Erosive Pustular Dermatosis

Presented by Hamish Dunwoodie, MBBS
The Pas,  Manitoba Canada

Abstract:  98 yo woman with exophitic tumor of the forehead

HPI:  The patient is a light complected Caucasian with a 4 month history of a keratotic lersion on the forehead.  She has a history of nonmelanoma skin cancer.  She is a poor historian.

O/E:  4 cm in diameter crusted tumor forehead.

Photos:

After crust removed

Procedure:  The lesion was compressed with a warm wet gauze pad for 10 minutes and the crust was easily removed.  A deep shave biopsy wes performed and the lesion was electrodessicated and curretted.

Histopathology:
The specimen shows cocally confluent ulceration with underlhying granulation tissue and a moderate to dense lymphoplasmacytic infiltrate.  This is consistent with erosive pustular dermatosis.

Diagnosis:  Erosive Pustular Dermatosis

Discussion:  Clinically, I thought this was a nonmelanoma skin cancer.  Most cases of EPD are on the scalp but they have been described in other sites.

Photo:  3 week post op:

Based on path report, she was treated with clobetasol ointment 0.05% b.i.d.  for two weeks; and after this pictures wwas taken she was switched to fluocinalone 0.025% ointment for two more weeks.

References:
1, emedicine.com Erosive Pustular Dermatosis

2. Erosive pustular dermatosis of the scalp and nonscalp.

Van Exel CE, English JC 3rd.
J Am Acad Dermatol. 2007 Aug;57(2 Suppl):S11-4.

University of Pittsburgh, Department of Dermatology, PA
Abstract; Erosive pustular dermatosis of the scalp is characterized by an idiopathic pustular eruption occurring in association with iatrogenic or incidental, antecedent trauma to actinically damaged skin. We present two cases of erosive pustular dermatosis, one of which occurred on the scalp, the other of which was primarily located on the face. (The editor can send a link to full text if you want.)

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Nonspecific Oral and Genital Lesions

Abstract:  92 year-old woman with 6 year history of painful oral and genital lesions

HPI:  The patient is an otherwise healthy and alert 92 year old woman with painful erosions of tongue and vulva for six years. She has marked pain with eating and urination.  Mouth lesions preceded genital lesions by a few years.  She has been followed for erosive lichen planus but it is unclear if any biopsies were positive.  She has lost weight because her painful tongue causes her to avoid eating.  She has been treated with clobetasol ointment,  Viscous Lidocaine was not effective.

O/E:  Erosive lesions of tongue and labia.  No bullae noted. Remainder of cutaneous exam unremarkable.

Clinical Photos:

Pathology:  Biopsy of genital labial skin shows papillary dermal fiborsis and a mild superficial perivascular mixed inflammatory cell infiltrate.  No lichenoid infiltrate.  Direct immunofluorescence from perilesional skin was negative.  PASD negative for fungi.

Diagnosis:  Painful erosions mouth and genitalia.  At present no support for lichen planus or bullous process histologically.

Questions:  What are your thoughts? Her oral pain makes eating difficult and negatively affects her quality of life.  Are there other therapies that we could consider?

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Norwegian Scabies Infestation

Abstract: An 84-year-old elderly woman with 3 months history of pruritic skin eruption

History: The patient is an 84-year-old nursing home resident who presents for evaluation  of pruritic skin eruption for the past three months. She first noted pruritic lesions on her hands when she was in a hospital for a back surgery. Since then her skin was progressively worsening and the eruption spread to her face, arms, abdomen and legs. She notes she was getting thick scales in the involved areas. She has chronic back pain and has gone through several operations past 3 months. Her other medical history includes congestive heart failure, hypertension, and COPD. Due to pain and her recent skin eruption, she has been taking prednisone, hydromorphone, doxepin, and Ativan. She feels sedated and confused at times. The patient is with her daughter who had significant contact and similar symptoms.

Clinical photos:

O/E: The skin exam shows a somewhat sleepy, but oriented, elderly woman in a wheelchair with yellow to green thick scaly plaques on the hands, forearms, elbows,  face,  ears, abdomen and legs. Dermoscopic evaluation shows multiple triangle jet signs with burrows. Scraping prep with mineral oil showed multiple scabies mites. Photo shows a portion of a 10X field on which 11 mites were seen.

