Raynaud's with Digital Ulcerations in a Smoker

The patient is a 63-year-old waitress who presents for evaluation of painful fingertip ulcerations that have been present for ten months. She has been seen by a number of physicians including the Wound Care Center. Nothing has helped.  She has had Raynaud’s Disease for 7 – 8 years.  She has been a heavy smoker for decades and has cut down from two packs per day to 1/2 ppd a month or so ago.

Medications: Nifedipine for her fingertip ulcers without any help.  She’s also taking lisinopril and carvedilol for hypertension. Her ulcerations were treated with Bactrim and oral clindamycin without effect.

The Examination shows a pleasant articulate woman. She has markedly erythematous hands that appear somewhat puffy. There are two purulent subungual digital ulcers.

Clinical Photos: (1/16/17)
The erythema was quite dramatic.  I should have taken my associate's hand next to the patient's hand for comparison.

IMPRESSION:

Raynaud’s disease with ulcerations. Should we consider progression to scleroderma?  Could this be Buerger’s Diasease?. Her history of smoking may be significant.

Lab: ANA 1:2560 Centromere, RF 25, C4 low 94, C3, nl.  Low titer antibeta 2GP Igm. Negative ACA and LAC, negative ENA, dsDNA, CCP
Xray: resorption of distal L small finger

Note:
Raynaud's occurs in two main types:

• Primary Raynaud's is the most common form of the disorder and is not connected to an underlying disease or related medical problem. It is also called Raynaud’s phenomenon.
• Secondary Raynaud's is also called Raynaud's phenomenon. This form is caused by an underlying, or related, problem. Secondary Raynaud's is less common than the primary form, but it tends to be a more serious disorder. Symptoms of secondary Raynaud's often first appear at later ages — around 40 — while people with the primary form often see symptoms earlier.

PLAN: I had a long discussion with the patient. We talked about the need to stop smoking and she promised to do so. A prescription for Bactroban ointment was given and she will be seen back in one week.

With her permission, she is being presented here on VGRD.

Follow-up:
The digital ulceration of the left fifth finger has progressed to distal reabsorption with bone protrusion.  X-rays and orthopedic consultation is pending.

Comments of Richard Sontheimer, M.D.
These are extended and useful remarks that begin with: "This patient’s digital ulceration and the puffiness of her fingers together argue strongly in favor of systemic sclerosis. The fingertip ulceration in your patient would be highly atypical for isolated benign Raynaud's disease.  And, Buerger's disease would not typically produce the sausage-like digital swelling,"  Please read the entire link as it is very helpful.
Also see Reference # 3 below.

Reference:

1. Ann Dermatol Venereol. 2013 Aug-Sep;140(8-9):549-54. doi: 10.1016/j.annder.2013.02.010. Epub 2013 Apr 11.
[Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation working group of the Société française de médecine vasculaire]. [in French]

Pistorius MA1, Carpentier PH; le groupe de travail « Microcirculation » de la Société française de médecine vasculaire.

Abstract: About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up.
  

2. A portable dermatoscope for easy, rapid examination of periungual nailfold capillary changes in patients with systemic sclerosis.
Muroi E, et. al. Rheumatol Int. 2011 Dec;31(12):1601-6. doi: 10.1007/s00296-010-1532-0. Epub 2010 May 27.

Abstract: excerpt “The presence of two or more enlarged capillaries in one or more fingers showed 83.1% sensitivity and 100% specificity for SSc. Among patients with SSc with anti-topoisomerase I antibody, the disease duration correlated negatively with the dermatoscopic number of enlarged capillaries and hemorrhages. Dermatoscope allows the easy and rapid identification of capillary nailfold morphological changes in SSc and should be routinely used for diagnosing SSc.”
 

