Unusual Linear Dermatosis

Presented by Dr. Wagdy El Kifty

Giza Skin Specialist Centre

Pamukkale Baths

The patient is a 38 year-old businesswoman who presents with a six-month history of a progressive linear dermatitis that began on her left hand and has spread centripetally over the ensuing months.  It is mildly pruritic. She takes no medications by mouth.  The rash began shortly after she visited the Pamukkale baths in Southwestern Turkey with her family.  At a Turkish bath, attendants scrape and abrade one's skin for a vigorous exfoliation.

She saw another dermatologist in Amman, Jordan three months ago and a biopsy was performed.

O/E:  The patient has Type IV skin

There is a linear dermatitis which follows Blaschko’s lines.  Acrally, it is erythematous, but more centrally it is hyperpigmented.  On her back the pattern is whorled.

Clinical Images:

 

Pathology:  We have not received the slide, but it was signed out as “Lichen planus.”

Diagnosis: Blaschkolinear Lichen Planus.

Discussion:   There are a number of rare and overlapping blaschkolinear dermatoses.  Histologically, some have features of lichen planus and some lichen striatus, with considerable overlap.  These are usually self-limited and resolve over time, but some can be followed by post-inflammatory hyperpigmentation in darker individuals.  Association with underlying pathology has not been found.

Questions: Do you have any specific comments?  It appears that potent topical corticosteroids can be helpful. Do you have any experience treating a similar patient?

References:

1. Adult blaschkolinear acquired inflammatory skin eruption (BLAISE) with simultaneous features of lichen striatus and blaschkitis.

Raposo I. et. al Dermatol Online J. 2018 Jan 15;24(1 Full Text.

Abstract

Blaschkitis and lichen striatus are generally distinguished in the literature by the age of onset, lesion distribution, and histopathology. However, there is currently no clear consensus among authors about whether to consider blaschkitis and lichen striatus different clinical entities or a spectrum ofthe same disease. We present a case of adult BLAISE with features of both lichen striatus and blaschkitis, which seems to support the theory that these clinical entities may in fact represent a spectrum of the same pathological process.

           

2. Lichen planus-like dermatosis with Blaschko line distribution: a case report. Stojanović S, Jovanović M, Vucković N. Acta Dermatovenerol Alp Pannonica Adriat. 2008 Sep;17(3):137-8. Full Text.

Abstract: The authors describe the case of a healthy 46-year-old woman with a unilateral linear papular band on the left side of the trunk that followed the lines of Blaschko from the lower back extending to the left anterior side of her abdomen. The lesions were flat-topped, slightly elevated, violaceous, agglomerated lichenoid papules. The biopsy specimen demonstrated the typical histology of lichen planus. A working diagnosis of linear lichen planus was confirmed. Because congenital and/or nevoid skin disorders in a blaschkolinear distribution may have a delayed onset after birth, these lesions must be differentiated from acquired dermatoses following the lines of Blaschko. This distinction should be made in cases with isolated lesions, such as the case presented here.

3. Linear lichen planus. Batra P.

Dermatol Online J. 2008 Oct 15;14(10):16. Full Text.

      

Cupping Comes to the Boondocks

The patient is a 65 yo man who came in for a dermatitis of his scalp.  He requested a complete skin exam  This was remarkable for circular ecchymoses on his left back.  I asked about this.  He sees a massage therapist for back pain and she recommended cupping.  His wife was horrified to see these bruises until he explained how they were acquired.

Clinical Image: 

 With cupping, traditionally, a cotton ball soaked in alcohol is burned inside the glass cup and removed right before placement to create the vacuum. Bamboo and other materials can be used as alternatives to glass. The procedure breaks superficial blood vessels in the papillary dermis, creating ecchymoses, purpura, and petechiae, which is seen as evidence that the ailment is being drawn from the body.

Clearly, this traditional Asian remedy is entering the mainstream in Western Massachusetts. Our Asian readers probably see this all the time.  
For a few laughs, see Cupping Video.

Reference:

1. Dermatoses caused by cultural practices: Therapeutic cultural practices.

Vashi NA. J Am Acad Dermatol. 2018 Jul;79(1):1-16.

