Sunday, May 21, 2017

PVLE Variant?

May 12, 2017
The patient is a 75 yo man with a 7 day history of a pruritic papular eruption on his legs.  This began on a recent trip to Florida after sun exposure.  He is on no photosensitizing medications.

O/E:  Erythematous non-blanching papules on both lower extremities.

Clinical Images:
Lab:  CBC, ANA normal

Pathology:  No evidence of vasculitis

Diagnosis:  Could this be an example of Papulo-vesicular Light Eruption?

References:
1.  Papulovesicular light eruption. A defined subset of polymorphous light eruption.
Elpern DJ, Morison WL, Hood AF.  Arch Dermatol. 1985 Oct;121(10):1286-8.
Abstract:  A distinctive photodermatitis is seen commonly in tourists visiting Hawaii. Analysis of 150 cases revealed that the eruption is acute in onset following exposure to sunlight, is confined to exposed areas, and mainly affects young to middle-aged white women. The clinical presentation is remarkably uniform, consisting of papules, papulovesicles, or vesicles. These findings are consistent with a diagnosis of polymorphous light eruption (PMLE) and the histologic picture supports this diagnosis. Other clinical variants of PMLE were not seen in our patients, however, which suggests that this condition is more monomorphous than polymorphous. We suggest papulovesicular light eruption as a suitable name for this common and distinctive subset of PMLE. 

2.  Papulovesicular light eruption. A defined subset of polymorphous light eruption.
Elpern DJ, Morison WL, Hood AF. Arch Dermatol. 1985 Oct;121(10):1286-8.
Abstract: A distinctive photodermatitis is seen commonly in tourists visiting Hawaii. Analysis of 150 cases revealed that the eruption is acute in onset following exposure to sunlight, is confined to exposed areas, and mainly affects young to middle-aged white women. The clinical presentation is remarkably uniform, consisting of papules, papulovesicles, or vesicles. These findings are consistent with a diagnosis of polymorphous light eruption (PMLE) and the histologic picture supports this diagnosis. Other clinical variants of PMLE were not seen in our patients, however, which suggests that this condition is more monomorphous than polymorphous. We suggest papulovesicular light eruption as a suitable name for this common and distinctive subset of PMLE.

A Case for Diagnosis

The patient is a 58 yo woman with asymptomatic lesions under breasts, in the axillae and groin.  She is on no medications,  She has breast cancer seven years ago treated with lumpectomy and chemotherapy (no radiation) and has been off therapy for 6 years.

O/E:  slightly hyperpigmented macules in affected areas.  KOH negative.

Clinical Photos:
Left axilla
Pathology:  2 representative punch biopsies were taken.

Diagnosis:  Pending  In the differential diagnosis is morphea, a variant of granuloma annulare

Saturday, May 20, 2017

Recessive X-Linked Ichthyosis

The patient is a 65-year-old engineer who presents for evaluation of skin lesions.  He grew up in a Southern state and during high school and college worked summers doing roadwork.  He wants a screening for skin cancer. The patient has had a B-cell lymphoma 3-4 years ago. 

He has had a form of ichthyosis since about age 2.  Other family members are affected.  They are all on his mother’s side.  His mother’s brother and some male maternal cousins are similarly affected. He always felt like an outsider in his school, and was bullied in grade and high school because of this scaly skin.  This has informed his self-image as an adult.

EXAMINATION:  A complete skin examination was done. He has scaly polygonal, somewhat “dirty-appearing” plaques on the legs, torso, and arms.  He has normal palms.  Head and neck are normal.  He has no lesions that are worrisome for melanoma, nonmelanoma skin cancer, or actinic keratoses. 

Clinical Images:  

Polygonal Scale
Normal Palm in X-Linked Ichthyosis
IMPRESSION:  Recessiive X-linked ichthyosis.

PLAN:  We discussed his ichthyosis.  One can get free Aquaphor from the drug company and we will order some for him. 

I will run his case by some pediatric dermatology colleagues.  I do not know whether any further testing is needed for a 65-year-old man who is not going to have any children.  

Corneal opacities are relatively common in these patients and may be evident with slit-lamp examination both of adult males who are affected and of women who are carriers. Cryptorchidism has been reported in 20% of patients. A few cases of testicular cancer have developed in patients with X-linked ichthyosis and cryptorchidism.

Reference:
FIRST: The Foundation for Ichthyosis and Related Skin Types is a great resource for patients and providers.  See FIRST’s page on Recessive X-Linked Ichthyosis.







Saturday, May 06, 2017

60 yo man with scaly rash upper chest

This 60 yo disabled man has had a sic moth history of a mildly pruritic eruption of his upper chest.  I expected the KOH prem to be postive, but it was not.  He has lots of psychosocial problems (wife an alcoholic, daughter a heroin addict) -- not enough time to properly evaluate; so a biopsy was done.  If that is negative for fungal elements I would think about Casal's necklace whigh I have never seen.

