A case of severe pemphigus vulgaris

Recently I saw this 40 year old woman who presented with severe blisters and erosions.  It started about 3 weeks ago and spread quite rapidly from the chest to the face and extremities.  Now her mouth is also involved.

Exam:  generalised bulla, majority are flaccid type.  Erosions extensive with crusts esp anterior chest wall.  Superficial erosions on the lower lips.  Nikolysky sign positive.

Skin biopsy:  Section shows skin composed of epidermis and dermis. A suprabasal bulla
containing acantholytic cells is seen. In areas, a tombstone appearance
is seen. Infiltrates of eosinophils and lymphocytes are seen in the
dermis.

Pemphigus vulgaris 

Blood counts and biochemistry are normal.   

She was treated as outpatient.  I have started her on oral prednisolone 40mg daily.  Added oral antibiotics cefuroxime 250mg bd.  Skin dressing with KMNO4 wet compress followed by fusidin/hydrocort cream bd.  Keep skin roof intact.

Questions:

1. I do not have IF facilities.  Will this lack of facilities affect her prognosis?
2. what kind of skin dressing would you use?
3. would you use steroid sparing agent like mycophenolate, MTX , azathioprine or ritazulimab at this juncture?

Thanks for your help.  Your comments are greatly valued.

 References:

Cochrane reviews: Interventions for pemphigus vulgaris and pemphigus foliaceus

Martin LK, Agero AL, Werth V, Villanueva E, Segall J, Murrell DF

Published Online: 

15 April 2009

This review of clinical trials aimed to find out which is the most effective and safest treatment option for pemphigus vulgaris and pemphigus foliaceus.

Pemphigus vulgaris and pemphigus foliaceus are rare diseases characterised by fragile blisters and sores on the skin and mucosa. They are auto-immune diseases which are caused by the body making an antibody against the person's own skin. These diseases are chronic and are not currently curable. Pemphigus vulgaris and foliaceus are managed with drugs which suppress the immune system. The aim of treatment is to suppress blister formation. Systemic glucocorticoids are the cornerstone of management in pemphigus, however adjuvant immunosuppressive and anti-inflammatory agents are commonly used. There are many treatments available, however it is not known which is the most effective or safest treatment option, or which is the best combination.

This review included data from 11 clinical trials involving 404 participants. The studies had very small numbers of participants, so can provide only limited information. Ten different active treatments were studied, including prednisolone, pulsed oral dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth factor and traditional Chinese medicine.

This review found insufficient information to conclude which is the most effective and safest treatment plan. We found that mycophenolate mofetil appears to be more effective than azathioprine in controlling disease, although no difference was seen in remission. We found that taking azathioprine and cyclophosphamide decreased the amount of glucocorticoids required. Topical epidermal growth factor decreased time required for lesions to heal by 6 days (median). We found no difference in withdrawal due to adverse events in any study, although differing adverse event profiles were observed for each intervention. We were not able to conclude which treatments are superior overall.

Multiple treatments are available for pemphigus vulgaris and pemphigus foliaceus and there is a variation in dosage plan and combination of drugs used, which makes choice of treatment schedule complex. In addition, response to treatment can vary between individuals. Treatments need to be chosen after careful consideration of the potential benefits and side effects, in the context of the individual's other medical conditions. This review found insufficient information to conclude which is the most effective and safest treatment regimen. Further studies are required to determine the optimal treatment regimen, especially to assess the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve harm:benefit analyses.

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Median Nail Dystrophy

Patient 1: A 65 yo woman with a 3 month history of a medial split in the right thumb nail. It is asymptomatic. No history of trauma. The physical exam showed the split begins at the proximal nail fold. There is a suggestion of erythema at its proximal end.

Patient 2: A 79 yo woman with a 3-6 months history of a medial split in the right thumb nail. It is painful with pressure otherwise asymptomatic. No history of trauma. The physical exam reveals the same finding as the patient 1 without a suggestion of erythema at the proximal fold. 

Clinical Images:  

Patient 1: Clinical View

Patient 1: Dermoscopic View

Patient 2: Clinical View

Patient 2: Dermoscopic View

Diagnosis: Median nail dystrophy.  Is this secondary to a subungual tumor? Is a biopsy indicated?

Reference:

Glomus tumor-induced longitudinal splitting of nail mimicking median canaliform dystrophy. Verma SB. Indian J Dermatol Venereol Leprol. 2008 May-Jun;74(3):257-9.

Abstract:Median canaliform deformity of the nail is an uncommon entity, where there is longitudinal splitting of the nail. Longitudinal splitting of the nail is a rare phenomenon and can also occur following number of growths arising in the nail matrix. On examination there was a longitudinal split in the nail plate, beginning in the distal nail fold and extending proximally all the way to the proximal nail fold. There was a small, almost indiscernible, swelling in that area, which was exquisitely tender. The split part of the nail showed a little discoloration. There was no discharge, bleeding, or subungual mass visible. 'Love test' was positive in this case. After nail avulsion, a small 2 mm x 4 mm nodule was exposed and excised. Histopathological examination of the tumor showed a mantle of glomus cells surrounding the blood vessels.  Free Full Text.

