Correspondence Address:
Debdeep Mitra
Department of Dermatology, Command Hospital Air Force Bangalore, Karnataka
India

Full Text



Sir,

Chronic regional pain syndrome (CRPS) is a disabling, complex, poorly understood neurovascular disorder and has two subtypes. Reflex sympathetic dystrophy (RSD) (type 1) is seen following noxious events, and type 2 is seen after definitive peripheral nerve damage. Both have characteristic hyperalgesia and continuing pain. Nail changes such as leukonychia, Beau's lines, nail-fold swelling, clubbing, acute paronychia, dystrophy and trachonychia are seen.[1] Herein, we report a case of CRPS type 1 following a cerebrovascular accident in a male who presented with pain in the left half of the body and dystrophy of all fingers and toenails.

A 54-years-old male with no comorbidities was admitted with a cerebrovascular accident (CVA) resulting in hemiplegia and pain in the left half of the body. Four months later, he complained of brownish discoloration of the fingers and toenails of the left side. It was preceded by pain, redness and swelling of the left hand 2 months back [Figure 1] and [Figure 2]. Over the next 1 week, he developed similar features in the toenails. There was brittleness of the finger and toenails along with swelling and pain of the nail folds. Dermatological examination revealed increased curvature of the left great toenail, brownish discoloration and dystrophy of all the nail plates of finger and toenails with sparing of the right great toenail. Rest of his dermatological examination was normal. His routine haematological and biochemical investigations were essentially normal. KOH mount and fungal culture were negative.{Figure 1}{Figure 2}

The Budapest criteria is the current standard for diagnosis for CRPS[2] [Table 1] However, diagnosis remains purely clinical without a gold-standard investigation. Our patient fulfilled the requisites of the Budapest criteria.{Table 1}

Cutaneous and nail changes are commonly associated but non-specific to CRPS; thus, they are frequently overlooked. There are three stages of progression of CRPS, but all stages may not occur [Table 2]. It can occur in the setting of underlying conditions such as trauma, infections, ischemic injuries, arthritis, immobilisation, nail surgeries and hemiplegia.[3]{Table 2}

Cutaneous manifestations such as erythema and oedema arising due to vascular components are prominent in CRPS, while papular lesions, folliculitis, skin atrophy, ulcers and bullae are rare. Localised nail dystrophy and non-specific nail changes have been reported with RSD with obscure pathophysiology. Neurological dysregulation and altered microvascular changes have been proposed. An increase in growth and transverse curvature of the nail plate is seen in the first stage, while the second and third stages show progressive brittleness of nails. In our case, there was a prominent and unusual nail involvement with minimal skin involvement.[4],[5]

Trachonychia in RSD is due to disrupted nail matrix differentiation and maturation resulting in the disordered arrangement of keratinocytes leading to nail thinning and brittleness. Beau's lines result from sympathetic denervation and matriceal oedema.[6] Our patient reported painful swelling of nail folds which improved with OTC medications. Over-curvature of the nail plate was seen in our case on the affected great toe along with nail dystrophy, which has been infrequently described. Onychomadesis and pyogenic granuloma due to post-operative immobilisation have been reported.[7] The case highlights unique nail changes in CRPS, exemplifying the fact that manifestations of this diverse condition can be extremely versatile.

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