922© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1210

Introduction

A 53-year-old woman presented with a slowly progressing nasal ulceration present for approximately 6 months. Clini-cal examination revealed an irregular but clearly demarcated crescent-shaped skin defect of the left nasal ala which was covered with a serous scab; the tip of the nose was spared (Figure 1).

The rest of the skin and mucous membranes were nor-mal. The patient had a history of arterial hypertension, hypercholesterolemia and chronic obstructive pulmonary disease. Eleven months earlier, she had experienced an ische-mic infarct involving the right common carotid artery/right internal carotid artery, with subsequent expressive aphasia.

Histological examination revealed a non-specific mixed cellular chronic fibrotic inflammatory reaction (Figure 2) with no signs of a malignant epithelial tumor or an infecti-ous pathology.

Unilateral painless nasal ulceration

Case for Diagnosis

DOI: 10.1111/ddg.12377

Marta Kogut, Corinna Hamsch, Eva Hadaschik, Alexander H. Enk, Wolfgang Hartschuh, Ferdinand Toberer

Department of Dermatology, Ruprecht-Karls-University of Heidelberg, Heidelberg, Germany

Figure 1 Clinical presentation: ulcerated lesion on the edge of the left nasal ala with clear edges and irregular margins.

Figure 2 Histologic examination. Nonspecific mixed dermal infiltrate and fibrosis of the dermis (HE; original magnification × 200).Your diagnosis?

Case for Diagnosis

923 © 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1210

Discussion

Trigeminal trophic ulcer or trigeminal trophic syndrome (TTS), first described by Wallenberg in 1901, is a chronic ul-ceration resulting from a defect of the afferent sensory fibers of the trigeminal nerve with subsequent paresthesia or dyses-thesia in parts of the area supplied [1]. Ulcerations usually result from chronic self-manipulation (rubbing, scratching) in the affected area, while patients often describe burning pa-resthetic sensations, sometimes pruritus, but usually no pain. Recurrent manipulation lead to skin defects with progressive loss of tissue [2]. Upon detailed history taking, our patient admitted repeatedly manipulating the area, sometimes sub-consciously.

Upon clinical examination, TTS usually appears as a strictly unilateral ulceration, usually involving the face, with the nose being most frequently affected, while rare cases in-volve the ears, forehead, temples or cheeks [1].

TTS usually affects female patients with a mean age of 57 years [1]. The time interval between the neurologic de-fect and the subsequent manifestation of the ulceration may range from a few weeks to several (on average: two) years [1]. The most frequent underlying pathophysiology is stroke, others include nerve injuries following neurosurgery (ablati-on of the trigeminal ganglion, surgical removal of an acoustic neuroma), infectious diseases such as herpes simplex infec-tions, syphilis or Mycobacterium leprae neuritis, encephalitis or birth trauma [3].

Diagnosis is based on clinical findings, wherein the clear-ly demarcated ulceration and the (not always easily obtained) information about burning sensations in the corresponding area are vital. In case of a nasal localization, the tip of the nose is usually spared, as its sensory innervation is transmit-ted via the ethmoidal nerve [2].

Differential diagnosis includes basal cell carcinoma, squamous cell carcinoma, Wegner granulomatosis and infec-tious disorders (recurrent herpes simplex, syphilitic ulcera-tions, lupus vulgaris) [3]. Histological examination is recom-mended to confirm the clinical diagnosis in most cases.

Treatment is often difficult and time-consuming. In-forming the patient about the pathophysiology is critical in order to motivate him or her to avoid any self-manipulati-on [2]. Supportively, temporary occlusive wound dressings may be used. Systemically, antidepressant and neuroleptic drugs (diazepam, chlorpromazine, pimozide) have been used, with varying results, functioning mainly through a reduction of the paresthetic sensations [4]. Plastic-reconst-ructive surgery (grafts or regional flaps) has achieved satis-fying results [5].

As the patient refused surgical management, local the-rapy with panthenol 5% cream, partially applied under an occlusive wound dressing, led to a stagnation of the progres-sive skin defect.

Conflict of InterestNone.

Correspondence to

Marta KogutDepartment of DermatologyRuprecht-Karls-University of Heidelberg

Im Neuenheimer Feld 44069120 Heidelberg, Germany

E-mail: marta.kogut@med.uni-heidelberg.de

References1 Pedicelli C, Paradisi A, Fazio M et al. Trigeminal neurotrophic

ulceration in Wallenberg’s syndrome. Int J Dermatol 2009; 48: 443–5.

2 Willis M, Shockley WW, Mobley SR. Treatment Options in Trigeminal Trophic Syndrome: A Multi-Institutional Case Series. Laryngoscope 2011; 121: 712–6.

3 Sadeghi P, Papay FA, Vidimos AT. Trigeminal trophic syndrome – report of four cases and review of the literature. Dermatol Surg 2004; 30: 807–12.

4 Rashid RM, Khachemoune A. Trigeminal trophic syndrome. J Eur Acad Dermatol Venereol 2007; 21: 725–31.

5 Hartschuh W, Adler D, Kohl PK. Das trigeminotrophe Ulkus des Nasenflügels; erfolgreiche Behandlung in zwei Fällen durch plastisch-rekonstruktive Eingriffe. Gegenwärtiger Stand der operativen Dermatologie; Fortschritte der operativen Dermatologie 1988; 4: 284–92.

Diagnosis:

Trigeminal trophic ulcer of the nasal ala