1576 Correspondence
negative results, as was recently reported for 10 of 13 cases of EBA with salt-split skin negativity, probably due to a low sensitivity of the technique.7 The aspect and the topography of our patients’ lesions did not support the hypothesis of EPS but could have resulted from the coexistence of two phenomena induced by D-penicillamine: abnormal elastic tissue and autoimmunity against collagen VII as in EBA. However, complete resolution of lesions contrasted with the classical irreversibility of EPS.8 The quick improvement after D-penicillamine withdrawal in our patients is a strong argument for the drug-induced lesions, without any relapse with trientine dihydrochloride. This latter drug, another copper chelator, acts by the renal elimination of copper but does not have any anti-inflammatory direct action that could have explained the improved condition. The immunoblot findings in the patient without clinical manifestations suggest that D-penicillamine could induce a symptomatic or nonsymptomatic anticollagen VII autoimmunity in some patients. However, the role of Wilson disease itself in the pathophysiological aspects of the phenomenon remains to be investigated. A genetic susceptibility could explain the simultaneity of similar lesions in the woman and her brother. Several subtypes of HLA class II are associated with autoimmune diseases. HLADRB1*08 is a susceptibility factor for pemphigus vulgaris,9 but only HLA-DR2 (DRB1*15-16) is known to be associated with EBA.10 Department of Dermatology, 3Department S. INGEN-HOUSZ-ORO1 7 of Pathology and Department of S. GROOTENBOER-MIGNOT2 Immunology, AP-HP, Henri Mondor N. ORTONNE3 Hospital, 51 avenue S. NAHON4 du Marechal de Lattre de Tassigny 94000, J. HORVATH5 Creteil, France C. BERNARDESCHI1 2 Department of Auto-immunity and E. LAFFITTE6 Hypersensitivity, Bichat Hospital, Paris, C . A N D R E7 France S. CHOLLET-MARTIN2 4 Department of Gastro-Enterology, P . W O L K E N S T E I N 1, 8, 9 Montfermeil Hospital, Montfermeil, France O . C H O S I D O W 1, 8, 9 5 Department of Neurology and 6 Clinic of Dermatology, Geneva University Hospitals, Geneva, Switzerland 8 Universite Paris-Est Creteil Val de Marne, Creteil, France 9 INSERM, Centre d’Investigation Clinique 006, AP-HP, Creteil, France Correspondence: Saskia Ingen-Housz-Oro. E-mail: [email protected]
2 Cetkovska P, Pizinger K, Skalova A. Epidermolysis bullosa acquisita-like reaction associated with penicillamine therapy for sclerodermatous graft-versus-host disease. J Am Acad Dermatol 2003; 49:1157–9. 3 Grootenboer-Mignot S, Descamps V, Picard-Dahan C et al. Place of human amniotic membrane immunoblotting in the diagnosis of autoimmune bullous dermatoses. Br J Dermatol 2010; 162:743–50. 4 Bardach H, Gebhart W, Niebauer G. ‘Lumpy-bumpy’ elastic fibers in the skin and lungs of a patient with a penicillamineinduced elastosis perforans serpiginosa. J Cutan Pathol 1979; 6:243–52. 5 Atzori L, Pinna AL, Pau M, Aste N. D-penicillamine elastosis perforans serpiginosa: description of two cases and review of the literature. Dermatol Online J 2011; 17:3. 6 Beer WE, Cooke KB. Epidermolysis bullosa induced by penicillamine. Br J Dermatol 1967; 79:123–5. 7 Terra JB, Jonkman MF, Diercks GFH, Pas HH. Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis. Br J Dermatol 2013; 169:164–7. 8 Carlesimo M, Narcisi A, Cortesi G et al. An 18-year follow-up of a case of D-penicillamine-induced elastosis perforans serpiginosa. Int J Immunopathol Pharmacol 2011; 24:257–9. 9 Yan L, Wang J-M, Zeng K. Association between HLA-DRB1 polymorphisms and pemphigus vulgaris: a meta-analysis. Br J Dermatol 2012; 167:768–77. 10 Zumelzu C, Le Roux-Villet C, Loiseau P et al. Black patients of African descent and HLA-DRB1*15:03 frequency overrepresented in epidermolysis bullosa acquisita. J Invest Dermatol 2011; 131:2386–93.
