Management of chronic wounds in patients with pemphigus

Figures & data

Table 1 Key clinical and laboratory findings in pemphigus disease

	Epidemiology	Autoantigens	Clinical features	Histopathology	Direct immunofluorescence	Indirect immunofluorescence	Variants
Pemphigus vulgaris	Primarily middle-aged adults	Desmoglein 1 and desmoglein 3, others	Mucosal erosions; flaccid bullae and erosions on skin	Suprabasal acantholysis; “row of tombstones” pattern of basal keratinocytes	Intercellular deposition of IgG	Intercellular deposition of IgG; monkey esophagus is preferred substrate	Pemphigus vegetans and pemphigus herpetiformis
Pemphigus foliaceus	Primarily middle-aged adults; endemic form most common in children and young adults	Desmoglein 1	Fragile blisters, shallow erosions, erythematous patches, crusts; mucosal involvement absent	Subcorneal or granular layer acantholysis	Intercellular deposition of IgG	Intercellular deposition of IgG; normal human skin or guinea pig esophagus is preferred substrate	Endemic pemphigus foliaceus (fogo sevalgem), pemphigus erythematosus, and pemphigus herpetiformis
Paraneoplastic pemphigus	Primarily adults, but may occur at any age	Envoplakin, periplakin, others	Severe stomatitis, variable cutaneous findings (eg, blisters, erosions, lichenoid lesions), bronchiolitis obliterans	Variable findings; suprabasal acantholysis, keratinocyte necrosis, and lichenoid interface dermatitis are most common	Intercellular and/or basement membrane zone deposition of IgG and/or C3	Intercellular deposition of IgG; rat bladder is preferred substrate
Subcorneal pustular dermatosis-type IgA pemphigus	Any age	Desmocollin 1	Vesicles, pustules, crusts on skin; annular, circinate, or herpetiform morphology; mucosal involvement usually absent	Subcorneal clefts and pustules; minimal acantholysis; mixed infiltrate in dermis	Intercellular deposition of IgA	Intercellular deposits of IgA on monkey esophagus*
Intraepidermal neutrophilic dermatosis	Any age	Poorly defined	Vesicles, pustules, crusts on skin; annular, circinate, or herpetiform morphology; mucosal involvement usually absent	Intraepidermal pustules; minimal acantholysis; mixed infiltrate in dermis	Intercellular deposition of IgA	Intercellular deposits of IgA on monkey esophagus^*

Notes:

* Indirect immunofluorescence is negative in around 50 percent of patients with IgA pemphigus. Test availability restricted to specialized laboratories. Reproduced with permission from: Hertl M, Sitaru C. Pathogenesis, clinical manifestations, and diagnosis of pemphigus. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on July 22, 2019). 1 1 Copyright© 2019 UpToDate, Inc. For more information visit www.uptodate.com.

Table 2 Pharmacological treatment for pemphigus

Topical options	First line	Other corticosteroid-sparing agents
For isolated lesions: Potent topical or intralesional steroids (clobetasol, betamethasone, triamcinolone)	Oral prednisone (0.5–1.0 mg/kg every 24 hrs as a single dose) – may increase every 5–7 days if blistering continues, or Pulsed intravenous prednisone for initial treatment of severe disease, or Anti-CD20 monoclonal antibodies: Rituximab (2 infusions of 1 g, 2 weeks apart – rheumatoid arthritis protocol)	If treatment failure occurs with a first-line adjuvant drug, consider switching to an alternate adjuvant agent. Methotrexate Cyclophosphamide Plasmapheresis or plasma exchange Intravenous immunoglobulin (IVIG) Dapsone
For oral lesions (paste or mouthwash): Triamcinolone acetonide 0.1% in adhesive paste (Adcortyl in Orabase®) Benzydamine hydrochloride 0.15% (Difflam Oral Rinse®) Betamethasone sodium phosphate 0.5 mg dissolved in 10 mL water Topical cyclosporine (5 mL of 100 mg/mL as mouthwash) Chlorhexidine gluconate 0.2% (Corsodyl), hexetidine 0.1% (Oraldene®) antiseptic mouthwash	May be combined with corticosteroid-sparing agents: Azathioprine 2–3 mg/kg daily, or Mycophenolate mofetil 2–3 g daily

Note: Data from Murrell et al,¹³ Ahmed et al,¹⁴ Gregoriou et al,¹⁵ Joly et al¹⁶ and Kridin.¹⁷

