Letters to the Editor
189
(a)
(b)
Figure 2 (a) Case 1. Histopathology showing a normal epidermis and a deep granulomatous infiltrate with multinucleated giant cells surrounded by lymphohistiocytes, occasional plasma cells and eosinophils. (b) Case 2. Three-layered granuloma with necrosis and neutrophils (arrow up), multinucleated giant cells (arrow right), and plasma cells and lymphocytes (arrow left).
Although mostly idiopathic, vegetative PG has been occasionally linked to associated disorders.4 To our knowledge, only few cases of PG and lung carcinoma have been described in the literature and this is the first report with evidence of granuloma formation on biopsy. Remarkably, our second patient also had an underlying disorder MGUS. These cases illustrate that granuloma formation in pyoderma gangrenosum is not limited to the benign vegetative variant. Our patients displayed an impressive clinical presentation with high fever and extensive skin involvement. This probably represents an overlap between different variants of pyoderma gangrenosum as pustules and ulcerations were also present. Therefore, after the histological findings of granulomas, the diagnosis of pyoderma gangrenosum should be kept in mind even in acutely ill patients where an infectious disease seems more probable. R. Speeckaert,1,†,* L. De Smet,1,† S. De Schepper,1 N. van Geel,1 J. Veramme,1 L. Vanderdonckt,1 L. Temmerman,2 J. Lambert,1 H. Beele1 1
Department of Dermatology, Ghent University Hospital, Gent, Belgium, 2 Department of Dermatology, Maria Middelares, Gent, Belgium *Correspondence: R. Speeckaert. E-mail: Reinhart.Speeckaert@ ugent.be † Equally contributed as first authors.
References 1 Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: a comprehensive review. Am J Clin Dermatol 2012; 13: 191–211. 2 Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venereol 2009; 23: 1008–1017. 3 Wilson-Jones E, Winkleman RK. Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum. J Am Acad Dermatol 1988; 18: 511–521. 4 Langan SM, Powell FC. Vegetative pyoderma gangrenosum: a report of two new cases and review of the literature. Int J Dermatol 2005; 44: 623– 629. DOI: 10.1111/jdv.12699
JEADV 2016, 30, 124–200
Assessing the quality of life over longer periods Editor We have read the manuscript by Kim et al. with great interest. The manuscript raises the perspective of time in quality of life (QoL) assessments. The Dermatology Life Quality Index (DLQI) is frequently used and shows the impact on QoL during the last week (LW).1 The authors investigated whether retrospective questions about chronic quality of life were better predictors of poor socioeconomic and medical outcomes than the current DLQI.2 In their study, lifetime (LT) and a last year’s (LY) DLQI were compared to the current standard LW DLQI. The authors found that DLQI showed a temporal correlation, i.e. DLQI mean value was 8.9 LW, 11.3 LY and 15.1 LT. The authors suggest that LT DLQI is a better predictor of patient outcomes related to weight, discrimination and depression than the standard LW DLQI. As all these factors are long-term consequences of the disease it may be discussed if the conclusion is simple circular reasoning or possibly a psychological mechanism justifying each patient’s current status. Different time frames have previously been studied for the DLQI, with similar results. In 2009, LW and LY DLQI were compared in a study aimed at capturing the trajectory of the QoLimpact over time for various skin diseases among 42 patients.3 The study found that current QoL impairment appeared to be lesser than long-term impairment. For all patients a median value of 8 LW and 10 LY (P = 0.03) was found. In particular, DLQI question 2 and 9 concerning embarrassment/self-consciousness and sexuality showed significant difference (P = 0.001 and 0.03) and this corresponds to the study by Kim et al. where greater LY DLQI was predictive of a greater likelihood of inhibiting sexual relationships (P = 0.007) and, among others, of more experiences of discrimination in social settings (P < 0.001). Whereas the study by Kim focussed on psoriasis, the earlier study was based on data from 13 diagnoses (11/42 had psoriasis), implying that that the observation is not disease-specific. More importantly, prospective data have been provided. Kim et al. found that patients with greater LT DLQI rated their psori-
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
190