Abstract: With globalization and widespread immigration, physicians increasingly encounter patients from varying backgrounds and diverse customs. Although certain cultural practices are widely performed, there is limited medical literature describing their dermatologic and systemic effects and complications. Population diversity and sharing of traditions make it increasingly important for dermatologists to understand the role of cultural practices and recognize physiologic and pathologic sequelae. In addition, dermatologists are often adjured to assess skin findings that may be mistaken for abuse. Child abuse misdiagnosis can be traumatizing to all those involved, and immigrant families with limited English proficiency may have difficulty explaining their traditional practices. The first article of this 2-part continuing medical education series begins with a review of therapeutic cultural practices, including traditional Chinese medicine, Ayurveda, acupuncture, cupping, moxibustion, and coining, and the clinically relevant complications that may occur. Therapeutic practices can cause a range of complications, including contact dermatitis, heavy metal toxicity, and severe cutaneous adverse reactions.

2019 HOT SPOTS IN DERMATOLOGY CONFERENCE

SAVE THE DATES!

32^nd  Annual Hot Spots in Dermatology

Turtle Bay Resort, Oahu, Hawaii

August 16 – 18, 2019

        

Please consider joining us for our 2019 meeting. Hot Spots addresses clinical dermatology, emerging technologies and humane aspects of medicine.  We keep the number of attendees small to assure meaningful informal sessions at which registrants and speakers have time to interact as colleagues.

Hot Spots specifically excludes speakers who are paid stipends from PhRMA or any special interest group.  Our registrants are not a captive audience to promote any products, either directly or subtly.

The Hot Spots web site, will answer most of your questions.

Nevus Lipomatosus Superficialis

There are some lesions that only a dermatologist could love.  Nevus Liopmatosus Superficialis (NLS) may be one.

Recently, a 63 yo woman presented with a pedunculated mass on her right costal margin.  It had been present for a few years and did not bother her; but her internist told her to see a dermatologist to have it removed.

Being compliant, she made an appointment.  It was a 3 cm, soft, fleshy, skin-colored nodule on a slender stalk.  At her request I removed it with a simple scissor snip and sent it for pathology.  The clinical diagnosis was “fibroepithelial polyp vs. nevus lipomatosus.”

Clinical Photo:

The pathology showed mature adipose tissue replacing large portions of the dermis.

Photomicrographs courtesy of Lynne Goldberg, dermatopathologist, Boston University Skin Path:

Diagnosis:  Solitary Nevus Lipomatosus Superficialis

Reference:  (PubMed has 30 citations on NLS)

1. Nevus lipomatosus superficialis: A rare cutaneous hamartoma.

Pujani M, et. al.  Indian Dermatol Online J. 2014 Jan;5(1):109-10. Free Full Text.

Acute Lymphedema

Presented by Dr. Henry Foong
Ipoh, Malaysia

The patient is a 50-yr-old man with a history of swelling of the right leg for 2 months. He noted that the right foot was swollen initially and a few weeks later the swelling has progressed to the right leg.  Over the past week the swelling has progressed to the right thigh. The swelling was mildly painful but overall the past week the swelling has progressed a lot.  The swelling was so severe that he was unable to wear his normal shoe. He has a history of hypertension and a history of “stroke” where he lost his consciousness and was told by his doctor he suffered from a minor stroke.  He was treated as cellulitis by his doctor with penicillin but did not improve.  In fact he had developed a generalised maculopapular eruptions over the trunk a week after starting on IV penicillin.

O/E: Shows a severe unilateral swelling of the right leg extending from the foot to the thigh.  There was superficial erythema, desquamation with small blisters. The affected areas are well-demarcated especially on right thigh.  The leg swelling was indurated and mildly pitting.  The measured circumference of the right leg at mid-calf level was 51 cm compared with the corresponding left leg of 39 cm.  His right inguinal nodes were markedly enlarged. Rest of exam was unremarkable.

Images: 

Differential Diagnosis

1.  Erysipelas right leg

2.  Filariasis

3.  Deep vein thrombosis

Lab:

Hb 13.5 gm%, TWBC 17,900 (N87%, L10%, E0.1%, B1% M2%) ESR 79mm/hr Biochemistry unremarkable. Culture from the right leg grew Staphylococcal aureus.  Doppler ultrasound right leg did not show any evidence of deep vein thrombosis but enlarged right inguinal nodes.

He was advised to stop penicillin and started on IV moxifloxacin 400mg od, IV hydrocortisone 200mg qid,  and wet compress. Blood was sent for microfilaria (x3) which was negative.

