A journey battling – ERYTHROMELALGIA

Erythromelalgia is a rare condition that primarily affects the feet and, less commonly, the hands  and sometimes face, cheeks and lips which is characterized by intense, burning  sensation and pain of affected extremities,  Due to the rareness of incidence, at the present scenario, no specific treatment line is  set fixed to manage the condition, only symptomatic transitory relief can be achieved . Considering the symptoms, this condition can be directly correlated to pitha prakopa lakshanas like daha, osha etc. Keeping this view in mind, the primary aim in this case was to treat this rare condition in the line of pittha prasamana. A 22 year old male presented with redness and burning sensation and burning pain in extremities was treated with pithahara medicines , snehapana , virechana, ksheeradhara , takradhara to head for 14days. The patient got complete relief from the symptoms which helped him to carry out his daily acivities thus improved his QOL. This case study helped to revealed the efficacy of ayurveda management even in very rare disease, by acknowledging the clinical symptoms in tridosha  perspective.

Key words

Erythromelalgia, pithahara management, daha

Introduction

Erythromelalgia is a very rare disease which is accepted by NORD .It is a type of   neurovascular peripheral pain disorder that affects the skin without a clearly defined pathogenesis (incidence of 0.6-2/100000). Erythromelalgia is a rare condition that primarily affects the feet and, less commonly, the hands (extremities). It is characterized by intense, burning pain of affected extremities, severe redness (erythema), and increased skin temperature that may be episodic or almost continuous in nature. This  rare disorder   was originally described in 1878. The overall age- and sex-adjusted average incidence rate per 100 000 people per year in a population-based study in the US was 1.3. Reports indicate that females are more affected than males. Although disorder onset appears to occur most commonly in middle age, associated symptoms may develop at any age

The prefix “erythro-” denotes redness, “mel-” is a combining form meaning limb or limbs, and the suffix “-algia” indicates pain. The specific underlying cause of erythromelalgia remains unknown. The condition is thought to result from vasomotor abnormalities or dysfunction in the normal narrowing (constriction) and widening (dilation) of the diameter (caliber) of certain blood vessels, leading to abnormalities of blood flow to the extremities. Erythromelalgia may be an isolated, primary condition or occur secondary to various underlying disorders. Primary erythromelalgia may appear to occur randomly for unknown reasons (sporadically) or may be familial, suggesting autosomal dominant inheritance.

Signs and symptoms – Erythromelalgia is characterized by severe, burning pain, marked redness (erythema) of the skin, swelling, and increased skin temperature, particularly of the feet. However, in some affected individuals, the hands maybe the primary sites of involvement. Although both sides of the body are usually affected (bilateral), involvement may sometimes be limited to one side (unilateral), particularly in cases in which erythromelalgia has occurred secondary to another underlying condition or disorder (secondary erythromelalgia). Symptoms may begin suddenly (acutely) may rapidly spread, increase in severity, and possibly become disabling over week. Episodes or intensification of symptoms are sometimes described as “flaring”, during which there is sudden (acute) redness, pain, sensation of heat, and swelling. Hallmarks of erythromelalgia include triggering or worsening of symptoms with exposure to heat (heat intolerance) or exercise and relief with cooling.

Causes In most cases, it is an apparently isolated, primary condition. Primary erythromelalgia may appear to occur randomly for unknown reasons or may be familial. In other, the condition may occur secondary to various underlying disorders, particularly certain bone marrow disorders characterized by abnormally increased production of particular blood cells (myeloproliferative disorders) and as acomplication of certain drugs also.

Diagnosis The diagnosis of erythromelalgia is established by a thorough evaluation of the characteristic symptoms and signs of the disease. Patient and family history can be helpful, and specialized tests may help to exclude certain disorders with similar symptoms

Treatment – In individuals with erythromelalgia, associated symptoms are typically relieved with cooling. More specifically, in almost all cases, affected individuals may experience pain relief by immersing the affected regions in ice water . Continuous  such activities may lead to  infection; non-healing ulcers, softening and breaking down of skin due to abnormally prolonged exposure to moisture (maceration); and/or localized tissue loss (necrosis).

Case report

A 22-year-old male patient from Oman  was admitted to our hospital, on 20-9-2017, complaining of diffused deep redness of skin, swelling, burning sensation and pain over bilateral foot, bilateral palms, and nose for the past two years. He also has episodes of epistaxis which subsides by itself.

