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Porokeratosis of Mibelli with mutilation: a case report
Farhad Handjani1, Shima Shahbaz, Fatemeh Sari Aslani
1Department of Dermatology, Shiraz University of Medical Sciences, Iran.
Abstract:
Porokeratosis is a rare keratinization disorder of the skin characterized by annular plaques with an atrophic center surrounded by a raised keratotic wall that spreads centrifugally. We report a case of porokeratosis of Mibelli with mutilation. A 30-year-old woman presented with atrophic plaques on the index fingers of both hands with a keratotic ridge in some margins of the plaques. There was loss of the distal phalanx of the left index finger. In the right hand, shortening of the right distal phalanx and flexion contracture of the distal interphalangeal joint were noted in the index finger.
Insights
Porokeratosis of Mibelli, a rare skin disorder, can lead to severe mutilation. This case highlights significant digital destruction, including bone loss and joint contractures, emphasizing the potential for disfigurement in this condition.
Area of Science:
- Dermatology
- Rare Skin Diseases
- Keratinization Disorders
Background:
- Porokeratosis is a rare genodermatosis characterized by abnormal keratinization.
- It typically presents as annular plaques with a raised keratotic border and central atrophy.
- Porokeratosis of Mibelli is a common subtype, but mutilating forms are exceptionally rare.
Observation:
- A 30-year-old woman presented with classic porokeratosis of Mibelli lesions on both index fingers.
- The lesions featured atrophic centers and raised keratotic ridges.
- Significant digital mutilation was observed, including distal phalanx loss on the left and shortening with contracture on the right.
Findings:
- The case demonstrates a severe mutilating variant of porokeratosis of Mibelli.
- Digital destruction, including phalangeal loss and joint deformities, occurred secondary to the skin condition.
- This presentation underscores the potential for aggressive and destructive manifestations of porokeratosis.
Implications:
- This case expands the known clinical spectrum of porokeratosis of Mibelli.
- Early recognition and aggressive management may be crucial to prevent severe mutilation.
- Further research into the pathogenesis of mutilating variants is warranted to guide treatment strategies.