Follow-up Photo after 5 days of IV moxifloxacin and IV hydrocortisone.

 

0

Marked improvement after 5 days of IV moxifloxacin and IV hydrocortisone.  There is a 5 cm reduction in the circumference of the right call. The patient would be scheduled for a MRI lower pelvis/right thigh soon, but he has refused since he is feeling so much better.
.

Your thoughts will be appreciated.

PRP and Kaposi’s: A Cautionary Tale

Our colleague, Professor Khalifa Sharquie from Baghdad, Iraq presents a 27-year old woman seen in his skin department with a dramatic facial rash and swelling of nine months duration.  Quite early on, the condition started as bruise-like discoloration and slight swelling around both eyes that slowly increased over a period of ~ three months “Figure. 1”.

The patient sought treatment at a  private clinic in Jordan.  A skin biopsy at that time showed "mucin deposition." She received peri-ocular plasma rich platelets (PRP) injections and afterwards, new lesions appeared more rapidly on her face; and then, after months, on other parts of her integument.  When seen at the Baghdad clinic she was severely ill with shortness of breath and difficulty in swallowing. “Figure. 2”.

Please see  the attached article.  and the click the link to download.
Your thoughts will be appreciated.

Figure 1

Figure 2

Reference:

Nathaniel Hawthorne:  The Birthmark

In which Beauty meets Science and is destroyed.  Science’s servant in this parable is called "Aminadab"!

Retroauricular Dermatitis: An Orphan Disease

The patient is an 11 year-old girl with a one-year history of a dermatitis in the right retroauricular sulcus.  She has a history of  an eczematous  eruption on her thighs. She was prescribed mupirocin and betamethasone valerate ointmens.  They both caused burning and pruritus and she refused to use them.  There is a history of a vaginal dermatitis a year or so ago, which was successfully treated with topical corticosteroids.

O/E:  There is erythema, crusting and mild fissuring in the above-mentioned area.  The left retorauricular area is mildly affected.  Presently there are no findings of atopic dermatitis in the usual areas.

Clinical Image:

Lab:  Wound culture was positive for 3+ Staph aureus and 3+ Group B Strep.

Diagnosis:  Retroauricular dermatitis.

We prescribed cephalexin 250 mg q.i.d. and a small amount of Vaseline for the dryness and fissuring.  Patch testing will be recommended if there are further symptoms suggestive of contact dermatitis.

Comments:  Retroauricular dermatitis, also called "infra-auricular fissures," appears to be an under-reported entity.  Although it is common in atopic dermatitis, there have been few articles about it. I see a few cases a year and the vast majority yield coagulase positive Staph aureus and occasionally strep when cultured. Most respond quickly to mupirocin ointment and a  low to moderate strength topical steroid.  This patient’s symptoms make her an outlier.  In addition, she has no findings of atopic dermatitis at this time.
Do any of you have thoughts on this entity?  The take home message here is that, as with atopic dermatitis, these lesions are frequently colonized with Staph aureus.

References

1. Infra-auricular fissures in atopic dermatitis.

Tada J, et. al. Acta Derm Venereol. 1994 Mar;74(2):129-31.

Abstract: Retro-auricular or auricular dermatitis in atopic dermatitis (AD) is common and important for the diagnosis of AD in infancy and even in adulthood. Particularly, "infra-auricular fissures", acute eczematous changes like fissures at the adhesive junction of ear lobes, seem to be prominent features for the diagnosis of AD. Of 137 patients with AD, 81.8% showed present or past existence of infra-auricular fissures, but only one of the 30 controls. Of the 46 patients with severe AD, 98% had infra-auricular fissures, compared to 74% in those with moderate and mild AD. Our findings suggest that infra-auricular fissures are important for the diagnosis of AD and should be cited in a list of criteria for the diagnosis of AD.

2. Streptococcal perianal disease in children. Kokx NP, Comstock JA, Facklam RR.  Pediatrics. 1987 Nov;80(5):659-63.  PubMed Link.

3. Psoriasis inversa: A separate identity or a variant of psoriasis vulgaris?

Omland SH, Gniadecki R. Clin Dermatol. 2015 Jul-Aug;33(4):456-61.

