2015.1-13

Ledderhose disease: A case report with palmar fibromatosis, keloid and partial response to oral retinoid

Sadr Eshkevari Shahriar, Nickhah Nahid

Department of Dermatology, Razi Medical Center, Guilan University of Medical Sciences, Rasht, Iran
Corresponding author: Ass. Prof. Shahriar Sadr Eshkevari, E-mail: dr. sadreshkevari@yahoo.com
Submission: 02.09.2014; Acceptance: 05.11.2014
DOI: 10.7241/ourd.20151.12

ABSTRACT

Ledderhose disease is a rare hyperproliferative disorder of fibrous tissue. We present the case of a 40-year-old man who presented with bilateral plantar fibromatosis, dupuytren contracture and keloid, with partial response to oral acitretin.

Key words:: Ledderhose disease; plantar fibromatosis; dupuytren contracture


INTRODUCTION

Plantar fibromatosis or ledderhose disease is a rare hyperproliferative disorder of fibrous tissue [1], occurring over the medial sole of the foot with tendency for local recurrence but do not metastasize [2]. Patient described in this report is a man with bilateral plantar fibromatosis, dupuytren contracture and keloid, with partial response to oral Acitretin.

CASE REPORT

A 40-years-old hospital employer man referred to our clinic with multiple painful nodules in plantar surface of his feet (Figs 1AD), in spring 2013.

Figure 1: A and D) The patient presents with multiple firm nodules on sole of both feet

20 years ago (1994) he noticed stiffness and flexion deformity in the third finger of his right hand. The patient underwent two surgical operations with the diagnosis of dupuytren contracture. Six years later (2002) slow growing nodule appeared in plantar surface of his left foot, and it was gradually progressive with a dull ache type of pain. Initial management consisted of a skin biopsy of this foot lesion which led to the confirmation of scar tissue, then a surgery performed and report of pathology, confirmed the previous diagnose. A few months later lesion recurred again at the site of operation on left sole and additionally the same nodules occurred in right sole (Figs 1AD). The lesions were painful, somewhat preventing him from working. On examination, there were multiple skin colour to violaceous firm nodules with sharp margin and scale-crust on some of them in plantar, medial and latral aspect of his feet (Figs 1AD). The size of biggest nodule on right sole was 2.5*2.9 cm and 3.5*3.0 cm on left sole. No flexion contracture of toes or neurovascular deficits was noted. Motion of the feet was within the normal range. Also he had a scar of dupuytren contracture surgery in right hand and two keloides in his chest and shoulder (Fig. 2). The patient reported no history of any diseases such as diabetes, seizure, trauma and he did not consume alcohol. Also there was no family history of similar lesions.

Figure 2: Scar of dupuytren contracture surgery and keloid in his chest

Laboratory data such as CBC/ESR/FBS/renal and liver function tests were normal. Pathology slides reviewed again and in microscopy the epidermis revealed hyperkeratosis, hypergranulosis and irregular acanthosis. In the dermis, there was proliferation of bland looking spindle shaped fibroblasts arranged vertically, within dense collagenous stroma suggestive of fibromatosis (Figs 3AC).

Figure 3: (A-C) Histopathology. Proliferation of bland looking spindle shaped fibroblasts arranged vertically, within dense collagenous stroma

Prior to the study, patient gave written consent to the examination and biopsy after having been informed about the procedure.

MRI of lesions revealed small masses without invasion to muscle or tendon sheets (Fig. 4).

Figure 4: Magnetic Resonance Imaging. Small masses without invasion to muscle or tendon sheets

To management, we recommended him to change his job and suggested him not to stand for long time any more. Then prescribed the patient Acitretin 20 mg/day. He consumed Acitretin for two months and stopped it for a month. However, the patient’s symptoms, including pain reduced a lot and some of the nodules became flat and smaller in size (The dimensions of biggest nodule on right sole were 2.5*2.7 cm and 3.5*2.6 cm on left sole). In addition scales and crusts disappeared significantly (Figs 5A,B and 6A,B).

Figure 5: (A) Sole lesions before treatment (left side) and after treatment (right side)
 
Figure 6: Lateral lesions before treatment (left side) and after treatment (right side)

The patient’s informed consent was obtained.

DISCUSSION

Fibromatoses are a family of fibroblastic proliferation sharing similar histology, an infiltrative pattern of growth and a tendency to recur after excision [2]. In general the superficial types are less aggressive than deep ones (such as desmoid) [3]. Plantar fibromatosis (ledderhose disease), palmar fibromatosis (Dupuytren contracture), penile fibromatosis (Peyronie’s disease) and knuckle pads are benign superficial neoplastic proliferation of fibroblasts and/or myofibroblasts. Patients with palmar fibromatosis have additional areas of plantar fibromatosis in 5% to 20% of cases and penile fibromatosis in 2% to 4% of cases [4]. Recently, Trybus et al. reported an association rate of 14.85% of Ledderhose in 101 cases of Dupuytren contracture [5]. An increased incidence of knuckle pads has been observed [6,7]. Plantar fibromatosis occurs less frequently than palmar lesions with incidence of 0.23% [8].