3. Proximal nailfold microhemorrhage events are manifested as distal cuticular (eponychial) hemosiderin-containing deposits (CEHD) (syn. Maricq sign) and can aid in the diagnosis of dermatomyositis and systemic sclerosis.
McBride JD, Sontheimer RD.  Dermatol Online J. 2016 Feb 17;22  Free Full Text Online

CONCLUSIONS AND RELEVANCE:

Cuticular (eponychial) hemosoderin-containing deposits (CEHD) are in fact hemosiderin-containing deposits that can reflect the active inflammatory phase of microvascular injury occuring in autoimmune disorders such as DM and SSc. CEHD can be visualized and documented at the bedside with tools commonly available to any dermatologist (portable dermatoscope and compact digital camera).

Neonatal Acne

Presented by Dr. Henry Foong, Ipoh Malaysia

21 day old boy

normal full term delivery

breast feeding

1 week history, initially forehead then spread too cheeks and nose area

mother is healthy and asymptomatic.

O/E: symmetrical/bilateral vesicles/ pustules/ papules/comedone on affected areas

 Dx: neonatal acne/ cephalic pustulosis

A benign condition.  i think it revolves with our treatment

Treatment:  reassurance!!

Comment by a pediatric dermatology colleague: "Neonatal acne is a common newborn eruption, and it can be seen in about 20% of healthy babies. The scalp, face, neck and back and chest are commonly affected, and open comedones are usually absent as this finding would make one consider infantile acne. The lesions are usually not present at birth, but typically appear within the first 2-4 weeks of life, and generally improve by about 4-6 months of age. This condition is self-limited, however, one can consider topical antifungal or low potency topical corticosteroid if the parents seek for medical treatment. Usually reassurance would comfort the parents." [The role of P. ovale is considered]

Reference:

Neonatal Malassezia furfur pustulosis.

Rapelanoro R, et. al.

Arch Dermatol. 1996 Feb;132(2):190-3.

Abstract

BACKGROUND:

Papulopustular eruptions of the face in neonates are frequently referred to as neonatal acne or sebaceous miliaria. Our findings suggest that there is an association between this type of eruption and Malassezia furfur infection.

OBSERVATIONS:

Direct examination of pustule smears showed M furfur yeasts in eight of 13 cases involving neonates with erythema and papulopustules of the face, neck, and scalp (mean age at onset, 22 days [range, 7 to 30 days]). The pustules were predominantly neutrophilic. Treatment with 2% ketoconazole cream applied topically twice daily was effective in 1 week.

CONCLUSION:

Malassezia furfur is frequently associated with a common nonfollicular pustulosis of the newborn, probably improperly termed neonatal acne.

Aquagenic Wrinkling of the Palms

The patient is a 19 yo woman who presents with a four month history of a peculiar eruption of her palms after showering.  She spends 15 - 20 minutes in the shower.  The process resolves within a 1/2 hour.  It is mildly pruritic.

O/E:  The eruption is comprised of 1 - 2 mm in diameter white papules, discrete and confluent, on the palms.  We demonstrated these lesions in the office and they cleared within 10 minutes.  Here feet are unaffected, by history.

Clinical Photos provided by patient: (directly after shower, and when returned to baseline.

 Diagnosis:  Aquagenic Wrinkling of the Palms

Question:  The literature states that this is more common in people with cystic fibrosis and CF carriers.  This patient has no history to suggest CF.  Should genetic testing be offerred? 

Reference: There are many case reports; but the Dermnet site is a great overview. 

CF Genetic Testing. (from Cystic Fibrosis Foundation web site)
In the United States, the number of people who carry a CF gene mutation is about:

• 1 in 29 Caucasian-Americans
• 1 in 46 Hispanic-Americans
• 1 in 65 African-Americans
• 1 in 90 Asian-Americans

Pretty, Ugly Thing

This 64 yo light-complected carpenter noticed a few rough spots on his chest. Since he vacations in Mexico two to three times a years, he is worried re: skin cancer.

A skin exam showed a 4 mm nodular BCC on his chest and a few hypertrophic AKs on the chest.  An incidental finding (that he was unaware of) was a 6 mm in diameter irregularly pigmented papule on the right upper back.  The dermatoscopic image is ugly and worrisome.

He is scheduled for an excisional biopsy.

This case is very similar to that presented on VGRD last week.  Therefore, this patient probably has a SSM that is < 1 mm thick.  We will see.

