No Worst, There Is None

The patient is a 26 yo man with an eight-year history of Hidradenitis supprativa (HS).  This began in his axillae but has progressed to groin, perineum and scrotum.  He has been treated doxycycline, minocycline, resection for groin and buttocks sinus tracts three years ago, and lastly Humira for over the past year, utilizing the standard doses recommended in the literature.*  He has constant pain for which he takes oxycodone 20 mg six times a day.  He was seen this week because of continuing pain, drainage and low grade fever (38 C).   He is essentially home bound and is cared for by a devoted mother.

The most affected area at present is his genitalia.   He has massive involvement of his scrotum with inflammatory draining lesions. 

Clinical Image:

Impression:  Ongoing hidradenitis stage III mostly affecting the scrotum at this point.  The picture is similar to what some label as “Scrotal Elephantiasis.”

Discussion: I do not think the Humira is impacting on the local disease at this time and he may need a surgical approach.  This could be ongoing inflammatory disease; but may eventuate into chronic lymphedema.  Another possibility is that the resection of the groin lesions may have caused impaired drainage.  Against this is the lack of leg edema.

We are looking for therapeutic suggestions and whether any of our members have successfully treated similar patients.  There are a few articles on surgical approaches in the urology literature.

Follow-up June 16, 2020:  Since we last reported on this patient he has had scrotal surgery and has been back on Humira for three months at 40 mg a week.  He cannot urinate easily and is home bound.  He feels his scrotum is getting worse, but comparison with the photo of June 2019, today's photo is less impressive.  We'll see if anyone has helpful suggestions:

June 16, 2020

 

References:

1.  Scholl L1, et. al. [Surgical treatment options for hidradenitis suppurativa/acne inversa].

[Article in German] Hautarzt. 2018 Feb;69(2):149-161.

Abstract:  Hidradenitis suppurativa/acne inversa (HS/AI) is a chronic inflammatory skin disease. Therapy consists of conservative and surgical treatment options. In Hurley stages II and III, surgical intervention is regarded as the method of choice for areas with irreversible tissue destruction. Resection techniques with different grades of invasiveness are described in the literature. Nevertheless, there is no generally accepted concept regarding resection and reconstruction techniques or specific postoperative care. Due to lack of definitions of recurrence after surgery and poor study quality, recurrence rates are difficult to determine.

2. Kimball AB, et. al. Two Phase 3 Trials of Adalimumab for Hidradenitis Suppurativa.

N Engl J Med. 2016 Aug 4;375(5):422-34. Full Free Text.

3. Hormonal therapies for hidradenitis suppurativa: Review.

Clark AK, Quinonez RL, Saric S, Sivamani RK. Dermatol Online J. 2017 Nov 12;23(10)..

Abstract: Hidradenitis suppurativa is a recurrent inflammatory skin condition characterized by abscesses and boils, predominantly in the groin, armpit, and buttocks areas. HS is not a life-threatening condition, but severely impairs quality of life in those affected. Finding a successful treatment approach for HS has been challenging, in part because of the lack of a gold-standard treatment method, limited research-based information, and the nature of clinical variation in the disease. Treatment commonly consists of antibiotics, anti-inflammatory therapy, hormonal therapy, and more invasive clinical procedures. Treatment is chosen by the degree of severity by which the condition presents and is modified accordingly. This review describes the roles of hormones in the pathogenesis of hidradenitis suppurativa and describes the use of hormonal therapy such as, finasteride, dutasteride, spironolactone, and oral contraceptives. The outcomes of the use of these modalities in various clinical studies are summarized.

*  The discounted retail cost of Humira for HS (40 mg weekly) is $10,000 USD a month or $120,000 year year.

 

Congenital Nail Dystrophy

The patient is a nine month old fraternal twin with mild developmental delays. No other pertinent history

She has opaque toenails that grow at an upward angle from the nail bed since birth. The great toenails continue to have an increasingly severe upward slant so that they are almost at a 90 degree angle to the nail bed. The nail plates show no thickening. Her fingernails are all normal. The skin of the feet and the rest of the body appear normal. No peeling or scale. No rash. The foot anatomy is grossly otherwise normal. The twin does not have the same condition. The parents are unable to put shoes on her because she seems to be in pain from her toenails. She does not seem bothered by socks or soft slippers. She is not walking yet, but should be within 2-3 months. they cut the nails short, but it doesn't offer enough relief to use shoes.  

