Indian Journal of Dermatology
: 2011  |  Volume : 56  |  Issue : 6  |  Page : 773--775

Multiple koenen tumors: An uncommon presentation

Basanti Devi, Manjulata Dash, Binodini Behera, Manas R Puhan 
 Department of Skin and VD, S. C. B. Medical College, Cuttack, Orissa 753007, India

Correspondence Address:
Basanti Devi
Department of Skin and VD, S. C. B. Medical College, Cuttack, Orissa 753007

How to cite this article:
Devi B, Dash M, Behera B, Puhan MR. Multiple koenen tumors: An uncommon presentation.Indian J Dermatol 2011;56:773-775

How to cite this URL:
Devi B, Dash M, Behera B, Puhan MR. Multiple koenen tumors: An uncommon presentation. Indian J Dermatol [serial online] 2011 [cited 2021 Apr 11 ];56:773-775
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Koenen tumors are a pathognomic feature and one of the major Gomez criteria of tuberous sclerosis complex (TSC), and usually appear after puberty in up to 50% of patients with TSC. We report an uncommon presentation of multiple Koenen tumors in a female aged 45 years, affecting all the finger and toe nails which were bigger in size and morphologically resembling periungual wart.

Tuberous sclerosis complex, the preferred name for the condition previously known as tuberous sclerosis, is an autosomal dominant genodermatosis, classically presenting as facial angiofibromas, shagreen patch, periungual fibromas, and ash-leaf white macules. Seizures, mental retardation, retinal hamartomas, subependymal nodules and internal organ hamartomas are common associations. [1] Koenen tumors are periungual or subungual fibromas appearing as reddish to flesh-colored, smooth, soft to firm papules and nodules emerging from nail folds. They are usually 5-10 mm in length, but may be very large. This can be the only clinically evident abnormality. [1],[2] In 50% cases of TSC, it appears at an age between 12 and 14 years and increases progressively in size and number with age. The tumors sometimes become hyperkeratotic, resembling fibrokeratoma. [3],[4] Histologically, Koenen tumors are characterized by fibrosis with stellate shaped strands of fibroblasts and dense collagen with numerous ectatic blood vessels. Multiple Koenen tumors, affecting all the fingers and toe nails, with a bigger size and morphologically resembling periungual wart, have not been reported earlier.

A 45-year-old female presented to us with complaints of fleshy lesions from the nail folds affecting all the fingers and toe nails, since 25 years of age. One of the big lesions of right middle toe had become ulcerated since 6 months [Figure 1]. On examination, it was clinically looking like periungual Koenen tumors with verrucous changes on the surface. History revealed features of adenoma sebaceum (facial angiofibroma) over face since childhood [Figure 2]. There was no other systemic abnormality. All the nails were dystrophic. There was no history of seizure. Routine tests like hemogram, urine, and stool were within normal limits. USG of abdomen and pelvis and X-ray of chest were normal. CT scan was not done as the patient was lost to follow-up. There was no family history of similar skin lesion. On histopathology, the lesions revealed dilated blood vessel surrounded by fibroblastic proliferation, which are features of angiofibroma [Figure 3].{Figure 1}{Figure 2}{Figure 3}

TSC is an autosomal dominant genodermatoses (MIM 191100). [1] Bournville, a French neurologist, described tuberous sclerosis (TS) in 1880. [5] Sherlock coined the now outmoded term "epiloia" indicating the diagnostic clinical triad of epilepsy, low intelligence and adenoma sebaceum. [6] Osborne et al. have estimated that the birth incidence may be in the region of 1 in 5800, which makes TSC one of more common single gene disorders. [1] Heredity is evident in approximately one-third of reported cases, the remaining ones being attributed to gene mutation. Half of the TSC families are linked to chromosome 9q34 with tumor suppressor activity protein tuberin (TSC1) and the other half to chromosome 16p13 with tumor suppressor activity protein hamartin (TSC2). [1]

In 1908, Vogt described the classical triad of adenoma sebaceum, epilepsy and mental retardation as the diagnostic criteria of TSC. [5] But the triad is not present in all cases. Now-a-days, TSC is diagnosed by the presence of either two major features or one major and two minor features of the clinical criteria developed by Gomez and revised by Osborne. [7]

 Major Features

Facial angiofibromas or forehead plaque, non-traumatic ungual or periungual fibromas, hypomelanotic maculae (>3 in number), shagreen patches, multiple retinal hamartomas, cortical tuber, subependymal nodule, subependymal giant cell astrocytoma, cardiac rabdomyoma, lmphangioleiomyomatosis and/or renal angiomyolipoma.

 Minor Criteria

Dental enamel pits, hamartomatous rectal polyps, bone cysts, cerebral white matter radial migration lines, gingival fibromas, non-rectal hamartoma, retinal achromic patch, confetti skin lesions, multiple renal cysts, skin tags, positive family history in 1° relatives.

Cutaneous markers are easy to find and are seen in abundance, which are most of the major criteria developed by Gomej and revised by Osborne. In our case, there were two major criteria like adenoma sebaceum and multiple Koenen tumors fulfilling the definitive diagnosis for TSC. Our case was uncommon as the tumors affecting all fingers and toe nails were very big in size and morphologically looking like periungual wart.


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