New Pigmented Lesion

Thiss 56 year-old man was brought into the office by his wife who has noticed a new pigmentedlesion on his mid-back for around a year.  He did not see the point in coming in, but she made the appointment and came in with him.

The exam showed a 1.1 cm pigmented nodule with a play of color and irregular borders.  The dermatoscopic exam shows whitish areas, black areas, and pigment dots (among other things).

Clinically, I thought this was a superficially spreading melanoma.  I could not be 100% sure it was not a seborrheic keratosis.

An excisional biopsy was done.  Pathology will be added in a few days.
Pathology:  Superficial spreading melanoma, at greatest, 0.68 mm thick.
I will recommend a WLE.  Not further studies.

Note:  Often, a wife will make the appointment for her husband who is reluctant to see the physician.  This patient insisted on going to work as a pipe-fitter after the excision.  When a wife finds a lesion on her skin and asks her husband to take a look; he often says to her, "If your worried, see a doctor."

A Case for Diagnosis

History: The patient is an otherwise healthy 67 year-old writer with a three month history of an intensely pruritic papular and pustular dermatitis in an otherwise.  He’s been on Welbutrin, HCTZ and Lipitor for years.   Previously treatments with triamcinolone 0.1% ointment and prednisone for two weeks were not helpful. 

O/E:  There are hundreds of 2 – 3 mm erythematous papules on arms, legs, torso, scalp.  Face spared.  No lesions on hands, feet or genitalia.

Clinical Images:

Pathology:

Lab:  CBC, Chemistries normal.  Wound culure grew 2+ SAUR sensitive to everything.
The patient was treated with Keflex 250 mg qid for a week and Prednisone starting at 40 mg a day.  He cleared quickly, but when he stopped the prednisone after ~ 3 weeks, the eruption and pruritus recurred.  The new lesions are distinct erythematous papules mostly on the torso.  Background looks normal.

Thoughts:  Could this be "subacute prurigo" othewise known as Itchy Red Bump Disease?  I will have slides reviewed and offer another biopsy to the patient.

Cutaneous Sarcoidal Granulomas

thinking outside of the box

57 year-old woman with a one-year history of an eruption on arms and legs

This 57 y.o. woman first noticed asymptomatic, erythematous patches on arms and legs a year ago.  She is in otherwise good health and was taking Losartan/ HCTZ and Pravastatin for hypertension and cholesterol by mouth.  She lived in Texas for five years in the 1980s, but otherwise spent her entire life in Western Massachusetts.

In September 2016, biopsies obtained from the right arm and left leg showed sarcoidal granulomatous dermatitis.

Clinical:
Her skin lesions, mostly on the arms and legs, are few and scattered. They are erythematous, slightly scaly, ill-defined plaques with irregular borders.

Pathology:
Photomicrographs graphs and interpretations courtesy of Dr. Deon Wolpowitz
Boston University, Department of Dermatology
The specimen exhibits superficial and deep, nodular well-formed collections of epithelioid histiocytes and multinucleated giant cells forming granulomas with sparse to mild lymphocytic rims and a moderate superficial and mid perivascular lymphocytic infiltrate. The histologic differential diagnosis includes sarcoidosis, a foreign body reaction, and infectious etiologies including mycobacterial infections. Polariscopic examination is negative. Fite stain is negative for mycobacteria. PAS stain is negative for fungal organisms.

Lab:  CBC and chemistries were normal, as was her chest x-ray.

Discussion:  This woman has no risk factors for sarcoidosis, but did spend five years in a geographic setting where sarcoid is more commonly seen. The only thing we came up with was that she was using a cat litter with silica in it and there is one reference in the literature to that being associated with sarcoidosis.

References:

1. Cat litter is a possible trigger for sarcoidosis.

Drent M, Wijnen PA, Boots AW, Bast A. Eur Respir J. 2012 Jan;39(1):221-2.  Free Full Text.  This is the fascinating report of a single case of pulmonary  sarcoidosis that appeared to be causally related to silica containing kitty litter.