asis while on various treatments as worse than their present psoriasis, which was in agreement with a lower LW DLQI (8.9) compared to LT DLQI (15.1).2 Similar results have been published for 34 patients with atopic dermatitis (AD) in a prospective study in 2012.4 Patients were given the age-appropriate questionnaires (DLQI, CDLQI or IDQOL) in 2003/04 and again in 2010 (if they had persistent disease) and asked to assess the severity of their AD, which has previously been suggested to be a valid patient reported outcome in AD.5 An almost 50% improvement of QoL was seen over the 6 years.4 Self-reported severity scores also showed an improvement of 33%. The study supports the validity of self-reported LT changes in QoL. It is suggested that these previous studies support the relevance of assessing the QoL over longer periods than the LW. In particular the prospective data implies that such a construct is valid, but require further argumentation and validation. S. Esmann, G.B.E. Jemec* Department of Dermatology, Roskilde Hospital, Health Sciences Faculty, University of Copenhagen, Roskilde, Denmark *Correspondence: G.B.E. Jemec. E-mail: [email protected]
References 1 Finlay AY, Khan GK. Dermatology Life Quality Index (DLQI): a simple practical measure for routine clinical use. Clin Exp Dermatol 1994; 19: 210–216. 2 Kim GE, Seidler E, Kimball AB. A measure of chronic quality of life predicts socioeconomic and medical outcomes in psoriasis patients. J Eur Acad Dermatol Venereol 2014. Doi:10.1111/JDV12503 [Epub ahead of print] 3 Esmann S, Jemec GB. Is the Dermatology Life Quality Index really time-sensitive? J Eur Acad Dermatol Venereol 2010; 24: 621–622. Epub 2009 Nov 19. 4 Vinding GR, Esmann S, Jemec GB. Quality of life in atopic dermatitis: changes over 6 years in patients who report persistent eczema. J Dermatol 2012; 39: 721–722. Epub 2012 Mar 6. 5 Holm EA, Wulf HC, Stegmann H, Jemec GB. Life quality assessment among patients with atopic eczema. Br J Dermatol 2006; 154: 719–725. DOI: 10.1111/jdv.12700
Table 1 Differential diagnosis of ABH Bullous diseases Lichen planus pemphigoides Linear IgA bullous bullous dermatosis Pemphigus Bullous pemphigoid Epidermolysis bullosa Haemostasis disorders Blood blisters resulting from endoscopic procedures Angiomatoid lesions of Rendu–Osler–Weber disease
biopsy of the periphery of a lesion found a normal mucosa without specific abnormality, and without acantholysis; no amyloid deposition was observed using Congo red stain, and immunofluorescence techniques were negative. As the oral blood-filled blisters could not be attributed to blood dyscrasia, systemic disease or blistering disease, the diagnosis of angina bullosa haemorrhagica (ABH) was retained (Table 1). ABH was first described by Badham et al. in 19671; however, the term angina is improper as there is no infection or swelling of the tonsils. The lesions usually occur mainly at the junction of the hard and soft palate or on other sites of the oral mucosa such as lateral and ventral border of the tongue, and oral mucosa; gums do not seem to be affected.2 Clinically, ABH initially corresponds to one or more dark red to purple blisters which can be painful.3 After a short time, blisters spontaneously break and leave a bleeding, painless ulcer that heals within 1 or 2 weeks, without scarring.2 Recurrences occur in 30% of patients, not necessarily in the same location.4 Although ABH is a benign pathology, acute upper airway obstruction caused by a blood-filled bulla in the orophanryx is possible, requiring tracheal intubation.5 The incidence of ABH remains unknown in general population, probably because it is an unknown and under-diagnosed disease. In patients seen in his Department of Oral pathology and Disease, Grispan D
Angina bullosa haemorragica Editor A 43-year-old woman, with no significant medical history, was referred to our hospital with a 15 year history of recurrent oral blood-filled blisters, located on the inside of her cheeks or on her tongue (Fig. 1). No obvious trigger was noticed. After rupture, the blisters healed spontaneously within 2 weeks, without scarring. Although they were not painful, any postbullous erosion was. The rest of the general examination was unremarkable. Blood cells count, coagulation tests were normal. Research for antinuclear antibodies, antibullous pemphigoid antibodies and antidesmoglein antibodies was negative; complement fractions and the distribution of immunoglobulin were normal. The
JEADV 2016, 30, 124–200
Figure 1 Angina bullosa haemorragica: blood bister on the tongue.
