the diagnosis and treatment of this patient resulted in permanent scarring alopecia.

4) See previous VGRD entry of a 9 yo girl with scarring alopecia. (January 2006)

5) Dr. Barry Ladiainski suggested this reference
Cervical Trophic Syndrome: A Distinct Clinical Entity?

Alison A. Fischer, MD; David M. Adelson, MD; Carlos Garcia, MD

Cutis. 2014 June;93(6):E6 - E7

Absract: Ulceration is not a typical feature of notalgia paresthetica or brachioradial pruritus; a history of self-mutilation due to underlying paresthesia is consistent with the diagnosis. This case report describes a patient with underlying spinal pathology; the authors discuss this case as a spectrum between trigeminal trophic syndrome and notalgia paresthetica whereby lesions of peripheral spinal nerves may lead to unilateral ulcerations restricted to a dermatome.

6) Trigeminal Trophic Syndrome: Report of 3 Cases Affecting the Scalp

Ranti S. Bolaji, MD; Barbara A. Burrall, MD; Daniel B. Eisen, MD. Cutis. 2013 December;92(6):291-296

Abstract: Trigeminal trophic syndrome (TTS) is a rare condition that results from a prior injury to the sensory distribution of the trigeminal nerve. Patients typically respond to the altered sensation with self-mutilation, most often of the nasal ala. We describe 3 patients with TTS who presented with self-induced ulcerations primarily involving the scalp. Two patients developed delusions of parasitosis (DOP) based on the resulting symptoms of TTS, which is a unique association. Trigeminal trophic syndrome may occur at extranasal sites and in any branch of the trigeminal nerve. The condition should be considered when ulcers are encountered in this nerve distribution. Symptoms such as formication may mimic DOP. Trigeminal trophic syndrome may be differentiated from DOP by the restriction of symptoms and ulcerations to the distribution of the trigeminal nerve.

 
 

Agminated Molluscum Contagiosum

Presented by Drs. Harold Blumenthal and Jerome Litt

Beachwood, Ohio

The patient is a 22 y.o. woman with a three month history of grouped papules at the left nostril and left upper lip.  They have been getting worse.  It was biopsied and treated with liquid nitrogen.  She has not been seen since.

Diagnosis:  Agminated molluscum contagiosum.

Reference:

1. J. L. Bunch. Agminated Molluscum Contagiosum. Proc R Soc Med. 1918; 11(Sect Study Dis Child): 44.  Free Full Text.

2. Demitsu T, et. al. Attenuated ubiquitination of molluscum bodies in agminated mollusca contagiosa associated with malignant lymphoma. Eur Acad Dermatol Venereol. 2007 May;21(5):691-2.

Red Face x Two

Two recent Red Face patients

1) 27 yo woman with few year history of unilateral erythema of right cheek. No papules when initially seen.  Erythema waxes and wanes -- has been worse by history.

Dx:  Unilateral Rosacea, Demodeciasis, other?

Rx?  I may start with doxycycline, cold compresses, ? ivermectin


2) 57 yo man with a long history of psoriasis.  Was using clobetasol ointment and clobetasol scalp solution for a number of years.  Last seen > 1 year ago.  He was also on methotrexate 10 mg per week.  Facial erythema and papules started in the past few months.  He also noted easy bruising, and loss of muscle mass in brachial area.  BP 160/100.  Exam also shows mild truncal obesity and ecchymoses.

Dx: Rosacea, steroid rosacea secondary to clobetasol scalp and body (Red Face Syndrome), Iatrogenic Cushing's Syndrome, Cushing's Disease

Plan: CBC, Chemistries, 8 am and 4 pm serum cortisol.  Endocrine consult.
Macrocytosis, plt 77,000
Alk phos 425 (18 - 210),  SGOT 182 (15 - 37)
Serum Cortisol a.m. 24, p.m. 11  AM ( 4-22),  (PM 3 - 17)

What are your thoughts?

Unilateral Facial Erythema

The patient is a 27 yo woman with a 3 year history of facial erythema, restricted, for the most part to the right cheek.  She is a pharmacist assistant, takes no meds by mouth other than thyroid.  She took doxycyclinc  a few years back but stopped because of G.I. upset and topical metronidazole was not helpful

Exam showed dramatic erythema of the right cheek and malar eminence.  There are no papules or pustules.  The left side of the face appears normal.