Ledderhose disease, described in 1897, characterized by one or more slow growing nodules, most often on the medial half of the mid foot. Most lesions are asymptomatic and if they invade the adjacent structures they become painful. The pain increased by prolonged standing or walking and rarely produces contracture [9]. Nodules may be multiple in 33% of cases [10]. The condition affect any age but more often in younger age group, most often age 30 [11,12] and men are affected twice as often as females. Bilateral involvement is seen in 20-50% of cases [13,14].

The etiology of ledderhose disease remains unknown but genetic predisposition as well as repeated trauma is thought to play a role in its pathogenesis [9]. Some association with diabetes, alcoholic liver disease and epilepsy have been described [2]. Our case had none of these associations or predisposing factors, also he didn’t indicate any family history of fibromatosis. Radiographs are frequently normal in Ledderhose disease, since the lesion is not encapsulated. Evaluation is most commonly performed with ultrasound and MRI. MRI is an excellent, non invasive method for delineating the extent of the lesion and planning surgical treatment [15], as it showed no invasion to muscle and tendon sheet in our patient.

In asymptomatic patients conservative managements such as footwear modification may be helpful [13]. For those suffering from pain of large lesions which cause disability, excision of the lesion with total fasciectomy is desirable with the lowest incidence of recurrence [16]. Kan and Hovius reported no recurrence after graft repair in two brothers, with a long term follow up, ranging from 14-25 years [17].

Adjuvant radiotherapy decreases the rate of recurrences but should be used selectively because of its side effects [18]. High-energy focused extracorporeal shockwave therapy reduces pain in plantar fibromatosis [19].

There are no specific medical treatment for plantar fibromatosis, other than symptomatic relief and supportive measures. In the other hand, Viera et al reported that retinoic acid and other vitamin A derivatives produce a marked reduction in human fibroblast proliferation by interfering with DNA synthesis in vitro. Retinoids also exhibit an inhibitory effect on TGF-β1-induced type I collagen gene expression in human fibroblasts [20]. Furthermore the proliferation and activity of cultured fibroblast cells and type III collagen synthesis are inhibited as the concentration of retinoids are increased in the medium [21]. Etretinate, free acid of etretinate and 13-cis retinoic acid(RA), reduce type IV collagen synthesis in vitro, the largest decrease being found with free acid of etretinate and 13-cis-RA [22].

So with regard to the results of a two monthes course of oral Acitretin in our case and above mentioned data, it may be a worth trying medical opportunity in cases, who deny surgery, with multiple surgical failure, or are not good candidate for surgery. However, these results require confirmation in future studies.

CONSENT

The examination of the patients was conducted according to the Declaration of Helsinki principles. Written informed consent was obtained from the patient for publication of this article and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

REFERENCES

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10. Morrison WB, Schweitzer ME, Wapner KL, Lackman RD, Plantar fibromatosis: a benign aggressive neoplasm with a characteristic appearance on MR imagesRadiology 1994; 193: 841-5.

11. Weiss SW, Goldblum JR, Enzinger FM, Fibromatoses in Enzinger and Weiss’ Soft Tissue Tumors2008; Philadelphia, Pa, USA: Mosby Elsevier; 227-228.

12. Fetsch JF, Laskin WB, Miettinen M, Palmar-plantar fibromatosis in children and preadolescents: A clinicopathologic study of 56 cases with newly recognized demographics and extended follow-up informationAm J Surg Pathol 2005; 29: 1095-105.

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15. Walker EA, Petscavage JM, Brian PL, Logie CI, Montini KM, Murphey MD, Imaging Features of Superficial and Deep Fibromatoses in the Adult PopulationHindawi Publishing Corporation Sarcoma 2012; Article ID 215810, 17 pages

16. Durr HR, Krodel A, Trouillier H, Lienemann A, Refior HJ, Fibromatosis of the plantar fascia: diagnosis and indications for surgical treatmentFoot Ankle Int 1999; 20: 13-7.

17. Kan HJ, Hovius SER, Long-term follow-up of flaps for extensive Dupuytren's and Ledderhose disease in one familyJ Plast Reconstr Aesthet Surg 2012; 65: 1741-5.

18. De Bree E, Zoetmulder FA, Keus RB, Peterse HL, Coevorden FV, Incidence and treatment of recurrent plantar fibromatosis by surgery and postoperative radiotherapyAm J Surg 2004; 87: 33-8.

19. Knobloch K, Vogt PM, High-energy focussed extracorporeal shockwave therapy reduces pain in plantar fibromatosis (Ledderhose's disease)BMC Res Notes 2012; 2: 542.

20. Viera MH, Amini S, Valins W, Berman B, Innovative therapies in the treatment of keloids and hypertrophic scarsJ Clin Aesthet Dermatol 2010; 3: 20-6.

21. Bakry OA, Samaka RM, Houla NS, Basha MA, Two Egyptian cases of lipoid proteinosis successfully treated with acitretinJ Dermatol Case Rep 2014; 8: 29-34.

Notes

Source of Support: Nil

Conflict of Interest: None declared.


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