Mohammad Reza Namazi

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206 ACTA DERMATOVENEROLOGICA CROATICAActa Dermatovenerol Croat 2011;19(3):206-211 LETTER TO THE EDITOREffective Treatment of Linear Darier’s Disease with Topical Retinoids: Case Report and Review of the LiteratureDarier’s disease is a rare genodermatosis caused by a defect in ATP2A2 gene located on chromosome 12q23-23 (1). This somatic mutation rarely occurs in the postzygotic phase of embryogenesis, leading to cutaneous mosaicism for the disease. The mosaic form of Darier’s disease is also called linear Darier’s disease and clinically presents with linear, zosteri-form or localized patterns located on Blaschko’s lines. A case of linear Darier’s disease successfully treated with topical 0.1% tretinoin therapy is presented.A 62-year-old male patient presented to derma-tology department for scaly eruption on the back for over a month. The patient had undergone Whipple’s operation (pancreatoduodenosplenectomy) for pan-creatic adenocarcinoma 3 months before. Dermato-logic examination revealed unilateral follicular hy-perkeratotic papules in an oblique linear configura-tion over the patient’s back. Some keratotic papules coalesced to form plaques, which were located on Blaschko’s lines (Fig. 1). The remaining dermatologic examination of the hair, nails and mucosa was unremarkable. A punch biopsy performed from one of the papules showed focal suprabasilar acantholysis accompanied by dys-keratotic keratinocytes both in the stratum spinosum and stratum corneum. Dyskeratotic cells in the stra-tum spinosum showed pyknotic nuclei and enlarged homogeneous eosinophilic cytoplasm, whereas ba-sophilic elongated nuclei were observed in dyskera-totic cells of the stratum corneum (Fig. 2). They were consistent with corps ronds and grains previously de-scribed in dyskeratotic dermatoses and particularly in Darier’s disease (2). With these histopathologic find-ings, the diagnosis of Darier’s disease was made. The clinical presentation composed of localized plaques parallel to Blaschko’s lines led to the definitive diag-nosis of linear Darier’s disease. Topical 0.1% tretinoin twice daily was prescribed and total improvement was accomplished after a month (Fig. 3).Darier’s disease is a rare autosomal dominant genodermatosis which is caused by a defect in AT-P2A2 gene on chromosome 12q23-23.1 (1). This gene’s product is a protein which functions as a sarco/endo-plasmic reticulum calcium pumping ATPase isoform 2 (SERCA2) and mutation of this protein leads to loos-ening of the cell-cell adhesions by causing defect in the formation or maturation of the tonofilament-des-mosome complex (3). The disrupted adhesion results in separation of the cells contributing particular focal suprabasilar acantholysis and dyskeratosis in kerati-nocytes known as corps ronds and grains histopatho-logically (2). The clinical findings in Darier’s disease include keratotic, crusted red-brownish papules dis-tributed over the seborrheic areas such as the trunk, scalp margins, face and lateral aspects of the face. The papules generally coalesce to form large verrucous plaques. Nearly half of the patients have flat, shiny warty papules on the dorsal aspects of the hands. Painless whitish papules may be observed in oral mu-cosa in 15% of patients (4). After reports of unilateral or zosteriform patterns in patients with Darier’s dis-ease, the synonyms acantholytic dyskeratotic epider-mal nevi, segmental Darier’s disease, linear Darier’s disease and localized Darier’s disease were started to be used for describing mainly the same localized clinical condition observed in Darier’s disease (5-7). In fact, 10% of patients with Darier’s disease are esti-mated to have linear or zosteriform pattern, localized on one-half of the body and linear Darier’s disease is accepted as a mosaic form of Darier’s disease (8,9).The reported treatment regimens for this rare mo-saic cutaneous disorder are few in the literature. Topi-cally applied adapalene was found to be effective by Cassals et al. (10). Short contact 0.1% tazarotene gel 207ACTA DERMATOVENEROLOGICA CROATICALetter to the editor Acta Dermatovenerol Croat2011;19(3):206-211Figure 1. Hyperkeratotic papules in linear and zosteriform configurations located on Blaschko’s lines over the