Felon

Presented by Yoon Cohen, D.O. & David Elpern, M.D.

Abstract: A 60-year-old woman with a 2-week history of skin infection on the tip of the 3rd finger

History: a 60-year-old woman presents with a 2-week history of skin infection on the tip of the left 3rd finger. She is diabetic and does a daily glucose check by finger prick. About 2 weeks ago, she has noticed a mild swelling where she had a finger prick, and treated herself at home with Epsom salt soak and cleaning with hydrogen peroxide solution. The lesion has been intensely tender to touch. She notes that had mild fever and chills 2 days prior to the visit.

The patient was hospitalized due to heart failure, and had a pacemaker and defibrillaor implanted 2 months prior. She handles horses and dogs at home, and she usually does not wear protective wears such as gloves.

O/E: A skin exam shows a well-appearing woman with a pink to erythematous markedly edematous abscess with yellowish drainage through a small punctum on the tip of the left 3rd finger. 

Clinical Photos:

At the initial visit

5 days after

Felon (Illustration by Renee L. Cannon)

Diagnostic Studies:

• Wound culture: Staphylococcus Aureus 3+; Serratia Liquefaciens 2+ (Gram negative rod)
• Radiograph: There is soft tissue swelling with likely ulcer formation involving the distal volar tip of the third phalanx. There is associated bony erosion involving the third distal phalanx worrisome for associated acute osteomyelitis

Diagnosis: Felon

Treatment: The patient was initially started on Keflex 500 mg four times daily with warm compress twice daily. Then we switched to Ciprofloxacin 250 mg twice daily after the wound culture report. We also lanced the lesion with a #11 blade to relieve pressure and drain. We are planning to refer the patient to an infectious disease specialist for a proper treatment of the underlying infection. 

Discussion: 

A felon is an abscess of the distal pulp or phalanx pad of the fingertip. The pulp of the fingertip is divided into small compartments by 15 to 20 fibrous septa that run from the periosteum to the skin. Abscess formation in these relatively noncompliant compartments causes significant pain, and the resultant swelling can lead to tissue necrosis. Because the septa attach to the periosteum of the distal phalanx, spread of infection to the underlying bone can result in osteomyelitis.

A felon usually is caused by inoculation of bacteria into the fingertip through a penetrating trauma. The most commonly affected digits are the thumb and index finger. Common predisposing causes include splinter, bits of glass, abrasions, and minor puncture wounds. A felon also may arise when an untreated paronychia spreads into the pad of the fingertip. Felons have been reported following multiple finger-stick blood tests.

Patients present with rapid onset of severe, throbbing pain, with associated redness and swelling of the fingertip. The pain caused by a felon is usually more intense than that caused by paronychia. The swelling will not extend proximal to the distal interphalangeal joint. Occasionally, the high pressure in the fingertip pad will cause a felon to spontaneously drain, resulting in a visible sinus.

If diagnosed in the early stages of cellulitis, a felon may be amenable to treatment with elevation, oral antibiotics, and warm water or saline soaks. Bone and soft tissue radiographs should be obtained to evaluate for osteomyelitis or a foreign body. Tetanus prophylaxis should be administered when necessary.

If fluctuance is present, incision and drainage are appropriate. Wound culture should be obtained to guide the optimal coverage of the underlying organisms. 

References:
1. Dwayne C, et al. Naval Hospital Jacksonville, Florida. Common Acute Hand Infections. Am Fam Physician. 2003. 1;68 (11):2167-76

Generalized Dermatitis in an 85 yo Woman

85 year old woman with wide-spread dermatitis.

HPI:  This 85 yo woman has had an evolving dermatitis for four to five months.  It began around two years ago with some spots on her legs.  Initially treated with clobetasol oint and prednisone.  She did well, but it recurred on her legs and has spread over the past few months.  It is moderately pruritic.  Initially, she was using clobetasol ointment.  She saw another provider recently who preformed a biopssy and ]prescribed calcipotriene which has caused increased pruritus.  Her only medications are levothyroxine and bisoprolol-hydrochlorthiazide which she's been on for a few years.  She has been under significant life-stress over the past two months.