2. Mahony J, Helms SE, Brodell RT.  The sarcoidal granuloma: A unifying hypothesis for an enigmatic response. Clin Dermatol. 2014 Sep-Oct; 32(5):654-9

Abstract: Although the cause of sarcoidosis is unknown, there is growing support for the concept that sarcoidal granulomas result from a hypersensitivity reaction producing a nonspecific response to an extrinsic or intrinsic (autoimmune) antigen in genetically susceptible individuals. The immune milieu associated with these antigens, localized in a specific cutaneous area, produces a variant of Ruocco's "immunocompromised district." This may explain the predilection for sarcoidal granulomas in association with foreign bodies, tattoos, herpes zoster-affected dermatomes, and scars. Similar antigenic stimulation produces sarcoidal granulomas surrounding internal tumors. Finally, systemic sarcoidosis, as manifested by hilar adenopathy, may reflect the lymphatic spread of foreign antigens.

Chronic Localized Folliculitis

The patient is a healthy 28 yo man with a five year history of erythematous papules and pustules on the central upper chest.  No improvement over the past few years.

O/E:  There are erythematous crusted papules and pustules mid upper chest.  No other cutaneous lesions.

Clinical Photos:

Path:  4 mm punch bx x 2: Both specimens show a dense perifollicular neutrophilic iinfiltrate forming abscesses and infiltrating follicular epithelium with admixed lymphocytes, plasma cells and histiocytes.  GMS and PAS and gram stain negative.
Deon Wolpowitz, M.D. of B.U. Skin Pathology provided these impressive microscopic photographs.

Lab:  Two bacterial cultures taken from a pustules a year apart show only coagulase negative staph. 

Diagnosis: Chronic Localized Folliculitis.  Simplistically, I am thinking about Majocci's granuloma or an atypical form of acne.

The patient is reluctant to try isotretinoin or systemic antibiotics.  He is a healthy person in all other respects and has fears about messing with is microbiome and has read about isotretinoin and is worried.

Chronic Recurrent Axillary Dermatitis

Six yo old boy with > 3 year history of recurrent dermatitis

HPI:  The patient is an otherwise healthy six year-old boy with a > three year history of a dermatitis in the left axilla.  There is no pertinent family history.  He has had similar areas since infancy.  A culture taken in February 2013 showed many coag + Staph aureus. He was treated then with mupirocin ointment and betamethasone valerate 0.1% cream with good results.

O/E:  Localized crusted erosions left axilla. No other lesions today.

Clinical Photos 10/31/16)

Lab: Repeat bacterial culture taken.

Sulzberger on Caring for Patients

Yoon Cohen sent this quotation to us.  Readers of VGRD will appreciate it:

I know of no better way to start teaching a student of medicine than by repeating over and over the old-time physician's concept of his responsibilities toward his patients: to cure sometimes; to help often; to comfort always.

Perhaps the most fundamental requirement to become a more than ordinary practitioner is to be able to put yourself as wholly as possible into the patient's place. This is not as easy to do as it sounds. I told my young colleagues: "As you sit opposite your patient, try to think about his or her problems so intensely that you lift yourself mentally into his shoes, his seat, his pants, his home, his work, his problems."

They were told over and over again: "Every patient who comes to us is in trouble. Whether the complaint seems serious or trivial to you, it is serious to the patient and deserves your full attention and your best efforts. You may have just seen ten patients with more grave or more interesting skin diseases, but to the patient you are now examining, his trouble is the most important in the world at that moment. You must be kind and patient even with those who are over-demanding, unreasonable, even antagonistic. Remember that those attitudes too are signs of illness and often the results of fear, anxiety, or ignorance."  

Marion B. Sulzberger

Florid Acneiform Eruption

Presented by: 
Marina Delgado, M.D.
Apache Junction, Arizona

The patient, a 21 woman  with a 9 year history of acne is studying in Arizona.  Her acne, present since age 12, was relatively quiescent until it flared three months ago when she was doing research in Southern China.  In the past, she had been treated with topicals, antibiotics and oral contraceptives.  None were effective; but her acne was not florid as it is now.

10.9.2016

 After treatment with prednisone and isotretinoin.

5.12.2017

Photos are presented with the young woman's consent.
 