back are seen on the left, and pinkish crusted papules seen on closer examination on the right.Figure 2. Focal suprabasilar acantholysis is indicated with arrows on the left; dyskeratotic cells in stratum spinosum with pyk-notic nuclei and enlarged homogeneous eosinophilic cytoplasm (corps ronds) are indicated with arrows, and dyskeratotic cells with basophilic elongated nuclei (grains) within stratum corneum are indicated with a circle on the right. (H&E x20 and x40 magnifications)for 6 weeks treated the disease successfully (3). In our experience, topically applied 0.1% tretinoin cream was also able to treat the lesions after twice a day ap-plication for 4 weeks. Tretinoin, also known as all-trans retinoic acid, is the acid form of vitamin A. In fact, all-trans retinoic acid is naturally formed by basal keratinocytes (11). Retinoids are believed to have antiproliferative ef-fects on keratinocytes modulating keratinocyte dif-ferentiation, normalizing abnormal differentiation, and down-regulating epidermal growth factor re-ceptors and hyperproliferative keratins. They bind to 208 ACTA DERMATOVENEROLOGICA CROATICALetter to the editor Acta Dermatovenerol Croat2011;19(3):206-211nuclear receptors RARs (retinoic acid receptors) or RXRs (retinoic X receptors) in cells and regulate gene expression by inhibiting transcription factors such as AP-1, which is a transcription complex controlling response to mitogenic injunctions (12). Retinoids also possess anti-inflammatory effects and they are shown to inhibit leukocytes, pro-inflammatory cy-tokine release and transcription factor expression (13). Topical retinoids are preferably used for local-ized cutaneous diseases for their low percutaneous absorption, rapid elimination and minor side effects. There are other local treatment options reported for Darier’s disease including 5-FU, cryotherapy and pho-todynamic therapy, which obviously are not equally cost effective when compared to short-term topically applied retinoids (14,15).Short periods of topically applied retinoids and retinoid analogues seem to be safe and effective for the treatment of linear Darier’s disease, which is etio-logically accepted as an inherited dyskeratotic mosaic cutaneous disorder. References1. Craddock N, Dawson E, Burge S, Parfitt L, Mant B, Roberts Q, et al. The gene for Darier’s disease maps to chromosome 12q23-24.1. Hum Mol Ge-net 1993;2:1942-3.2. Brazelli V, Prestinari F, Barbagallo T, Vassallo C, Agozzino M. Linear Darier’s disease successfully treated with 0.1% tazarotene gel “short-contact” therapy. Eur J Dermatol 2006;16:59-61.3. Munro CS, Cox NH. An acantholytic dyskeratotic epidermal nevus with other features of Darier’s di-sease on the same part of the body. Br J Dermatol 1992;127:168-71.4. Burge SM, Wilkinson JD. Darier-White disease: a review of the clinical features in 163 patients. J Am Acad Dermatol 1992;27:40-50.5. Starink TM, Woerderman MJ. Unilateral systema-tized keratosis follicularis. A variant of Darier’s disease or an epidermal naevus (acantholytic dyskeratotic epidermal naevus)? Br J Dermatol 1981;105:207-14.6. Vakilzadeh F. Case demonstration: segmental fol-licular dyskeratosis (Darier). Z Hautkr 1974;49:579-80. 7. Kellium RE, Haserick JR. Localized linear keratosis follicularis. Response to intralesional vitamin A and simultaneous occurrence of warty dyskera-toma. Arch Dermatol 1962;86:450-4. 8. Cockayne EA. Darier’s disease. In: Cockayne EA, editor. Inherited Abnormalities of the Skin and Its Appendages. Oxford: Oxford University Press; 1993. pp. 134-8.9. Happle R. A rule concerning the segmental ma-nifestation of autosomal dominant skin disorders: review of clinical examples providing evidence for dichotomous types of severity. Arch Dermatol 1997;133:1505-9.10. Casals M, Campoy A, Aspiolea F, Carrasco MA, Camps A. Successful treatment of linear Darier’s disease with topical adapalene. J Eur Acad Der-matol Venereol 2009;23:237-8.11. Prystowsky JH. Topical retinoids. In: Wolverton SE, editor. Comprehensive Dermatologic Drug Thera-py. Philadelphia: W.B. Saunders Company; 2001. pp. 578-94.12. Michel, S, Jombard A, Démarchez M. Pharmacolo-gy of adapalene. Br Assoc Dermatol 1998;139:3-7.Figure 3. Total improvement of the lesions accom-plished with 0.1% tretinoin cream after a month. 