Short th e r apeutic r epo r t Dermabrasion in treatment ofmorbus Darier K E Y WORDS Darier's disease, therapy, dermabrasion, deep Dermoabraswn - metlwd of clwice in treatment of morbus Darier Z. Periš S UMMARY Presented are patients with typical changes in morbus Darier, successfully treated with the method of deep abrasion. After experimental abrasions the regenerated epidermis revealed ali the characteristics of morbus Darier, while the regenerated epidermis after deep abrasion, when the regeneration resulted from remnants of epidermal adnexes, became completely normal both clinically and pathohistologically. Abrasion of large surfaces is performed under complete anesthesia. Morbus Darier is the only hereditary keratoderma in which after deep dermoabrasion the clinically and pathohistologically normal epidermis is regenerated and remains permanently unchanged. A highly probable explanation is that during the embriogenesis the epithelial adnexes had been formed before the pathological process characteristic far morbus Darier started. Introduction Morbus Da rie r or dyskeratosis follicularis is a spe- cial form of the clisturbance of the follicular keratini za- tion, which appears clinically on the skin, mucous mem- branes and nails. Approximately, it affects one to two persons in 100.000, and is predominantly inherited by anauto soma! do minant gene (1). Its genetic expres- sivity is variable, ancl often spontaneous mutations oc- cur. The deficiency of the tonofilament-desmosome complex is responsible for this anomaly. The creclit for the description of the disease belongs to Darier ancl White who had provicled it in 1889 (2,3). But since Darier hacl been the first to clescribe the pathohistological changes, his name is used as eponym for the disease. The beginning of this disease is gradual, and it occurs mostly between the ages of 10 and 15, appearing as keratotic follicul ar papu les of dirty brown color up to the length of 5 mm. With the removal of the pink plug or small warts, which also appear at times, there emerge warty vegetations emitting an unpleasant smeli. Patients also complain of itching. The rudimenta1y forms a re also clescribecl. The buccal mucous membrane, tongue, pharynx, la1ynx and rectum may also be involvecl. An abnormal function of T-cells as well as mencal retarcla- Acta Dermatoven APA Vol 10, 2001, No 3 ------- ---------- -------- --- ------ 111 Short therapeutic repo rt Figure 1. Male, 25 years, with characteristic manifestations of morbus Darier on the chest. Figure 2. The same patient on whom a test abrasion had been performed six months ago with completely regenerated normal skin (the smaller clear part) and with the second recent test abrasion (bloody area) Dermabrasion in treatment of morbus Darier tion and cystic changes in the bones are also mentioned. The nails display white or reddish streaks, thickenings or subungual keratosis . With tirne the symptoms may become more prominent. External influences, warmth and UV exposure may have a negative effect on the disease. Diagnosis is made clinically and pathohistolo- gically. In the epidermis, round the follicle , dyskera- totic cells are seen, usually in the stratum spinosum (corps ronds) or in the stratum granulosum (grains). With the electron microscopy the damaged tonofila- ment-desmosome complex is visible . By differential diagnosis should be excluded the seborrheic dermati- tis, acanthosis nigricans, pemphigus chronicus familiaris benignus (Hailey-Hailey) , hyperkeratosis follicularis et perifollicularis in cutem penetrans (morbus Kyrle). Ver- rucous lesions on the dorsa of hands and legs are de- scribecl by some writers as acrokeratosis verruciformis Hopf. Prognosis of the clisease is not favorable. Kera- tolytics , the retinoic acid (tretinoin) or corticosteroid preparations are used locally. Oral retinoids have a fa- vorable effect, unfortunately lasting only cluring the treatment ( 4). Patients and treatment Male, 25 years, with negative family anamnesis . In his case the changes began to appear at the age of 15 when he noticecl small brownish papules, first on the chest, that in the following years spread all over the trunk, they were fewer on the back than on the chest. He was unsuccessfully treatecl with various ointments until he receivecl