10.5826/dpc.1202a77
text/xml
2160-9381
1909
en
Nilgun Atakan
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
salt-split skin test
Vesiculobullous Erythema Elevatum Diutinum: A Rare Variant with Epidermolysis Bullosa Acquisita - like Immunofluorescence Findings
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-05-02
2021-07-28
2022-05-02
2022-05-24
e2022077
Dermatol Pract Concept
Nilgun Atakan
2022/05/02
10.5826/dpc.1202a77
e2022077
Dermatology and Venereology, Private Clinic
2160-9381
salt-split skin test
en
e2022077
Aftab MN, Dee A, Helm TN. Erythema elevatum diutinum arising in the setting of dermatitis herpetiformis. Cutis. 2006;78(2):129-132. PMID: 16983903.
Vesiculobullous Erythema Elevatum Diutinum: A Rare Variant with Epidermolysis Bullosa Acquisita - like Immunofluorescence Findings
https://dpcj.org/index.php/dpc/article/download/1909/1598
        Vesiculobullous Erythema Elevatum Diutinum:
          a Rare Variant With Epidermolysis Bullosa
         Acquisita-like Immunofluorescence Findings
            Basak Yalici-Armagan1, Deniz Ates-Ozdemir2, Gökçe Yeter2, Nilgun Atakan3


1 Department of Dermatology and Venereology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
2 Department of Pathology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
3 Dermatology and Venereology, Private Clinic, Ankara, Turkey



Key words: Direct immunofluorescence, Epidermolysis bullosa acquisita, Erythema elevatum diutinum, Salt-split skin test
Citation: Yalici-Armagan B, Ates-Ozdemir D, Yeter G, Atakan N. Vesiculobullous Erythema Elevatum Diutinum: A Rare Variant with
Epidermolysis Bullosa Acquisita-like Immunofluorescence Findings. Dermatol Pract Concept. 2022;12(2):e2022077. DOI: https://doi.
org/10.5826/dpc.1202a77
Accepted: October 4, 2021; Published: April 2022
Copyright: ©2022 Yalici-Armagan et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Basak Yalici-Armagan, MD, Department of Dermatology and Venereology, Hacettepe University, Faculty of
Medicine, Ankara, Turkey. E-mail: basakarmagan@gmail.com




Introduction                                                             dorsal region of hands. Hematoxylin and eosin staining from
                                                                         2 punch biopsies of the foot and knee showed nodular and
Erythema elevatum diutinum (EED) is a rare, chronic der-                 diffuse inflammation rich in neutrophils under uninvolved
matosis characterized by red–violet to red–brown papules,                epidermis (Figure 2A). Small vessels are damaged by neutro-
plaques, and nodules that favor extensor surfaces. Vesiculob-            phil rich inflammation and leukocytoclastic debri, resulting
ullous variant is a rare form of EED [1]. Herein, we present             in leukocytoclastic vasculitis (LCV) (Figure 2B) and stori-
a case of vesiculobullous EED with epidermolysis bullosa                 form fibrosis at the dermis are consistent with EED. The
acquisita (EBA)-like with direct immunofluorescence (DIF)                biopsy of the hand dorsum demonstrated subepidermal sepa-
findings.                                                                ration with perivascular and interstitial inflammation rich in
                                                                         eosinophils with fibrin accumulation in the dermis (Figure 2,
                                                                         C and D). DIF from perilesional skin of the hand revealed
Case Presentation
                                                                         linear deposition of immunoglobulins (IgG, IgA, IgM) and
A 65-year-old man presented with progressively increasing                complement (C3) along the basal membrane zone (BMZ).
nodules on feet for 5 years, pruritic papules on knees and               Location of deposits were on the floor of the blister in salt-
elbows for 1 year (Figure1, A–E) and blisters on hands for               split skin test with IgG (Figure 2E). Full blood count showed
1 month. Dermatological examination revealed violaceous                  iron deficiency anemia. Serology tests for HIV and hepa-
nodules on feet, erythematous flat-topped papules on el-                 titis-B and C were negative. Dapsone was initiated 50 mg
bows and knees with multiple tense blisters on palmar and                twice daily. At 5 weeks follow-up, a significant improvement



Research Letter | Dermatol Pract Concept. 2022;12(2):e2022077                                                                            1
    Figure 1. (A-E) Multiple tense blisters on palmar and dorsal region of hands (A,B); erythematous flat-topped papules on elbows and
    knees (C,D) and violaceous nodules on feet (E), improving of the vesiculobullous and papular lesions located on the hands (F,G) and
    knees (H).




     Figure 2. (A-E) Diffuse dense inflammation composed of predominantly neutrophils and dermal fibrosis are observed under un-
     involved epidermis. (A) Fibrin deposition is present around dermal vessels (H&E, 40x magnification). (B) Endothelial swelling,
     leukocytoclastic debri and fibrin deposition are visible in dermal vessels (H&E, 200x magnification). (C) Subepidermal separation is
     seen with fibrin deposition and mild to moderate perivascular and interstitial dermal inflammation (H&E, 40x magnification). (D)
     Subepidermal separation and mild to moderate inflammation are present with eosinophils (H&E, 200x magnification). (E) Linear
     IgG deposition is observed at the base of the split cavity (direct immunofluorescence with Ig G in salt split skin, 200x magnification).


2                                                                               Research Letter | Dermatol Pract Concept. 2022;12(2):e2022077
was observed in hands, knees and elbows (Figure 1, F-H),           with none or little inflammation depending on the clinical
whereas there was a partial improvement in feet.                   subtype. While dapsone is an effective treatment option for
                                                                   EED, EBA is usually refractory to many systemic agents in-
                                                                   cluding dapsone.
Discussion
EED is a rare skin disorder that is associated with a variety
                                                                   Conclusions
of systemic diseases. Histopathologically, it is characterized
by early changes of LCV with an infiltrate of polymorphonu-        Dermal inflammation with eosinophils and fibrin deposition
clear cells, occasionally eosinophils and deposition of fibrin     in histopathologic examination and dramatic response to
which resolves with fibrosis. The association of EED and au-       dapsone therapy suggested the diagnosis of vesiculobullous
toimmune bullous diseases, such as dermatitis herpetiformis        EED rather than EBA in the current case. The present novel
(DH) has been described previously [2]. Recently, it is argued     DIF and salt-split skin test findings expand the clinicopatho-
that these cases may be more compatible with vesiculobul-          logical spectrum of vesiculobullous EED.
lous EED than with DH. Although, perivascular deposition
of IgG, IgA, IgM, complement, and fibrin has been demon-
                                                                   References
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case of vesiculobullous EED [1]. To our knowledge, this is            Salamanca-Sánchez M, Linares-Barrios M. Vesiculobullous
                                                                      variant of erythema elevatum diutinum. Clin Exp Derma-
the first case of vesiculobullous EED with linear IgA, IgG,
                                                                      tol. 2017;42(7):777-780. DOI: 10.1111/ced.13166.. PMID:
IgM and C3 deposition in BMZ. The presence of the de-
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posits in the base of the bulla in salt-split skin test was also   2. Aftab MN, Dee A, Helm TN. Erythema elevatum diutinum arising
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pitfall for EBA in the differential diagnosis. Histopathologi-        132. PMID: 16983903.
cal findings of EBA include subepidermal blister formation




Research Letter | Dermatol Pract Concept. 2022;12(2):e2022077                                                                         3