Pathology:  The biopsy showed a mild superficial spongiotic dermatitis with no evidence offungal elements.

Follow-up:  When seen back for suture removal, another KOH prep was done.  It was positive for numerous septate hyaline hyphae.  The original KOH prep was taken from the advancing scaly border as was the biopsy.  Sampling error caused the misdiagnosis.  The patient was started on terbinafine 250 mg daily for six weeks.  Follow-up photos will be posted when he returns.

Thursday, April 27, 2017

63 y.o. Man with P.R.-like Eruption

The patient is a 63 yo man with a one month history of an asymptomatic eruption on the torso.  He has a history of non-Hodgkin's lymphoma treated successfully four years ago.

O/E:  Healthy-appearing man with Type III skin.  He has .5 to 1 cm in diameter plaques on his torso and proximal extremities.  On his left forehead, he has three follicular papules.

Clinical Images


Comment:  This looks banal; but considering the patient's history biopsy is necessary.  Two representative lesions were sampled with 4-mm trephines.

Pathology: Biopsy showed no atypical features and this most likely  represents Pityriasis rosea in a somewhat immunocompromised man.  Since his lymphoma treatment, his white count is ~ 1800 - 2000 but he feels well otherwise.

Monday, April 10, 2017

Pruritic Nodules

The patient is a 32 year old Hispanic woman with a 2 year history of pruritic nodules on her knees, right ankle and dorsum of right hand.Her health is good otherwise and she takes no medications by mouth.  She denies kneeling, rubbing or manipulating the skin.  Her primary physician has give her various topical steroids which were not effective.

O/E:  There are a few hyperpigmented nodules in these areas.  She has Type IV skin.

Clinical:
Lab:  None

Diagnosis: Similar lesions have been seen after  trauma from habitual pressure from kneeling, surfing, praying: but thepatient denies these.

I am not hopeful about impacting here.  What thoughts do you have?  Are any lab tests or biopsy indicated?

Thursday, April 06, 2017

78 yo man with scalp lesions

Abstract:  78 yo man with 1 year history of scalp lesions.

HPI:
May 9,2016:  Cryotherapy 4 keratoses on vertex of scalp
August 11, 2016: Crusted lesions at site of cryotherapy.  Clinical diagnosos of erosive pustular dermatosis of scalp made.  Treated with mupirocin oint and clobeyasol ointment  Initially improved.
Sept 9 2016:  Resolved
10/24/2016: Continued to do well
April 4, 2017: Recurrent lesions on vertex of scalp.  Thick crusted lesions (see photo)  The crusts were brownish and dirty looking, but unfortunately I removed them before taking the photo of April, 2017.

O/E:  What was initially hypertrophic keratosit papules were transformed into ~ 1 cm crusted erosive lesions.

Clinical  Photos:
Note pustule
4.4.17 (after crusts removed)
s/p 1 week Chlorhexidine, 4 days tacrolimus 0.1%

Lab:  August 11, 2016: + Staph aureus from lesion Vx scalp - usual sensitivities

Diagnosis:
This is either erosive pustular dermatosis of the scalp or squamous cell ca.  The rapid worsening since cryotherapy suggests the former.  It appears that biopsy may be necessary.  Not all cases of EPD respond to clobetasol ointment.

Questions:  Is this EPD or are these lesions squamous cell carcinomas.  Patient is reluctant to have a biopsy done. This process appears to have been gtriggered by the trauma of liquid nitrogen and did respond initially to clobetasol.  10 - 20% of EPD cases appear to be non-responders ro clobetasol.

Follow-up.  Marked improvement following chlorhexidine wash daily and topical tacrolimus 0.1% ointment at the suggestion of a colleague.  He recurred after clobetasol ointment.

References:
1. Erosive pustular dermatosis of the scalp: Clinical, trichoscopic, and histopathologic features of 20 cases.  [Current and thorough review]
Starace M, et. al.: J Am Acad Dermatol. 2017 Feb 14.
BACKGROUND: Erosive pustular dermatosis of the scalp is a chronic eruption that leads to scarring alopecia.
OBJECTIVE: The clinical, dermoscopic, and histopathological features and the course of the disease in 20 patients were reviewed and compared with the reports in the literature.
RESULTS:The mean age was 59.4 years. Androgenetic alopecia was present in 12 patients, 6 of whom showed actinic damage. Trauma was reported in 9 patients. Four patients were affected by autoimmune disorders. The vertex was the most common location. In all 20 patients trichoscopy showed an absence of follicular ostia with skin atrophy. Histopathology revealed 3 different features, depending on the disease duration. A reduction of inflammatory signs was observed in 14 patients treated with topical steroids and in all 3 patients treated with topical tacrolimus 0.1%.
CONCLUSIONS: The relatively high number of patients allowed us to identify a better diagnostic approach, using trichoscopy, and a more effective therapeutic strategy, with high-potency steroids or tacrolimus, which should be considered as first-line treatment.