Diagnosis: Norwegian scabies

Questions: We'd appreciate your recommendations on this patient's care. 

Discussion: Crusted Norwegian scabies is an uncommon hyperkeratotic variant of scabies infestation. Norwegian scabies are known to be as opportunistic pathogens. Our patient has been under significant stress physically and emotionally due to recent back surgeries and her skin eruption has progressed under the eyes of a medical staff.  Her medications contributed to the development of Norwegian scabies by suppressing her immune response (prednisone) and blunting her itch perception (Dilaudid, doxepin and Ativan).  In a way, this was a perfect storm.   Due to severity of involvement, the patient will be treated with oral ivermectin 200 micrograms per kilogram weekly for 3 weeks and permethrin 5% cream weekly for 4 - 5 weeks. Her daughter and all close family members will be also treated with topical medication. The nursing home will be informed.  It is estimated that the mite load of patients with Norwegian scabies can be as high as 15 million which accounts for the  highly contagious nature of this variant.  All of the staff and residents of her nursing home should receive scabies treatment.

References:
1. Towersey L, Cunha MX, Feldman CA, et al. Dermoscopy of Norwegian scabies in a patient with acquired immunodeficiency syndrome. An Bras Dermatol. 2010;85(2):221-223

2. Problems in diagnosing scabies, a global disease in human and animal populations.

Walton SF, Currie BJ.  Clin Microbiol Rev. 2007 Apr;20(2):268-79.  Excellent review, available open access fulltext.

3. Reflectance confocal microscopy for quantification of Sarcoptes scabiei in Norwegian scabies. Cinotti E, Perrot JL, et. al J Eur Acad Dermatol Venereol. 2013 Feb;27(2):e176-8.  PubMed listing.

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Complicated Speckled Lentiginous Nevus

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

Abstract: An 8-year-old girl with large congenital nevus on the chin, the anterior neck and the right aspect of shoulder and the chest

History: The patient is an 8-year-old girl who was born with congenital nevi on the mid anterior neck and possibly another similar lesion over the right side of the mandible. Over the years, she has developed many 1-3 mm in diagmeter tan papules in and around the chin and submental region. She was seen recently for follow up. Her nevus is slowly maturing but still benign in appearance and there are no outlier lesions in the large defect. Some of the tumor is bilateral.

Clinical photo:

O/E: The skin examination shows a healthy, pleasant child with numerous scattered 1 to 3 mm brown macules and papules in a background of light tan pigmentation on the chin, the anterior neck and the right aspect of the upper chest. There is a well marginated 25 mm in diameter brown patch on the lower mid anterior neck. There is a 3.5 x 4.0 cm hypopigmented patch on the right shoulder

Diagnosis: Complicated Speckled Lentiginous Nevus (SLN)

Questions: Please share your experiences in managing patients with SLN similar conditions.  Do you feel there is a concern that this lesion may undergo malignant transformation.  What do you think of the hypopigmented area?

Discussion: A speckled lentiginous nevus (SLN, Nevus Spilus) can be a congenital or acquired pigmented lesion. More recently arguments are in favor of speckled lentiginous nevi as subtype of congenital nevi including the following observations: frequent presence at birth or noted soon therafter; patterns of distribution reflecting embryonic development; hamartomatous behavior with various types of nevi; and histologic features of congenital melanocytic nevi within the lesions. There are two types of speckled lentiginous nevus -- Nevus spilus maculosus and nevus spilus papulosus. 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, nevus spilus papulosus is defined by dark papules that are of different sizes and rather unevenly distributed, reminiscent of a star map. Perhaps, the most pressing concern is its potential malignant transformation. The propensity to develop Spitz nevi appears to be the same in both types of speckled lentiginous nevus whereas the development of malignant melanoma has been reported far more commonly in nevus spilus maculosus. Torchia and her colleague argue that malignant transformation of melanocytes might occur in SNL not because the hyperpigmented background features any specific procarcinogenic abnormality but because there are more melanocytes with SNL, yielding a slightly higher likelihood of developing any type of melanomcytic lesion. It is our best interest that we monitor our patients with SNL pediodically to detect any early signs of malignant transformation.

