Rosacea is a very common skin disorder in the clinical practice that primarily affects the convex areas of the face. Extrafacial rosacea lesions have occasionally been described, but extensive involvement is exceptional. In the absence of its typical clinical or histological features, the diagnosis of extrafacial rosacea may be problematic. We describe an unusual case of rosacea with very exuberant extrafacial lesions, when compared with the limited involvement of the face

Pereira, TM

Sousa-Basto, A

Vieira, AP

English

Institutional repository of Hospital de Braga

 International Journal of Dermatology  2008,  47 , 52–55 © 2008  The International Society of Dermatology 52 Abstract Rosacea is a very common skin disorder in the clinical practice that primarily affects the convex areas of the face. Extrafacial rosacea lesions have occasionally been described, but extensive involvement is exceptional. In the absence of its typical clinical or histological features, the diagnosis of extrafacial rosacea may be problematic. We describe an unusual case of rosacea with very exuberant extrafacial lesions, when compared with the limited involvement of the face. Blackwell Publishing LtdOxford, UKIJDnternational Jour al of Dermatology0011-9059© 2007 The Internati nal Society f DermatologyXXXCase report Extrafacial rosacea; RosaceaPereira  et al.Case reportRosacea with extensive extrafacial lesions Teresa M. Pereira, Ana Paula Vieira, and A. Sousa Basto From the Department of Dermatology and Venereology, Hospital de São Marcos, Braga, Portugal Correspondence Teresa M. Pereira,  MD Department of Dermatology and Venereology Hospital de São Marcos Apartado 2242 4701-965 Braga Portugal E-mail: teresa-per@netcabo.pt Introduction Rosacea is a skin disorder frequently observed in the clinicalpractice. It is characterized by the primary involvement ofconvex areas of the face. 1  However, a wide spectrum of clin-ical findings is often observed. 2  We describe an unusual caseof rosacea with exuberant extrafacial involvement. Case Report A 53-year-old man presented with an extensive exudativeexanthema consisting of papules and erythematous nodules,pustules, and large granulomatous, nodulo-cystic lesions involv-ing the face, neck, and upper chest, and extending from theleft shoulder along the whole left arm (Fig. 1). The dermatosisrelatively spared the central region of the face, being moreexuberant in its lateral aspects and ears. On the upper half ofthe chest and the left arm (external aspect) the lesions weredensely confluent, very inflammatory and exudative, with acrusty surface (Fig. 2). The lesions were pruritic only after sunexposure. The dermatosis had about 6 years of evolution,always localized to the face. The patient reported exacerba-tion of the lesions, which extended to the chest and left arm,in the past 2 months, following a period of mourning for thedeath of a member of his family. At this time, he was medi-cated with oral prednisolone, with transitory improvement ofthe lesions followed by severe exacerbation. Subsequently,admitting the clinical diagnosis of pustular dermatophytosis,the patient was medicated with itraconazol, with continuedworsening of the cutaneous lesions. He had no other patho-logical antecedents of interest and the remaining physicalexamination was normal.Bacteriological and mycological tests of the contents ofthe pustules were negative. Baseline investigations, includingcomplete blood count, liver and renal functions, autoimmunescreen, serology for human immunodeficiency virus, and urinebromides and iodides levels were negative or normal. Photo-testing with ultraviolet A (100 J/cm 2  daily) and ultraviolet B(1,5 MED daily) failed to induce the clinical lesions. Histologicalexamination of a lesional biopsies fromy, the face and upperarm, revealed the same features: slight irregular acanthosis,polymorphous inflammatory infiltrate, and formation ofepithelioid granulomas in the superficial dermis, and concomi-tant inflammation of the acute type with formation of follicularpustules (Fig. 3). These findings were consistent with the clinicaldiagnosis of rosacea. The presence of  Demodex folliculorum was not noticed.The patient was treated with deflazacort 30 mg (3 weeks),azithromycin (500 mg, 3 days a week, for 4 weeks), andisotretinoin (10 mg daily, for 1 year, with gradual reductionof the dose in the last months). He was instructed to avoidsun exposure, and a titanium dioxide- and zinc oxide-basedsunscreen was prescribed. Gradual