1
P.W. and O.C. contributed equally to this paper.
References 1 Bialy-Golan A, Brenner S. Penicillamine-induced bullous dermatoses. J Am Acad Dermatol 1996; 35:732–42. British Journal of Dermatology (2014) 171, pp1555–1608
Funding sources: no external funding. Conflicts of interest: none declared.
Hidradenitis suppurativa fulminans: a clinically distinct phenotype? DOI: 10.1111/bjd.13147 DEAR EDITOR, Hidradenitis suppurativa (HS) is a chronic suppurative skin condition characterized by recurrent inflammatory nodules, cysts, abscesses and sinus tracts in apocrine-glandbearing sites. The clinical presentation is highly variable both in terms of the cutaneous features, distribution, presence of complications (e.g. fistula formation, lymphoedema) extracutaneous features (arthritis, interstitial keratitis)1 and associated constitutional symptoms (fever, malaise). Despite this apparent phenotypic heterogeneity it is only recently that attempts have been made to classify the disease. The patient cohort with c-secretase mutations described by Pink et al.2 represents the first genetically defined phenotypic subgroup of HS, but mutations appear rare in the disease population. A recent latent class analysis of over 600 patients initiated the formal process of classifying HS into three distinct clinical phenotypes: © 2014 British Association of Dermatologists
Correspondence 1577
axillary–mammary, follicular and gluteal.3 In this report we document a rare but clinically distinct subset of patients with HS with what we have termed ‘HS fulminans’, and discuss how they relate to this recent phenotypic classification. A small number of patients with HS were observed to present with severe cutaneous disease, extracutaneous manifestations including arthritis and keratitis, and marked constitutional upset associated with disease flares. Medical records of all patients with HS attending tertiary referral clinics at St John’s Institute of Dermatology and King’s College Hospital, London, were examined to identify patients with a similar disease presentation. A total of seven such patients were identified (Table 1). All seven patients were male and of AfroCaribbean descent. The mean age at disease onset was 265 years (range 14–34). Five patients described gradual disease onset prior to a severe and persistent flare of cutaneous disease. All seven patients had Hurley grade 3 disease at presentation, comprising extensive sinus tracts, nodules, abscesses and comedones at involved sites. Four had axillary involvement, six had inguinoscrotal disease and six had gluteal involvement. Two individuals had HS involving atypical sites including the head, neck, upper trunk and lower back. Sartorius scores could not be accurately assessed due to the disease severity and confluent nature of the sinus tracts. Six of the seven patients presented with associated genital lymphoedema. All patients underwent magnetic resonance imaging of the pelvis, which demonstrated anal fistulae in two patients. All seven cases were associated with multisystem inflammation comprising fever or night sweats (n = 6), recent weight loss (n = 4), arthralgia (n = 6) or reactive ocular keratitis (n = 1). Five individuals were diagnosed with seronegative inflammatory arthritis by a rheumatologist (axial disease in two, peripheral oligoarthritis in three) on the basis of typical clinical and radiological findings. Six patients had a raised erythrocyte sedimentation rate (ESR), the average ESR in the group being 899 mm h 1 (range 19–151). Four of the seven patients presented with a microcytic, iron-deficiency anaemia (mean haemoglobin 1025 g dL 1, range 88–115, not measured in two individuals). All individuals had a normal body mass index (mean 241), an unusual finding in HS.4 All patients were resistant to initial medical therapy (single and combination antibiotics in seven, isotretinoin in five, acitretin in three and oral steroids in six) and required treatment with biological therapies (five infliximab, two adalimumab), often in combination with incision and drainage of problematic lesions (in four patients) to achieve control of this severe presentation. We have identified a subset of male Afro-Caribbean patients in whom an explosive onset of severe multifocal disease and genital lymphoedema was associated with systemic involvement characterized by fever, raised inflammatory markers and an inflammatory arthritis. This is similar to acne fulminans, a severe form of acne with acute onset characterized by necrotic skin lesions and systemic features including arthralgia, fever, raised inflammatory markers, leucocytosis and osteolytic bone lesions.5 However, neither a leucocytosis nor osteolytic © 2014 British Association of Dermatologists
Table 1 Characteristics of seven patients with ‘fulminant’ hidradenitis suppurativa (HS) Age at symptom onset (years), mean (range) Male sex Afro-Caribbean racial group Smoker (current) Body mass index (kg m 2), mean (range) Fulminant presentation Explosive disease Fever/night sweats Weight loss Joint symptoms Low haemoglobin (mean 1025 g dL 1) High ESR (mean 899 mm h 1) Distribution of cutaneous disease Axillae Inguinoscrotal Gluteal Lower back Head/neck Upper trunk Other follicular disorders Acne Dissecting cellulitis Local complications Genital lymphoedema Enterocutaneous fistula Other systemic features Inflammatory arthritis Ocular keratitis Low mood Suicidal ideation/intent Family history of HS Family history of other follicular disorder
265 7 7 4 241
(14–34) (100) (100) (57) (227–263)
7 6 4 6 4 6
(100) (86) (57) (86) (57) (86)
4 6 6 1 1 1
(57) (86) (86) (14) (14) (14)
5 (71) 3 (43) 6 (86) 2 (29) 5 1 7 4 3 2
(71) (14) (100) (57) (43) (29)
Values are n (%) unless stated otherwise. ESR, erythrocyte sedimentation rate.
changes were detected in the patients with HS fulminans. The presentation seen in our cohort is not completely consistent with any of the three clinical phenotypes described by Canoui-Poitrine et al.3 The male predominance, disease severity and duration, hypertrophic scarring and association with acne vulgaris would indicate that these patients overlap with, and may form a subgroup within, the ‘follicular’ phenotype. The ability of latent class analysis to detect phenotypic subgroups is dependent on the nature and number of clinical variables that are analysed. We agree with Ingram and Piguet6 that further phenotypic data should be incorporated into future analyses to increase the sensitivity of detection of small phenotypic subgroups such as this. The phenotypic subgroup presented here represents a severe and ‘fulminant’ form of HS. A number of rare phenotypes have now been reported to comprise features of HS including PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, suppurative hidradenitis),7 PASH (pyoderma gangrenosum, acne, suppurative hidradenitis),8 SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis)1 and HS and keratitis,1 which need to be considered in the evolving disease classificaBritish Journal of Dermatology (2014) 171, pp1555–1608
1578 Correspondence 6 Ingram JR, Piguet V. Phenotypic heterogeneity in hidradenitis suppurativa (acne inversa): classification is an essential step toward personalized therapy. J Invest Dermatol 2013; 133:1453–6. 7 Marzano AV, Trevisan V, Gattorno M et al. Pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH): a new autoinflammatory syndrome associated with a novel mutation of the PSTPIP1 gene. JAMA Dermatol 2013; 149:762–4. 8 Braun-Falco M, Kovnerystyy O, Lohse P, Ruzicka T. Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) – a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol 2012; 66:409–15.
tion. We believe a multidisciplinary approach encompassing genetics, immunology, histopathology and clinically led research will expedite refinement of the current phenotypic classification of HS. 1
Department of Dermatology, King’s College Hospital, Denmark Hill, London SE5 9RS, U.K. 2 St John’s Institute of Dermatology, Guy’s and St Thomas’ NHS Foundation Trust, London, U.K. E-mail: [email protected]
B. MORIARTY1 A. PINK2 D. CREAMER1 N. DESAI2
Funding sources: none. Conflicts of interest: none declared.