Past Medical History:  As a teenager, during World War II, the patient was sent to California from her home in Hawaii.  During this stressful period, she had an eczematous eruption on her extremities.

Exam:  There is a generalized dermatitis consisting of erythematous scaly patches,  It covers all body surfaces including the face.

Clinical Photos: (June 18, 2014)

Lab:

Pathology:  We've asked for the path report.

Diagnosis: Eczematous eruption in an octogenarian.

Acral Melanoma in a Malay Woman

Abstract: 70 yo woman with acral melanoma

Presented by Dr. Henry Foong, Ipoh Malaysia

HPI: The patient is a 70-year-old Malay woman who presented with a one-year history of a pigmented lesion on the left foot.  She has seen at least 4 doctors and I am sure all have advised her to have a biopsy done. It was occasionally painful but otherwise asymptomatic.  The lesion had been gradually increasing in size.

Her medical history includes diabetes, hypertension and hypercholesterolemia.  She is on glibenclamide, metformin, perindopril, aspirin, hydrochlorothiazide and lovastatin.

She lives in a rural area south of Ipoh, Malaysia. She has 10 children.  There was no family history of skin cancer.

O/E:  shows a localised pigmented tumor 3 x 3 cm with superficial ulcerations on the medial aspect of the sole of left foot.  It has an irregular margin but was well circumscribed.  The nodule is firm on deep palpation.

Clinical Images:

Skin Biopsy

Nests of melanoma cells are seen invading the dermis. The tumour cells are pleomorphic, have vesicular nuclei and eosinophilic cytoplasm. There is increased mitotic activity. Many of the cells contain melanin pigment.

Diagnosis:  Left foot biopsy Malignant melanoma, acrolentiginous type, nodular

Discussion and Questions: We rarely see melanoma here in Malaysia.  The prevalence rate is reported to be about 0.4 per 100 000 population.  I have not had a single case of melanoma the whole of last 2 years.  This patient waited for a year before a diagnosis was made.  What has gone wrong? 

The histopath report unfortunately did not indicate the thickness of the tumor neither is there any mitotic rate or Clark’s level of invasion.  In a study in Malaysia most of the cases are located on the sole of the foot as in this patient.  (12/24 cases) Histologically majority are of the nodular type.  I think based on the report, our patient has a nodular type of melanoma.

Plan: 

Dermatologists in Malaysia don't manage malignant melanoma.  Instead, they are referred to surgeons for excision.  Sentinal node biopsy and CT scan abdomen and chest would be useful for staging of the tumor.  Would PET scan give more useful information for this patient?  Immunotherapy and BRAF inhibitors are probably too expensive  for her.

References:

1. Malaysian J Pathol 2012; 34(2) : 97 – 101

Cutaneous malignant melanoma: clinical and histopathological

review of cases in a Malaysian tertiary referral centre

Jayalakshmi PAILOOR, Kein-Seong MUN and Margaret LEOW*

Departments of Pathology and *Surgery, Faculty of Medicine, University of Malaya

Abstract

Melanoma is a lethal skin cancer that occurs predominantly among Caucasians. In Malaysia, the incidence of melanoma is low. This is a retrospective study of clinical and histopathological features of patients with cutaneous melanoma who were seen at the University Malaya Medical Centre from 1998 to 2008. Thirty-two patients with cutaneous melanoma were recorded during that period. Of these, 24 had sought treatment at the onset of disease at our centre. Chinese patients constituted the largest group (19 cases). The median age of these 24 patients at the time of presentation was 62 years. 16 patients had melanoma involving the lower limb with 12 affecting the sole of the foot. None had melanoma arising from the face. Histopathology showed nodular melanoma in 22 cases (91.6%), with superficial spreading and acral lentiginous melanoma diagnosed in 1 case each. The majority of patients (62.5%) were found to be in Stage III of the disease at the time of diagnosis.