References:
1. Happle R. Speckled lentiginous naevus: which of the two disorders do you mean? Clin Exp Dermatol. 2008;34:133-135
2. Paraskevas LR, Halpern AC, Marghoob AA. Utility of the Wood's light: five cases from a pigmented lesion clinic. Br J Dermatol. 2005 May; 152(5): 1039-44
3. Menon K. Dusza SW, Marghoob AA, et al. Classification and prevalence of pigmented lesions in patients with total body photographs at high risk of developing melanoma. J Cutan Med Surg. 2006 Mar-Apr; 10(2):85-91
4. Schaffer J. Orlow S, Lazova R, et al. Speckled Lentiginous Nevus Within the Spectrum of Congenital Melanocytic Nevi. Arch Dermatol. 2001; 137:172-78
5. Torchia D. Schachner L. Is speckled lentiginous nevus really prone to dysplasia/neoplasia? Pediatr Dermatol. 2012 Jul-Aug; 29(4):546-7

Comments:
1. Dr. Ashfaq Marghoob: 
These patients (SLN of maculosa type) are at slightly higher risk for developing MM. Continued surveillance for focal change is what I do for these patients.

2. Dr. Rudolph Happle:
Thank you very much for letting me see this interesting case! Apparently, this is a classical example of papular SLN (papular nevus spilus).  Why is your diagnosis "Complicated SLN"? I would call this a complicated case if a melanoma would develop, but the risk is rather low because It's not a macular nevus spilus.

3. Dr. Harper Price: 

Thank you for sharing this interesting case.  It does look very classic for a SLN.

The risk of malignant transformation is very low and likely starts at puberty so I would follow her yearly and if there was a lot of change, even more frequently and use photography to help.  These SLN can have many other nevi within in as stated, including Spitz and blue nevi.

Hers looks quite good and not worrisome.  The area of hypopigmentation, if new, may represent vitiligo or if present early on, a hypochromic nevis (“twin spotting”).

These SNL certainly carry a much lower risk than a typical congenital melanocytic nevus of this size for melanoma development. 
Lifelong follow-up is my recommendation

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Hemorrhagic Blister

Abstract: A 58-year-old man with 2-day history of deep purple to black papule

History: A 58-year-old man presents for evaluation of a skin lesion on the right lower abdomen that has been present approximately 2 days. The patient does not remember having any trauma on that area. It seems the lesion appeared quite abruptly and the color has been darkened over past 2 days. No history of non-melanoma skin cancers or melanoma.

O/E: The skin exam shows a healthy and pleasant man with skin type III. There is a sharply demarcated 6 mm deep dark purple oval shaped papule on the right lower abdomen. It is somewhat compressible.

Clinical & Dermatoscopic Photos:

Polarized Dermatoscopic Photo

Non-Polarized Dermatoscopic Photo

Diagnosis: Hemorrhagic blister

Discussion: The lesion was lanced with an 11 blade. Blood product was drained and confirmed the diagnosis of hemorrhagic blister. Initially, the lesion did not obviously look as  a hemorrhagic blister. The dermatoscopic image did not show a typically appearing hemangioma. Our biggest concern was to make sure this is not a melanoma. Conventionally, melanoma would not appear in a 2-day period. A careful history led us to consider a hemorrahagic blister with a simple lancing. 

Reference:
1. Bullous malignant melanoma: an unusual differential diagnosis of a hemorrhagic friction blister.

Vogt T, Brunnberg S, Hohenleutner U, et al. Dermatol Surg. 2003 29(1): 102-4

Source

BACKGROUND:

A 66-year-old woman presented to our outpatient clinic with a 3 x 2.5-cm tense, hemorrhagic-appearing bulla on her forefoot. Histopathology and immunohistochemistry confirmed a transtumoral-transepidermal blister formation within an advanced acrolentiginous malignantmelanoma (MM).

OBJECTIVE:

To study bullous malignant melanoma.

