Letters to the Editor
could have improved clinical outcome, as has also been reported for Mycobacterium tuberculosis infection.6,7 We decided to continue treatment in our patient as the CD was still active and we did not risk a deterioration of symptoms for the patient. During the treatment with minocycline, we only observed a slight improvement, which stands in line with literature. In many reports, treatment failure was reported under tetracycline therapy as doxycycline or minocycline. After changing the therapy to clarithromycin, we saw a fast healing of the lesions and the immunosuppressive treatment had not to be stopped. Maybe in other cases, the treatment failure under immunosuppressive treatment could be explained by the use of insufficient agents such as tetracyclines and a change of antibiotic agents rather than a discontinuation in the immunosuppressive therapy should have been considered. However, clinical trials are needed to further investigate this point. D. Belz,1,* I. Tantcheva-Poor,1 H. Rasokat,1 M. Fabri,1,2 M. Schlaak1
515
confused with dissecting cellulitis as it develops into a suppurative folliculitis and granulomatous dermatitis with mixed inflammatory infiltrate, alopecia and negative PAS.1 There are six cases of TC mimicking dissecting cellulitis in the literature.2– 6 We present three additional cases to discuss diagnostic challenges and describe the role of trichoscopy in diagnosis. A 14-year-old African American girl presented to an outside dermatologist with a 3-month history of erythema, boggy induration, serosanguinous drainage and alopecia involving the vertex of the scalp. Biopsy showed dense, mixed lymphoplasmacytic and neutrophilic infiltrate with negative PAS consistent with dissecting cellulitis. After 2 months of doxycicline 200 mg daily and clobetasol 0.01% foam, she presented to our clinic with persistence of tender scalp nodules, scalp erythema, severe alopecia, pustular lesions and cervical lymphadenopathy (Fig. 1a). Trichoscopy showed numerous comma and corkscrew hairs
(a)
1
Department of Dermatology, 2Center for Molecular Medicine, University of Cologne, Cologne, Germany *Correspondence: D. Belz. E-mail: [email protected]
References 1 Huang Y, Xu X, Liu Y et al. Successful treatment of refractory cutaneous infection caused by mycobacterium marinum with a combined regimen containing amikacin. Clin Interv Aging 2012; 7: 533–538. 2 Fabricius S, Fogh H, Jemec GB, Baslund B, Agner T. Widespread mycobacterium marinum infection. Acta Derm Venereol 2009; 89: 91–92. 3 Cheung JP, Fung B, Ip WY, Chow SP. Review article: Mycobacterium marinum infection of the hand and wrist. J Orthop Surg (Hong Kong) 2012; 20: 214–218. 4 Nenhoff P, Klapper BM, Mayser P, Paasch U, Handrick W. Kutane Infektionen durch Mycobacterium marinum. Der Hautarzt 2011; 62: 266–271. 5 Garzoni C, Adler S, Boller C, Furrer H, Villiger PM. Possible role of antiTNF monoclonal antibodies in the treatment of Mycobacterium marinum infection. Rheumatology (Oxford) 2010; 49: 1991–1993. 6 Wallis RS, Kyambadde P, Johnson JL et al. A study of the safety, immunology, virology, and microbiology of adjunctive etanercept in HIV-1-associated tuberculosis. AIDS 2004; 18: 257–264. 7 Wallis RS, Van VC, Potgieter S. Adalimumab treatment of life-threatening tuberculosis. Clin Infect Dis 2009; 48: 1429–1432.
(b)
DOI: 10.1111/jdv.12911
Pitfalls in the diagnosis of kerion Editor Tinea capitis (TC) is a dermatophytosis of variable clinical presentation, from superficial, non-inflammatory fungal infection to inflammatory kerion. There are four distinct subtypes of kerion: type 1 and 2 are predominantly periodic acid-Schiff (PAS) positive and type 3 and 4 are PAS negative.1 Type 3 can be
JEADV 2016, 30, 446–556
Figure 1 (a) Affected scalp at presentation to our clinic after receiving outside treatment for dissecting cellulitis. (b) Trichoscopy showing numerous comma and corkscrew hairs.
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
516
(a)
(b)
Figure 2 (a) Horizontal sections at level of infundibulum showing diffuse dense mixed-cell inflammation in the dermis (Haematoxylin&Eosin, 94). (b) Horizontal sections at level of low follicle in the subdermis showing diffuse dense mixed-cell inflammation (Haematoxylin&Eosin, 94).