History

This 22-year-old male patient was a corporation worker living in the high altitudes in Oman. He met with a road traffic accident on March 2015. During the accident, he faced severe maxillo facial injuries which was sutured and managed under GA. After the trauma, he was oriented, conscious, with a GCS that was 14/15 and no limb injuries were reported. No change was reported in his neurological or physical status post trauma. After 2 months, the patient developed an episode of epistaxis which was subsided by a cold pack by himself. Gradually redness of cheeks,  nose, palms and feet developed and within 3 months burning he experienced burning sensation in his feet and palms. Sometimes the gradation of his burning sensation moved to a burning pain which was intolerable. Some topical applications and cold treatments gave him temporary relief. He experienced migratory joint pain less frequently  and this was in bearable limits. Later on 10 months back the redness in upper  sole of  bilateral feet turned into bullae, which  infected with severe pain and burning sensation that  affected his daily routine. The wound was debrided, cleaned and healed in somedays. Frequent episodes of epistaxis, redness and burning pain in palms and foot increased which started to affect the quality of his life. Blood investigations including platelet counts, RBC, ESR, CRP, RA factor,ANA, Anti CCP antibodies,LFT were all found to be normal. He was non-diabetic and a non-hypercholestremic patient, without any significant family history pertaining to his present condition. Subsequent clinical examinations were done to rule out other autoimmune diseases and  myeloproliferative diseases and neuropathies  which concluded to diagnose the condition as erythro melalgia of idiopathic origin.

  • On admission, the skin colour of nose, cheeks, palms and feet was in deep red to purple colour as it flared up frequently with severe burning pain and sensation.
  • He was not taking any regular medications.

Physical examination- His neurological assesement post TBI according to revised Rancho los Amigos scale revealed as 10. Clinical examinations were done to rule out joint pathologies and neuropathies

Investigations : Blood investigations-CBC,Rheumatoid panel, LFT, FBS,Hba1C – normal levels. Venous and arterial colour Doppler study- normal

Management – Considering the symptoms all internal and external therapies was in the aim of pitha prasamana from 20.9.2017 to 3.10.17.

External Treatments done were Kashayadhara with dasamoola and Ksheeradhara with pithasamana drugs, Virechanam, Snehapana with tiktha rasa pradhana ghrita and Takradhara for head.

Internal  medicines – which are tiktha madhura kashayarasa predominant like vasaguluchyadi were given. Vasa swarasam in honey was also given daily.

Discussion

Acharya Charaka depicts the pitha vikaras as 40 in number and among them, the 1st 4- dosha, plosha, daha, davadhu, dhoomaka are different kinds of burning sensations which are felt in the external part of body.1  He  gives the liberty to treat symptoms  by analysing doshas, rather as a disease in whole. The management of pitha vikaras should be with madhura, tiktha, kashaya, seethe upakrama  which includes snehana, virechana, pradeha, parisheka, abhyanga. ‘Virechanantu sarvakramebhya pithapradhanamanyante bhishaja’. 2   

Taking into account above perspectives all the internal and external drugs and therapies were selected  has madhura, tiktha, kashaya rasa and seetha snighdha guna.  As a symptomatic approach we started the treatment from the 1st day itself with  kashayadhara and   Ksheeradhara during the morning and evening. From the 3rd day onwards patient showed relief in burning sensation. Virechanam cleansed the body. This was followed by Snehapanam with pithahar ghritam  and subsequent virechanam helped to reduce the burning pain (visual assesement, scale of pain showed a reduction of score from 7 to 3 in 9th day). Redness of skin of feet, cheeks and palms also reduced completely. On the 14th day pain score reduced to zero and colour of skin became normal. No episode of epistaxis was reported during the  course of the treatment. The internal medicines which include Vasa swarasam  (agrayaoushada for rakta pita), guluchi, etc  have predominant pitta hara property which reduced  daha. Ksheeradhara with the inherent daha upasamana property of ksheera and additive tikta rasa drugs reduced the burning sensation substantially.  Dasamoola is well known for its anti-inflammatory and analgesic property. The medium of takra and ksheera helps in maintaining the seetha guna as well. The combined effect of these drugs helped in breaking the immunological reaction, removal of toxic substance from body, relieving pain and burning sensation and inflammation by controlling the circulation to extremities through the normalisation of vessels. Thus by clearly differentiating the symptoms, coalescing into the tridosha aspect helps to treat even the rarest of diseases through ayurveda.

Conclusion

The rare case of erythromelalgia  which has the very close resemblance to pitha vikaras  was successfully managed with pithaprasamana upakrama. The judicious and prudent know how of ayurveda helps in managing  this type of sparse and hard to treat condition in an effortless manner.

References

  1. Vaidya Yadvjitrikamji Acharya, Sree Chakrapani Virachita Ayurveda Deepika Vibhushita Charaka Samhitha-Chapter 20,  13
  2. Vaidya Yadvjitrikamji Acharya, Sree Chakrapani Virachita Ayurveda Deepika Vibhushita Charaka Samhitha-Chapter 20,  15
  3. http://rarediseases.org

Dr.A M Anvar ,BAMS, MD

Chairman and Managing Director ,

Punarnava Ayurveda Hospital , Kochi ,Kerala

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