2.  Whiteman DC1, Pavan WJ, Bastian BC. The melanomas: a synthesis of epidemiological, clinical, histopathological, genetic, and biological aspects, supporting distinct subtypes, causal pathways, and cells of origin. Pigment Cell Melanoma Res. 2011 Oct;24(5):879-97.  Free Full Text

Abstract:  Converging lines of evidence from varied scientific disciplines suggest that cutaneous melanomas comprise biologically distinct subtypes that arise through multiple causal pathways. Understanding the respective relationships of each subtype with etiologic factors such as UV radiation and constitutional factors is the first necessary step toward developing refined prevention strategies for the specific forms of melanoma. Furthermore, classifying this disease precisely into biologically distinct subtypes is the key to developing mechanism-based treatments, as highlighted by recent discoveries. In this review, we outline the historical developments that underpin our understanding of melanoma heterogeneity, and we do this from the perspectives of clinical presentation, histopathology, epidemiology, molecular genetics, and developmental biology. We integrate the evidence from these separate trajectories to catalog the emerging major categories of melanomas and conclude with important unanswered questions relating to the development of melanoma and its cells of origin.

A case of severe pemphigus vulgaris

Recently I saw this 40 year old woman who presented with severe blisters and erosions.  It started about 3 weeks ago and spread quite rapidly from the chest to the face and extremities.  Now her mouth is also involved.

Exam:  generalised bulla, majority are flaccid type.  Erosions extensive with crusts esp anterior chest wall.  Superficial erosions on the lower lips.  Nikolysky sign positive.

Skin biopsy:  Section shows skin composed of epidermis and dermis. A suprabasal bulla
containing acantholytic cells is seen. In areas, a tombstone appearance
is seen. Infiltrates of eosinophils and lymphocytes are seen in the
dermis.

Pemphigus vulgaris 

Blood counts and biochemistry are normal.   

She was treated as outpatient.  I have started her on oral prednisolone 40mg daily.  Added oral antibiotics cefuroxime 250mg bd.  Skin dressing with KMNO4 wet compress followed by fusidin/hydrocort cream bd.  Keep skin roof intact.

Questions:

1. I do not have IF facilities.  Will this lack of facilities affect her prognosis?
2. what kind of skin dressing would you use?
3. would you use steroid sparing agent like mycophenolate, MTX , azathioprine or ritazulimab at this juncture?

Thanks for your help.  Your comments are greatly valued.

 References:

Cochrane reviews: Interventions for pemphigus vulgaris and pemphigus foliaceus

Martin LK, Agero AL, Werth V, Villanueva E, Segall J, Murrell DF

Published Online: 

15 April 2009

This review of clinical trials aimed to find out which is the most effective and safest treatment option for pemphigus vulgaris and pemphigus foliaceus.

Pemphigus vulgaris and pemphigus foliaceus are rare diseases characterised by fragile blisters and sores on the skin and mucosa. They are auto-immune diseases which are caused by the body making an antibody against the person's own skin. These diseases are chronic and are not currently curable. Pemphigus vulgaris and foliaceus are managed with drugs which suppress the immune system. The aim of treatment is to suppress blister formation. Systemic glucocorticoids are the cornerstone of management in pemphigus, however adjuvant immunosuppressive and anti-inflammatory agents are commonly used. There are many treatments available, however it is not known which is the most effective or safest treatment option, or which is the best combination.

This review included data from 11 clinical trials involving 404 participants. The studies had very small numbers of participants, so can provide only limited information. Ten different active treatments were studied, including prednisolone, pulsed oral dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth factor and traditional Chinese medicine.