Abstract: Psoriasis is a chronic skin disorder affecting approximately 2% of the European and American population. The most common form of psoriasis is the chronic plaque type. Inverse psoriasis, also named flexural or intertriginous psoriasis, is not considered a separate disease entity but rather a special site of involvement of plaque psoriasis, characterized by its localization to inverse/intertriginous/flexural body sites. We review current evidence and establish whether inverse psoriasis is a separate disease entity based on characteristics in terms of epidemiology, pathogenesis, clinical and histologic presentation, microbiology, and treatment.

.

Subtle Facial Lesion in a Four Year-old Boy

The patient is a four year-old boy who was referred for evaluation of a slightly rough patch on his right cheek that has been present for over a year.  He is otherwise well.

O/E:  There is a subtle 1.5 cm in diameter erythematous patch on his right cheek.  Dermatoscopy revealed a group of sharply demarcated plugs that appear to be comedones.  Clinically, this was not as evident.

Clinical and Dermatological Images:

 Diagnosis:  Small subtle nevus comedonicus.

Discussion:  I feel this is probably a nevus comedonicus.  I’ve only seen a few of these and all were obvious: not so with this case.  Not much is known about the evolution of these lesions.  Topical retinoids are of some value; but since this doesn’t bother the patient I am reluctant to have his mother rub a topical agent on the area for two to three months.  Does anyone feel that tazarotene is preferable to tretinoin in similar cases?  Comedone extraction would be easy, but can be traumatizing in a young child.  Once I hear other ideas I will discuss options with his parents.

References:

1. Dermoscopy on nevus comedonicus: a case report and review of the literature.  Kamińska-Winciorek G1, Spiewak R.

Postepy Dermatol Alergol. 2013 Aug;30(4):252-4.  Free Full Text.

2. Nevus comedonicus: an updated review. Tchernev G, et. al. Dermatol Ther (Heidelb). 2013 May 25;3(1):33-40. Full Free Text.

Erysipelas in a 69 yo Woman

presented by Henry Foong
Ipoh, Malaysia

A 69-yr-old woman was seen with a 2-day history of rapidly enlarged swelling on the right cheek.  Initially she felt some discomfort on the right ear and then involved the right cheek.  She had no fever.

There was no history of trauma.  She had a history of hypertension. 

Examination showed unilateral swelling on the right cheek with increased temperature, tenderness and redness extending from right forehead to the right lower jaw.  It appeared oedematous, with swelling of the ipsilateral upper eyelid. It had a well defined raised border. The rash did not cross the bridge of the nose to the opposite cheek. No blisters were seen. Her regional nodes were not enlarged.

Clinically she has erysipelas.

TWBC 12,900 (N84%)

blood sugar  8.5 mol/l

BU and serum electrolytes normal

LFT normal

Erysipelas affects the superficial layer of the skin while cellulitis affects the deeper part of the dermis. There were no clusters of vesicles or erosions to suggest herpes zoster. She was not keen to be admitted and preferred oral medication as an outpatient  She was treated with oral cefuroxime 500mg bd for 10 days with wet compress dressing on the affected face. The erysipelas cleared with the treatment.

Pemphigus Vulgaris in a 43 yo Woman

The patient is a 43 yo Hispanic female. with several months of bleeding gums, sloughing tissue, and generalized oral pain.   She is otherwise healthy and takes no meds or supplements.  She has no skin or vaginal lesions.

Lab: Normal CBC and metabolic panel.

Her oral surgeon did a biopsy biopsy of normal mucosa adjacent to sloughing attached gingiva on anterior mandible and sent this for direct immunoflouresence (DIF).

Clinical Image:

Path:

H&E: Acantholysis

DIF:  Intercellular deposition of C3 and IgG.

Diagnosis: Pemphigus vulgaris

Questions:
This patient does not live close to an academic center and travel there would be difficult.
Does it make sense to initiate therapy with rituximab (if her insurance covers that) + prednisone. or should therapy be initiated with prednisone and conventional  adjunctive added later if nexessary?


References:

1. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial.

Joly P. et. al.   Lancet. 2017 May 20;389(10083):2031-2040.

This is a paper by the French study group on autoimmune bullous skin diseases.