Neotigason, w hich was effective, but only while he was taking it. Upon his admission to the clinic the trunk was coverecl with thickly disseminated brown- ish papules. They were rangecl in a follicular pattern, the size of millet grains (Figure 1). Incliviclual papules could also be observed on the proximal parts of upper arms. The lesions caused intense itching. His psychical state was normal. Two test dermoabrasions w ere pre- formed: one small six months ago revealing normally regeneratecl healthy skin (the clear, rouncl part), ancl a freshly abraclecl larger surface. (Figure 2). Female, 34 years, married. Family anamnesis negative. The clisease started at the age of 12 on the chest ancl the lateral parts of neck where minute papules appearecl ancl were slowly spreacling. The changes worsen in summer, especially during sun bathing. She was treatecl with vari- ous ointrnents and retinoids that were helping her, but only while she was taking them. Upon her aclmittance e1ythematous skin on her breasts, below ancl between them was visible, with numerous small papules of a red- Acta Dermatoven APA Vol 10, 2001, No 3 ----------------------------------113 Dermabrasion in treatment o{ morbus Darier clish color located follicularly. (F(gure 3). Incliviclual pap- ules have also been observecl on the lateral parts of her neck and below her navel. The psychic conclition was regular. In Figure 3 we can also observe the result of a test dermoabrasion performed 6 months earlier. Male, 34 years. His history contained the information that one of his uncles suffered from this clisease. In our patient the disease appeared in puberty on the chest, ancl at the age of 20 it spread over the en tire trunk reach- ing the inguinal region. (Figure 4). Moreover, smaller inclividual lesions developed on the dorsal parts of the lower extremities. He was treated with various ointments and Tigason orally that was of help until he was taking it. The changes causecl itching. The trunk was cliffusely covered with brownish minute papules, more to the fore ancl placed follicularly. Two smaller surfaces cov- ered with similar changes are displayed on the extensory parts on both shins . The nails on both hands were thick- ened with longitudinal streaks. A few papules could also be observed on the oral mucosa. According to the anam- nesis the patient's condition worsened considerably after a sunning speli. His psychic condition was regular. All the patients were completely examined to ex- clude possible contraindications concerning dermo- abrasion. After biopsy and pathohistological examina- tion, a test abrasion was first performecl uncler local anesthesia on the site of the most pronouncecl skin changes (Figure 1). The patients, seeing the results of abrasions after 5 to 6 months, agreed to the procedure. Naturally, large surfaces coulcl not be abraded under local anesthesia. The operation was performecl under general anesthesia at the surgical clinic. When the bleed- ing stopped, the abraded part was strewn with antibi- otic powder and left to dry in the air, regardless of the size of the planed surface. From under the crust, which cleveloped already the next day, epithelization developed quickly. The crusts began to fall off after ten days. Afi:er 6 months, in Figure 5, a border can be seen between the treated area ancl the limiting part that have not been abraded. It must be stressed that the patients with a large abraded surface sufferecl great pains during the post- operative period. The crust was cracking at the slight- est stir, sneezing, coughing and strain causing much pain to them. During the first week they were continu- ally receiving analgesics. After five to six months, when all the patients were checked, the abraded parts were clini- cally and pathohistologically completely normal. Discussion Curtin's technique of dermoabrasion, which in the world at large has attracted numerous followers, has Short therapeutic repo rt also been used for decades in Croatia. (5-11). It is based on the lasting regenerative ability of the skin. Full at- tention must be paid to prevent too deep abrasions since regeneration is possible starting from the remnants of the skin adnexes. The surface planing above the papil- lae does not cause bleeding. Bleecling starts when co- rium