This review found insufficient information to conclude which is the most effective and safest treatment plan. We found that mycophenolate mofetil appears to be more effective than azathioprine in controlling disease, although no difference was seen in remission. We found that taking azathioprine and cyclophosphamide decreased the amount of glucocorticoids required. Topical epidermal growth factor decreased time required for lesions to heal by 6 days (median). We found no difference in withdrawal due to adverse events in any study, although differing adverse event profiles were observed for each intervention. We were not able to conclude which treatments are superior overall.

Multiple treatments are available for pemphigus vulgaris and pemphigus foliaceus and there is a variation in dosage plan and combination of drugs used, which makes choice of treatment schedule complex. In addition, response to treatment can vary between individuals. Treatments need to be chosen after careful consideration of the potential benefits and side effects, in the context of the individual's other medical conditions. This review found insufficient information to conclude which is the most effective and safest treatment regimen. Further studies are required to determine the optimal treatment regimen, especially to assess the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve harm:benefit analyses.

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Median Nail Dystrophy

Patient 1: A 65 yo woman with a 3 month history of a medial split in the right thumb nail. It is asymptomatic. No history of trauma. The physical exam showed the split begins at the proximal nail fold. There is a suggestion of erythema at its proximal end.

Patient 2: A 79 yo woman with a 3-6 months history of a medial split in the right thumb nail. It is painful with pressure otherwise asymptomatic. No history of trauma. The physical exam reveals the same finding as the patient 1 without a suggestion of erythema at the proximal fold. 

Clinical Images:  

Patient 1: Clinical View

Patient 1: Dermoscopic View

Patient 2: Clinical View

Patient 2: Dermoscopic View

Diagnosis: Median nail dystrophy.  Is this secondary to a subungual tumor? Is a biopsy indicated?

Reference:

Glomus tumor-induced longitudinal splitting of nail mimicking median canaliform dystrophy. Verma SB. Indian J Dermatol Venereol Leprol. 2008 May-Jun;74(3):257-9.

Abstract:Median canaliform deformity of the nail is an uncommon entity, where there is longitudinal splitting of the nail. Longitudinal splitting of the nail is a rare phenomenon and can also occur following number of growths arising in the nail matrix. On examination there was a longitudinal split in the nail plate, beginning in the distal nail fold and extending proximally all the way to the proximal nail fold. There was a small, almost indiscernible, swelling in that area, which was exquisitely tender. The split part of the nail showed a little discoloration. There was no discharge, bleeding, or subungual mass visible. 'Love test' was positive in this case. After nail avulsion, a small 2 mm x 4 mm nodule was exposed and excised. Histopathological examination of the tumor showed a mantle of glomus cells surrounding the blood vessels.  Free Full Text.

Cervical Nodules in a Healthy 65 Year-old Woman

Abstract:  One month history of three nodules in the right posterior cervical area in a 65 year-old woman.

HPI:  The patient is an otherwise healthy, immunocompetent woman who has noted three slightly painful nodules that appeared in the right posterior nuchal area. She has a history of  scalp excoriations.  She has two cats, one of which sleeps with her.

O/E:  Initially, there were three firm nodules measuring 0.8 to 1.5 cm in diameter.  She has had superficial scalp erosions secondary to excoriation for about a year.

Clinical Photos (courtesy of Yoon Cohen, D.O.)

Scar is biopsy site

Lab:  CBC normal, Chemistries normal.  Chest Xray normal.  Cat Scratch serology drawn six weeks after onset:  Bartonella Henselae IgG 1:1280 and Bartonella Quintana IgG 1:640  (IgM negative for both. Negative is <1:320)

Pathology: (photomicrographs courtesy of Lynne Goldberg, M.D., Boston University Skin Path) Because this was thought to be lymphadenopathy, a deep incisional biopsy was performed.  To our surprise, no lymphatic tissue was seen.  Rather, the pathology showed stellate subcutaneous microabscesses with a surrounding lymphohistiocytic infiltrate and fibrosis.