© 2014 European Academy of Dermatology and Venereology
189
(a)
(b)
Figure 2 (a) Case 1. Histopathology showing a normal epidermis and a deep granulomatous infiltrate with multinucleated giant cells surrounded by lymphohistiocytes, occasional plasma cells and eosinophils. (b) Case 2. Three-layered granuloma with necrosis and neutrophils (arrow up), multinucleated giant cells (arrow right), and plasma cells and lymphocytes (arrow left).
Although mostly idiopathic, vegetative PG has been occasionally linked to associated disorders.4 To our knowledge, only few cases of PG and lung carcinoma have been described in the literature and this is the first report with evidence of granuloma formation on biopsy. Remarkably, our second patient also had an underlying disorder MGUS. These cases illustrate that granuloma formation in pyoderma gangrenosum is not limited to the benign vegetative variant. Our patients displayed an impressive clinical presentation with high fever and extensive skin involvement. This probably represents an overlap between different variants of pyoderma gangrenosum as pustules and ulcerations were also present. Therefore, after the histological findings of granulomas, the diagnosis of pyoderma gangrenosum should be kept in mind even in acutely ill patients where an infectious disease seems more probable. R. Speeckaert,1,†,* L. De Smet,1,† S. De Schepper,1 N. van Geel,1 J. Veramme,1 L. Vanderdonckt,1 L. Temmerman,2 J. Lambert,1 H. Beele1 1
Department of Dermatology, Ghent University Hospital, Gent, Belgium, 2 Department of Dermatology, Maria Middelares, Gent, Belgium *Correspondence: R. Speeckaert. E-mail: Reinhart.Speeckaert@ ugent.be † Equally contributed as first authors.
References 1 Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: a comprehensive review. Am J Clin Dermatol 2012; 13: 191–211. 2 Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venereol 2009; 23: 1008–1017. 3 Wilson-Jones E, Winkleman RK. Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum. J Am Acad Dermatol 1988; 18: 511–521. 4 Langan SM, Powell FC. Vegetative pyoderma gangrenosum: a report of two new cases and review of the literature. Int J Dermatol 2005; 44: 623– 629. DOI: 10.1111/jdv.12699
JEADV 2016, 30, 124–200
Assessing the quality of life over longer periods Editor We have read the manuscript by Kim et al. with great interest. The manuscript raises the perspective of time in quality of life (QoL) assessments. The Dermatology Life Quality Index (DLQI) is frequently used and shows the impact on QoL during the last week (LW).1 The authors investigated whether retrospective questions about chronic quality of life were better predictors of poor socioeconomic and medical outcomes than the current DLQI.2 In their study, lifetime (LT) and a last year’s (LY) DLQI were compared to the current standard LW DLQI. The authors found that DLQI showed a temporal correlation, i.e. DLQI mean value was 8.9 LW, 11.3 LY and 15.1 LT. The authors suggest that LT DLQI is a better predictor of patient outcomes related to weight, discrimination and depression than the standard LW DLQI. As all these factors are long-term consequences of the disease it may be discussed if the conclusion is simple circular reasoning or possibly a psychological mechanism justifying each patient’s current status. Different time frames have previously been studied for the DLQI, with similar results. In 2009, LW and LY DLQI were compared in a study aimed at capturing the trajectory of the QoLimpact over time for various skin diseases among 42 patients.3 The study found that current QoL impairment appeared to be lesser than long-term impairment. For all patients a median value of 8 LW and 10 LY (P = 0.03) was found. In particular, DLQI question 2 and 9 concerning embarrassment/self-consciousness and sexuality showed significant difference (P = 0.001 and 0.03) and this corresponds to the study by Kim et al. where greater LY DLQI was predictive of a greater likelihood of inhibiting sexual relationships (P = 0.007) and, among others, of more experiences of discrimination in social settings (P < 0.001). Whereas the study by Kim focussed on psoriasis, the earlier study was based on data from 13 diagnoses (11/42 had psoriasis), implying that that the observation is not disease-specific. More importantly, prospective data have been provided. Kim et al. found that patients with greater LT DLQI rated their psori-
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
190
