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.

Annular Lesions in a 50 yo woman

This image was sent by Dr. Yogesh Jain from India for diagnostic suggestions.
The only history provided was that the process is present on the hands and feet and has been ongoing for 25 years. The lesions regress after a number of months.

Other than a variant of granuloma annulare or elastosis perforans serpiginosa, what are your thoughts?

Biopsy is important but so is the dermatopathologist who reads it.

What is Right Care?

The patient is a 90 year-old man, homebound with a dementia.  His 87 year-old wife is a steadfast, loving and loyal caregiver.  His dermatologist has made house calls for the past four months. 

There are non-melanoma skin cancers on the left cheek  and mid upper lip.  The former lesion has increased from 1.3 to 1.5 mm in diameter and the the lip lesion has increased from 1.8 to 2.2 mm in diameter in the past two months.  Both are somewhat inflammatory and crusted. He picks on the lip lesion, but because of his dementia he can not articulate what it is that bothers him.

7.28/15

March 2016

6.28.2016

8.11.16

Thoughts: The question is what is the best treatment for this man.  The lesion on the left cheek could be curetted and desiccated in the office. The lesion on the lip is a more complicated problem. 

A trial of topical 5-FU plus imiquimod may be helpful, especially for the lesion on the lip, as a palliative procedure.  The lesion on the malar eminence which grew rapidly ~ 6 months ago and is either a squamous cell or a keratoacanthoma) could be curetted and dessicated.

The patient can not make a decision for himself and his wife wants to just watch these lesions.  She understands that treatment is not likely impact on his quality of life at this point and want’s to spare him the trauma or surgery.

As physicians, we feel compelled to “do something.”  Is this the right time to “not just do something, but to sit there.”

Reference:

1. Linos E. Treatment of nonfatal conditions at the end of life: nonmelanoma skin cancer.  JAMA Intern Med. 2013 Jun 10;173(11):1006-12

CONCLUSIONS AND RELEVANCE: Most NMSCs are treated surgically, regardless of the patient's life expectancy. Given the very low tumor recurrence rates and high mortality from causes unrelated to NMSC in patients with limited life expectancy, clinicians should consider whether these patients would prefer less invasive treatment strategies.  PubMed.  PMC Free Full Text.

2.  Knocking on Heaven’s Door by Katie Butler is an honest, sobering book that describes what awaits so many elderly people and their caregivers, who are often family members.  It is relevant to how one manages a patient such as the man described and discussed here.

Hypopigmentation in an African

The patient is a 39 yo man from Ghana.  His wife noticed these spots on his back recently.  My first diagnosis was tinea versicolor; but KOH prep showed only spores.  Is this just quiescent T.v.?  It's symmetrically distributed over upper back (no where else).  In differential diagnosis was vitiligo -- but this is incomplete hypopigmentyation (which can occur with vitiligo, but less commonly).  I suggested ketoconazole cream and a follow-up in 3 months.  If still present, may do a biopsy.
What are your thoughts?

References:

1. The utility of dermoscopy in the diagnosis of evolving lesions of vitiligo.

Thatte SS1, Khopkar US.  Indian J Dermatol Venereol Leprol. 2014 Nov-Dec;80(6):505-8.

BACKGROURD: Early lesions of vitiligo can be confused with various other causes of hypopigmentation and depigmentation. Few workers have utilized dermoscopy for the diagnosis of evolving lesions of vitiligo.

CONCLUSION: Pigmentary network changes, and perifollicular and perilesional hyperpigmentation on polarized light examination, and a diffuse white glow on ultraviolet light examination were noted in evolving vitiligo lesions. Histopathological examination was comparatively less reliable. Dermoscopy appears to be better than routine histopathology in the diagnosis of evolving lesions of vitiligo and can obviate the need for a skin biopsy. Free Full Text.

2. Dermoscopy as an ancillary tool for the diagnosis of pityriasis versicolor.

Zhou H, Tang XH, Chen MK. J Am Acad Dermatol. 2015 Dec;73(6):e205-6. (this is only reference in PubMed on T.v. and dermsocopy and it is not particularly helpful)

3. Dermatoscope--the dermatologist's stethoscope.

Lallas A, Argenziano G.  Indian J Dermatol Venereol Leprol. 2014 Nov-Dec;80(6):493-4. Full Free Text 

This is an interesting somewhat philosophical article.  The references are extensive and helpful.

