10.5826/dpc.1304a218
text/xml
2160-9381
3192
en
Cheng Tan
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Epidermolysis bullosa pruriginosa; blister; pruritus; scar; flagellate
Widespread Pruriginous Lesions with Flagellate Scarring
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Image Letter
2023-10-31
2023-01-29
2023-10-31
2024-01-15
e2023218
Dermatol Pract Concept
Cheng Tan
2023/10/31
10.5826/dpc.1304a218
e2023218
Department of Dermatology, Affiliated Hospital of Nanjing University of Chinese Medicine,Nanjing, China
2160-9381
Epidermolysis bullosa pruriginosa; blister; pruritus; scar; flagellate
en
e2023218
Clawson RC, Duran SF, Pariser RJ: Epidermolysis bullosa pruriginosa responding to dupilumab. JAAD Case Rep 2021, 16:69-71.1.doi: 10.1016/j.jdcr.2021.07.036.
Widespread Pruriginous Lesions with Flagellate Scarring
https://dpcj.org/index.php/dpc/article/download/3192/2361
                          Wide-spread Pruriginous Lesions
                             with Flagellate Scarring
                                                       He Fu1, Cheng Tan1


1 Department of Dermatology, Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, China



Citation: Fu H, Tan C. Wide-spread Pruriginous Lesions with Flagellate Scarring. Dermatol Pract Concept. 2023;13(4):e2023218.
DOI: https://doi.org/10.5826/dpc.1304a218
Accepted: April 13, 2023; Published: October 2023
Copyright: ©2023 Fu 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: Cheng Tan, MD, Department of Dermatology, Affiliated Hospital of Nanjing University of Chinese Medicine,
155 Hanzhong Road, Nanjing, China, 210029. Phone No: +862586617141 E-mail: tancheng@yeah.net




Case Presentation                                                        associated with intense itch. Due to scratching, makes in-
                                                                         tact blister are rarely observed. Recurrent excoriations may
A 47-year-old man had pruriginous nodules evolving into                  stimulate skin to develop hypertrophic scars in a linear or
flagellate scarring for 5 years. Family history was negative.            figurate configuration [1]. Histology shows cell-poor sub-
On examination, pruriginous papules and nodules were                     epidermal splits or blisters. Direct and indirect immunoflu-
present on the trunk and extremities associated with hyper-              orescence studies are negative. EBP can be confirmed by a
trophic scars in a flagellate or linear configuration (Figure 1).        mutation analysis of COL7A1, which disrupts the normal
A biopsy of a pruritic papule showed a subepidermal blis-                functions of anchoring fibrils and leads to sublamina densa
ter with a dermal proliferation of fibroblasts. The mutation             blistering.
was identified in the COL7A1 gene: c.6846G>C, compatible                      The late-onset and widespread lesion in EBP differs
with epidermolysis bullosa pruriginosa (EBP). Thalidomide                from the pretibial form of dystrophic epidermolysis bullosa.
100 mg/day was used for three months with some relief from               The intense pruritus in EBP may similarly appear in lichen
itching.                                                                 simplex, nodular prurigo, pemphigoid nodularis, and li-
                                                                         ­
                                                                         chen planus. The subepidermal blistering made all these

Teaching Point                                                           skin ­
                                                                              disorders unlikely in our patients. Treatment strat-
                                                                         egies for this ­
                                                                                        condition include topical and intralesional
EBP is a hereditary disease that commonly arises in infancy              corticosteroids, ­
                                                                         ­                thalidomide, cyclosporine, phototherapy,
or early childhood. Occasionally, it occurs in the third decade          and dupilumab. However, most patients are refractory to
or the fifties. EBP starts as pruriginous papules or plaques             them [2].




Image Letter | Dermatol Pract Concept. 2023;13(4):e2023218                                                                          1
Figure 1. (A) Numerous violaceous papules and nodules are seen on the trunk and extremities with excoriations. (B) Hypertrophic scars are
scattered among them or arranged in flagellate or linear configurations.


References                                                                 2013;149(6):727-731. DOI: 10.1001/jamadermatol.2013.155.
                                                                           PMID: 23616197.2. Clawson RC, Duran SF, Pariser RJ. Epi-
 1. Vivehanantha S, Carr RA, McGrath JA, Taibjee SM, Madhog-               dermolysis bullosa pruriginosa responding to dupilumab. JAAD
    aria S, Ilchyshyn A.. Epidermolysis bullosa pruriginosa: a case        Case Rep. 2021;16:69-71.DOI: 10.1016/j.jdcr.2021.07.036
    with prominent histopathologic inflammation. JAMA Dermatol.            . PMID: 34522751. PMCID: PMC8427235.




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