From blisters to nodules: clinical clues to epidermolysis bullosa pruriginosa

Omkar Gopal Jaju; Aishwarya Kalpande; Mahesh Unni

doi:10.18203/issn.2455-4529.IntJResDermatol20263748

Authors

Omkar Gopal Jaju Department of Dermatology, Maharashtra Institute of Medical Sciences and Research and Yashwantrao Chavan Rural Medical College, Latur, Maharashtra, India
Aishwarya Kalpande Department of Dermatology, Venereology and Leprology, Gode Medical College, Amravati, Maharashtra, India
Mahesh Unni Department of Dermatology, Maharashtra Institute of Medical Sciences and Research and Yashwantrao Chavan Rural Medical College, Latur, Maharashtra, India

DOI:

https://doi.org/10.18203/issn.2455-4529.IntJResDermatol20263748

Keywords:

Epidermolysis bullosa pruriginosa, Dystrophic epidermolysis bullosa, Dermoscopy, Nail dystrophy, Milia, COL7A1

Abstract

Epidermolysis bullosa pruriginosa (EBP) is an uncommon variant of dystrophic epidermolysis bullosa characterised by severe pruritus, prurigo-like nodules, milia, nail dystrophy, and scarring. Delayed diagnosis is common because blistering may become inconspicuous with age. The case report demonstrates a 44-year-old woman with a 10–12-year history of intensely pruritic papules and nodules involving the scalp, trunk, and extremities. Detailed history revealed recurrent trauma-induced blistering since childhood, delayed wound healing, recurrent great toenail shedding, and similar illness in her sister. Examination showed multiple excoriated hyperpigmented papules and nodules, milia, atrophic scarring, and dystrophic toenails. Dermoscopy demonstrated milia-like white clods and scar-like white areas, while histopathology showed acanthosis, compact hyperkeratosis, papillary dermal fibroplasia, and infundibular cyst formation. Clinicopathological correlation established the diagnosis of epidermolysis bullosa pruriginosa. Recognition of childhood blistering, nail dystrophy, milia, scarring, and family history is essential for timely diagnosis and appropriate management.

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