resolution of the lesionsoccurred. One year later, there was still some facial erythema,but complete resolution was achieved on the chest, left shoulderand arm (Fig. 4). Comment Usually rosacea is a straightforward diagnosis. However, inthe absence of its typical clinical or histological features, thediagnosis of extrafacial rosacea may be problematic. Initially,the clinical differential diagnosis suggested were the manysubtypes of infectious folliculitis such as dermatophytosis, © 2008  The International Society of Dermatology International Journal of Dermatology  2008,  47 , 52–55 53 Pereira  et al. Extrafacial rosacea; Rosacea Case report bacterial folliculitis, demodicosis or inflammatory folliculitis,namely, actinic superficial folliculitis, eosinophilic pustularfolliculitis, iododerma, or bromoderma. The diagnosis ofinfectious folliculitis and eosinophilic pustular folliculitis wasexcluded by the clinical history, bacteriological and mycolog-ical tests, and biopsy. Rare patients with actinic superficialfolliculitis develop sterile pustules 24–36 h following intensesunlight exposure, with good response to isotretinoin. 3,4  Onthe contrary, the dermatosis described in our patient showedgradual progression with asymmetric distribution, was nottriggered or maintained by sunlight or heat (just caused prurituswith sun) and, inclusively, the phototesting was negative. Thepatients with halogenoderma (iododerma or bromoderma)tend to develop indurated pustulonodular lesions on the faceand extremities, after longer exposure to higher amount ofiodides or bromides. Our patient denied ingestion of medicationsand a measure urine bromides and iodides level was negative.Figure 1 Clinical features of the lesions, with extensive extrafacial involvementFigure 2 Detail of the lesions on the left forearmFigure 3 Formation of follicular pustules (hematoxylin and eosin, ×100)Figure 4 Complete resolution of the lesions after one year of treatment with isotretinoin International Journal of Dermatology  2008,  47 , 52–55 © 2008  The International Society of Dermatology 54 Case report Extrafacial rosacea; Rosacea Pereira  et al. Therefore, in this case, the presence clinically of inflammatorypapules, pustules, and nodules on a congestive background,with spread from the typical midfacial involvement to otherareas of the body, and the presence of poorly organized epi-thelial granulomas and follicular pustules in the biopsy allowthe diagnosis of extrafacial rosacea.Rosacea is a common skin disorder that is generally local-ized to the face, more frequently affecting the nose, cheeks,chin, forehead, and glabela, typically sparing the periocularand perioral regions. 2  The presence of rosacea with extrafaciallocation has been occasionally reported. 5–7  Extrafacial lesionsusually complicate the more serious cases of rosacea but,occasionally, as was the case of our patient, they may accom-pany slight facial lesions. Involvement of the lateral contoursof the face, ears, scalp (particularly in old patients), and neckoccurs mainly in erythematotelangiectatic rosacea. 2  Theseextrafacial manifestations are relatively uncommon andgenerally arise in areas of chronic solar lesions or flushing. 8 Involvement, by acneiform-like lesions, of the chest, axillae,shoulders, arms, and legs (thighs and knees) is described in theliterature, but is quite exceptional and generally occurs in thecontext of the granulomatous variant of rosacea, 5,9,10  as observedin our patient.Granulomatous rosacea is a variant characterized bypapules and monomorphic nodules with erythematous orbrownish tonality, generally with periorificial location. 1  Usually,the patient does not have persistent facial erythema. 10  In ananatomoclinical review of 53 cases of granulomatous rosa-cea, extrafacial lesions were found in 15% of the patients. 11 The authors noticed the presence of those lesions mainly inareas rich in apocrine and pilosebaceous glands, which ledthem to the conclusion that there is a relationship betweengranulomatous rosacea and other acneiform eruptions, suchas supurative hidrosadenitis. 11 The cause for the formation of granulomas in rosacea isstill unclear. They arise as local macrophage proliferation inresponse to a persistent antigen, hence, the suggestion by manyauthors as to the pathogenic role of  Demodex folliculorum . 