Clinical Images:

Diagnosis: Unilateral Telangiectatic Rosacea or Unilateral Facial Telangiectasia

References:

1. Unilateral Swelling and Erythema of the Face.

Burgess N. Proc R Soc Med. 1938 Dec;32(2):85-6.

2. Unilateral demodectic rosacea.  Shelley WB1, Shelley ED, Burmeister V.  J Am Acad Dermatol. 1989 May;20(5 Pt 2):915-7.

Abstract: A unilateral rosacea-like chronic dermatitis of the right side of the face was shown to harbor innumerable Demodex folliculorum and D. brevis. Treatment with oral metronidazole suppressed the dermatitis but did not significantly reduce the Demodex population. Treatment with topical crotamiton eliminated the Demodex and was curative. These observations support the view that D. folliculorum and D. brevis may be pathogenic when they are present in extremely large numbers.

We could find no useful articles on unilateral rosacea without papules or pustules. Perhaps, even so, a scraping for demodex mites should be done. 

Subungual Pigmentation

The patient is a 49 yo woman who noted a discolored right great toe nail for ~ a week.  After a web search she became quite agitated and presented at the office as a walk-in patient.  The area was painless and there was no history of trauma.

O/E:  The patient is an anxious-appearing Korean woman. There was a dark purple area at the proximal and lateral portion of the right great toe nail.  Dermoscopically no brown or black color could be seen.  The nail was scraped down and the base appeared reddish.

Unfortunately the second photograph is a bit blurry.

Impression:  She short history and the pigment suggest subungual hematoma.  It would be unusual for a subungual melanoma to present this rapidly.

Plan:  I will follow this.  I expect it will take many months for this to grow out.

34 yo woman with micropapules

The patient is a 34 yo woman with a 1 year history of a slightly pruritic eruption on her upper arms, elbows, mid back.  Her health is good and she takes no meds p.o.  She commutes between Seoul, New York City and Paris for her work in fashion.

The lesions are symmetrically placed in these areas.

Pathology:  Two 3 mm punch biopsies taken.

There is an interstitial proliferation of lymphocytes and histiocytes forming granulomas with focally increased dermal mucin and central necrobiosis, and with islands of intervening normal dermal collagen and a mild superficial and mid perivascular lymphocytic infiltrate with occasional plasma cells. These findings support the histologic diagnosis of granuloma annulare.

Diagnosis: Granuloma annulare

The clinical picture is unusual, but in retrospect, it fits.  Perhaps, this is an example of generalized G.a.  The patient is otherwise healthy but we have no recent lab tests.  It may be prudent to obtain A1c, chemistry and lipid profile.

What are your thoughts?

Reference: 
1. Remission of generalized erythematous granuloma annulare after improvement of hyperlipidemia and review of the Japanese literature.
Watanabe S et.a.l  Dermatol Pract Concept. 2014 Jan 31;4(1):97-100.  Free Full Text Online

Splinter Hemorrhages

The patient is a 48 year-old woman with a two week history of splinter hemorrhages in all ten finger nails.  Her toe nails are obscured with nail polish.  She has a history of appendiceal cancer, has had two surgeries and is left with residual disease.  She is being treated with leucovoran, avastin and 5FU.  She feels well.  No fever, chills or night sweats.  She has had recent normal cardiac echos.

Diagnosis and Discussion:  It's hard not to conclude that these are typical splinter hemorrhages and that she needs to be worked up for subacute bacterial endocarditis.  Some antineoplastic agents, such as paclitaxel, can cause splinter hemorrhages but I could find not reference to the drugs she is on.  The splinter hemorrhages of SBE are more often proximal and all of these are distal, arguing for a relationship with her chemotherapy; but appropriate blood cultures seem indicated.

To Treat or Not to Treat: that is the question

Elani Linos and colleagues wrote a milestone paper on the treatment of nonmelanoma skin cancer (NMSC) that was published in JAMA – Internal Medicine in June 2013. In it, they stated:

“Nonmelanoma skin cancer (NMSC) is the most common cancer and predominantly affects older patients. Because NMSCs do not typically affect survival or short-term quality of life, the decision about whether and how to treat patients with limited life expectancy (LLE) is challenging, especially for asymptomatic tumors.

“The current standard of care in the United States is to treat NMSCs, and no guidelines exist about whether physicians should consider patient age or functional status in choosing treatments.  Treatment decisions for patients with NMSC with LLE require consideration that the benefits of treatment may not occur within the patient's
remaining life span, but any risks are immediate.”

