Article Text

Download PDFPDF

Median canaliform dystrophy of Heller presenting as a body-focused repetitive behaviour
Free
  1. Christopher Jude Pinto1,2,
  2. Shadab B Maldar3 and
  3. Prakash K Wari3
  1. 1Department of Family Medicine, Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, Michigan, USA
  2. 2Department of Medicine, Karnataka Institute of Medical Sciences Hubballi, Hubli, Karnataka, India
  3. 3Department of Paediatric Medicine, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India
  1. Correspondence to Dr Christopher Jude Pinto; christopher.pinto{at}wmed.edu

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Description

A mid-teen male boy presented to the dermatology outpatient with deformed nails of the thumb and bilateral great toes (figures 1 and 2). On a detailed history taking, he mentioned that for the last 2 years he had been traumatising the nails of his thumbs and great toes with the nails of the right hand (dominant), hence leading to a longitudinal palpable furrow with transverse ridges relatively sparing the right thumb (figure 1A,B). Macrolunula was noted over the right thumb with a few transverse ridges indicative of resolved prior self-infliction.1 The nail pattern observed on the toes were symmetric in appearance (figure 2A,B). Physical examination showed excoriated patches of skin with mild inflammation over the hands, elbows, feet and knees with moderate lichenification. There were no features of onychomycosis or lichen planus.

Figure 1

Nails of the hands showing relative sparing of the right thumb, with surrounding epidermal lichenification. (A) Left hand (non-dominant) showing a central furrow with inverted parallel transverse ridges (marked in black) and inflamed hypertrophied cuticles (marked in red). (B) Right hand (dominant) showing macrolunula (marked in blue).

Figure 2

Nails of the feet showing morphological central furrows with inverted parallel transverse ridges (marked in black) and inflamed hypertrophied cuticles (marked in red) affecting both feet with surrounding epidermal lichenification with moderate inflammation. (A) left foot, (B) right foot.

On psychiatric evaluation, the patient mentioned that he had issues with anxiety but did not get evaluated to date. He often felt restless and that his habit of picking at greater digits were precipitated during moments of stress during long study hours for the preparation of his examinations. He also reported irritability and constant worry with regards to his results, around same time when he started inflicting his nail beds.

The patient was diagnosed with median canaliform dystrophy of Heller (MCD). In addition, the diagnosis of unspecified obsessive–compulsive and related disorders leading to body-focused repetitive behaviour (BFRB) was also confirmed by psychiatry and dermatology multidisciplinary care as per DSM-5-TR. The patient was followed up on a weekly basis for cognitive–behavioural therapy. The patient was offered topical triamcinolone 0.1% for MCD and moisturisers for his atopic dermatitis. At the second month follow-up visit, the patient agreed to nail taping to prevent further self-infliction as the topical triamcinolone proved ineffective due to persistent repeated trauma.2–5

This disorder of MCD of Heller seen here belongs to rare heterogeneous class of nail disorders characterised by a longitudinal split or furrow in the nail plate with transverse ridges usually affecting bilateral thumbs and great toes.2 Its aetiology is multivariate, with its most known cause to be repeated trauma affecting nail formation, hence indicating the need for a psychiatric evaluation.2 The causation of the longitudinal furrow in MCD could be due repetitive damage along the entire length of the nail plate leading to a collapse in the nail bed. Primarily, this disorder has to be identified and delineated from habit tic nail disorder where a longitudinal furrow is not seen. Other conditions to consider would be of onychomycosis, lichen planus, subungual skin tumours affecting nail plate growth, digital mucous cysts and psoriasis.6 Some cases are noted to be familial in origin and/or noticed at birth.2–4 Other causes may include oral retinoid use, glomus tumour and cryotherapy induced.4–6

Treatment modalities of BFRB with psychiatric conditions may leave dermatological interventions ineffective as the physical infliction may persist despite focused dermatological care. Meticulous evaluation and history taking is crucial in identifying cases wherein patients are unaware of their habitual tics.7 Topical retinoids and immunomodulators have shown good results in normalising the process of nail bed keratinisation. Full length nail taping could also aid in reducing physical infliction as well as acting as reminder to prevent repetitive behaviour.7 Psychiatric treatment with selective serotonin reuptake inhibitors could act in adjunct with cognitive behavioural therapy. Inconsistent response to any single mode of treatment necessitates a multidisciplinary approach to further elucidate underlying conditions.1–7

Patient’s perspective

I had these lines along my nails for a very long time. I understood it could have been due to me constantly fidgeting with them. Now after being diagnosed with a type of OCD and a developed habit, applying a tape would seem like the best option to me. I am going through therapy and its definitely helping but I often catch myself picking at my nails over and over again, but I am hopeful that I would eventually stop.

Learning points

  • Median canaliform dystrophy of Heller is a rare entity characterised by a longitudinal ridge in the nail plate with transverse ridges angled backwards usually affecting bilateral thumbs and great toes.

  • As per the DSM-5-TR, this body-focused repetitive behaviour is classified under unspecified obsessive–compulsive and related disorders, requiring cognitive–behavioural therapy and dermatological management.

  • Due to inconsistent responses to dermatological intervention alone, patients may have to undergo multiple sessions of cognitive–behavioural therapy and medications with selective serotonin reuptake inhibitors.

Ethics statements

Patient consent for publication

References

Footnotes

  • Twitter @corizot

  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: CJP, SBM and PKW. The following authors gave final approval of the manuscript: CJP, SBM and PKW.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.