One of our pediatric dermatology colleagues suggested that this woman has pyoderma faciale.

We recommended isotretinoin plus prednisone but, because of iPledge, the patient has to wait a month to qualify for isotretinoin.

Have you managed similar patients?  What suggestions do you have?  What do you see as the role for prednisone and how long shoould it be continued?

References:

1. Pyoderma faciale: Successful treatment with isotretinoin

Victor J. Marks, Robert A. Briggaman

J Am Acad Dermatol 17, 1062–106. 1987  PDF.

2. Henry Foong. Pyoderma faciale, Virtual Grant Rounds in Dermatology, October 2001.

3.

Combination of low-dose isotretinoin and pulsed oral azithromycin in the management of moderate to severe acne: a preliminary open-label, prospective, non-comparative, single-centre study.  De D1, Kanwar AJ. Clin Drug Investig. 2011;31(8):599-604.

RESULTS: Sixty-two (93.9%) of 66 eligible patients had complete clearance of disease activity after a mean treatment duration of 21 weeks. The mean total cumulative dose of isotretinoin was 49.6 mg/kg. Seven (11.3%) patients had a relapse of disease during the post-treatment follow-up period. Fifty-three adverse effects were observed. Three patients had initial aggravation of disease that was managed with prednisolone and disappeared with continuation of treatment.

CONCLUSION: A combination of low-dose isotretinoin and oral azithromycin pulse is effective in severe acne and has a reasonably acceptable adverse-effect profile and low post-treatment relapse rates.  Abstract.

 

Extensive Darier's Disease

This 55 yo man presents with a flare of Darier’s disease. In his own words, he suffers from “mental illness” and has been on lithium for decades. He very embarassed about his skin and feels that he looks “hideous” because of this. Two yeas ago he had squamous cell carcinoma of the base of the tongue that was treated with surgery, radiation and chemothreapy.  This is in remission presently.

O/E: The examination shows widespread discrete and confluent reddish-brown greasy papules on the the chest and back. 

We have treated him successfully with isotretinoin in the past and were concerned about the possible interaction between lithium and isotretinoin, but the patient is at his wits end with his disease.

PLAN: He was requalified for iPledge today. He will be started on 40 mg of isotretinoin a day. In a month, we will do biochemistry survey, CBC, lipid profile, and lithium level.  We will obtain his last lithium level, in addition.

Have you managed similar patients and if so, what are your recommendations?

The patient was treated with 40 mg of isotretinoin daily.  After a few months, he is completely clear and his dosage is being tapered.

 

References

Isotretinoin treatment of Darier's disease.
J Am Acad Dermatol. 1982 Apr;6(4 Pt 2 Suppl):721-6.
Dicken CH, et, al.

Airborn Contact Dermatitis?

The patient is an 83 yo woman who has had a recurring pruritic dermatitis located mostly on face, neck and upper chest for two years.  It seems to be more prominent seasonally.  She has a history of a lymphoma ~ five years ago.  She had alopecia universalis for many years that spontaneously remitted ~ two years ago.

O/E:  Florid erythema of face and neck.  Submental area does not appear to be spared.

This woman has a somewhat "leonine" facies.

Diagnosis:  Initially, I thought she had a contact dermatitis or the "red face syndrome" from overuse of topical corticosteroids. She has been off the latter for > 1 year.  The has needed prednisone to control this; but I prefer a long-term medication with less side-effects.  Given her history of lymphoma, further evaluation may be necessary.


References:

Azathioprine versus betamethasone for the treatment of parthenium dermatitis: a randomized controlled study.

Verma KK1, Mahesh R, Srivastava P, Ramam M, Mukhopadhyaya AK.

Indian J Dermatol Venereol Leprol. 2008 Sep-Oct;74(5):453-7

Author information: prokverma@hotmail.com

Abstract

CONCLUSIONS: Azathioprine and betamethasone appear to be almost equally effective (P=0.0156 vs. 0.0005) in the treatment of parthenium dermatitis. However, adverse effects and relapses were observed to be more frequent in patients treated with betamethasone. Free Full Text

Postiive Band-Aid Sign

The patient is a 77 y.o. man who presented with a number of skin lesions.  He has a past history of non-melanoma skin cancer.