Course:  The patient continues to feel well.  An abscess was drained and around 1 cc of viscous pus was withdrawn.  It has refilled.  Since the patient feels well, we have not prescribed antibiotics.

Diagnosis: Cutaneous abscesses in Cat Scratch Disease.

Discussion: There are no reports of cutaneous abscesses in patients with CSD.  It may be possible that the suppuration we noted occurred as a sequela to an infected lymph node.  A review of the medical literature was not helpful here.  Recommendations for antibiotic therapy of immunocompetent with no systemic signs or symptoms are equivocal and we have elected to observe our patient for the time being.  If we decide to recommendad treatment, it will probably be with erythromycin.

References:

1.  eMedicine.comhttp://emedicine.medscape.com/article/214100-overview:  Catscratch disease (CSD), also known as catscratch fever or subacute regional lymphadenitis, is a bacterial infection affecting lymph nodes that drain the sites of inoculation. Bartonella henselae, a gram-negative rod, is considered the principal etiologic agent. CSD is a common cause of chronic lymphadenopathy in children and adolescents.

Patients with CSD usually have a history of sustaining a scratch or bite from a cat (typically a kitten). The initial symptom is formation of a papule at the inoculation site, followed by solitary or regional lymphadenopathy within 1-2 weeks (see the images below). In most patients, the disease resolves spontaneously within 2-4 months.

2. Abscess-forming lymphadenopathy and osteomyelitis in children with Bartonella henselae infection.  Ridder-Schröter R, et. al. J Med Microbiol. 2008 Apr;57(Pt 4):519-24. doi: 10.1099/jmm.0.47438-0.

Abstract: Bartonella henselae is the agent of cat-scratch disease (CSD), a chronic lymphadenopathy among children and adolescents. A systemic infection is very rare and most of these cases are found in patients with immunodeficiency. Here, cases involving four children of 6-12 years of age are reported. In immunocompetent patients, infection affects skin and draining lymph nodes; however, prolonged fever of unknown origin as in the fourth patient indicated a systemic complication of CSD. Free full text.

Median Nail Dystrophy

The patient is a 65 yo woman with a 3 month history of a medial split in the right thumb nail.  It is asymptomatic.  No history of trauma.

O/E:  The split begins at the proximal nail fold.  There is a suggestion of erythema at its proximal end.

Clinical Images:  Courtesy of Yoon Cohen

Diagnosis: Median nail Dystrophy.  Is this secondary to a subungual tumor?

Reference:

Glomus tumor-induced longitudinal splitting of nail mimicking median canaliform dystrophy. Verma SB. Indian J Dermatol Venereol Leprol. 2008 May-Jun;74(3):257-9.

Abstract:Median canaliform deformity of the nail is an uncommon entity, where there is longitudinal splitting of the nail. Longitudinal splitting of the nail is a rare phenomenon and can also occur following number of growths arising in the nail matrix. On examination there was a longitudinal split in the nail plate, beginning in the distal nail fold and extending proximally all the way to the proximal nail fold. There was a small, almost indiscernible, swelling in that area, which was exquisitely tender. The split part of the nail showed a little discoloration. There was no discharge, bleeding, or subungual mass visible. 'Love test' was positive in this case. After nail avulsion, a small 2 mm x 4 mm nodule was exposed and excised. Histopathological examination of the tumor showed a mantle of glomus cells surrounding the blood vessels.  Free Full Text.

Birt-Hogg-Dube Syndrome

Presented by Yoon Cohen, D.O. 
Alta Dermatology 
Mesa, Arizona

This patient's case courtesy of Bradley Kurgis, D.O. in San Luis Obispo, CA

Abstract: 54 yo woman with multiple white firm papules on the face since her 30s. 

HPI: This is a 54 yo woman with multiple scattered 2-4 mm white firm papules on the face and upper neck. She has first noticed her facial lesions at her late 30s. Previously a biopsy was obtained on the right cheek which showed sebaceous hyperplasia. 