is reached where the superficial plexus of blood vessels is placed. If during abrasion fat droplets are noticed, it means that the subcutis has been reached. If, however, during abrasion epiclermal adnexes (seba- ceous and sweat glands, hair follicles) are removed, the defect does not regenerate, but is just repaired by scar- ring. Treatment of keratoderma is a great problem for any dermatologist, but even a greater one for the pa- tient, depending on the localization and spread of the disease. Great hopes were pinnecl on retinoids, but due to side effects the treatment will sooner or later have to be cut short. ( 4). Over the years we applied abrasion in numerous disorders of keratinization by always per- forming test abrasions on a limited surface where the pathological changes were most pronounced (ichtyosis, mal de Meleda, karatodermia figurata variabilis, psoria- sis vulgaris, etc.). Unfortunately, the results were dis- appointing, on the abraded surface the original disease with its typical changes kept reappearing. Satisfacto1y results cannot be achieved in any other keratoderma. That is why we were pleasantly surprised when by deep dermoabrasion a clinically ancl pathohistologically normal skin was obtained in Darier's disease. A highly probable explanation is that during the embriogenesis the epithelial adnexes had been formed before the pathological process characteristic for morbus Darier started. Those were patients in completely normal psy- chic conclitions who were firmly clecided to recover and regain their normal appearances. After the treatment their life accorcling to their own words acquired a higher quality. Now, even after 12 years, on the abraded parts no pathological changes are visible. Therefore we sug- gest that dermabrasion should be more widely used for treatment of morbus Darier. Conclusion Thme patient must be an aclult, in a period after twenty years of age that tends to regain his or her normal ap- pearance ancl has no psychic problems. The team per- forming dermabrasion must be protectecl (protective de- vices, masks, surgical gowns), since with the spouting of blood droplets and particles of abraded tissue, an infec- tion with AIDS or hepatitis may take place (12). 114 ~----------------------------------Acta Dermatoven APA Vol 10, 2001, No 3 Short therapeutic repo rt Figure 3. Female, 34 years, with a performed test abrasion and normal regenerated ski after six months. Figure 4. Male, 34 years, with characteristic changes of morbus Darier from the neck to inguinal regions Figure 5. The same patient after six months. The limit of the abraded fronta! part with completely regenerated normal skin, and the posterior stili non-abraded part on which the changes are less expressed. Dermabrasion in treatment of morbus Darier Acta Dermatoven APA Vol 10, 2001, No 3 ------------ - - ------------------ - ~ 1 JJ Dermabrasion in treatment ofmorbus Darier Short therapeutic repo rt R E F E H E N C E S l. Milj kovic J, Kecelj N, Balkovec Vet al. Darier's disease in Slovenia. Acta Dermatoven APA 2000; 9: 10-7. AUTHOR'S ADDRESS 2. Darier J. De la psorospermose vegetante. Ann Dermatol 1889; 10: 597. 3. White JC. A case of keratosis (ichtyosis) follicularis. J Cutan Genito Urin Dis 1889; 7: 201. 4. Saurat}H. Side Effects of sytemic Retinoids and theri Clinical Management. J Am Acad Derrnatol 1992; 27: 523-528. 5. Curtin A. Corrective surgical planin of Skin. Arch dermatol 1953; 68-389. 6. Vukas A. Brušenje kože u dermatologiji. Llječ Vjesn 1960; 32:315-321. 7. Periš Z. Gligora M. Morbus Bourneville-Pringle. Medica Iadertina 1976; 3-4: 31. 8. Periš Z. Dermabrasion in Darier's disease. Acta Derm lug 1991; 4:223. 9. Periš Z. Dermabrasion in a girl with congenital poikiloderma. Acta dermatovenerologica APA 1997; 275-77. 10. Periš Z. How we treat Rhinophyma? Acta Dermatoverol. Croat 1999; 7 (1): 25-27. 11. Periš Z. Corrective abrasion of solar elastosis facial skin. Acta Dermato venerol Croat 1999; 7(3) , 117-120. 12. Wentzell JM, Robinson JK, Schwartz DE, Carlson SE. Pcysical properties of Aerosols Produced by Dermabrasion. Arch Dermatol 1989; 25: 1637-43. Zdravko Periš MD, PhD, jull projessor oj dermatology, S. Krautzeka 92 b, 51000 Rijeka, Croatia 116 - - --------------- - - -------- ----- Acta Dermatoven APA Vol 10, 2001, No 3