Alopecia Universalis in a Teenage Girl

Presented by Henry Foong, Ipoh, Malaysia

Here is a patient I saw recently.  She is a 17-year-old girl who has a history of severe alopecia since the age of 12 years.  It was abrupt and sudden with marked loss of scalp hair followed by eye brows and other body hair.  Within a month, she had developed alopecia universalis.  She was initially treated with intralesional triamcinolone and topical minoxidil but did not help.  Subsequently she had NB-UVB 3 times weekly in the local hospital but that also did not do much good.  She was advised to go to tertiary centres in KL but was disappointed with only one visit.  She could not go to NSC in Singapore because of financial constraints.  As a Malay, she always wears a tudong to cover her scalp.  There was no other systemic complaints.  She is the 4th in the family of 5 siblings.  No family history of alopecia. 

She saw me yesterday.  Am trying out DPCP diphencyprone sensitisation for her.  She had a previous sensitisation done but quit after one treatment.  She had total alopecia affecting the scalp, eyebrow, eyelash, axillary and suprapubic area. Used 0.1% DPCP concentration, left on the scalp for 24 hours and reviewed the next day.  She does have good reaction with small vesicles and plan to do it weekly till her hair grows.  The eyebrow hair loss was treated with intralesional triamcinolone acetonide injection of 10 mg/ml strength.  According to literature, topical minoxidil 5% solution, topical clobetasol ointment and weekly methotrexate 25mg/wk do help too.  Other novel therapy would include JAK inhibitors.
Comment:  Who has had real success treating patients with AU? Are there any lab tests of real value?

After DPCP sensitization

References:

1. Clinical Efficacy of Diphenylcyclopropenone in Alopecia Areata: Retrospective Data Analysis of 50 Patients.  Chiang KS, Mesinkovska NA, Piliang MP, Bergfeld WF. J Investig Dermatol Symp Proc. 2015 Nov;17(2):50-5.

Abstract: Diphenylcyclopropenone (DPCP) is widely considered the most effective topical immunotherapy for refractory or extensive alopecia areata (AA), but questions regarding how long to try DPCP therapy before terminating and what factors are prognostic of therapeutic success still remain unanswered. In this retrospective study of 50 AA patients, we evaluated DPCP efficacy and identified patient factors predictive of therapeutic success/failure. The median duration of DPCP treatment was 3 years, with 47% patients experiencing their first regrowth in the first 6 months of DPCP therapy, 20% between 6 months-1 year, and 8% between 1-2 years. In our study, treatment success, defined as ⩾50% terminal hair regrowth, was reached in 71% of alopecia totalis patients and in 56% of alopecia universalis patients. Three factors were statistically significant predictors of poor treatment outcome-extent of hair loss before DPCP treatment, history of thyroid disease, and extent of body hair involvement. Relapse was observed in 44% of patients and significantly associated with history of thyroid disease. Common side effects were itching, rash, and local lymphadenopathy. The results of this study support our belief that DPCP therapy is a viable treatment option, can be successfully accomplished at home, and should not be terminated before 2 years.

2.  Pulse corticosteroid therapy for alopecia areata: study of 139 patients.

Nakajima T1, Inui S, Itami S. Dermatology. 2007;215(4):320-4.

Author information

Abstract: Of the patients, 72.7% had hair loss on > 50% of their scalp area. Among the recent-onset group (duration of AA < or = 6 months), 59.4% were good responders (> 75% regrowth of alopecia lesions), while 15.8% with > 6 months duration showed a good response. Recent-onset AA patients with less severe disease (< or = 50% hair loss) responded at a rate of 88.0%, but only 21.4% of recent-onset patients with 100% hair loss responded. No serious adverse effects were observed.

3. Association between vitamin D levels and alopecia areata.

Mahamid M, Abu-Elhija O, Samamra M, Mahamid A, Nseir W. Isr Med Assoc J. 2014 Jun;16(6):367-70.

RESULTS: Mean CRP values were significantly higher in the AA group than the control group (1.1 +/- 0.7 mg/dl vs. 0.4 +/- 0.8 mg/ dl, P < 0.05). Vitamin D levels were significantly decreased in the AA group (11.32 +/- 10.18 ng/ml vs. 21.55 +/- 13.62 ng/ml in the control group, P < 0.05). Multivariate analysis showed that CRP (odds ratio 3.1, 95% confidence interval 2.6-4.2, P = 0.04) and serum vitamin D levels < 30 ng/ml (OR 2.3, 95% CI 2.2-3.1, P = 0.02) were associated with AA.