2.  Disseminated Erosive Pustular Dermatosis also Involving the Mucosa: Successful Treatment with Oral Dapsone (Free Full Text)
Jamison D. Feramisco.  Acta Derm Venereol. 2012 Jan; 92(1): 91–92.

3.  Erosive pustular dermatosis of the scalp: a review with a focus on dapsone therapy.
Broussard KC. J Am Acad Dermatol. 2012 Apr;66(4):680-6
Abstract
BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory disorder of unknown origin characterized by pustules, erosions, and crusting in areas of alopecia that tend to be atrophic, actinically damaged, or both. The most common treatments reported include antibiotics and topical anti-inflammatories, which can be ineffective. In the search for effective treatment for EPDS, we share our experience with topical dapsone 5% gel.
OBSERVATIONS:We present 4 patients with EPDS, all with classic clinical presentations and histologic findings of EPDS, who had failed a variety of treatments including oral, intralesional, or topical steroids, tacrolimus, and antibiotics. All patients demonstrated rapid improvement or resolution with topical dapsone 5% gel.
CONCLUSION: Our observations demonstrate topical dapsone 5% gel to be a novel, safe, and efficacious therapeutic alternative for mild to moderate EPDS.

Monday, April 03, 2017

Herpes Zoster in a 10 year-old



The patient is an otherwise healthy ten year-old boy with a two day history of grouped zosteriform vesicles on the left arm, anterior shoulder and upper back.  He has mild discomfort.  No known illnesses and on no medication.  He had two immunizations for varicella at the approopriate ages.

Observation:  All of the grouped vesicles appeared to be of uniform size.

Lab:  A Tzanck smear was positive for multinucleated giant cells.

Diagnosis:  Herpes zoster roughly C3 - 6.

Herpes zoster in children is unusual but not all that rare.  There are a few cases of HZ after vaccination for varicella.  As he felt well, and as the effect of specific antiviral therapy is not striking; after discussion with his mother it was elected to simply follow.  In out opinion, in healthy children and young adults the course of HZ is usually relatively mild and almost never followed by post-herpetic neuralgia.  Immunity seems to wear off over time and it appears that this attenuated vaccine is capable of causing H.Z.  The other possibility is that this is zosteriform simplex.  We did not culture for that. 
While researching this case, we looked up "zosteriform simplex."  An observation (ref 4) indicates that in these patients the vesicles are of uniform size (as we see here).  This would tilt us towards a diagnosis of Zosteriform Herpes Simplex here, and not of vaccine failure.  Should this child get a recurrence, that would clinch the diagnosis.


Reference:
1. Herpes zoster in children.
Peterson N, Goodman S, Peterson M. Cutis. 2016 Aug;98(2):94-5.

Abstract:

Herpes zoster (HZ) in immunocompetent children is quite uncommon. Initial exposure to the varicella-zoster virus (VZV) may be from a wild-type or vaccine-related strain. Either strain may cause a latent infection and subsequent eruption of HZ. We present a case of HZ in a 15-month-old boy after receiving the varicella vaccination at 12 months of age. A review of the literature regarding the incidence, clinical characteristics, and diagnosis of HZ in children also is provided. 
 
2. Herpes zoster and zosteriform herpes simplex virus infections in immunocompetent adults.

Kalman CM, Laskin OL. Am J Med. 1986 Nov;81(5):775-8.

Abstract: Among 111 immunocompetent patients referred to a general hospital setting with the clinical diagnosis of herpes zoster, viral cultures were obtained from 47 patients. Six of these patients (13 percent) had herpes simplex virus isolated, with four of the six infections involving the facial distribution, and the other two involving the T4 (breast) distribution. Excluding those in whom herpes simplex virus was isolated, the mean age (+/- SD) of the remaining 105 patients was 50 +/- 19 years. Thirty-two percent of the patients were at least 65 years old; however, 39 percent were younger than 40 years of age. Thus, herpes zoster frequently occurs in young, immunocompetent adults. Also, since zosteriform rashes may be caused by herpes simplex virus, viral cultures of lesions are useful to differentiate infections caused by herpes simplex virus from those due to varicella-zoster virus. The need to distinguish between these two viruses may be important with the advent of antiviral drugs and for use of the proper epidemiologic isolation procedures.
3. Varicella Vaccine (Wiki)
Vaccines are less effective among high-risk patients, as well as being more dangerous because they contain attenuated live virus. In a study performed on children with an impaired immune system, 30% had lost the antibody after five years, and 8% had already caught wild chickenpox in that five-year period.


4. Zosteriform herpes simplex and herpes zoster: A clinical clue

Sanath Aithal, Sheela Kuruvila, and Satyaki Ganguly. Indian Dermatol Online J. 2013 Oct-Dec; 4(4): 369.  Free Full Text.

Excerpt: An important clinical observation by many authors that the vesicles of herpes simplex are uniform in size in contrast to the vesicles seen in herpes zoster, which vary in size. In other words, vesicles of herpes simplex are uniform within a cluster.