Table 3 Properties of dressing materials and topical agents

Type	Actions	Indications/use	Precautions/contraindications	Brand Names
Alginates	Absorb fluid Promote autolytic debridement Conformability to wound bed	Moderate to high exuding wounds. Spécial cavity présentations in the form of rope or ribbon. Can combine with silver for antimicrobial activity.	Do not use on dry/necrotic wounds. Use with caution on friable tissue (may cause bleeding). Do not pack cavity wounds tightly.	Algiderm (Bard) Algisorb™ (Calgon-Vestal) Algosteril® (KCI) Curasorb™ (The Kendall Co.) Kalginate® (DeRoyal) Kaltostat® (ConvaTec)
Foams	Absorb fluid Moisture control Conformability to wound bed	Moderate to high exuding wounds. Spécial cavity présentations in the form of strips or ribbon. Can combine with silver or PHMB for antimicrobial activity.	Do not use on dry/necrotic wounds or those with minimal exudate. Low-adhèrent versions are availabié for patients with fragile skin.	Allevyn® (Smith & Nephew United) Curafoam™ (The Kendall Co.) Hydrasorb® (Tyco/Kendall Co.) Mepilex® (Molnlycke Health Care) V.A.C. GranuFoam™ (KCI)
Hydrocolloids	Absorb fluid Promote autolytic debridement	Clean, low to moderate exuding wounds. Combined présentation with silver for antimicrobial activity.	Do not use on dry/necrotic wounds or high exuding wounds. May encourage overgranulation. May cause macération.	Comfeel® (Coloplast Sween, Inc.) Cuti nova® (Smith & Nephew) Duoderm® (ConvaTec) NuDerm® (KCI)
Hydrogels	Rehydrate wound bed Moisture control Promote autolytic debridement Cooling sensation	Dry/low to moderate exuding wounds. Combined présentation with silver for antimicrobial activity.	Do not use on highly exuding wounds or where anaérobie infection is suspected. May cause macération.	Clearsite® (Conmed Corp.) Curafil™ (The Kendall Co.) Elasto-Gel™ (SW Technologies) Normgel® (SCA Hygiene Products) 2nd Skin® (Spenco Medical, Ltd.) Transigel™ (Smith & Nephew) Vigilon® (CR Bard)
lodine	Antimicrobial action	Low to high exuding wounds. Critically colonized wounds or clinical signs of infection.	Do not use on dry necrotic tissue. Do not use with known sensitivity to iodine. Short-term use recommended (reduce systemic absorption).	lodosorb gel (Cadexomer iodine)
Low-adherent wound contact layer (silicone)	Protect new tissue growth Atraumatic to peri-wound skin Conformable to body contours	Low to high exuding wounds. Use as a contact layer on superficial low exuding wounds.	May dry out if left in place for too long. Do not use with known sensitivity to silicone.	ADAPTIC TOUCH™ (KCI) Cardinal Health™ Silicone Contact Layers (Cardinal Health) ComfiTel™ (DermaRite Industries) Cuticell® Contact (Essity) Mepitel® (Mölnlycke Health Care)
Odor control Dressing	Odor absorption	Malodorous wounds (due to excess exudate). May require additional antimicrobial if odor due to increased bioburden.	Do not use on dry wounds.	Exuderm Odorshield Dressing (Medline) CarboFLEX® (Convatec) ACTISORB® Silver 220™ (KCI)
PHMB (polyhexamethylene biguanide)	Antimicrobial action	Low to high exuding wounds. Critically colonized wounds or clinical signs of infection. May require secondary dressing.	Do not use on dry/necrotic wounds. Do not use with known sensitivity to PHMB.	Kerlix AMD™, Excilon AMD™, Telfa AMD™ (Tyco HealthCare Group) XCell® (Xylos Corp, Langhorne, Pa)
PMD (Polymeric membrane dressings)	Wound cleansing Absorb fluid Provide hydration Decrease pain	Exudative wounds. Venous, diabetic or pressure ulcers of all stages. Chronic wounds. Infected Wounds.	May require multiple dressing changes for high exudative wounds. Can be used on dry or infected wounds unlike traditional foam dressings.	Polymem® (Ferris Corp.)
Polyuréthane film	Moisture control Breathable bacterial barrier Transparent (visualization of wound)	Primary dressing over superficial low exuding wounds. Secondary dressing over alginate or hydrogel for rehydration of wound bed.	Do not use on patients with fragile/ compromised peri-wound skin.	BIOCLUSIVE™ (KCI) Finesse PU Film Dressing (Finesse Medical, LTD) Kendall™ Window Transparent Film Dressing (Cardinal Health)
Protease modulating	Active or passive control of wound protease levels	Cleans wounds that are not progressing despite correction of underlying causes, exclusion of infection, and optimal wound care.	Do not use on dry wounds or those with leathery eschar.	PROMOGRAN™ (KCI)
Silver	Antimicrobial action	Critically colonized wounds or clinical signs of infection. Low to high exuding wounds. Combined presentation with foam and alginates/ CMC for increased absorbency. Also in paste form.	May cause discoloration. Do not use if known sensitivity to silver. Discontinue after 2 weeks if no improvement and reevaluate.	Aquacel Ag® (ConvaTec) Acticoat 7 (Smith and nephew) ACTISORB® Silver 220 (KCI) Silversorb (medline) Silvercel (KCI)

Note: Data from Dabiri et al,¹⁰ Armstrong,²⁷ Powers et al,²⁸ Broussard and Powers,²⁹ and Borda et al.³⁰

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