CONCLUSIONS: 32.5% of our patients with moderate to severe pemphigus vulgaris failed prednisone and traditional CAT treatment and required rituximab therapy. Rituximab reduced the monthly prednisone intake in these patients by 73%. This suggests that a subset of patients with moderate to severe pemphigus may benefit from early institution of rituximab therapy. Rituximab significantly reduces the monthly prednisone requirement among CAT-resistant pemphigus vulgaris patients to levels on par with CAT-responsive patients.

2. Comparison of rituximab and conventional adjuvant therapy for pemphigus vulgaris: A retrospective analysis.

Agarwal A, Hall RP 3rd, Bañez LL, Cardones AR.

PLoS One. 2018 Sep 25;13(9):e0198074.  Free PMC Article

CONCLUSIONS: 32.5% of our patients with moderate to severe pemphigus vulgaris failed prednisone and traditional CAT treatment and required rituximab therapy. Rituximab reduced the monthly prednisone intake in these patients by 73%. This suggests that a subset of patients with moderate to severe pemphigus may benefit from early institution of rituximab therapy. Rituximab significantly reduces the monthly prednisone requirement among CAT-resistant pemphigus vulgaris patients to levels on par with CAT-responsive patients.

3. 

JAMA Dermatol. 2015 Aug;151(8):878-82.

Intralesional Rituximab in the Treatment of Refractory Oral Pemphigus Vulgaris.

Vinay K, Kanwar AJ, Mittal A, Dogra S, Minz RW, Hashimoto T.

JAMA Dermatol. 2015 Aug;151(8):878-82.

Abstract

IMPORTANCE:

Oral lesions of pemphigus vulgaris are usually recalcitrant and respond slowly to treatments. Corticosteroid injection is considered to be the most effective local treatment in oral pemphigus vulgaris. However, intralesional corticosteroids are not effective in all remnant lesions. In 3 such patients with pemphigus vulgaris, we evaluated the utility of 2 injections (on days 1 and 15) of intralesional rituximab, 5 mg/cm², in terms of accelerated healing, limitation of the use of systemic immunosuppressants, and reduction of their adverse effects.

OBSERVATIONS:

Three patients (1 man and 2 women) received 2 doses of intralesional rituximab in March and April 2013. All 3 patients responded to the treatment. In patients 1 and 2, the objective severity score was reduced to 0 at the final visit from a baseline score of 4 and 5, respectively (range, 0-11). The subject severity score in these patients was reduced to 1.0 and 0 from a baseline score of 22.0 and 22.5, respectively. After clinical remission was achieved, patient 3 developed a relapse of mucosal lesions. At the final visit, all of the patients were satisfied with the treatment, with a mean satisfaction score of 8 (maximum score, 10). We found a marked decline in the CD19 cell count from a pretreatment mean count of 287 cells/µL to 6 cells/µL on day 15 after a single intralesional rituximab injection. Adverse events were limited to local pain in 1 patient.

CONCLUSIONS AND RELEVANCE:

Intralesional rituximab administration lacks the adverse effects of intravenous administration. This method reduces the amount of drug administered and therefore is less expensive. Encouraging results from our study should prompt further evaluation of this novel route of rituximab administration in patients with refractory oral pemphigus vulgaris.

17 Year-old Girl with Unexplained Bruising

The patient is a 17 year-old girl whose first episode of bruising occurred after trauma sustained while playing soccer in March of 2018.  The middle child of three, she comes from a stable family.  She is an active varsity athlete.

For purposes of VGRD, the present illness consists of episodes of unexplained ecchymoses occurring every few months since March 19th, 2018.  This initial ecchymosis was tender and persisted for a month.  She has had three more episodes of bruising since then, not all related to antecedent trauma.

The patient has had poorly explained pelvic pain for two years, menometrothagia, and significant urinary retention necessitating self-catherization.  Extensive pediatric urologic evaluation at a major medical center found only a “lazy bladder.”

Other constitutional symptoms include nausea, vomiting and weight loss.  Over the past two years she has consulted multiple primary pediatricians, a pediatric endocrinologist, a neurosurgeon, a pediatric nephrologist, two pediatric urologists, a pediatric gastroenterologist, a pediatric gynecologist, a neurologist, and a pediatric hematologist. 

Thorough hematologic/coagulation workup was normal except for a minor platelet defect on electron microscopy that was felt insufficient to be causing the ecchymoses.

