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Images in Clinical Practice
ARTICLE IN PRESS
doi:
10.4103/ijdvl.IJDVL_772_19
pmid:
32270768
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Giant Koenen tumors involving 20 nails

Departments of Dermatology Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India
Plastic Surgery, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India
Corresponding author: Dr. Rashmi Jindal, Department of Dermatology, Venereology and Leprosy, Himalayan Institute of Medical Sciences, Swami Ram Nagar, Doiwala, Dehradun - 248 140, Uttarakhand, India. rashmijindal98@gmail.com
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This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Jindal R, Dvivedi S, Sethi S. Giant Koenen tumors involving 20 nails. Indian J Dermatol Venereol Leprol 0;0:0.

A 63-year-old lady presented with multiple periungual growths which progressively increased in size and number to involve all 20 nails for last 30 years. There was no history of seizures or psychosis. Both her daughters had similar but smaller lesions on a few nails. On examination, she had multiple smooth to verrucous, firm, flesh-colored excrescences emerging from proximal nail folds of all nails, largest on the left thumb being 4 cm × 3 cm in size [Figure 1]. Dermoscopy revealed multiple erythematous to hyperpigmented polypoidal and digitate growths, garlic - clove morphology with a yellow-brown scale at the tip of the central one and multiple longitudinal striations on nail [Figure 2]. She also had multiple firm, red-brown papules on nasolabial folds and cheeks. There were multiple soft to firm skin-colored plaques on lumbosacral area and multiple ovoid hypopigmented macules on trunk and extremities. Histopathology from one of the nailfold growths revealed a central core of dense collagen with prominent interspersed fibroblasts and dilated capillaries at the distal end with overlying orthokeratosis suggestive of angiofibroma. Fundoscopy, computer tomography scan of brain, magnetic resonance imaging of brain and ultrasonography of abdomen were normal. Diagnosis of tuberous sclerosis complex was established with four major features that included facial angiofibromas, ungual fibromas (Koenen tumors), hypomelanotic macules and shagreen patch. Patient was offered excision of large angiofibromas to reduce functional impairment.

Figure 1:: Multiple, smooth to verrucous, firm, flesh-colored excrescences emerging from proximal nail folds of all nails
Figure 2:: Multiple, erythematous to hyperpigmented, polypoidal and digitate growths. Garlic clove morphology with a yellow-brown scale at the tip of the central growth, and longitudinal striations on nail (Dermoscopy using Dermlite DL200 Hybrid, ×10)

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name 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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