Maria Pichler

Systematic review of surgical treatment of pyoderma gangrenosum with negative pressure wound therapy or skin grafting.

Editor Pyoderma gangrenosum (PG) is a rare autoinflammatory ulcerative skin disease. Fewer than 50% of the patients achieve wound healing after 6 months of immunosuppressive therapy and relapses occur in 30% to 60% of the cases. Progression of existing lesions or development of new lesions after surgery or minor trauma is reported in up to 30% of PG patients (pathergy phenomenon). Therefore, the role of surgical interventions such as split thickness skin grafting (STSG) and negative pressure wound therapy (NPWT) is controversially discussed, as these procedures might pose a trigger and further aggravate the condition, especially if performed without immunosuppression. A broad search of the PubMed, Medline, EBSCO Biomedical Reference Collection and Cochrane databases was performed and a total of 61 relevant articles describing 94 patients could be retrieved and complemented with our personal multicentre experience of 21 patients (see Table 1). A total of 115 patients with PG (69% females, median age 53) were identified. Ulcers were mainly situated on the lower extremities (n = 90; 78%), followed by breasts (n = 11; 10%), abdomen (n = 7; 6%) and other localizations (12; 10%). Surgical treatment consisted of split thickness skin grafts (STSG) alone (n = 53; 46%), STSG together with NPWT (n = 35; 30%), NPWT alone (12; 10%) or other forms of skin grafting (cultured cells, suction blisters, full thickness skin grafts, pinch grafts n = 15, 13%). Systemic immunosuppression consisted mainly of systemic corticosteroids (n = 107; 93%), supplemented with cyclosporine (21; 19%), dapsone (12; 9%), TNF-alpha antibodies (11; 9%), mycophenolate mofetil (9; 8%), azathioprine (7, 6%) and others used in less than five patients. Treatment was successful in 84 cases (73%), 18 patients healed but had a recurrence (16%), eight improved (7%), treatment failed in three patients and two patients died due to sepsis. The lower relapse rates observed in this review is probably due to the short follow-up in the many single patients reported. No case of pathergy was observed, neither at the side of PG nor at the skin graft donor sites. On the basis of the literature review and personal experience, we developed a surgical treatment algorithm with proposed immunosuppressive and adjuvant measures shown in Fig. 1. The basic immunosuppression consists of CS. As a second step, we prefer dapsone as anti-inflammatory steroid sparing agent over cyclosporine, as it does not affect renal function or blood pressure and for its well-known additional antimicrobial properties. Indeed, mortality rates from 11% to 17% have been reported for PG and were mostly related to sepsis and septical complications were the cause of death in the two deceased patients from the literature review. As a third step, if necessary, the anti-TNF-alpha antibody infliximab is used as the sole biologic with a positive randomized, double-blinded, placebocontrolled trial for PG. We further suggest the use of pentoxyphylline as adjuvant treatment for its known rheologic, immunomodulatory and anti-TNF-alpha activities. Indeed, it is advised by a Cochrane review for leg ulcers and also successfully used in other diseases with neutrophil dysfunction like oral aphthous ulcers or Behc et’s disease. Another drug with anti-inflammatory activity and positive effects on wound healing demonstrated recently in a blinded, randomized clinical trial is simvastatin. The adjuvant use of prostaglandins (iloprost) is suggested to facilitate skin grafting. Its positive effects on inflammation, ischaemic, but also venous leg ulcers are well known. This first comprehensive review of surgical treatment for PG confirms that NPWT and STSG comprises a valuable and safe treatment option for PG if performed under adequate immunosuppressive therapy. A paradigm change in the treatment of PG towards surgical approaches with STSG is suggested as this approach significantly accelerates healing time. A sustained immunosuppressive treatment with slow tapering and follow-up is suggested to avoid or manage recurrences as fast as possible.

Reconstruction of large rectangular infraorbital and malar skin defects in elderly patients with a modified “reading man flap” using local tumescent anesthesia

The classical “reading man flap” is a recently described flap named after its appearance and mainly used for reconstruction of circular malar or infraorbital skin defects. It avoids surgical complications such as lower lid retraction and ectropion but is limited to circular skin defects. Local tumescent anesthesia uses a diluted local anesthetic together with diluted epinephrine to anesthetize large skin areas without the need for general anesthesia.

Premature newborns with fatal intrauterine herpes simplex virus‐1 infection: first report of twins and review of the literature

Neonates with blistering skin diseases are dermatologic emergencies. The pathologies involved can pose diagnostic difficulties and there exists a variety of potential life‐threatening differential diagnoses.

Fehldiagnostiziertes mikrozystisches Adnexkarzinom an der seitlichen Stirn und Herausforderungen bei der Rekonstruktion eines bis zum Knochen reichenden Defekts.