METHODS:

Blistering in MMs represents a rare but clinically important pitfall in clinical differential diagnosis. The blisters are typically due to the disruption of the cohesion between neoplastic cells and keratinocytes, but physical friction may also contribute.

RESULTS:

Hemorrhagic blistering is, in many cases, a relatively insignificant finding in which frictional forces are imposed.

CONCLUSION:

The case reported here underscores that in rare cases MMs, particularly if acrally located, can be complicated by hemorrhagic blistering. Because of the life-threatening consequences, one should be aware of this rare differential diagnosis.

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Rosacea-Like Demodeciasis

Abstract:  47 yo woman with ab 8 month history of a facial eruption
 

The patient is a 47 yo woman with a rosacea-like eruption for eight months.  Dr. Yoon Cohen suggested demodicosis and performed a scraping.  It was loaded with demodex mites.  We are considering this an example of rosacea-like demodexinfestation and treated the patient with ivermectin 250 microgram/kg x 2 a week apart and permethrin 5% cream 2 - 3 times a week. 

Photos:

 After one week:

Diagnosis:  Rosacea-like Demodeciasis (most probably)

Discussion: It seems that this acneiform eruption is due to Demodex, but we need more time to be certain. Since Samuel Ayers, Jr. described this entity in 1930 it has been in the literature but there are still doubters in the dermatologic community.

Reference:

1. Dermatol Online J. 2007 Oct 13;13(4):9.

Granulomatous rosacea-like demodicidosis. Free Full Text

Lee JY, Hsu CK.

Abstract

Demodicidosis may present as pityriasis folliculitis, papulopustular lesions, rosacea-like eruptions, and granulomatous rosacea-like eruptions. We report a case of demodex granuloma presenting with recurrent granulomatous rosacea-like papules on the face in a middle-aged woman. The diagnosis of demodicidosis was made by finding extrafollicular mites in the perifollicular inflammatory infiltrate. The papules resolved after 3 weeks of systemic and topical metronidazole, and low-dose oral prednisolone therapy. In summary, demodex granuloma may be mistaken for granulomatous rosacea-like papules. Correct diagnosis can be facilitated by finding extrafollicular demodex mites in skin biopsy specimens.

2.  Cutis. 2007 Aug;80(2):149-51.

Recalcitrant papulopustular rosacea in an immunocompetent patient responding to combination therapy with oral ivermectin and topical permethrin.  Full Text PDF

Allen KJ, Davis CL, Billings SD, Mousdicas N.

Abstract

A 68-year-old healthy man presented with papulopustular rosacea (PPR) recalcitrant to multiple therapies, including permethrin cream 5%. Histologic examination detected the presence of chronic folliculitis and numerous Demodex organisms. A diagnosis of rosacealike demodicidosis was rendered, and the patient was treated with oral ivermectin and permethrin cream 5%, resulting in resolution of the folliculitis. Demodex infestation should be considered in any patient with rosacealike dermatitis resistant to conventional rosacea therapies. If infestation is demonstrated in these patients, oral ivermectin in combination with topical permethrin is a safe and effective therapeutic option.

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Follicular Mucinosis?

Abstract: 80 yo man with intensely pruritic papules face and neck for 2 months

HPI:  This 80 yo man has a 2 month history of 2 – 3 mm erythematous papules, mostly on face and neck. These are few in number.  A trial cobetasol ointment was without relief.  His medications are: amlodipine, HCTZ, metroprolol, Vytorin (ezetimibe and eimvastatin), Pantoprazole.  He is in good general health.

ClinicalPhotos: These show how subtle the lesions are.

Because of symptoms a Biopsy was done.

Histopath shows: focal erosion and ulceration and ectatic blood vessels.  There is a moderate to dense superficial and deep perivascular perifollicular lymphohistiocytic infiltrate with numerous eosinophils, marked mucin deposition and conspicuous disruption of the follicular epitheliumThe pathologic diagnosis includes, “infectious etiology,” eosinophilic folliculitis, and follicular mucinosis.

Diagnosis:  Acneiform Follicular Mucinosis might be the best fit.

Reference:

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