Guest guest Posted July 16, 2006 Report Share Posted July 16, 2006 Hailey Hailey Disease This comes under the category of Bullous skin disease as a type of pemphugus, meaning that it is a blistering condition with vesicles and bullae. The main TCM categories are Heart-Fire, Spleen Damp, Damp Accumulation. It is not the same as Bullous pemphigoid. Some authors also differentiate Hailey-Hailey Disease and 'true pemphigus'. This is because Hailey-Hailey is not an autoimmune disease, it is genetic, but I am note sure this is relevant in TCM terms. The integrity of the skin structure is compromised and fluid is excreted. This is most likely to occur in flexure areas of friction, and the the Dampness leads to erosion with the likelihood of secondary infection. As long as the Dampness is present, the erosion and damage will continue. It is important to understand that this is a Shi condition, not a Xu one, and therefore draining is required. However, the nature of the disease damages fluids, Spleen and Kidney over time. Management of acute phases with steroids and antibiotics is likely to be required. Ref Dermatology in TCM, Xu Yihou Qing Pi Chu Shi Yin Jia Jian Chu fu ling 15g Sheng di 15g Lian qiao 15g Yin chen hao 15g Cang zhu 10g Bai zhi 10g Mai dong 10g Ze xie 10g Zhi ke 10g Zhi zi 6g Huang qin 6g Chi xiao dou 30g Bai hua she she cao 30g Actions: Clear Heat and Transform Dampness Fortify the Spleen and Harmonise the Stomach Cool the Blood and Relieve Toxicity Enrich the Yin and protect Body Fluids Hailey-Hailey disease information Hailey-Hailey disease, sometimes called Œfamilial benign chronic pemphigus¹ is a rare hereditary blistering skin disease. The Hailey brothers first described it in 1939. Hailey-Hailey disease usually appears in the third or fourth decade, although it can occur at any age. It typically begins as a painful erosive skin rash in the skin folds. Common sites include the armpits, groins, and neck, under the breasts and between the buttocks. The lesions tend to come and go and leave no scars. As the lesions get bigger the centre clears leaving a typical ring shape. If the lesions are present for some time they may become thickened. The skin then tends to macerate leaving quite painful cracks. Secondary bacterial infection, which is not uncommon, can give rise to an unpleasant smell. White bands on the fingernails and pits in the palms can also occur. Heat, sweating and friction often exacerbates the disease, and most patients have worse symptoms during the summer months. Hailey-Hailey disease is a inherited skin disorder, although occasionally sporadic cases arise without a family history. The defect responsible has now been identified on a gene called ATP2C1 found on chromosome 3q21-24. This gene codes for the protein SPCA1 (Secretory Pathway Calcium/manganese-ATPase), a calcium and manganese pump. The skin cells (keratinocytes) stick together via structures called desmosomes and it seems the desmosomes do not assemble properly if there is insufficient calcium. The genetic defect in Hailey-Hailey disease causes the skin cells to become unstuck from one another. Normally the cells are packed together tightly in much the same way as bricks and mortar. Patients with Hailey-Hailey disease have defective ´mortar´ and the cells fall apart, like a dilapidated brick wall. Many patients have long remissions and an improvement with age does occur. Stefan Chmelik MRCHM MBAcC MATCM MBHMA DipCHM DipAc DipCHM (Dermatology) ClinAc (Nanjing) ClinCHM (Hangzhou) CST I/II Oriental Medicine Physician T: 07956 448085 stefan-herb www.newmedicinegroup.com Clinical appointments: New Medicine Group The Life Centre 144 Harley Street, W1G 7LE 15 Edge St, W8 7PN T: 07956 448085 T: 020 7221 4602 New Medicine Group ³There¹s more to healthcare than medicine² > <Chinese Medicine > > No Reply <notify-dg-Chinese Medicine > > 16 Jul 2006 11:40:17 -0000 > <Chinese Medicine > > Digest Number 1456 > > > There are 2 messages in this issue. > > Topics in this digest: > > 1a. Hailey Hailey disease > coastacu > 1b. Re: Hailey Hailey disease > Tom Verhaeghe > > > Messages > ______________________ > > 1a. Hailey Hailey disease > Posted by: " coastacu " coastacu coastacu > Sat Jul 15, 2006 1:29 pm (PDT) > > Hello All, > > I have a 29-year old female patient who has been diagnosed with Hailey > Hailey disease, an inherited skin disease. This disorder manifests with severe > redness, cracking, burning of skin in the folds of the body, or wherever > perspiration accumulates. My patient has exhibited these symptoms for the > last 7 years, under her arms, in the groin area, on the sternum. When I say > severe, I mean it. The skin under the arms resembles a very yin Xu tongue - > purply-red, severly cracked, oozing fluids. It is very prone to staph > infections, hence the frequent antibiotic and steroid use. She is otherwise > healthy, albeit frustrated, because she cannot run or exercise without greatly > exascerbating her skin situation. This recent weather, hot and humid, has been > terrible for her. > > Her pulse is thin and slightly rapid, tongue is red, with red dots at the > tip, raised edges, puffy, thin white coat, and a dip in the tip. She tends to > agitation (who wouldn't), and covers it all up with a huge smile. She has dark > shadows under her eyes. She had exerercise-induced asthma since the age of 14, > used an inhaler for years, now it is no longer a problem. (Aha? Perhaps the > pathogen has moved to a deeper level?) Periods are regular and normal. Her > bowels are very loose, with urgency. She is thin, moves rapidly, speaks > quickly, is very sensitive and intuitive. She is open to Chinese medicine as > nothing else has really had more than a palliative effect. My diagnosis is Ht > blood Xu, Lv blood xu, toxic heat in the blood, blood stasis, spQi xu. > > I have treated her 4 times and have given her Yin Care to use topically > 3x/day, along with the Kan formulation Drema Wind Release, xiao feng san. She > came in today looking guilty because she had to go to the emergency room, it > was so bad. They of course immediately gave her antibiotics and oral steroids, > along with topical steroid cream. > > This genetic defect occurs statistically in 1 in a million people. I would > love to help her. Does anyone have any ideas? > > Thankyou! > > Beata Booth > ______________________ > Check out AOL.com today. Breaking news, video search, pictures, email and IM. > All on demand. Always Free. > > > Quote Link to comment Share on other sites More sharing options...
Recommended Posts
Join the conversation
You are posting as a guest. If you have an account, sign in now to post with your account.
Note: Your post will require moderator approval before it will be visible.