References 1 Alzaga Fernandez AG, Demirci H, Darnley-Fisch DA, Steen DW. Interstitial keratitis secondary to severe hidradenitis suppurativa: a case report and literature review. Cornea 2010; 29:1189–91. 2 Pink AE, Simpson MA, Desai N et al. c-Secretase mutations in hidradenitis suppurativa: new insights into disease pathogenesis. J Invest Dermatol 2013; 133:601–7. 3 Canoui-Poitrine F, Le Thuaut A, Revuz JE et al. Identification of three hidradenitis suppurativa phenotypes: latent class analysis of a cross-sectional study. J Invest Dermatol 2013; 133:1506–11. 4 Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol 2009; 60:539–61. 5 Karvonen SL. Acne fulminans: report of clinical findings and treatment of twenty-four patients. J Am Acad Dermatol 1993; 28:572–9.
(a)
Ultraviolet-induced linear IgA bullous dermatosis: a case report and literature survey DOI: 10.1111/bjd.13154 DEAR EDITOR, Linear IgA bullous dermatosis (LABD) is an autoimmune blistering disorder mediated by IgA autoantibodies against leucocyte adhesion deficiency (LAD)-1, a domain of the BP180 antigen in the basement membrane zone (BMZ).1 Most cases are idiopathic; however, the role of different triggering agents has been well documented, including
(b)
(d)
(c)
(e)
Fig 1. (a–c) Clinical characterization of the patient. Numerous tense vesicles and blisters, some of them showing annular arrangement localized on (a) the forehead, (b) the chest and (c) the upper back. (d, e) Laser scanning confocal microscopy study. Linear IgA deposits (shown in green) are located below laminin-332 (red) (d), and above type IV collagen (red) (e). British Journal of Dermatology (2014) 171, pp1555–1608
© 2014 British Association of Dermatologists
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
negative results, as was recently reported for 10 of 13 cases of EBA with salt-split skin negativity, probably due to a low sensitivity of the technique.7 The aspect and the topography of our patients’ lesions did not support the hypothesis of EPS but could have resulted from the coexistence of two phenomena induced by D-penicillamine: abnormal elastic tissue and autoimmunity against collagen VII as in EBA. However, complete resolution of lesions contrasted with the classical irreversibility of EPS.8 The quick improvement after D-penicillamine withdrawal in our patients is a strong argument for the drug-induced lesions, without any relapse with trientine dihydrochloride. This latter drug, another copper chelator, acts by the renal elimination of copper but does not have any anti-inflammatory direct action that could have explained the improved condition. The immunoblot findings in the patient without clinical manifestations suggest that D-penicillamine could induce a symptomatic or nonsymptomatic anticollagen VII autoimmunity in some patients. However, the role of Wilson disease itself in the pathophysiological aspects of the phenomenon remains to be investigated. A genetic susceptibility could explain the simultaneity of similar lesions in the woman and her brother. Several subtypes of HLA class II are associated with autoimmune diseases. HLADRB1*08 is a susceptibility factor for pemphigus vulgaris,9 but only HLA-DR2 (DRB1*15-16) is known to be associated with EBA.10 Department of Dermatology, 3Department S. INGEN-HOUSZ-ORO1 7 of Pathology and Department of S. GROOTENBOER-MIGNOT2 Immunology, AP-HP, Henri Mondor N. ORTONNE3 Hospital, 51 avenue S. NAHON4 du Marechal de Lattre de Tassigny 94000, J. HORVATH5 Creteil, France C. BERNARDESCHI1 2 Department of Auto-immunity and E. LAFFITTE6 Hypersensitivity, Bichat Hospital, Paris, C . A N D R E7 France S. CHOLLET-MARTIN2 4 Department of Gastro-Enterology, P . W O L K E N S T E I N 1, 8, 9 Montfermeil Hospital, Montfermeil, France O . C H O S I D O W 1, 8, 9 5 Department of Neurology and 6 Clinic of Dermatology, Geneva University Hospitals, Geneva, Switzerland 8 Universite Paris-Est Creteil Val de Marne, Creteil, France 9 INSERM, Centre d’Investigation Clinique 006, AP-HP, Creteil, France Correspondence: Saskia Ingen-Housz-Oro. E-mail: [email protected]