Das mikrozystische Adnexkarzinom (MAC) ist ein seltenes kutanes Malignom, das durch aggressive lokale Infiltration und starke Tendenz zur perineuralen Invasion gekennzeichnet ist. Da es histologisch leicht mit benignen adnexalen Tumoren verwechselt werden kann, kommt oft zu einer unzureichenden Erstbehandlung. Daher müssen nach verzögerter chirurgischer Exzision häufig ausgedehnte Hautbereiche entfernt werden, was die anschließende Rekonstruktion erschweren kann.

Using the Peng flap for a wide dorsal nasal defect.

Reconstructions of large surgical defects of the central part of the nose pose a challenge for the surgeon due to its cosmetically prominent location. Treatment options include second intention healing, full‐thickness skin grafts and various local flaps. In the present case the “Peng flap” was used for the reconstruction of a 3.1 × 7 cm sized deep defect of the convex curve of the central nasal area in a 66‐year‐old woman. The Peng flap was performed as single‐stage procedure without complications. Good cosmetic outcome was provided by the use of adjacent skin from the lax perinasal area, the placement of the scar lines between facial cosmetic units, and the excellent tissue match for the sebaceous nasal skin. Considering the few complications of a single‐stage procedure associated with an excellent aesthetic outcome, the Peng flap should be considered not only as an effective choice for reconstruction of the cosmetically complex midline nasal tip but also the convex curve of the central nose.

Misdiagnosed microcystic adnexal carcinoma on the lateral forehead and challenges in reconstruction of a large and bone-deep defect.

Microcystic adnexal carcinoma (MAC) is a rare cutaneous malignancy characterized by aggressive local infiltration, including a high propensity for perineural invasion. Histologically it can be easily confused with benign adnexal tumors, which often leads to inappropriate initial treatment. As a consequence delayed surgical excision often requires removal of large cutaneous masses which can be followed by challenges in reconstruction.

Direct comparison between negative wound pressure therapy and negatively charged polystyrene microspheres in wound bed preparation for split skin grafting in two large symmetric whole circumference leg ulcers, a single patient experience

ciated with scarce epithelioid granulomas. After a short course of antibiotics, the area of debridement was skin-grafted. The diagnosis of CD revealed by psoas abscesses and NF was retained. Azathioprine was started 2 months after surgery. Six months later, digestive continuity was reestablished and the patient remained asymptomatic under aziathioprine with complete skin healing. Digestive fistulas are well-known CD complications. Our patient’s cruralgia probably reflected a non-specific clinical manifestation of her psoas abscesses. Psoas abscess is a rare condition usually associated with gastrointestinal tract diseases among which CD is the most frequent. Its prevalence in CD patients ranges from 0.6% to 10% and is also considered to be rare. Bowel to psoas fistulas and enterocutaneous fistulas with necrotizing fasciitis in CD patients were previously reported. In our observation, the daily ketoprofen injections probably delayed diagnosis of our patient0s psoas abscesses and might have worsened the initial presentation. In addition, her psychiatric history could have led to misdiagnosis of faecal self injection. Although digestive fistulas leading to NF are rare complications of CD when polymicrobial necrotizing fasciitis is suspected, clinicians should actively search and eliminate a digestive portal of entry (e.g. CD fistulas).

Cupping is contraindicated in Haily‐Haily disease – the seamy site of alternative medicine

Hailey-Hailey disease (HHD) or familiar benign chronic pemphigus is a rare autosomal-dominant genodermatosis characterized by recurrent erythematous plaques with predilection for intertriginous areas. Histologically, HHD is characterized by incomplete suprabasal acantholysis due to different mutations in the ATP2C1 gene which encodes for a calcium transport ATPase essential for keratinocyte adhesion. Btadini et al. conducted heat-shock experiments on different types of fibroblasts and demonstrated an increase in ATP2C1 expression in all of them. They conclude that a combination of genetic and environmental factors is necessary for the manifestation of HHD. Cupping is a widely used ancient traditional technique to treat pain and various other conditions. After a specific point selection, a vacuum by manual or electromechanical suction is produced.