CONCLUSIONS: We found a significant correlation between AA and vitamin D deficiency. Vitamin D deficiency can be a significant risk factor for AA occurrence.

4. Pulse corticosteroid therapy with oral dexamethasone for the treatment of adult alopecia totalis and universalis JAAD, May 2016  Link.

Chronic Stasis Dermatitis

Abstract: 68 yo man with chronic dermatitis of left leg

History: The patient has had a chronic dermatitis of left leg for over ten years.  He has been seen at the Wound Care Clinic for over three years.  What worked best in the past was an Unna boot but these are not used as commonly anymore as we have newer, more high-tech types of bandages. 

O/E: He has chronic lymphedema of the left leg, which periodically develops elephantiasis-like changes. The examination woody edema left leg, that is erythematous with crusted areas.  It looks clean today and there is no evidence of secondary infection.

Impression:  Chronic dermatitis of his left leg.  It is most likely a chronic stasis eczema. 

Comments: He has been diligent about home care.  For the time being, he will use wet dressings with a dilute bleach (a half cup of Chlorox per 40 gallons of water in a bathtub) and zinc oxide-like paste.  He will be seen back in two weeks.  Consider going back to an Unna boot.

What are your thoughts?

Unusual Tumor in a 53 yo Man

53-year-old man who was seen for a general skin examination.  He has Crohn's disease and has been on 6-MP for the past 8-10 years.  His gastroenterologist said because of this he should have a skin examination. 

O/E:  The patient has type II skin.  He has a 6 mm in diameter pink papule on the calf of the left leg.  Dermoscopically, this lesion has a dotted vascular pattern.

IMPRESSION:  Lesion left calf.  Etiology unclear.  

Plan:  Excisional biopsy was obtained. 

Reference:
Inflammatory Bowel Disease Treatment and Non-melanoma Skin Cancer: Jacqueline F De Luca, MD, Yun Sun Lee, MD, and Douglas Johnson, MD, Hawaii J Med Public Health. 2012.7:324–325.

Abstract:Immunosuppressant medications for Inflammatory Bowel Disease can help with both symptoms and disease progression. However, like immunosuppressants used in transplant patients, they are now suspect of contributing to nonmelenoma skin cancer (NMSC). Presented is a case of a 57-year-old Jewish man with Crohn's Disease who was diagnosed with a total of 84 NMSCs. We hope to elucidate the risk of immunosuppressants, particularly the thiopurines, on the development of NMSC.

 

Congenital Triangular Alopecia

The patient is an 11 y.o boy seen for an unrelated problem.  His mother noted an alopecic area in her newborn and attributed it to something the obstetrician did,

O/E:  There is a 1.5 xm area of alopecia on the left fronto-temporal area.

Diagnosis: Temporal (Congenital) Triangular Alopecia

We have presented two similar cases in the past.

Here is a free full text article from the Indian Journal of Dermatology

Labial Macules

The patient is a 33 year-old woman who recently noted pigmented macules on her lower lips.  She admits to having an anxiety disorder and being preoccupied with her health (which is otherwise excellent).  Her anxiety dates back to the death of her father from colon cancer at age 52 (when she was 10 years old).  She was seen for an unusual cheilitis in 2010 and her case was presented on VGRD then.

O/E:  There are six lightly pigmented macules on her lower lip.  No other pigmentation on her oral mucosae. No other pigmented macules noted.

Discussion: While these macules could be post-inflammatory changes from her allergic cheilitis, it's hard to ignore her family history and the possibility that they could be a forme fruste of Peutz-Jeghers Syndrome.  Endoscopy seems, to me at least, to be indicated.  With classical PJS the pigmented macules may be present even in infancy or childhood.  Visualizing her G.I. tract is important here, if only to allay this woman's anxiety.

Your thoughts will be appreciated.

Bullae and Erosions in a Newborn

Presented by Dr. Henry Foong

Ipoh, Malaysia

This 20-day-old baby presented with multiple blisters on the hands and feet.  The mother first noticed them on Day 2 after a normal delivery when there were blisters on the hands.  Subsequently, she noticed blisters on the feet as well.  These blisters ruptured easily on friction or minimal rubbing. Recently he has difficulty in swallowing as well. There was no fever.  No family history of similar problems and no history of consanguinity in the family.

He has seen paediatricians and dermatologists but his condition did not improve.