2 Cetkovska P, Pizinger K, Skalova A. Epidermolysis bullosa acquisita-like reaction associated with penicillamine therapy for sclerodermatous graft-versus-host disease. J Am Acad Dermatol 2003; 49:1157–9. 3 Grootenboer-Mignot S, Descamps V, Picard-Dahan C et al. Place of human amniotic membrane immunoblotting in the diagnosis of autoimmune bullous dermatoses. Br J Dermatol 2010; 162:743–50. 4 Bardach H, Gebhart W, Niebauer G. ‘Lumpy-bumpy’ elastic fibers in the skin and lungs of a patient with a penicillamineinduced elastosis perforans serpiginosa. J Cutan Pathol 1979; 6:243–52. 5 Atzori L, Pinna AL, Pau M, Aste N. D-penicillamine elastosis perforans serpiginosa: description of two cases and review of the literature. Dermatol Online J 2011; 17:3. 6 Beer WE, Cooke KB. Epidermolysis bullosa induced by penicillamine. Br J Dermatol 1967; 79:123–5. 7 Terra JB, Jonkman MF, Diercks GFH, Pas HH. Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis. Br J Dermatol 2013; 169:164–7. 8 Carlesimo M, Narcisi A, Cortesi G et al. An 18-year follow-up of a case of D-penicillamine-induced elastosis perforans serpiginosa. Int J Immunopathol Pharmacol 2011; 24:257–9. 9 Yan L, Wang J-M, Zeng K. Association between HLA-DRB1 polymorphisms and pemphigus vulgaris: a meta-analysis. Br J Dermatol 2012; 167:768–77. 10 Zumelzu C, Le Roux-Villet C, Loiseau P et al. Black patients of African descent and HLA-DRB1*15:03 frequency overrepresented in epidermolysis bullosa acquisita. J Invest Dermatol 2011; 131:2386–93.
1
P.W. and O.C. contributed equally to this paper.
References 1 Bialy-Golan A, Brenner S. Penicillamine-induced bullous dermatoses. J Am Acad Dermatol 1996; 35:732–42. British Journal of Dermatology (2014) 171, pp1555–1608
Funding sources: no external funding. Conflicts of interest: none declared.
Hidradenitis suppurativa fulminans: a clinically distinct phenotype? DOI: 10.1111/bjd.13147 DEAR EDITOR, Hidradenitis suppurativa (HS) is a chronic suppurative skin condition characterized by recurrent inflammatory nodules, cysts, abscesses and sinus tracts in apocrine-glandbearing sites. The clinical presentation is highly variable both in terms of the cutaneous features, distribution, presence of complications (e.g. fistula formation, lymphoedema) extracutaneous features (arthritis, interstitial keratitis)1 and associated constitutional symptoms (fever, malaise). Despite this apparent phenotypic heterogeneity it is only recently that attempts have been made to classify the disease. The patient cohort with c-secretase mutations described by Pink et al.2 represents the first genetically defined phenotypic subgroup of HS, but mutations appear rare in the disease population. A recent latent class analysis of over 600 patients initiated the formal process of classifying HS into three distinct clinical phenotypes: © 2014 British Association of Dermatologists
Correspondence 1577
axillary–mammary, follicular and gluteal.3 In this report we document a rare but clinically distinct subset of patients with HS with what we have termed ‘HS fulminans’, and discuss how they relate to this recent phenotypic classification. A small number of patients with HS were observed to present with severe cutaneous disease, extracutaneous manifestations including arthritis and keratitis, and marked constitutional upset associated with disease flares. Medical records of all patients with HS attending tertiary referral clinics at St John’s Institute of Dermatology and King’s College