(Fig. 1b). Diagnosed with probable TC, she began terbinafine 250 mg daily for 6 weeks. Culture grew Trichophyton species. Two months later, inflammatory changes resolved, but some areas of scarring alopecia remained. A 15-year-old Caucasian boy with a 5-year history of patchy alopecia and scalp pruritus, presented with an erythematous patch on the parietal scalp with superficial scale and erosions. Trichoscopy showed severe inflammation and comma hairs. Biopsy showed dense inflammatory infiltrate with predominant eosinophils around the follicles in the reticular dermis and adipose tissue. Fungal stains were negative. Despite negative KOH and fungal culture, the patient was treated with itraconazole 200 mg daily based on dermoscopic findings. At 1-month follow-up, inflammatory changes resolved, though the alopecic patch showed no regrowth. A 68-year-old Caucasian woman presented with a 3-month history of scalp pruritus and painful scalp nodules unimproved after a 1-month course of doxycycline and topical steroids. Trichoscopy showed diffuse redness and pustules. Biopsy showed diffuse, dense, mixed-cell inflammation filling the entire dermis and subdermis and increased number of telogen follicles (Fig 2a,2b). Both PAS and Grocott’s methenamine silver (GMS) stains for fungal organisms were negative. KOH was negative, but fungal culture grew Trichophyton species. After a course of terbinafine 250 mg for 6 weeks, the patient achieved complete resolution. Inflammatory TC is rare, however, prompt diagnosis can prevent scarring alopecia. Some subtypes of kerion are misdiagnosed due to clinical and histological similarities to dissecting cellulitis. Differential diagnosis is particularly difficult in patients of African descent. Our three cases underline the difficulties encountered and the importance of utilizing multiple diagnostic tools, including trichoscopy and mycology. In fact, pathology, which is an important tool in the diagnosis of fungal infection, fails to indentify fungi in some cases of kerion,1,4,6 including our three cases. On pathology,
JEADV 2016, 30, 446–556
a diagnostic clue is that inflammation shows dense foci of suppurative folliculitis, affecting not only the deep but also the superficial dermis,2 which is usually spared in dissecting cellulitis. Although KOH and cultures are the gold standard for diagnosis of TC, mycology was negative in one of our patients, possibly due to suboptimal sample. Trichoscopy is useful for rapid diagnosis of TC, even though it is often negative in typical kerion (unpublished data). The presence of comma and corkscrew hairs are characteristic of TC and may serve as diagnostic markers.7,8 Biopsy should be taken from comma hairs identified on trichoscopy, preferably outside of the boggy inflamed areas. In two of our patients, empiric treatment was initiated based on dermoscopic evidence of comma hairs even before fungal culture results were known. C.E. LaSenna,1,* M. Miteva,2 A. Tosti2 1
University of Miami Miller School of Medicine, 2Department of Dermatology and Cutaneous Surgery, University of Miami, Miami, FL, USA *Correspondence: C.E. LaSenna. E-mail: [email protected]
References 1 Isa-Isa R, Arenas R, Isa M. Inflammatory tinea capitis: kerion, dermatophytic granuloma, and mycetoma. Clin Dermatol 2010; 28: 133–136. 2 Miletta NR, Schwartz C, Sperling L. Tinea capitis mimicking dissecting cellulitis of the scalp: a histopathologic pitfall when evaluating alopecia in the post-pubertal patient. J Cutan Pathol 2014; 41: 2–4. 3 Stein LL, Adams EG, Holcomb KZ. Inflammatory tinea capitis mimicking dissecting cellulitis in a postpubertal male: a case report and review of literature. Mycoses 2013; 56: 596–600. 4 Torok RD, Bellet JS. Tinea capitis mimicking dissecting cellulitis. Pediatr Dermatol 2013; 30: 753–754. 5 Twersky JM, Sheth AP. Tinea capitis mimicking dissecting cellulites: a distinct variant. Int J Dermatol 2005; 44: 412. 6 Sperling LC. Inflammatory tinea capitis (kerion) mimicking dissecting cellulitis: occurrence in two adolescents. Int J Dermatol 1991; 30: 190. 7 Slowinska M, Rudnicka L, Schwartz RA et al. Comma hairs: a dermatoscopic marker for tinea capitis. J Am Acad Dermatol 2008; 59: S77– S79.