The child looks fretful.  Superficial erosions with crusts were mainly noted on his hands and feet.  A superficial bulla 0.5 x 0.5 cm was present on the left index finger.  Mucosa of the lips appeared eroded.  These was no nail involvement.

Do you see this condition? How do you manage this in your institution? I guess this will be a tough management problem for the child and the family.

 Diagnosis:  This child most likely has epidermolysis bullosa, probably recessive dystrophic EB, although the Hurlitz type needs to be considered as well. 

Our pediatric dermatologists can help with management suggestions. 

Definitive diagnosis can be obtained with biopsy.  In the past, electron microscopy was utilized, but there may be simpler studies.

Note:  At an appropriate time, if the diagnosis is more certain, the parents might want to visit the DEBRA site. There is a resources in Malaysian, in addition: DebraMalaysia.

References:
1. Recessive Dystrophic Epidermolysis Bullosa

A Review of Disease Pathogenesis and Update on Future Therapies

Luis Soro, DO et. al  J Clin Aesthet Dermatol. 2015. 8(5): 41–46.

Abstract: Objective: Review the pathogenesis of recessive dystrophic epidermolysis bullosa and provide an update on research currently underway that is aimed at treating and potentially curing this severe skin disorder. Design: Review article. Setting: Private practice and large teaching hospital. Participants: None. Measurements: N/A. Results: Currently, patients with recessive dystrophic epidermolysis bullosa are managed with only supportive care. However, there are several promising new treatment avenues that may help patients in the future. These include gene therapy, cell therapy, and protein-based therapy. Each approach offers distinct advantages and disadvantages. Conclusions: The advances in understanding the molecular basis for epidermolysis bullosa over the last few decades has led to significant progress in devising new treatment options. Though many of these approaches remain several years away from regular implementation, it is an exciting time for research in the field.  Free Full Text.

2. Recessive Dystrophic Epidermolysis Bullosa: Advances in the Laboratory Leading to New Therapies. Woodley DT, Chen M. J Invest Dermatol. 2015;135(7):1705-7.  Free Full Text.

In the Maze of the Technical Exhibit

zombies roam these halls

collecting priceless samples

in overstuffed bags

after exhausting themselves in the cavernous maze, a relaxing dinner sustained the weary tropers and their consorts.

Rwecalcitrant Warts

The patient is an 11 yo girl with difficult-to-treat warts present for 4 years.  Her pediatrician has used cryotherapy, and a vesicant.  They have tried salicylic acid under occlusion for a month and the warts have grown.

The child is very self-conscious.  What would you do?

A Thing of Beauty

The patient is a 7 year-old boy with a lesion present since infancy.  He was adpopted at age 11 months and prior history is unknown.  The lesion has changed only minimumly over the past few years.

O/E:  7 mm lesion with macular dots and two darker pigmented papules on the left abdomen. He has a small area of segmental vitiligo on the left neck.

Ckinical and Dermatoscopic Images:

Workup:  None at this time

Diagnosis:  Nevus spilus, Speckled Lentiginous Nevus
Note:  An association of segmental vitiligo with SLN has not been reported.

Reference:

Speckled lentiginous naevus: which of the two disorders do you mean?

Happle R1.  Clin Exp Dermatol. 2009 Mar;34(2):133-5

Abstract: Speckled lentiginous naevus (synonym: naevus spilus) no longer represents one clinical entity, but rather, two different disorders can be distinguished. Naevus spilus maculosus is consistently found in phacomatosis spilorosea, whereas naevus spilus papulosus represents a hallmark of phacomatosis pigmentokeratotica. The macular type is characterized by dark speckles that are completely flat and rather evenly distributed on a light brown background, resembling a polka-dot pattern. In contrast, naevus spilus papulosus is defined by dark papules that are of different sizes and rather unevenly distributed, reminiscent of a star map. Histopathologically, the dark spots of naevus spilus maculosus show a 'jentigo' pattern and several nests of melanocytes involving the dermoepidermal junction at the tips of the papillae, whereas most of the dark speckles of naevus spilus papulosus are found to be dermal or compound melanocytic naevi. The propensity to develop Spitz naevi appears to be the same in both types of speckled lentiginous naevus, whereas development of malignant melanoma has been reported far more commonly in naevus spilus maculosus.

Rhinophyma

The patient is a 73-year-old semi-retired carpenter who presents for evaluation of lesions on his back and eyelids.  Surprisingly, he did not mention his nose.

O/E: He has two epidermal inclusion cysts on the back some and some small skin tags around the right upper and lower lids (these lesions were snip excised).

