Tuesday, November 29, 2016

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.

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

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.

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.

Friday, October 28, 2016

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.

Thursday, October 27, 2016

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.

Sunday, October 09, 2016

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.
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?

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.

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.

Monday, October 03, 2016

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?

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

Thursday, September 22, 2016

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.

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
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

Tuesday, September 06, 2016

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.