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
517
8 Hughes R, Chiaverini C, Bahadoran P, Lacour JP. Corkscrew hair: a new dermoscopic sign for diagnosis of tinea capitis in black children. Arch Dermatol 2011; 147: 355–356. DOI: 10.1111/jdv.12912
High prevalence of cryofibrinogenaemia in dermatitis herpetiformis Editor Cryofibrinogenaemia (CFE) is an under-recognized condition in which a precipitate (cryofibrinogen) is formed in cold (4 °C) plasma but not in serum. CFE has a prevalence of 0–7% in healthy population, and 8–13% among hospitalized patients. The cryoprecipitate mainly consists of fibrinogen, fibrin, fibronectin and factor VIII.1,2 CFE can be primary or secondary to various conditions, e.g. autoimmune disorders, malignancies or infections. CFE may exist asymptomatically, but skin findings such as acral purpuras, cold intolerance, Raynaud phenomenon may develop.3,4 Dermatitis herpetiformis (DH) is mostly the skin manifestation of gluten sensitive enteropathy with transglutaminase-2 and 3 (TG3) autoimmunity, but it has not been recognized as a CFE associated disease. Here, we studied DH patients for CFE, because (i) fibrin, fibrinogen, fibronectin, TG3 and IgA co-localize in the papillary dermis in early DH lesion5–7; (ii) acral purpura is a common symptom in DH; (iii) efficacy of high-dose heparin, as solely treatment in DH has been reported8 and (iv) dapsone is effective in DH, as well as in CFE associated skin diseases.9 Here, 88 DH patients (60 men and 28 women, mean age 36.5 17.4) have been screened for the presence of cryoproteins regardless of their clinical and serological activity markers. Cryofibrinogen and serum cryoglobulin was assessed qualitatively in accordance with standard protocols.3,4 We retrospectively also analysed data of 233 non-DH patients as controls (56
men and 177 women, mean age 52.9 17.4) with suspected CFE and cryoglobulinaemia (CGE), who had Raynaud phenomenon, systemic lupus erythematodes, dermatomyositis, cold urticaria, acral ulceration, livedo reticularis and different forms of vasculitis. The prevalence of isolated CFE, isolated CGE and CFE with CGE is summarized in Table 1. Among unselected DH patients, the prevalence of isolated CFE was unexpectedly high, reaching 48.9% (43/88), compared to 27.5% (64/233) measured in the controls. Contrary, isolated CGE was present in 2.3% (2/ 88) of DH patients, but in 9.4% (22/233) of controls. The prevalence of mixed CFE and CGE showed similar values in DH and non-DH patients with 9.1% (8/88) and 6.9% (16/233) respectively. We further analysed the presence of isolated CFE in DH patients under normal diet without dapsone (DPS) treatment (n = 55), under gluten-free diet (GFD) without DPS (n = 25) and under GFD with concomitant DPS treatment (n = 8). Isolated CFE was detectable in 33/55 (60%) patients under normal diet without DPS, whereas in patients already on GFD but no DPS CFE was present in 10/25 (40%). In the group of patients on GFD and concomitant DPS treatment CFE was not detectable in the eight studied patients (Table 1). These data clearly show the unusually high prevalence of isolated CFE among 88 DH patients. The highest rate of CFE (60%) was detected in the group of untreated patients, it decreased under GFD (40%) and was not detected in patients under GFD and dapsone treatment. Interestingly, earlier we also observed the reduction in cryofibrinogen level in dapsone treated, non-DH patients with CFE.9 Although this study has limitations mainly due to the relatively small group sizes, it provides evidence of a so far unrecognized association between CFE and DH. These data suggest that CFE may be at least partially responsible for the acral purpuras in DH, and may indicate a new way of dapsone efficacy in the disease.
Financial disclosure No other author has a financial or proprietary interest in any material or method mentioned.