asis while on various treatments as worse than their present psoriasis, which was in agreement with a lower LW DLQI (8.9) compared to LT DLQI (15.1).2 Similar results have been published for 34 patients with atopic dermatitis (AD) in a prospective study in 2012.4 Patients were given the age-appropriate questionnaires (DLQI, CDLQI or IDQOL) in 2003/04 and again in 2010 (if they had persistent disease) and asked to assess the severity of their AD, which has previously been suggested to be a valid patient reported outcome in AD.5 An almost 50% improvement of QoL was seen over the 6 years.4 Self-reported severity scores also showed an improvement of 33%. The study supports the validity of self-reported LT changes in QoL. It is suggested that these previous studies support the relevance of assessing the QoL over longer periods than the LW. In particular the prospective data implies that such a construct is valid, but require further argumentation and validation. S. Esmann, G.B.E. Jemec* Department of Dermatology, Roskilde Hospital, Health Sciences Faculty, University of Copenhagen, Roskilde, Denmark *Correspondence: G.B.E. Jemec. E-mail: [email protected]
References 1 Finlay AY, Khan GK. Dermatology Life Quality Index (DLQI): a simple practical measure for routine clinical use. Clin Exp Dermatol 1994; 19: 210–216. 2 Kim GE, Seidler E, Kimball AB. A measure of chronic quality of life predicts socioeconomic and medical outcomes in psoriasis patients. J Eur Acad Dermatol Venereol 2014. Doi:10.1111/JDV12503 [Epub ahead of print] 3 Esmann S, Jemec GB. Is the Dermatology Life Quality Index really time-sensitive? J Eur Acad Dermatol Venereol 2010; 24: 621–622. Epub 2009 Nov 19. 4 Vinding GR, Esmann S, Jemec GB. Quality of life in atopic dermatitis: changes over 6 years in patients who report persistent eczema. J Dermatol 2012; 39: 721–722. Epub 2012 Mar 6. 5 Holm EA, Wulf HC, Stegmann H, Jemec GB. Life quality assessment among patients with atopic eczema. Br J Dermatol 2006; 154: 719–725. DOI: 10.1111/jdv.12700
Table 1 Differential diagnosis of ABH Bullous diseases Lichen planus pemphigoides Linear IgA bullous bullous dermatosis Pemphigus Bullous pemphigoid Epidermolysis bullosa Haemostasis disorders Blood blisters resulting from endoscopic procedures Angiomatoid lesions of Rendu–Osler–Weber disease
biopsy of the periphery of a lesion found a normal mucosa without specific abnormality, and without acantholysis; no amyloid deposition was observed using Congo red stain, and immunofluorescence techniques were negative. As the oral blood-filled blisters could not be attributed to blood dyscrasia, systemic disease or blistering disease, the diagnosis of angina bullosa haemorrhagica (ABH) was retained (Table 1). ABH was first described by Badham et al. in 19671; however, the term angina is improper as there is no infection or swelling of the tonsils. The lesions usually occur mainly at the junction of the hard and soft palate or on other sites of the oral mucosa such as lateral and ventral border of the tongue, and oral mucosa; gums do not seem to be affected.2 Clinically, ABH initially corresponds to one or more dark red to purple blisters which can be painful.3 After a short time, blisters spontaneously break and leave a bleeding, painless ulcer that heals within 1 or 2 weeks, without scarring.2 Recurrences occur in 30% of patients, not necessarily in the same location.4 Although ABH is a benign pathology, acute upper airway obstruction caused by a blood-filled bulla in the orophanryx is possible, requiring tracheal intubation.5 The incidence of ABH remains unknown in general population, probably because it is an unknown and under-diagnosed disease. In patients seen in his Department of Oral pathology and Disease, Grispan D
Angina bullosa haemorragica Editor A 43-year-old woman, with no significant medical history, was referred to our hospital with a 15 year history of recurrent oral blood-filled blisters, located on the inside of her cheeks or on her tongue (Fig. 1). No obvious trigger was noticed. After rupture, the blisters healed spontaneously within 2 weeks, without scarring. Although they were not painful, any postbullous erosion was. The rest of the general examination was unremarkable. Blood cells count, coagulation tests were normal. Research for antinuclear antibodies, antibullous pemphigoid antibodies and antidesmoglein antibodies was negative; complement fractions and the distribution of immunoglobulin were normal. The
JEADV 2016, 30, 124–200
Figure 1 Angina bullosa haemorragica: blood bister on the tongue.
© 2014 European Academy of Dermatology and Venereology