12 Ayres, 9  for instance, considers extrafacial rosacea to be causedby a disseminated infestation by  D. folliculorum , normallyinhabiting follicles and sebaceous glands. However, follicularinflammation may be observed in the absence of parasites,and parasites may be observed without associated inflamma-tion. 5,10  A limitative factor in these studies has to do with thefact that  D. folliculorum  is not easily detected in histologicalpreparations: the skin biopsy only shows the parasites thatreside superficially in the follicle, but not those located deeperin the follicle or the parasite of  Demodex brevis  residing insebaceous glands. 2 Concerning the treatment, we opted for the use of very lowdoses of isotretinoin (10 mg/day), regardless of body weight,for a long period, with the gradual reduction of the dose to 3–5 days a week. Some studies have established the safety andefficacy of this type of therapeutic approach in difficult casesof rosacea. 13,14  In the long term, cumulative doses above1075 mg/kg have not caused significant radiological changes. 15 In our patient, the annual cumulative dose is, on average,much lower than that (i.e. 60 mg/kg). The benefit of concom-itant short-term corticotherapy has been reported, particularlyfor granulomatous rosacea. 10  Thus, we also combinedisotretinoin with oral corticoids in the first 3 weeks oftreatment with good results. In the first month of treatmentwe also used, azithromycin 3 days a week, in accordance withrecent studies that demonstrated the efficacy and safety of thisantibiotic in the management of rosacea. 16,17 Reports of extrafacial rosacea are rather scarce. However,it may be more common than it is thought, because it is gen-erally not considered and/or not researched. We presented anunusual setting of extrafacial rosacea that is remarkable forthe exuberant and extensive involvement, when comparingwith facial involvement, the initial diagnostic difficulty andthe good therapeutic results obtained. This case leaves a numberof questions for further study, namely, the potential of extra-facial symptoms in rosacea, the sensitivity to sunlight, the psy-chogenic factors, and the hypothetical role of  D. folliculorum . References 1 Wilkin J, Dahl M, Detmar M,  et al . Standard classification of rosacea: report of the National Rosacea Society Expert Committee on the Classification and Staging of Rosacea.  J Am Acad Dermatol  2002;  46 : 584–587.2 Crawford GH, Pelle MT, James WD. Rosacea: etiology, pathogenesis and subtype classification.  J Am Acad Dermatol  2004;  51 : 327–341.3 Norris PG, Hawk JL. Actinic folliculitis – response to isotretinoin.  Cli Exp Dermatol  1989;  14 : 69–71.4 Jaeger C, Hartschuh W, Jappe U. Actinic superficial folliculitis.  J Eur Acad Dermatol Venereol  2003;  17 : 562–565.5 Marks R, Jones EW. Disseminated rosacea.  Br J Dermatol  1969;  81 : 16–28.6 Wilkin JK. Epigastric rosacea.  Arch Dermatol  1980;  116 : 584.7 Dupont C. How common is extrafacial rosacea? (letter).  J Am Acad Dermatol  1986;  14 : 839.8 Gajweska M. Rosacea on common male baldness.  Br J Dermatol  1975;  93 : 63–66.9 Ayres S Jr. Extrafacial rosacea is rare but does exist.  J Am Acad Dermatol  1987;  16 : 391–392.10 Plewig G, Kligman AM.  Acne and Rosácea , 3rd edn. Berlin, Germany: Springer-Verlag, 2000: 456–503.11 Helm KF, Menz J, Gibson LE,  et al . A clinical and histopathologic study of granulomatous rosacea.  J Am Acad Dermatol  1991;  25 : 1038–1043.12 Forton F. Démodex et inflammation périfolliculaire chez l’homme: revue et observation de 69 biopsies.  Ann Dermatol Venereol  1986;  113 : 1047–1058. © 2008  The International Society of Dermatology International Journal of Dermatology  2008,  47 , 52–55 55 Pereira  et al. Extrafacial rosacea; Rosacea Case report 13 Erdogan FG, Yurtsever P, Aksoy D,  et al . Efficacy of low-dose isotretinoin in patients with treatment-resistant rosacea.  Arch Dermatol  1998;  134 : 884–885.14 Hofer T. Continuous “microdose” isotretinoin in adult recalcitrant rosacea.  Clin Exp Dermatol  2004;  29 : 196–205.15 Ling TC, Parkin G, Islam J,  et al . What is the cumulative effect of long-term, low-dose isotretinoin on the development of DISH?  Br J Dermatol  2001;  144 : 630–632.16 Bakar O, Demircay Z, Gurbuz O. Therapeutic potential of azithromycin in rosacea.  Int J Dermatol  2004;  43 : 151–154.17 Fernandez-Obregon A. Oral use of azithromycin for the treatment of acne rosacea.  Arch Dermatol  2004;  140 : 489–490.

Rosacea with extensive extrafacial lesions

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Rosacea with extensive extrafacial lesions

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