Table 1 Presence of cryofibrinogenaemia, cryoglobulinaemia or both in dermatitis herpetiformis and control patients (GFD = gluten free diet, DPS = dapsone) Dermatitis herpetiformis patients
Group (n=)
Cryofibrinogen+
Cryoglobulin+
Cryofibrinogen+Cryoglobulin+
Non selected
88
43 (48.9%)
2 (2.3%)
8 (9.1%)
No GFD no DPS
55
33 (60%)
0 (0%)
4 (7.27%)
GFD no DPS
25
10 (40%)
2 (8%)
2 (8%)
GFD + DPS
8
0 (0%)
0 (0%)
2 (25%)
22 (9.4%)
16 (6.9%)
Control patients
JEADV 2016, 30, 446–556
233
64 (27.5%)
© 2014 European Academy of Dermatology and Venereology
could have improved clinical outcome, as has also been reported for Mycobacterium tuberculosis infection.6,7 We decided to continue treatment in our patient as the CD was still active and we did not risk a deterioration of symptoms for the patient. During the treatment with minocycline, we only observed a slight improvement, which stands in line with literature. In many reports, treatment failure was reported under tetracycline therapy as doxycycline or minocycline. After changing the therapy to clarithromycin, we saw a fast healing of the lesions and the immunosuppressive treatment had not to be stopped. Maybe in other cases, the treatment failure under immunosuppressive treatment could be explained by the use of insufficient agents such as tetracyclines and a change of antibiotic agents rather than a discontinuation in the immunosuppressive therapy should have been considered. However, clinical trials are needed to further investigate this point. D. Belz,1,* I. Tantcheva-Poor,1 H. Rasokat,1 M. Fabri,1,2 M. Schlaak1
515
confused with dissecting cellulitis as it develops into a suppurative folliculitis and granulomatous dermatitis with mixed inflammatory infiltrate, alopecia and negative PAS.1 There are six cases of TC mimicking dissecting cellulitis in the literature.2– 6 We present three additional cases to discuss diagnostic challenges and describe the role of trichoscopy in diagnosis. A 14-year-old African American girl presented to an outside dermatologist with a 3-month history of erythema, boggy induration, serosanguinous drainage and alopecia involving the vertex of the scalp. Biopsy showed dense, mixed lymphoplasmacytic and neutrophilic infiltrate with negative PAS consistent with dissecting cellulitis. After 2 months of doxycicline 200 mg daily and clobetasol 0.01% foam, she presented to our clinic with persistence of tender scalp nodules, scalp erythema, severe alopecia, pustular lesions and cervical lymphadenopathy (Fig. 1a). Trichoscopy showed numerous comma and corkscrew hairs
(a)
1
Department of Dermatology, 2Center for Molecular Medicine, University of Cologne, Cologne, Germany *Correspondence: D. Belz. E-mail: [email protected]
References 1 Huang Y, Xu X, Liu Y et al. Successful treatment of refractory cutaneous infection caused by mycobacterium marinum with a combined regimen containing amikacin. Clin Interv Aging 2012; 7: 533–538. 2 Fabricius S, Fogh H, Jemec GB, Baslund B, Agner T. Widespread mycobacterium marinum infection. Acta Derm Venereol 2009; 89: 91–92. 3 Cheung JP, Fung B, Ip WY, Chow SP. Review article: Mycobacterium marinum infection of the hand and wrist. J Orthop Surg (Hong Kong) 2012; 20: 214–218. 4 Nenhoff P, Klapper BM, Mayser P, Paasch U, Handrick W. Kutane Infektionen durch Mycobacterium marinum. Der Hautarzt 2011; 62: 266–271. 5 Garzoni C, Adler S, Boller C, Furrer H, Villiger PM. Possible role of antiTNF monoclonal antibodies in the treatment of Mycobacterium marinum infection. Rheumatology (Oxford) 2010; 49: 1991–1993. 6 Wallis RS, Kyambadde P, Johnson JL et al. A study of the safety, immunology, virology, and microbiology of adjunctive etanercept in HIV-1-associated tuberculosis. AIDS 2004; 18: 257–264. 7 Wallis RS, Van VC, Potgieter S. Adalimumab treatment of life-threatening tuberculosis. Clin Infect Dis 2009; 48: 1429–1432.
(b)
DOI: 10.1111/jdv.12911
Pitfalls in the diagnosis of kerion Editor Tinea capitis (TC) is a dermatophytosis of variable clinical presentation, from superficial, non-inflammatory fungal infection to inflammatory kerion. There are four distinct subtypes of kerion: type 1 and 2 are predominantly periodic acid-Schiff (PAS) positive and type 3 and 4 are PAS negative.1 Type 3 can be
JEADV 2016, 30, 446–556
Figure 1 (a) Affected scalp at presentation to our clinic after receiving outside treatment for dissecting cellulitis. (b) Trichoscopy showing numerous comma and corkscrew hairs.
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
516
(a)
(b)
Figure 2 (a) Horizontal sections at level of infundibulum showing diffuse dense mixed-cell inflammation in the dermis (Haematoxylin&Eosin, 94). (b) Horizontal sections at level of low follicle in the subdermis showing diffuse dense mixed-cell inflammation (Haematoxylin&Eosin, 94).