Hospital, London, were examined to identify patients with a similar disease presentation. A total of seven such patients were identified (Table 1). All seven patients were male and of AfroCaribbean descent. The mean age at disease onset was 265 years (range 14–34). Five patients described gradual disease onset prior to a severe and persistent flare of cutaneous disease. All seven patients had Hurley grade 3 disease at presentation, comprising extensive sinus tracts, nodules, abscesses and comedones at involved sites. Four had axillary involvement, six had inguinoscrotal disease and six had gluteal involvement. Two individuals had HS involving atypical sites including the head, neck, upper trunk and lower back. Sartorius scores could not be accurately assessed due to the disease severity and confluent nature of the sinus tracts. Six of the seven patients presented with associated genital lymphoedema. All patients underwent magnetic resonance imaging of the pelvis, which demonstrated anal fistulae in two patients. All seven cases were associated with multisystem inflammation comprising fever or night sweats (n = 6), recent weight loss (n = 4), arthralgia (n = 6) or reactive ocular keratitis (n = 1). Five individuals were diagnosed with seronegative inflammatory arthritis by a rheumatologist (axial disease in two, peripheral oligoarthritis in three) on the basis of typical clinical and radiological findings. Six patients had a raised erythrocyte sedimentation rate (ESR), the average ESR in the group being 899 mm h 1 (range 19–151). Four of the seven patients presented with a microcytic, iron-deficiency anaemia (mean haemoglobin 1025 g dL 1, range 88–115, not measured in two individuals). All individuals had a normal body mass index (mean 241), an unusual finding in HS.4 All patients were resistant to initial medical therapy (single and combination antibiotics in seven, isotretinoin in five, acitretin in three and oral steroids in six) and required treatment with biological therapies (five infliximab, two adalimumab), often in combination with incision and drainage of problematic lesions (in four patients) to achieve control of this severe presentation. We have identified a subset of male Afro-Caribbean patients in whom an explosive onset of severe multifocal disease and genital lymphoedema was associated with systemic involvement characterized by fever, raised inflammatory markers and an inflammatory arthritis. This is similar to acne fulminans, a severe form of acne with acute onset characterized by necrotic skin lesions and systemic features including arthralgia, fever, raised inflammatory markers, leucocytosis and osteolytic bone lesions.5 However, neither a leucocytosis nor osteolytic © 2014 British Association of Dermatologists
Table 1 Characteristics of seven patients with ‘fulminant’ hidradenitis suppurativa (HS) Age at symptom onset (years), mean (range) Male sex Afro-Caribbean racial group Smoker (current) Body mass index (kg m 2), mean (range) Fulminant presentation Explosive disease Fever/night sweats Weight loss Joint symptoms Low haemoglobin (mean 1025 g dL 1) High ESR (mean 899 mm h 1) Distribution of cutaneous disease Axillae Inguinoscrotal Gluteal Lower back Head/neck Upper trunk Other follicular disorders Acne Dissecting cellulitis Local complications Genital lymphoedema Enterocutaneous fistula Other systemic features Inflammatory arthritis Ocular keratitis Low mood Suicidal ideation/intent Family history of HS Family history of other follicular disorder
265 7 7 4 241
(14–34) (100) (100) (57) (227–263)
7 6 4 6 4 6
(100) (86) (57) (86) (57) (86)
4 6 6 1 1 1
(57) (86) (86) (14) (14) (14)
5 (71) 3 (43) 6 (86) 2 (29) 5 1 7 4 3 2
(71) (14) (100) (57) (43) (29)
Values are n (%) unless stated otherwise. ESR, erythrocyte sedimentation rate.