Clinical Image:

Has anyone seen a similar patient?  What are your thoughts?

Note:  The infant will be seen by a dermatologist in a few days and a KOH prep will hopefully be done.

Infiltrating BCC of the Ala

The patient is a 58 yo man in fair health.  He suffers from anxiety and depression as well as diabetes and coronary heart disease and is status past CABG.  He was brought in by his female companion who noticed a lesion of the left ala.

O/E There is a nine mm indurated lesion with some surface erosion.

Image:

A 3 mm punch biopsy was difficult because of his severe agitation.  The pathology showed a deeply infiltrating basal cell.

Questions:

Given this patient’s pervasive anxieties, should one consider XRT over Mohs surgery?  The latter might also cause some deformity and may require a complicated reconstruction.  Of course, I will lay out the choices to the patient and his companion; but I thought this was a good question for our members to consider and weigh in on.  Some great unknown medical sage said, “Sometimes, it is may be more important to treat the patient who has the lesion, than it is to simply treat the lesion the patient has.”

Extensive Pruritic Vulvar Plaque in an 84 year old Woman

An 84-yr-old woman presented with a pruritic vulvar plaque for more than a year.  It was increasing in size extending from the vulva to the surrounding areas. She was otherwise well and did not have any constitutional symptoms such as fever or weight loss. She had seen few doctors and a gynaecologist but did not improve. She was treated with topical fucidin cream, clotrimazole cream and moisturisers. Her past medical history was insignificant.  She is a housewife.

Examination showed an irregular asymmetrical extensive erythematous plaque on the vulva extending to the suprapubic, groins, perianal areas and anus.  Some of the lesions on the periphery appeared hyperpigmented and nodular.  Her regional nodes were not enlarged.

Diagnosis: Extramammary Paget Disease

Differentials needed to be considered included Bowen disease and malignant melanoma

A skin biopsy was done and pending results.  She may need assessment of other malignancy eg urological or gynaecological cancers.

Treatment could be challenging as the plaques are so extensive.  Surgical excision probably not advisable at her age  - may need multi disciplinary surgeons eg O & G, urologist, plastic and colorectal surgeon. Not sure about role of radiotherapy but am starting her on topical imiquimod cream every other day.

A Young Girl with Ulcerated Lips

A 14-yr-old girl had severe blisters on the lower lip of 5 days duration.  It was painful and developed into superficial painful ulcerations of the lips.  Then she experienced eye discomfort with eye discharge esp in early morning.  There were no red eyes though.  She did not have any fever or any polyarthralgia.  No genital ulcerations. She is a secondary school student and stays with parents with no unusual habits. There was no family history of similar illness.  There was no recent drug history including OTC products, supplements and traditional chinese medicines. 

Examination was unremarkable except superficial ulcerations on the lower lip and to a certain extent on the upper lip too. The ulcerations was covered with yellowish slough and crusts.  Superficial erosions were noted on the inner buccal mucosa. No genital ulcerations.  No blisters elsewhere. 

Rest of exam unremarkable.

Diagnosis

Aphthous ulcerations - severe

Differentials considered were First episode orolabial HSV infection, drug eruptions, pemphigus vulgaris, erythema multiforme.

Blood counts and biochemistry was done as well as ANA serology.  HSV I and II serology was not done due to financial reasons. If she does not improve I think this patient may require a biopsy.

She was treated with oral prednisolone 20mg bd, topical triamcinolone gel bd and oral azithromycin 250mg daily. Your comments on this patient would be highly appreciated.

References:

1. Mucosal erosions as the presenting symptom in erythema multiforme: a case report. Spencer S, Buhary T, Coulson I, Gayed S. Br J Gen Pract. 2016 Mar;66(644):e222-4.  Free Full Text.

Follow up: Good response to treatment with oral prednisolone and azithromycin. Lesions were drying up and clearing.

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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.