(Fig. 1b). Diagnosed with probable TC, she began terbinafine 250 mg daily for 6 weeks. Culture grew Trichophyton species. Two months later, inflammatory changes resolved, but some areas of scarring alopecia remained. A 15-year-old Caucasian boy with a 5-year history of patchy alopecia and scalp pruritus, presented with an erythematous patch on the parietal scalp with superficial scale and erosions. Trichoscopy showed severe inflammation and comma hairs. Biopsy showed dense inflammatory infiltrate with predominant eosinophils around the follicles in the reticular dermis and adipose tissue. Fungal stains were negative. Despite negative KOH and fungal culture, the patient was treated with itraconazole 200 mg daily based on dermoscopic findings. At 1-month follow-up, inflammatory changes resolved, though the alopecic patch showed no regrowth. A 68-year-old Caucasian woman presented with a 3-month history of scalp pruritus and painful scalp nodules unimproved after a 1-month course of doxycycline and topical steroids. Trichoscopy showed diffuse redness and pustules. Biopsy showed diffuse, dense, mixed-cell inflammation filling the entire dermis and subdermis and increased number of telogen follicles (Fig 2a,2b). Both PAS and Grocott’s methenamine silver (GMS) stains for fungal organisms were negative. KOH was negative, but fungal culture grew Trichophyton species. After a course of terbinafine 250 mg for 6 weeks, the patient achieved complete resolution. Inflammatory TC is rare, however, prompt diagnosis can prevent scarring alopecia. Some subtypes of kerion are misdiagnosed due to clinical and histological similarities to dissecting cellulitis. Differential diagnosis is particularly difficult in patients of African descent. Our three cases underline the difficulties encountered and the importance of utilizing multiple diagnostic tools, including trichoscopy and mycology. In fact, pathology, which is an important tool in the diagnosis of fungal infection, fails to indentify fungi in some cases of kerion,1,4,6 including our three cases. On pathology,
JEADV 2016, 30, 446–556
a diagnostic clue is that inflammation shows dense foci of suppurative folliculitis, affecting not only the deep but also the superficial dermis,2 which is usually spared in dissecting cellulitis. Although KOH and cultures are the gold standard for diagnosis of TC, mycology was negative in one of our patients, possibly due to suboptimal sample. Trichoscopy is useful for rapid diagnosis of TC, even though it is often negative in typical kerion (unpublished data). The presence of comma and corkscrew hairs are characteristic of TC and may serve as diagnostic markers.7,8 Biopsy should be taken from comma hairs identified on trichoscopy, preferably outside of the boggy inflamed areas. In two of our patients, empiric treatment was initiated based on dermoscopic evidence of comma hairs even before fungal culture results were known. C.E. LaSenna,1,* M. Miteva,2 A. Tosti2 1
University of Miami Miller School of Medicine, 2Department of Dermatology and Cutaneous Surgery, University of Miami, Miami, FL, USA *Correspondence: C.E. LaSenna. E-mail: [email protected]
References 1 Isa-Isa R, Arenas R, Isa M. Inflammatory tinea capitis: kerion, dermatophytic granuloma, and mycetoma. Clin Dermatol 2010; 28: 133–136. 2 Miletta NR, Schwartz C, Sperling L. Tinea capitis mimicking dissecting cellulitis of the scalp: a histopathologic pitfall when evaluating alopecia in the post-pubertal patient. J Cutan Pathol 2014; 41: 2–4. 3 Stein LL, Adams EG, Holcomb KZ. Inflammatory tinea capitis mimicking dissecting cellulitis in a postpubertal male: a case report and review of literature. Mycoses 2013; 56: 596–600. 4 Torok RD, Bellet JS. Tinea capitis mimicking dissecting cellulitis. Pediatr Dermatol 2013; 30: 753–754. 5 Twersky JM, Sheth AP. Tinea capitis mimicking dissecting cellulites: a distinct variant. Int J Dermatol 2005; 44: 412. 6 Sperling LC. Inflammatory tinea capitis (kerion) mimicking dissecting cellulitis: occurrence in two adolescents. Int J Dermatol 1991; 30: 190. 7 Slowinska M, Rudnicka L, Schwartz RA et al. Comma hairs: a dermatoscopic marker for tinea capitis. J Am Acad Dermatol 2008; 59: S77– S79.