We saw two such patients recently in our dermatology practice.  They are presented for your thoughts and discussion.

1. The patient is a 94 yo woman, status post CVA (12/24/13) with right hemiparesis.  She has a two year history of a rodent ulcer on the right nasolabial fold measuring 2.4 x 1.4 cm.  It itches and she picks it.  Biopsy shows “infiltrating basal cell carcinioma.”  She is a retired executive secretary, never married with no close relatives nearby.  Mentally, she is alert and oriented.  We discussed active surveillance, surgery and radiotherapy.  She is confined in a nursing home and was not keen on having XRT considering the number of treatments.

2.  This 89 yo man has a tumor of the mid upper lip for ~ 10 months.  The 1.4 cm in diameter lesion is firm with rolled borders.  Clinically, this is BCC, but it has not yet been biopsied.  His general health is good, but he has moderately advanced dementia and lives independently with his wife.  The couple have children who live at some remove.  We discussed active surveillance, XRT and surgery.  The latter would be fairly simple; but we recognize that the tumor may not ever significantly impact on his quality of life or longevity.

Discussion:  Both of these lesions could be treated or watched.  Lesion # 2 would be easy to excise and that may make management easier.  Excision of lesion #1 would entail a long trip for micrographic surgery which is difficult logistically.  In our opinion, how to proceed with these cases is a value judgement and input from the patient and/or the family is important.

Dr. Linos’ article (1) is helpful but each case presents unique management quandaries.  It has been said that “often it is more important to treat the patient with the disease, than it is to treat the disease the patient has.”  These two cases are examples of this conundrum.

An additional thought:   Topical imiquimod can be helpful in the management of superficial and nodular basal cell carcinomas.(2)  The marked inflammatory response is often difficult for patients to tolerate, but less frequent applications may allow for palliation and slowing of tumor progression.

You thoughts will be appreciated.

 Reference:

1. Treatment of nonfatal conditions at the end of life: nonmelanoma skin cancer.  Linos E, Parvataneni R, Stuart SE, Boscardin WJ, Landefeld CS, Chren MM.  JAMA Intern Med. 2013 Jun 10;173(11):1006-12.

Available Free Full Text.

2.  Surgical excision versus imiquimod 5% cream for nodular and superficial basal-cell carcinoma (SINS): a multicentre, non-inferiority, randomised controlled trial.

Bath-Hextall F, et. al.  Lancet Oncol. 2014 Jan;15(1):96-105.

Full article free on Lancet site.

Sacral Herpes Simplex

The patient is a 77-year-old woman who presents for evaluation of a recurrent localized blistering eruption on the right buttock.  This has happened off and on for 2-3 years.  Before this, she noticed a pain in the right buttock to hip that was attributed to some form of trauma and has had physical therapy for the pain.

EXAMINATION:  The examination shows grouped vesicles on an erythematous base on the right buttock. 

Clinical Picture:

Lab: Tzanck smear was positive for multinucleated giant cells. 

IMPRESSION:  Sacral herpes simplex.  Her buttock and hip pain may be related.  

PLAN:  Acyclovir 400 mg three times a day for seven to ten days.  If her hip pain improves, I would continue the acyclovir for a few months at 400 mg twice to three times a day to see if that impacts the chronic hip pain for which she has had physical therapy without much relief.

Discussion:  Sacral herpes simplex is seen with some regularity, although it has not been well-studied.  In 1974, Lenzer and Conant mentioned sciatica with sacral herpes simplex. I have seen a few memorable cases over the years.  One, in particular was a 70 yo man with sciatica and urinary symptoms that resolved completely when his recurrent sacral HSV was treated with acyclovir and he was maintained on suppressive therapy.

Patient reports:  I completed the full ten day regimen of acyclovir with apparent success - healing of the lesion and elimination  of the ache in my buttock which I had thought was a lingering result of the fall that I had almost two years ago. I am wondering if I should continue with prophylactic use of the acyclovir.

References:

1. Neuralgia in Recurrent Herpes Simplex

Robert B. Layzer, MD; Marcus A. Conant, MD

Arch Neurol. 1974;31(4):233-237.