Two weeks ago she had another spontaneous episode of ecchymoses on her abdomen and neck, that are illustrated in photos.  Although her past ecchymoses have been tender, this most recent extensive bruise on the neck was very painful, and exquisitely tender to light touch.  Over the past two weeks these are slowly resolving.

On questioning both patient and her parents deny any adverse childhood experiences and nothing suggests a factitial etiology.

Clinical Photos:

Lab: Extensive laboratory studies have been normal.

An intradermal autoerythrocyte sensitization test has not been done yet.  Among the many studies done, an MRI showed a small pituitary microadenoma that was considered to be an incidentaloma.

Diagnosis:  The history and clinical appearance suggests Gardner Diamond Syndrome (Autoerythrocyte Sensitization Syndrome).

Questions:  GDS is a controversial diagnosis. 

1. What are your thoughts regarding this entity, especially in reference to this young woman?  She will see a pediatric rheumatologist and a pediatric  dermatologist and a pediatrician with a special interest in adolescent medicine. 

2. How can you tie together her disparate pelvic and urologic symptoms, as well as her unexplained nausea and vomiting with her bruising?

One can imagine how unsettling and scary the past two years have been for this young person and her family.  Your thoughts and suggestions will be appreciated.

Primary Biliary Cholangitis and Pruritus

Primary Biliary Cholangitis and Pruritis

This 57 yo woman was first seen in February 2018 for generalized pruritus.  She suffered with insomnia secondary to the symptoms. Her long-term medications include lithium, Effexor (venlafaxine) and Abilify (aripiprazole).   Because of abnormal LFTs she was referred to a gastroenterologist.  A liver biopsy “was suspicious for primary biliary cholangitis vs. primary sclerosing cholangitis.”  She was started on ursadiol 500 mg b.i.d. and cholestyramine 4 gm b.i.d. 

O/E: This woman appeared tired, depressed and older than stated age.  There were excoriated papules on her arms and legs and accessible areas of torso.

Clinical Photo:

Lab: Because of initial symptoms, CBC and Chem profile were ordered in February 2018

CBC normal

Initial Chen Profile: Alk phos 718 IU   (nl 18 – 218)

                                 SGOT 309            (nl 15 – 37)

                                 SGPT 446            (nl 15 – 56)

Repeat 10.18)          AlK Phos 319

                                SGOT  40

                                SGPT  57

                                Bilirubin has always been wnl.

Pathology: Biopsy of a papules on her right leg showed irregular epidermal hyperplasia, markedly dilated and disrupted follicles containing necrotic debris and a few PAS + spores. Gram stain negative.  DX: Perifollicular fibrosis suggestive of perforating folliculitis.

Comment:  In spite of normalization of LFTs on ursadiol and cholestyramine her pruritus did not improve.  The etiology of pruritus in PBC is unclear.  She has been started on Rifampin 150 mg b.i.d. and Narrow Band UVB.  I assume her skin lesions are secondary to PBC pruritus and excoriations. Your comments will be helpful.

Reference:
Excellent reference suggested by Dr. Richard Sontheimer:

1. A systematic approach to the management of cholestatic pruritus in primary biliary cirrhosis.  Hegade VS, wt. al.  Frontline Gastroenterol. 2016 Jul;7(3):158-166.   Free Full Text

Dr. Amanda Oakley suggested the leg lesions look like porokeratosis.  The association has been reported once in the literature.

2.Porokeratosis in primary biliary cirrhosis during plasmapheresis.  Venencie PY, Verola O, Puissant A. J Am Acad Dermatol. 1986 Oct;15(4 Pt 1):709-10.  Free Full Text.

Erosive Pustular Dermatosis of the Scalp

The patient is an 81 yo man with a long history of chronic lymphocytic leukemia.  His white counts are ~ 130,000 and platelets ~ 60,000.  He has had scores of nonmelanoma skin cancers (mostly squamous cell carcinomas) on the head and neck, torso and  all four extremities.  

He presents with yellow-brown crusts over his bald pate.  These have a mousy, earthy odor.  They were gently debrided and the subjacent areas were erosions.