She is not a smoker; however, she had four episodes of spontaneous pneumothorax in the past at her early 20s. At that time, she was informed that she had multiple "blebs" in her right lower lobe which resulted in the right lower lobectomy. Recently she had a colonoscopy with multiple benign polyps, otherwise, the only other heath issue is hypertension. Her father and paternal grandmother had the same skin findings on the face and the neck.  Her paternal grandmother also had episodes of pneumothorax in her right lower lobe and died of renal failure. No actual renal or thyroid cancers history in the family. No GI related malignancy in the family. 

The patient will be back in 2 weeks for another biopsy. If it shows fibrofolliculoma or trichodiscoma, we will obtain her renal ultrasound, abd/pelvic CTs and chest Xray. 

Family medical history of the patient

O/E: There are multiple scattered 2-4 mm white firm shiny papules throughout the face, the ear lobes and the upper neck. There are multiple acrochordons in the axillary areas which can be common findings in any individuals in her age group. No gingival papules or palmoplantar keratoses noted that you commonly observe in Cowden syndrome.

Clinical Photos (with permission of the patient): 

Dermatoscopic view shows well-demarcated areas of pallor with central follicular opening

Close-up view of dermatoscopic view

 Jarrett R, Walker L, Side L, et al. Dermoscopic features of Birt-Hogg-Dube Syndrome. Arch Dermtol. 2009; 145 (10): 1208.

Patient's father with the same facial lesions, deceased 2 years prior

Pathology: The repeated biopsy will be obtained 

Presumptive Diagnosis: Most likely Birt-Hogg-Dube Syndrome, but we also consider Cowden synydrome

Discussion: 
Birt-Hogg-Dubé syndrome (BHDS) was originally described in 1977 as the grouping of 3 skin tumors-the fibrofolliculoma, trichodiscoma, and acrochordon-in family members with an autosomal dominant inheritance pattern. [1] The condition is caused by germline mutations in the FLCN gene, which encodes folliculin; the function of this protein is largely unknown.[3] The recent years it has become clear that these 3 lesions likely represent only 1 of these tumors, the fibrofolliculoma. More important, evidence now supports a definite susceptibility to malignant renal tumors and pulmonary disease in patients with BHDS. Clinical recognition of this entity is possible in spite of the fact that several syndromes exist that are characterized by the presence of multiple firm facial papules.[1] [5] Jarrett, et al reported dermatoscopic findings of BHDS which showed well-demarcated pallor with central follicular opening. [2] The notably similar dermatoscopic findings were found in this patient. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.[4]

The patient reported that she has seen many physicians including dermatologists for her facial lesions in the past. However, she was repeatedly told that they could not help due to the generalized nature of the lesions, and she was dismissed without any further work-ups. Her case demonstrates our orphan patient today. 

Questions: 
1. What are your thoughts? Any suggestions for diagnostic studies?
2. The patient's most concern at this time is her facial appearance. Any treatment suggestions would be greatly appreciated.  

References:
1. Vincent A, Chan E, James W. Birt-Hogg-Dube syndrome: A review of the literature and the differential diagnosis of firm facial papules. J Am Acad Dermatol. 2003;49:698-705.

2. Jarrett R, Walker L, Side L, et al. Dermoscopic features of Birt-Hogg-Dube Syndrome. Arch Dermtol. 2009; 145 (10): 1208.

3. Menko FH, van Steensel MA, Giraud S, et al. Birt-Hogg-Dube syndrome: diagnosis and management. Lancet Oncol. 2009;10(12):1199-206.

4. Lencastre A, et al. Birt-Hogg-Dube syndrome. An Bras Dermatol. 2013;88:203-5.

5. Warwick Gm et al. Renal cancer associated with recurrent spontaneous pneumothorax in Birt-Hogg-Dube syndrome: a case report and review of the literature. J Med Case Reports. 2010;4:106.