209ACTA DERMATOVENEROLOGICA CROATICA13. Wolf JE. Potential anti-inflammatory effects of to-pical retinoids and retinoid analogues. Adv Ther 2002;19:109-18.14. Yoon TY, Kim JW, Kim MK. Successful treatment of Darier disease with topical 5-fluorouracil. Br J Der-matol 2006;154:1210-2.15. Van’t Westeinde SC, Sanders CJ, Van Weelden H. Photodynamic therapy in a patient with Darier’s di-sease. J Eur Acad Dermatol Venereol 2006;20:870-2.Sibel Dogan1, Ayşen Karaduman1, Gul Erkin1, Ozay Gokoz21Department of Dermatology, 2Department of Pathology, Faculty of Medicine, Hacettepe Uni-versity, Ankara, TurkeyCorresponding author:Sibel Dogan, MDDepartment of DermatologyFaculty of Medicine, Hacettepe UniversitySihhiye 06100, AnkaraTurkey sibel.dogan@hacettepe.edu.trReceived: October 21, 2010Accepted: July 15, 2011Letter to the editor Acta Dermatovenerol Croat2011;19(3):206-211Generalized Pustular Psoriasis-Like Dermatophytosis  due to Trichophyton rubrumTrichophyton rubrum (T. rubrum) accounts for ap-proximately 90% of chronic superficial dermatophyte infections in human host (1). In particular, it is fre-quently implicated in tinea corporis caused by anthro-pophilic fungi, in which it can produce large annular lesions with a poorly defined border and erythema or scaling (2). In 1968, Ive and Marks (3) used the term “tinea incognito” for cases of epidermomycosis, erro-neously treated with topical steroids, having clinical manifestations that mimicked other skin conditions such as seborrheic dermatitis, lichen planus, folliculi-tis, and rosacea (1). We reported on a patient with generalized tinea incognito caused by T. rubrum, who presented two weeks after being cured by fluconazole, with pustular psoriasis-like generalized tinea corporis.A 56-year-old previously healthy woman presented to dermatology clinic (Faghihi Hospital, Shiraz, Iran) with a 2-year history of pruritic erythematous lesions involv-ing the left leg, back, shoulders, right arm and forearm. The lesions were treated erroneously as eczema in three other medical centers, and she received topical cortico-steroids, emollients and antihistamines. Her past medi-cal history did not reveal any significant disease. In terms of drug history, she did not use any drug on regular basis except for the above-mentioned drugs.On physical examination, erythematous, sharply demarcated lesions were observed on the above-mentioned areas of her body (Fig. 1). Other physical examinations showed nothing abnormal. Routine laboratory tests were within the normal limits. Micro-scopic examination of skin scrapings from the lesions with potassium hydroxide (KOH) revealed dermato-phyte hyphae. T. rubrum was detected in the culture of skin scrapings. A course of 4-week fluconazole (150 mg daily) was started. The patient did not present to our clinic for follow up.210 ACTA DERMATOVENEROLOGICA CROATICATwo weeks after ending the treatment course, the patient presented again to one of the above-men-tioned dermatologic centers. They suspected pustular psoriasis and decided to start therapy with methotrex-ate. Before starting the new treatment, the patient pre-sented to our dermatologic clinic. On physical exami-nation, previous lesions were found to have changed to well-demarcated and round plaques with scaling and pustules on the back, face, arms, and right leg (Fig. 2). KOH smear and fungal culture indicated the diag-nosis of T. rubrum dermatophytosis again, thus the pre-vious 4-week course of fluconazole was reintroduced. After four weeks, complete resolution of all lesions was observed. Six-month follow up was normal.Tinea corporis may be caused by either zoophilic fungi or anthropophilic organisms, less frequently by geophilic fungi (2). As a result of misdiagnosis or presence of pre-existing pathologies treated with local steroids, the clinical appearance of the lesions may mimic other skin conditions (1). Crawford et al. (4) report on not only corticosteroids but also new class topical non-steroidal medications including pimecrolimus and tacrolimus to have induced tinea incognito.Generalized dermatophyte infections of the skin are typically observed in immunocompromised pa-tients (e.g., patients receiving long-term immunosup-pressive therapy, patients with HIV infection or with underlying lymphoproliferative disorders) (5). There are