A case of Hailey‐Hailey herpeticum

eczema herpeticum refers to a severe disseminated herpes simplex virus (HSV) infection superimposed on a preexisting eczematous skin disease, in most cases atopic dermatitis. Kaposi’s varicelliform eruption (KVE), on the other hand, designates a disseminated HSV infection superimposed on any preexisting dermatosis, including dyskeratosis follicularis (Darier’s disease) [ 1 ] , mycosis fungoides, Sézary syndrome, Wiskott-Aldrich syndrome, seborrheic dermatitis, pemphigus foliaceus, ichthyosis vulgaris, patients with burns, and Hailey-Hailey disease [ 2, 3 ] . The most common predisposing factor is the skin disease-induced disruption of the stratum corneum, which affects the integrity of the epidermis [ 4 ] . Beginning with a monomorphic eruption of multiple umbilicated vesicles and pustules in the affected areas, KVE may subsequently spread to the entire integument. Hailey-Hailey disease (HHD), also known as familial benign chronic pemphigus, was fi rst described by the Hailey brothers in 1939. Marked by autosomal dominant inheritance, it is a rare blistering skin disorder caused by mutations in the ATP2C1 gene, which codes for a portion of a calcium pump essential for proper keratinocyte differentiation and adhesion. Predominantly affecting the skin in the intertriginous areas, HHD usually presents in the third or fourth decade. Clinically, it is characterized by vesicles, erosions, and crusts. Histopathology typically shows acantholytic dyskeratosis of the epidermis. Simultaneous occurrence of KVE and HHD is a possible rare, yet severe dermatological condition. One pathogenetic factor contributing to HSV infection is the disruption of the compact keratinocyte layer caused by HHD. Similar to atopic dermatitis, this results in unmasking of the desmosomal protein nectin-1, which acts as one of the relevant entry receptors for HSV. Accordingly, an impaired skin barrier may also lead to antimicrobial peptide dysregulation, resulting in impaired antiviral activity of cathelicidin peptide LL-37 [ 5, 6 ] . Only 14 cases of KVE occurring in HHD have so far been described in the medical literature (Table 1 ).

Ein Fall von Morbus Hailey‐Hailey in Verbindung mit einem Eczema Herpeticatum

Literatur 1 Thyssen JP , White JM . Epidemiological data on consumer allergy to p-phenylenediamine . Contact dermatitis 2008 ; 59 : 327 – 43 . 2 Helaskoski E , Suojalehto H , Virtanen H et al. Occupational asthma, rhinitis and contact urticaria caused by oxidative hair dyes in hairdressers . Ann Allergy Asthma Immunol 2014 ; 112 : 46 – 52 . 3 Kind F , Scherer K , Bircher AJ . Contact dermatitis to paraphenylenediamine in hair dye following sensitization to black henna tattoos–an ongoing problem . J Dtsch Dermatol Ges 2012 ; 10 ( 8 ): 572 – 8 . 4 Goebel C , Coenraads PJ , Rothe H et al. Elicitation of the immune response to p-phenylenediamine in allergic patients: the role of dose and exposure time . Br J Dermatol 2010 ; 163 : 1205 – 11 . 5 Birnie AJ , English JS . Immediate hypersensitivity to paraphenylendiamine . Contact Dermatitis 2007 ; 56 : 240 – 5 . 6 Wong GAE , King CM . Immediate-type hypersensitivity and allergic contact dermatitis due to para-phenylendiamine in hair dye . Contact dermatitis 2003 ; 48 : 166 . 7 Pot LM , Scheitza SM , Coenraads PJ et al. Penetration and haptenation of p-phenylenediamine . Contact Dermatitis 2013 ; 68 : 193 – 207 . spezifi schen IgE von PPD oder von dessen Derivaten im RadioAllergo-Sorbent-Test (RAST) ist nicht beschrieben [ 2 ] . Der zur Sensibilisierung und Auslösung führende Mechanismus von PPD wurde für Typ-IV-Allergien untersucht, ist bislang allerdings nicht vollständig verstanden. PPD hat eine hohe Oxidationsneigung und wird zu Bandrowski-Base beziehungsweise zu p-Benzochinon konvertiert. Es wird davon ausgegangen, dass nicht nur PPD, sondern auch seine Oxidationsprodukte zur Sensibilisierung führen. Das höchste sensibilisierende Potenzial wird dabei der Bandrowski-Base zugeschrieben [ 7 ] . Bezüglich der Typ-I-Sensibilisierung ist nicht bekannt, inwiefern PPD oder eines seiner Oxidationsprodukte als Sensibilisator fungieren. Ebenso ist der genaue Mechanismus, wie das Hapten Paraphenylendiamin zur Mastzelldegranulation führen soll, nicht klar. Differenzialdiagnostisch ist an eine nicht allergisch bedingte Urtikaria zu denken. Allerdings ist der positive Reibtest auf die Haarcoloration und der positive Pricktest auf PPD, dessen Spezifi tät durch einen Kontrollprobanden bestätigt wurde, auch bei fehlender Provokationstestung ein klarer Hinweis für eine Typ-I-Sensibilisierung auf PPD als Ursache der Anaphylaxie. Aufgrund des potenziell vital bedrohlichen Verlaufs hat die Typ-I-Sensibilisierung auf PPD trotz ihrer niedrigen Prävalenz eine hohe klinische Relevanz und sollte Tabelle 1 Positive Reaktion im Reibetest auf die verwendete Haarcoloration sowie im Pricktest auf p-Phenylendiamin. Negative Reaktion im Pricktest auf p-Toluylendiamin, 3-Aminophenol, p-Aminophenol, Resorcin, Propylenglykol, Duftstoffmix I/II.