Cervical Nodules in a Healthy 65 Year-old Woman

Abstract:  One month history of three nodules in the right posterior cervical area in a 65 year-old woman.

HPI:  The patient is an otherwise healthy, immunocompetent woman who has noted three slightly painful nodules that appeared in the right posterior nuchal area. She has a history of  scalp excoriations.  She has two cats, one of which sleeps with her.

O/E:  Initially, there were three firm nodules measuring 0.8 to 1.5 cm in diameter.  She has had superficial scalp erosions secondary to excoriation for about a year.

Clinical Photos (courtesy of Yoon Cohen, D.O.)

Scar is biopsy site

Lab:  CBC normal, Chemistries normal.  Chest Xray normal.  Cat Scratch serology drawn six weeks after onset:  Bartonella Henselae IgG 1:1280 and Bartonella Quintana IgG 1:640  (IgM negative for both. Negative is <1:320)

Pathology: (photomicrographs courtesy of Lynne Goldberg, M.D., Boston University Skin Path) Because this was thought to be lymphadenopathy, a deep incisional biopsy was performed.  To our surprise, no lymphatic tissue was seen.  Rather, the pathology showed stellate subcutaneous microabscesses with a surrounding lymphohistiocytic infiltrate and fibrosis.

Course:  The patient continues to feel well.  An abscess was drained and around 1 cc of viscous pus was withdrawn.  It has refilled.  Since the patient feels well, we have not prescribed antibiotics.

Diagnosis: Cutaneous abscesses in Cat Scratch Disease.

Discussion: There are no reports of cutaneous abscesses in patients with CSD.  It may be possible that the suppuration we noted occurred as a sequela to an infected lymph node.  A review of the medical literature was not helpful here.  Recommendations for antibiotic therapy of immunocompetent with no systemic signs or symptoms are equivocal and we have elected to observe our patient for the time being.  If we decide to recommendad treatment, it will probably be with erythromycin.

References:

1.  eMedicine.comhttp://emedicine.medscape.com/article/214100-overview:  Catscratch disease (CSD), also known as catscratch fever or subacute regional lymphadenitis, is a bacterial infection affecting lymph nodes that drain the sites of inoculation. Bartonella henselae, a gram-negative rod, is considered the principal etiologic agent. CSD is a common cause of chronic lymphadenopathy in children and adolescents.

Patients with CSD usually have a history of sustaining a scratch or bite from a cat (typically a kitten). The initial symptom is formation of a papule at the inoculation site, followed by solitary or regional lymphadenopathy within 1-2 weeks (see the images below). In most patients, the disease resolves spontaneously within 2-4 months.

2. Abscess-forming lymphadenopathy and osteomyelitis in children with Bartonella henselae infection.  Ridder-Schröter R, et. al. J Med Microbiol. 2008 Apr;57(Pt 4):519-24. doi: 10.1099/jmm.0.47438-0.

Abstract: Bartonella henselae is the agent of cat-scratch disease (CSD), a chronic lymphadenopathy among children and adolescents. A systemic infection is very rare and most of these cases are found in patients with immunodeficiency. Here, cases involving four children of 6-12 years of age are reported. In immunocompetent patients, infection affects skin and draining lymph nodes; however, prolonged fever of unknown origin as in the fourth patient indicated a systemic complication of CSD. Free full text.

Median Nail Dystrophy

The patient is a 65 yo woman with a 3 month history of a medial split in the right thumb nail.  It is asymptomatic.  No history of trauma.

O/E:  The split begins at the proximal nail fold.  There is a suggestion of erythema at its proximal end.

Clinical Images:  Courtesy of Yoon Cohen

Diagnosis: Median nail Dystrophy.  Is this secondary to a subungual tumor?

Reference:

Glomus tumor-induced longitudinal splitting of nail mimicking median canaliform dystrophy. Verma SB. Indian J Dermatol Venereol Leprol. 2008 May-Jun;74(3):257-9.

Abstract:Median canaliform deformity of the nail is an uncommon entity, where there is longitudinal splitting of the nail. Longitudinal splitting of the nail is a rare phenomenon and can also occur following number of growths arising in the nail matrix. On examination there was a longitudinal split in the nail plate, beginning in the distal nail fold and extending proximally all the way to the proximal nail fold. There was a small, almost indiscernible, swelling in that area, which was exquisitely tender. The split part of the nail showed a little discoloration. There was no discharge, bleeding, or subungual mass visible. 'Love test' was positive in this case. After nail avulsion, a small 2 mm x 4 mm nodule was exposed and excised. Histopathological examination of the tumor showed a mantle of glomus cells surrounding the blood vessels.  Free Full Text.