ABSTRACT: Five patients with recurrent herpes simplex of the skin had unusual neuralgic pains preceding the eruptions by 24 hours or more. Although prodromal neuralgia is an uncommon feature of recurrent herpes, about 15 similar cases have been reported previously. The pain is often diffuse and aching in character and, in contrast with herpes zoster, leaves no sensory or motor deficit. Stereotyped cycles of pain and herpes simplex may occur repeatedly for as long as 20 years. The fact that pain precedes the eruption supports the theory that a persistent latent infection of sensory ganglia is activated during recurrences of herpes simplex.

2. [Recurrent herpes with neuralgia and zones of cutaneous hypoesthesia].

[Article in French]

de la Sayette V, er. Al

Abstract: A 52-year old man presented with recurrent Herpes simplex of the thigh and buttock of 30 years duration. The skin eruption was preceded by pain and sciatica. Surgical excision of the skin area involved modified the site of recurrence. During an attack, the patient developed severe pain and hypoaesthesia in the left half of his chest. The skin lesions were unmodified, and a type 2 Herpes simplex virus was isolated from a vesicle. A clinical examination performed 5 weeks later showed reduced sensitivity to pin prick in the previously painful D5 to D12 territory. Three points are of interest in this case: the site of recurrence moved after surgical excision, pain extended over a wide area and, most of all, persistent hypoaesthesia occurred during a recurrence.

3. Although this review (below) does not mentione HSV neuropathy, I suppose it belongs in this group.

Infectious neuropathies.

Sindic CJ1. Curr Opin Neurol. 2013 Oct;26(5):510-5

Abstract

PURPOSE OF REVIEW: Infectious neuropathies are heterogeneous neuropathies with multiple causes. They still represent an important world health burden and some of them have no current available therapy.

RECENT FINDINGS: Leprosy incidence has decreased by 50% during the last years, but leprosy-related neuropathies still cause severe disability. The pure neuritic leprosy is a diagnostic challenge that may require nerve biopsy or nerve aspiration cytology. The treatment itself may lead to a 'reversal reaction', which further causes injuries to the nerve. HCV-related neuropathies may be related or not to the presence of cryoglobulins. The absence of vasculitis, the most frequent form is a peripheral sensory neuropathy involving small nerve fibers, and more accurately diagnosed by pain-related evoked potentials. HIV-related neuropathy has become the major neurological complication of HIV infection. Both HIV-induced neuropathy and antiretroviral toxic neuropathy are clinically indistinguishable. The existence of an isolated chronic polyneuropathy due to Borrelia burgdorferi remains highly controversial. Lastly, an active infectious ganglioneuritis caused by varicella zoster virus, producing shingles, is the most frequent infectious neuropathy in the world and may cause various neurological complications. Zoster sine herpete remains frequently undiagnosed.

SUMMARY: Recent data have improved our knowledge and diagnostic tools of infectious neuropathies. Treatment of the injured nerves is not yet available, and prevention and rapid diagnosis remain the main priorities for the clinician.

Tay Syndrome (Trichothiodystrophy)

Presented by  Amira Abdel Azim MD, MRCP (UK) and  Rasha El Barbary  MD Egypt (the case presented to us at AL Zahraa University hospital Egypt)

HPI:  An 11 years old girl presented with skin and hair problems dating since birth. She was deaf  (had previously been subjected to a failed trial of cochlear transplantation) and had severe eye problems causing blindness expect for light recognition of one eye. Mentality was normal in proportion to her sensory defect. There was no history of consanguinity nor history of similar conditions in the family.

O/E:  On examination the child was cooperative, alert and responsive to the directions of her mother. She was completely deaf, almost completely blind except for slight light recognition of one eye.

Her skin was very dry with fine scales dating since birth yet there was no history of collodion baby.  There were erythematous plaques on the flexures as well as perioral, she had mild ectropion and  palmoplantar keratoderma.

On examination of the scalp: she had scaly scalp, areas of hypotrichosis and yellowish dull lusterless brittle hair. There was loss of the eyelashes and eyebrows.

The child was physically not compatible with her age and was very thin. She also had skeletal abnormalities  in the form of  asymmetry of the lower limbs and syndactyly.

Clinical images: 

 Lab: no abnormality detected.

Therapy:  Topical  moisturizers and  keratolytics in the form of urea 10% cream was  given  for her scaly skin.

She has already been consulted by an ENT specialist, ophthalmologists and orthopedics.