Bacterial Culture:
3+ Pseudomonas
3+ Staph aureus

Clinical Images:

After initial debridement and chlorhexidine scrubs and mupirocin ointment for 33 days

Diagnosis:  Erosive Pustular Dermatosis of the Scalp

Plan: 

References:

1. Thuraisingam T1, Mirmirani P. Erosive Pustular Dermatosis: A Manifestation of Immunosenescence A Report of 8 Cases. Skin Appendage Disord. 2018 Aug;4(3):180-186\

Abstract: Erosive pustular dermatosis (EPD) is a rare condition of the scalp and legs that is marked by crusted erosions or superficial ulcerations that may result in scarring alopecia and chronic wounds. The condition predominantly affects elderly female as compared to male patients. Its pathogenesis remains poorly understood. The majority of the cases in the literature are from the United Kingdom and continental Europe. In this series, we present 8 North American patients with EPD of the scalp, one of whom also had involvement of the legs and another with the involvement of the face. All our patients were advanced in age and had a predisposition to chronic actinic damage, which are common characteristics of EPD previously reported in the literature. We hypothesize that immunosenescence leads to an aberrant immune response to wound healing and, along with other factors such as a loss of the normal epidermal barrier, ultraviolet damage, and hormonal factors, may contribute to the development of this condition.

2 Wilk M1 et. al. Erosive pustular dermatosis of the scalp: reappraisal of an underrecognized entity. J Dtsch Dermatol Ges. 2018 Jan;16(1):15-19.

Abstract: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory dermatosis of unknown etiology. Herein, we present a review of the disease and report our own clinical and histopathological experience in eleven patients. EPDS tends to spontaneously affect bald areas of the scalp in elderly individuals. A history of previous surgery at the same site - as observed in four of our patients - is common. Coronary artery disease, cerebrovascular insult, arterial hypertension, diabetes mellitus, and severe cases of cancer were frequent comorbidities. Most patients show an undulating clinical course despite topical anti-inflammatory treatment; in some individuals, the lesions heal with scarring. Histopathology reveals scaly crusts or erosions and granulation tissue-like changes in the dermis, evolving into a scar in more advanced stages. Apart from actinic/local damage, impaired immunity and microcirculation may be predisposing factors of the disease. Similar to pyoderma gangrenosum, EPDS must be considered in the context of nonhealing wounds in the elderly after the differential diagnoses mimicking EPDS have been ruled out. Given that previous or concomitant adjacent basal cell or squamous cell carcinoma is a common finding and that infiltrative variants extending beyond the clinically visible tumor may occur, histological mapping of the surrounding skin may be advisable in doubtful cases.

Facial Eruption in a 2 year-old

Presented by Dr. Hamish Thain,  Dundee, Scotland
22 September, 2018

The patient is a 2 yr and 10 month old girl with a few month history of a facial eruption. It began as hive-like plaques but has evolved over the past few weeks.  Her paternal grandmother has Alpha-1 Antitrypsin disease and the child is a carrier.  No family history of collagen vascular disease.  She takes no medications save vitamins.

EXAMINATION:  The examination shows an erythematous papular and nodular eruption on both malar eminences and the left lower lid.   The remainder of the cutaneous exam is unremarkable.

Clinical Photos:

Lab:
CBC, BUN, ANA: all normal or negative.  ESR 7
Urine analysis normal

Pathology:  The slides were read by Deon Wolpowitz and the photmicrographs were taken by Erin Tabata, both of Boston University Department of Skin Pathology.
Intermittent compact hyperkeratosis with parakeratosis, intermittent basal layer vacuolization with squamatization of the basal cell layer and occasional individually necrotic keratinocytes and mild lymphocytic exocytosis, papillary dermal edema that is focally prominent in a few papillae, ectatic blood vessels, and a moderate superficial and deep perivascular and interstitial, and focally periappendageal, lymphohistiocytic infiltrate with few extravasated erythrocytes. 

Diagnosis:  Polymorphous Light variant.  This is not the common papulovesicular variant of PMLE.

Plan:  Initially, she will use broad-spectrum sun-blocks containing zinc oxide or titanium dioxide and a large floppy hat whilst outdoors.  As the season changes, and the light wanes, she should improve.  Topical corticosteroids, calcineurin inhibitors, and hydroxychloroquine will be considered if her parents want further therapy.  Phototesting at a centre is disruptive for this child, at this time.

What are your thoughts.

References
1.Alexis L. Dougherty, Cloyce L. Stetson, MD, Dr. Khachemoune. What are These Facial Plaques in a 4 year-old Child.  The Dermatologist. 8.20.2013  Link