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CASE REPORT
Year : 2022 | Volume
: 26
| Issue : 3 | Page : 19-20
Bullous pemphigoid: Associating with the multifaceted phenomenon of koebner
Sudhir U. K. Nayak1, Shukriti D Shenoi2, S Smitha Prabhu1, Raghavendra Rao1
1 Department of Dermatology, Venereology and Leprosy, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, India
2 Department of Dermatology, Venereology and Leprosy, Kasturba Medical College, Manipal Academy of Higher Education, Manipal; Department of Dermatology, Venereology and Leprosy, Kanachur Institute of Medical Sciences, Mangalore, Karnataka, India
Correspondence Address:
Dr. Sudhir U. K. Nayak
Department of Dermatology, Venereology and Leprosy, Kasturba Medical College, Manipal Academy of Higher Education, Manipal - 576 104, Karnataka
India
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jdds.jdds_142_20
Koebner phenomenon (KP) or isomorphic phenomenon often helps in diagnosis of certain conditions and often reflects disease activity. Although commonly reported with psoriasis, vitiligo, and lichen planus, KP has been occasional reported with other conditions. Bullous pemphigoid has a rare association with this and we report a case with scratching being the inciting factor. A 50-year-old female with no comorbidities presented with itchy fluid-filled lesions all over the body for 15 days. Examination showed generalized distribution of bullae and vesicles with linear bulla on the forearms. Histopathology and immunofluorescence studies were conclusive of bullous pemphigoid. KP has been reported in general due to mechanical trauma, therapy, dermatoses, or reactions. KP in bullous pemphigoid is rarely reported and it is mentioned in the category IV of Boyd and Nelder classification. The linear nature of bulla in our patient was suggestive of scratching being the inciting factor for KP, which has not been reported to the best of our knowledge.
Keywords: Bullous pemphigoid, immunofluorescence, Koebner phenomenon
Koebner's phenomenon is the development of isomorphic lesions on traumatized apparently uninvolved skin. It has been reported with a number of dermatoses and might reflect disease activity. The occurrence of Koebner's phenomenon in bullous pemphigoid has been considered questionable. We report a case of Koebner's phenomenon in bullous pemphigoid to further strengthen the association of this occurrence.
Case ReportA 50-year-old female presented with itchy fluid-filled lesions all over the body for 15-day duration. There was no preceding history of any drug intake before the onset of lesions. There was no preceding history of wheals or lesions in the oral cavity. Examination revealed multiple vesicles and bullae of varying size along with erosions all over her body including the scalp and genitalia. Some of the bullae on the forearms were arranged in a linear pattern, suggesting a Koebner phenomenon (KP) [Figure 1]. A clinical diagnosis of bullous pemphigoid was made.
Figure 1: Koebner phenomenon in bullous pemphigoid evident by linear whealsHistopathology of a vesicle showed a subepidermal blister filled with eosinophils. Direct and indirect immunofluorescence studies showed a linear staining of the basement membrane zone with IgG and C3, with roof (epidermal) band in areas of split [Figure 2]. On the basis of clinical, histopathological, and immunofluorescence studies, a final diagnosis of bullous pemphigoid was made. The patient was started on tapering doses of systemic corticosteroids along with daily cyclophosphamide with complete resolution of lesions. At a 1-year follow-up, the patient was in clinical remission
Figure 2: Direct immunofluorescence showing linear IgG and C3 deposits along the dermoepidermal junction (×20) Discussion Koebner or isomorphic phenomenon (Gk: Equal shape), described first by Heinrich Koebner in 1876, is the occurrence of isomorphic lesions on traumatized uninvolved normal skin. Boyd and Nelder classified diseases with KP into four subcategories. True KP (Category I) has been described with psoriasis, vitiligo, and lichen planus. Pseudokoebnerization (category II) is due to contact of infectious agents with trauma site and includes molluscum contagiosum and verruca. Category III Koebner phenomenon is occasionally occurring KP and includes Darier disease, erythema multiforme and perforating disorders. Category IV includes those disorders with questionable trauma-induced process and whose association with KP is doubtful. Bullous pemphigoid has been reported in this category and is thus rare.[1] Reverse KP in bullous pemphigoid due to tight clothing has described.[2] The presence of KP usually indicates disease activity and poor initial response to therapy. The exact pathogenesis of KP is not known. Various factors which have regarded as basis are vascular, dermal, immunological, enzymatic, neural, growth, inhibitory, hormonal, and genetic.[3] The occurrence of lesions in bullous pemphigoid has been attributed subsequent to burns, surgical scars, photochemotherapy, radiation therapy, central venous lines, and fistulas.[4]
Itch is one of the most common symptoms of bullous pemphigoid. We could not find any case report of KP in bullous pemphigoid secondary to scratching in bullous pemphigoid. In our patient, the lesions occurred secondary to scratching as was evident by the linear nature of bullae on the forearms mimicking the scratch process. This provides ample proof of KP in bullous pemphigoid. Perhaps, it is also time now to move bullous pemphigoid from category IV (questionable) to category III of occasionally occurring.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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