The lesion in question has been present for a few months.  It is an almost 5 cm in diameter exophytic tumor.

Diagnosis:  Probable Squamous Cell Carcinoma.

I anesthetized the lesion and shaved it off.  There was a fair amount of bleeding.  I curretted it and cautrized the base. It was not as soft as a typical SCC or BCC.  Specimen submitted and I'll attach a follow-up with the path.

Pathology:  Well-differentiated squamous cell carcinoma

This is a particularly good example of the "Positive-Band Aid" sign.  Most of us know this, but it has not been well-reported in the literature.  We presented this sign on the VGRD Blog in 2007.

The Tortured Tube

The patient is a 25 yo man with a 4 mo hx of an eyelid dermatitis.  His mother, a health professional, gave him 0.1% triamcinalone oinment to apply ~ 2 months ago.  It has run out and he came in for an appointment.  He is healthy and has a history of atopic dermatitis that is now quiescent.

Diagnosis and Discussion: I think this is an example of "steroid acne."  It's hard to tell what preceded it.  Most topical corticosteroids when applied for weeks or more to thin skin such as is seen on the face (expecially eyelids or around the mouth) or the genitalia can cause this.  It's a type of steroid addiction.

The standard treatment is to stop the topical steroid, apply cold compresses two time a day and doxycycline 100 mg b.i.d. for a month or more.  The longer this has been going on, the harder it is to treat.

Reference:  Dr. Ken Fowler and I reported a similar patient in 2001.

Tortured tube" sign. Fowler KP, Elpern DJ.  West J Med. 2001 Jun;174(6):383-4. Free FullText Online.

Linear Pruritic Lesions

Dr. Yogesh Jain would appreciate your comments about the following patient:

18 year old man with no significant past medical history, presented with these lesions to the OPD.  He revealed that he has been having such lesions ever since he was 2 years of age. These excoriative lesions are very itchy, but not painful. They extends from the left groin till the medial malleolus in a continous pattern. And also involve the left arm in a similar fashion affecting the palm as well. He is not on any medications.   There are no other systemic positive finding.

Dermatitis Neglecta

The patient is a 15 yo boy with a three month history of a dermatosis of his cheeks.

O/E: Slightly greenish symmetrical dermatosis of cheeks.  Othewise, normal.

Photos taken by patient's mother and emailed to me.

Dermatoscopic images before and after area was cleansed with an alcohol pledget.

Diagnosis:  Dermatitis Neglecta

There are no descriptions of the dermatoscopic appearance of this disorder.

Laugier Hunziker syndrome

The patient is a 74 yo man with a long history of oral hyperpigmentation.  He was presented on VGRD in 2012, but we have further history now.  The pigmentation has been present many years. His mother had a similar process by history.

He has a history of colon polyps. His paternal grandmother had colon cancer. His mother had colonic polyps and breast cancer. His father and his father’s brother both had leukemia.

O/E:  There are multiple dark brown irregular lenticular hyperpigmented macules of 2–5 mm diameteron the lower lip ant tongue.  No other hyperpigmentation was noted.

Clinical Image:

Diagnosis: Laugier Hunziker syndrome vs Peutz Jeghers syndrome

Case to be discussed at Hot Spots 2016

References:

1. Laugier–Hunziker Syndrome: A Rare Cause of Oral and Acral Pigmentation

Silonie Sachdeva, Shabina Sachdeva, and Pranav Kapoor

J Cutan Aesthet Surg. 2011 Jan-Apr; 4(1): 58–60.

Abstract: Laugier–Hunziker syndrome (LHS) is an acquired, benign pigmentary skin condition involving oral cavity including lower lip in the form of brown black macules 1–5 mm in size, frequently associated with longitudinal melanonychia. There is no underlying systemic abnormality or malignant predisposition associated with LHS, and therefore the prognosis is good. Important differential diagnoses include Peutz Jeghers syndrome and Addison’s disease among other causes of oral and acral pigmentation.  PubMed Central.