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
517
8 Hughes R, Chiaverini C, Bahadoran P, Lacour JP. Corkscrew hair: a new dermoscopic sign for diagnosis of tinea capitis in black children. Arch Dermatol 2011; 147: 355–356. DOI: 10.1111/jdv.12912
High prevalence of cryofibrinogenaemia in dermatitis herpetiformis Editor Cryofibrinogenaemia (CFE) is an under-recognized condition in which a precipitate (cryofibrinogen) is formed in cold (4 °C) plasma but not in serum. CFE has a prevalence of 0–7% in healthy population, and 8–13% among hospitalized patients. The cryoprecipitate mainly consists of fibrinogen, fibrin, fibronectin and factor VIII.1,2 CFE can be primary or secondary to various conditions, e.g. autoimmune disorders, malignancies or infections. CFE may exist asymptomatically, but skin findings such as acral purpuras, cold intolerance, Raynaud phenomenon may develop.3,4 Dermatitis herpetiformis (DH) is mostly the skin manifestation of gluten sensitive enteropathy with transglutaminase-2 and 3 (TG3) autoimmunity, but it has not been recognized as a CFE associated disease. Here, we studied DH patients for CFE, because (i) fibrin, fibrinogen, fibronectin, TG3 and IgA co-localize in the papillary dermis in early DH lesion5–7; (ii) acral purpura is a common symptom in DH; (iii) efficacy of high-dose heparin, as solely treatment in DH has been reported8 and (iv) dapsone is effective in DH, as well as in CFE associated skin diseases.9 Here, 88 DH patients (60 men and 28 women, mean age 36.5 17.4) have been screened for the presence of cryoproteins regardless of their clinical and serological activity markers. Cryofibrinogen and serum cryoglobulin was assessed qualitatively in accordance with standard protocols.3,4 We retrospectively also analysed data of 233 non-DH patients as controls (56
men and 177 women, mean age 52.9 17.4) with suspected CFE and cryoglobulinaemia (CGE), who had Raynaud phenomenon, systemic lupus erythematodes, dermatomyositis, cold urticaria, acral ulceration, livedo reticularis and different forms of vasculitis. The prevalence of isolated CFE, isolated CGE and CFE with CGE is summarized in Table 1. Among unselected DH patients, the prevalence of isolated CFE was unexpectedly high, reaching 48.9% (43/88), compared to 27.5% (64/233) measured in the controls. Contrary, isolated CGE was present in 2.3% (2/ 88) of DH patients, but in 9.4% (22/233) of controls. The prevalence of mixed CFE and CGE showed similar values in DH and non-DH patients with 9.1% (8/88) and 6.9% (16/233) respectively. We further analysed the presence of isolated CFE in DH patients under normal diet without dapsone (DPS) treatment (n = 55), under gluten-free diet (GFD) without DPS (n = 25) and under GFD with concomitant DPS treatment (n = 8). Isolated CFE was detectable in 33/55 (60%) patients under normal diet without DPS, whereas in patients already on GFD but no DPS CFE was present in 10/25 (40%). In the group of patients on GFD and concomitant DPS treatment CFE was not detectable in the eight studied patients (Table 1). These data clearly show the unusually high prevalence of isolated CFE among 88 DH patients. The highest rate of CFE (60%) was detected in the group of untreated patients, it decreased under GFD (40%) and was not detected in patients under GFD and dapsone treatment. Interestingly, earlier we also observed the reduction in cryofibrinogen level in dapsone treated, non-DH patients with CFE.9 Although this study has limitations mainly due to the relatively small group sizes, it provides evidence of a so far unrecognized association between CFE and DH. These data suggest that CFE may be at least partially responsible for the acral purpuras in DH, and may indicate a new way of dapsone efficacy in the disease.
Financial disclosure No other author has a financial or proprietary interest in any material or method mentioned.
Table 1 Presence of cryofibrinogenaemia, cryoglobulinaemia or both in dermatitis herpetiformis and control patients (GFD = gluten free diet, DPS = dapsone) Dermatitis herpetiformis patients
Group (n=)
Cryofibrinogen+
Cryoglobulin+
Cryofibrinogen+Cryoglobulin+
Non selected
88
43 (48.9%)
2 (2.3%)
8 (9.1%)
No GFD no DPS
55
33 (60%)
0 (0%)
4 (7.27%)
GFD no DPS
25
10 (40%)
2 (8%)
2 (8%)
GFD + DPS
8
0 (0%)
0 (0%)
2 (25%)
22 (9.4%)
16 (6.9%)
Control patients
JEADV 2016, 30, 446–556
233
64 (27.5%)
© 2014 European Academy of Dermatology and Venereology