Diagnosis and comments:  Our clinical diagnosis was icthyosis with brittle hair suggestive of Tay syndrome.

References:
1. Brittle hair and ichthyosis in the newborn: A case of Tay syndrome
Paula Karina N Gonzales-Carait, Marie Eleanore O Nicolas
Indian J Paediatric Dermatol. 2014:15;127-129
Tay syndrome is a rare autosomal recessive disorder characterized by brittle hair and congenital ichthyosis. It is one of the syndromes of trichothiodystrophy - a group of DNA repair disorders with wide range of phenotypic expressions unified by the presence of sulfur-deficient brittle hair.  Free Full Text Online  

2. Trichothiodystrophy: Photosensitive, TTD-P, TTD, Tay syndrome.

Lambert WC1, Gagna CE, Lambert MW. 

Adv Exp Med Biol. 2010;685:106-10.

Abstract

Nevi of Interest

In this post, we will present photos of interesting pigmented tumors.  Please feel free to send us interesting photos with brief captions.

7/25/2015
1) 15 y.o. girl with a 6 mm diameter congenital nevus on upper back.  Looks benign to me but has an interesting play of color.

2) 10 y.o. girl with a planaria shaped nevus on the right 4th toe.  Pigment globules likely represent growth.

3) 14 yo girl last seen 10 years ago.  Congenital nevus noted on l. upper back then, 9 mm diameter.  7.28.15 the lesion is 20 mm diameter. It has find terminal hairs.  There are two (?) satellite lesions near to it, the largest being 7 mm diameter.  The new lesion has a peculiar pattern dermatoscopically.  Still, I think it is benign.  I gave them a follow-up for 6 months.

A Diagnostic Dilemma

presented by Hamish Dunwoodie
Tracadie, New Brunswick

The patient is a 60 yo man who presented with a six months history of two asymptomatic erythematous nodules on the torso.  He has been in his usual state of health otherwise. No history of fever, chills or night sweats.

O/E:  There are two erythematous nodules located on the right abdomen and the left upper back.  They measure 3 - 4 cm in diameter. No other cutaneous findings.

Clinical Photos:

New Lesion 10.25,16 R, Upper Back)

Pathology:
A superficial and deep nodular and interstitial infiltrate of CD20 positive B-lymphocytes admixed with CD3 positive T-lymphocytes with slight preponderance of B-cells.  There is a scattering of CD30 positive lymphocyres.  There appears to be a Grenz zone.  Gene rearrangement studies are not indicative of either a clonal T or B cell lymphoproliferative disorder.

 

CD 3

CD 20

Lab: CBC, Chemistries, Serum protein electrophoresis all normal. IgG, IgM and IgG were all normal.

Diagnosis: The differential is between an atypical lymphoid infiltrate and a cutaneous lymphoma.  We are leaning towards the former.  Note: The last clinical photo was taken ~ 1 month after the others and shows progression.  We plan to excise this recurring tumor for help with diagnosis.

Questions:  

Should we treat? and if so how?

Should we follow with active surveillance?
Would any of you make a a more specific diagnosis?
Is any further testing indicated at this time?

Follow-up 2.3.16
The patient's lesions come and go.  All tests for systemic disease are negative.  New lesion (see photo) on left chest 2 cm in diameter seen today.  Will try to treat with clobetasol ointment.

10.16.16.  Old lesions have disappeared and new lesions develop.  Bo evidence of systemic disease.

Reference:
1. Atypical lymphoid proliferations: the pathologist's viewpoint. Hussein MR. Expert Rev Hematol. 2013 Apr;6(2):139-53. doi: 10.1586/ehm.13.4.

Abstract: Lymphoid proliferations are traditionally thought to be either benign conditions (reactive hyperplasia and lymphadenitis) or malignant lymphomas. However, not all lymphoid lesions at present can be precisely placed into one of these categories. 

2. Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management. Wilcox RA.  Am J Hematol. 2015 Jan;90(1):73-6. Free Full Text Online.  

Difficult Leg Ulcer

The patient is an otherwise healthy 84 y.o. woman with a two year history of a progressively expanding and debilitating leg ulcer.  It has not improved after attention from three wound care centers (two of which were associated with medical schools).

This woman, a compliant retired teacher, is depressed about her debilitating leg ulcer; especially so since her PCP has been talking about "end of life care."What can we do to put life into her years?

Photos taken by her visiting nurse.