More significantly, the skin of the distal nose it grossly thickened and patulous.

Clinical Photos:

Diagnosis:  Rhinophyma, Grade 3.

Although he did not initially express concerns regarding his nose, when I mentioned that there are treatments, the patient was very interested.

References:

1. Basal cell carcinoma masked in rhinophyma.

De Seta D1, Russo FY, De Seta E, Filipo R.  Case Rep Otolaryngol. 2013;2013:201024.  Free Full Text.

2. Basal cell carcinoma and rhinophyma. Leyngold M et. al. Ann Plast Surg. 2008 Oct;61(4):410-2.

Abstract: Rhinophyma, the end stage in the development of acne rosacea, is characterized by sebaceous hyperplasia, fibrosis, follicular plugging, and telangiectasia. Although it is commonly considered a cosmetic problem, it can result in gross distortion of soft tissue and airway obstruction. Basal cell carcinoma (BCC) is a rare finding in patients with rhinophyma. The objective of this study is to review the literature of BCC in rhinophyma and report on a case. A 70-year-old male presented with long-standing rosacea that resulted in a gross nasal deformity. The patient suffered from chronic drainage and recurrent infections that failed conservative treatment with oral and topical antibiotics. The patient decided to proceed with surgical intervention and underwent tangential excision and dermabrasion in the operating room. Since 1955 there have been 11 cases reported in the literature. In our case, the pathology report noted that the specimen had an incidental finding of a completely resected BCC. The patient did well postoperatively and at follow-up remains tumor-free. Despite the uncommon occurrence of BCC in resection specimens for rhinophyma, we recommend that all specimens be reviewed by a pathologist. If BCC is detected, re-excision may be necessary and careful follow-up is mandatory. Larger studies would be needed to determine the correlation between the 2 conditions.

Cryotherapy Gold Mine

Percent Reimbursement for Cryo per Medicare Payment

CPT Codes

17000 – Cryotherapy for One Lesion Reimbursed at: $59.88

17003 – Cryotherapy for > 1 Lesion   Reimbursed at: $8.05/lesion

Cryotherapy pattern of six dermatologists with practices in same area of New England.  All in private practice.  This only shows Medicare reimbursements (that probably account for 34 -- 40% of gross income).  

Excoriated Acne with Hyperpigmentation

A 37 yo Haitian woman was seen complaining about hyperpigmented lesions on face and back.  By history, these followed acne which she has excoriated.  Her health is good otherwise and she takes no medication by mouth.

O/E:  Hyperpigmented papules and macules on face and torso in a young woman with Type V skin.  Some lesions show mild excoriation.

Clinical Photos:

Diagnosis:  Post-inflammatory hyperpigmentation in excoriated acne.

Comment:  In my area, we have few patients with Type V and VI skin.  This is a common problem, but I do not have much experience treating it.  What is your protocol?  I started her on tretinoin 0.05% cream as this may be covered by her insurance.

Note:  A Pubmed search for hyperpigmented acne scars retrieves only three references and all are to laser surgery which this patient can not afford. This problem must be extraordinarily common; yet the biomedical literature is strangely silent about it.

Active Nevus

17 yo boy seen for another problem.  Atypical nevus noted on left shoulder.

O/E:  Type III-IV skin. 5 mm papule with slight play of color clinically.  Pt. unaware of lesion.

Dermoscopic images shows pigment dots of varying sizes.

Dx:  Actively growing nevus.  Easy to excise with a 6 mm punch.  Best to do this of just observe?  Patient will be leaving for college in another city in a few months.

Unusual Facial Dermatosis

Abstract:  16 yo girl with 3 month history of annular dermatosis mid-forehead.

HPI:  This healthy 16 yo has had a 4 cm roughley circular plaque on the mid-forehead.  Takes no medications by mouth and is not aware of any OTC meds taken infrequently,  She has bued 1% hydrocortisone cream and also a topical imidazole without improvement.

O/E:  The lesion has fairly sharp borders.  The surface is somewhat gready.  KOH prep failed to demonstrate hyphae or spores.  The remainder of the cutaneous examination is unremarkable.

Clinical Photo:

Diagnosis:  The picture is suggestive of seborrheic dermatitis, but the circular nature is unusual. What are your thoughts?

Plan:  Betamethasone valerate 0.1% cream b.i.d. x 2 weeks sand then Elidel cream.  If it does not respond or if it recurs a biopsy will be done.