a few descriptions in the literature of widespread tinea corporis in immunocompetent patients (6), but there are some reports of widespread and chronic Figure 1. Erythematous, sharply demarcated lesions on the leg and back, with active margin and scaling surface.Figure 2. Erythematous, well demarcated round plaques with scaling and pustules on the back and arm.Letter to the editor Acta Dermatovenerol Croat2011;19(3):206-211211ACTA DERMATOVENEROLOGICA CROATICALetter to the editor Acta Dermatovenerol Croat2011;19(3):206-211dermatophyte infections in atopics (2) and in patients with Cushing’s syndrome (7).The novel case described herein presented with pustular psoriasis-like generalized tinea corporis le-sions. Serarslan (8) reports on a patient with tinea in-cognito characterized by pustular inflammatory skin lesions after receiving steroid and emollient therapy, as in our patient. T. rubrum is a fungus that most fre-quently induces both widespread dermatophytosis and atypical features and may cause pustular reac-tion (2).IIkit et al. (9) report on plaques with erythema and papules in the neck and breast area due to T. rubrum in a 20-year-old female. She was treated with corti-costeroids for a long time. They conclude that tinea incognito could present in various clinical features, usually due to wrong treatment modalities. They sug-gest investigation of fungi in atypical erythematous plaques. Kastelan et al. (10) report on a 72-year-old woman treated unsuccessfully with topical steroid cream for 3 months. Multiple nummular scaly pap-ules and plaques were found on her arms and trunk. They describe circular and erythematous lesions with sharp demarcated and raised scaly edges. Finally, T. rubrum was cultured on Sabouraud’s agar. They sug-gest that in patients previously treated with steroids, disseminated scaly infiltrate lesions should be inves-tigated for fungal infection.Although we treated our patient with fluconazole, terbinafine remains a good first-line treatment be-cause T. rubrum is still highly sensitive to terbinafine (11). In conclusion, concerning clinical features of the case presented, dermatophyte infection may be con-sidered on differential diagnosis of generalized pus-tular dermatoses. References 1. Romano C, Maritati E, Gianni C. Tinea incognito in Italy: a 15-year survey. Mycoses 2006;49:383-7. 2. Gorani A, Schiera A, Oriani A. Case report. Wides-pread tinea corporis due to Trichophyton rubrum. Mycoses 2002;45:195-7. 3. Ive FA, Marks R. Tinea incognito. Br Med J 1968;3:149-52. 4. Crawford KM, Bostrom P, Russ B, Boyd J. Pime-crolimus-induced tinea incognito. Skinmed 2004;3:352-3. 5. Hoetzenecker W, Schanz S, Schaller M, Fierlbeck G. Generalized tinea corporis due to Trichophyton rubrum in ichthyosis vulgaris. J Eur Acad Dermatol Venereol 2007;21:1129-31. 6. Vittorio CC. Widespread tinea corporis in an im-munocompetent patient resistant to all conven-tional forms of treatment. Cutis 1997;60:283-5. 7. Canizares O, Shatin H, Kellert AJ. Cushing’s syn-drome and dermatomycosis. Arch Dermatol 1959;80:705-12. 8. Serarslan G. Pustular psoriasis-like tinea incognito due to Trichophyton rubrum. Mycoses 2007;50:523-4. 9. Ilkit M, Karakas M, Yuksel T. A tinea incognito case caused by Trichophyton rubrum with clinical and mycological cure and review of the literature. Mik-robiyol Bul 2010;44:149-53. (in Turkish)10. Kastelan M, Massari LP, Brajac I. Tinea incognito due to Trichophyton rubrum – a case report. Coll Antropol 2009;33:665-7.11. Diogo HC, Melhem M, Sarpieri A, Pires MC. Eva-luation of the disk-diffusion method to determine the in vitro efficacy of terbinafine against subcu-taneous and superficial mycoses agents. An Bras Dermatol 2010;85:324-30.Amir Feily1, Mohammad Reza Namazi2, Hami-dreza Seifmanesh31Infectious Research Center and Department of Dermatology, Jundishapur University of Medical Sciences, Ahvaz; 2Shiraz Skin Research Center, Shiraz University of Medical Sciences, Shiraz; 3Tehran University of Medical Sciences, Tehran, Iran Corresponding author: Mohammad Reza Namazi, MDDepartment of DermatologyFaghihi HospitalShirazIran namazi_mr@yahoo.comReceived: May 24, 2010Accepted: July 14, 2011

Generalized Pustular Psoriasis-Like Dermatophytosis due to Trichophyton rubrum

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