changes were detected in the patients with HS fulminans. The presentation seen in our cohort is not completely consistent with any of the three clinical phenotypes described by Canoui-Poitrine et al.3 The male predominance, disease severity and duration, hypertrophic scarring and association with acne vulgaris would indicate that these patients overlap with, and may form a subgroup within, the ‘follicular’ phenotype. The ability of latent class analysis to detect phenotypic subgroups is dependent on the nature and number of clinical variables that are analysed. We agree with Ingram and Piguet6 that further phenotypic data should be incorporated into future analyses to increase the sensitivity of detection of small phenotypic subgroups such as this. The phenotypic subgroup presented here represents a severe and ‘fulminant’ form of HS. A number of rare phenotypes have now been reported to comprise features of HS including PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, suppurative hidradenitis),7 PASH (pyoderma gangrenosum, acne, suppurative hidradenitis),8 SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis)1 and HS and keratitis,1 which need to be considered in the evolving disease classificaBritish Journal of Dermatology (2014) 171, pp1555–1608
1578 Correspondence 6 Ingram JR, Piguet V. Phenotypic heterogeneity in hidradenitis suppurativa (acne inversa): classification is an essential step toward personalized therapy. J Invest Dermatol 2013; 133:1453–6. 7 Marzano AV, Trevisan V, Gattorno M et al. Pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH): a new autoinflammatory syndrome associated with a novel mutation of the PSTPIP1 gene. JAMA Dermatol 2013; 149:762–4. 8 Braun-Falco M, Kovnerystyy O, Lohse P, Ruzicka T. Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) – a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol 2012; 66:409–15.
tion. We believe a multidisciplinary approach encompassing genetics, immunology, histopathology and clinically led research will expedite refinement of the current phenotypic classification of HS. 1
Department of Dermatology, King’s College Hospital, Denmark Hill, London SE5 9RS, U.K. 2 St John’s Institute of Dermatology, Guy’s and St Thomas’ NHS Foundation Trust, London, U.K. E-mail: [email protected]
B. MORIARTY1 A. PINK2 D. CREAMER1 N. DESAI2
Funding sources: none. Conflicts of interest: none declared.
References 1 Alzaga Fernandez AG, Demirci H, Darnley-Fisch DA, Steen DW. Interstitial keratitis secondary to severe hidradenitis suppurativa: a case report and literature review. Cornea 2010; 29:1189–91. 2 Pink AE, Simpson MA, Desai N et al. c-Secretase mutations in hidradenitis suppurativa: new insights into disease pathogenesis. J Invest Dermatol 2013; 133:601–7. 3 Canoui-Poitrine F, Le Thuaut A, Revuz JE et al. Identification of three hidradenitis suppurativa phenotypes: latent class analysis of a cross-sectional study. J Invest Dermatol 2013; 133:1506–11. 4 Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol 2009; 60:539–61. 5 Karvonen SL. Acne fulminans: report of clinical findings and treatment of twenty-four patients. J Am Acad Dermatol 1993; 28:572–9.
(a)
Ultraviolet-induced linear IgA bullous dermatosis: a case report and literature survey DOI: 10.1111/bjd.13154 DEAR EDITOR, Linear IgA bullous dermatosis (LABD) is an autoimmune blistering disorder mediated by IgA autoantibodies against leucocyte adhesion deficiency (LAD)-1, a domain of the BP180 antigen in the basement membrane zone (BMZ).1 Most cases are idiopathic; however, the role of different triggering agents has been well documented, including
(b)
(d)
(c)
(e)
Fig 1. (a–c) Clinical characterization of the patient. Numerous tense vesicles and blisters, some of them showing annular arrangement localized on (a) the forehead, (b) the chest and (c) the upper back. (d, e) Laser scanning confocal microscopy study. Linear IgA deposits (shown in green) are located below laminin-332 (red) (d), and above type IV collagen (red) (e). British Journal of Dermatology (2014) 171, pp1555–1608
© 2014 British Association of Dermatologists
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
