Conjluent and reticulate papillomatosis (Gougerot-Carteaud) Conjluent and reticzilate papillomatosis (Gougerot-Carteaud) R. Fink-Puches, J. Smolle and HP. Soyer SUMMARY A 17 year-old girl with the diagnosis of confluent and reticulate papillomatosis (CRP) is presented. Clinical and histiopathologic differential diagnostic considerations as well as the pathogenesis of CRP are discussed. Introduction Confluent ancl reticulate papillomatosis (CRP) was first clescribecl by Gougerot ancl Carteaucl in 1927 (1). It consists of grayish-brown pigmentecl papules that later coalesce, most often localizecl in the intermammary ancl interscapular regions, neck ancl abdomen. It usually starts after puberty, primarily in females (2). The etiology remains unclear but many observations are in favor of the role of Pityrosporum orbiculare in CRP (3). A case of confluent ancl reticulatecl papillomatosis responcling to treatment with minocycline is reportecl. Case report A 17-year-old girl with asymptomatic, brownish, papules that coalesced into sharply circumscribed plaques in the nuchal ancl interscapular region was admitted to our Department (Figure 1). Similar skin lesions were found to a smaller extent in the sternal region. The first clinical cliagnosis was atypical pityriasis versicolor; further differential diagnosis included acanthosis nigricans. Histopathogy of a biopsy specimen from the nuchal region showed basket-wave hyperkeratosis, acanthosis ancl papillomatosis and a sparse, preclominantly lym- phocytic infiltrate around the superficial vascular plexus (Figure 2a). Closer scrutiny revealecl foci of numerous small spores in the cornifiecl layer clearly visible already with hematoxylin-eosin staining (Figure 2b). The most probable histopathologic diagnosis was seborrhoic keratosis; further clifferential cliagnosis consiclerations were acanthosis nigricans ancl epiclermal nevus. How- ever, ali these histopathological differential cliagnoses were not consonant with the clinical cliagnosis proviclecl on the case report slip, namely, atypical pityriasis versi- color. Further investigations: The mycological examination from the nuchal re- gion revealed numerous spores but no hyphae thus Case repo r t K E Y WORDS confluent and reticulate papillomatosis, Pityrosporum orbiculare, pityriasis versicolor, tetracyclines 28 acta dermatovenerologica A.P.A. Vol 8, 99, No 1 Confluent and reticulate papillomatosis (Gougerot-Carteaud) ruling out pityriasis versicolor, because microscopy of the scales of pityriasis versicolor always reveals thick- walled sphe rical yeast forms and coarse septate mycelium often broken up into short filaments. Also, PAS-staining of the biopsy specimen revealed spores but no hyphae within the cornified layer. So, the diag- nosis confluent and reticulated papillomatosis Gouge- rot-Carteaud (CRP) was made. Previous treatment with antifungal agents was in- effective. Thus, treatment with minocycline (l00mg/ daily) was started. The patient experienced significant improvement after 3 weeks and 6 weeks later her skin was completely clear. Discussion The diagnosis of CRP in our patient has been establi- shed by the typical clinical aspect, namely slightly ve- rrucous brownish papules and confluent plaques in the nuchal and interscapular region in addition to the histopathologic findings - basket-wave hyperkeratosis, acanthosis and papillomatosis and cluster of spores in the cornified layer. So, CRP is a typical example in which the definite diagnosis is achieved by clinico-pathologic correlation. Based on a review of 48 cases reported in the literature from 1978 to 1992 Lee et al. defined CRP by the following criteria: i) hyperpigmented papules and plaques involving at least the central portion of the chest or back with central confluence and peripheral reticu- lation; ii) no hyphae demonstrable, either by performing mycological examination or by conventional light microscopy of a biopsy specimen; and iii) basket-wave hyperkeratosis, acanthosis and papillomatosis and a sparse, superficial perivascular infiltrate histopatho- logically (4) . The most probable clinical diagnosis in our patient was pityriasis versicolor. The diagnosis of pityriasis versicolor, however, as a rule, is based on the myco- logical examination revealing spherical, thick-walled spores and hyphae that are often fragmented to short filaments. Histopathologically, in pityriasis versicolor the prominent basket-wave stratum corneum is situated above a rather normal epidermis. Also, in pityriasis versicolor short hyphae are always present together with spores in the cornified layer. Therefore , the clinical diagnosis of p ityriasis versicolor in our patient has been excluded by the mycological and histopathologic examination . The exact pathogenesis of CRP remains unknown. On the one hand it was thought that CRP represents an abnormal bost reaction to Pityrosporum orbiculare (ovale) and therefore treatment with topical and sys- temic antifungal agents is carried out with variable success. The mechanism by which the Pityrosporum 30 Fig. 1. Confluent and reticulated papillomatosis in the nuchal region of a 17-year-old girl. Fig. 2a. Hyperkeratosis, acanthosis and papillomatosis and a sparse lymphocytic infiltrate arround the superficial vascular plexus (Hematoxylin-eosin stain< x 100). Cas e repo rt acta dermatovenerologica A.P.A. Vol 8, 99, No 1 Ca se r e p o rt -- Confluent and reticulate papillomatosis (Gougerot-Carteaud) yeasts induce the skin changes are not known (3) . Direct lipase activity or antibody-mediated epidermal damage are discussed. On the other hand CRP is regarded as a benign disorder of keratinization and thus drugs with an "antikeratinizing effect" such as retinoids have been used more or less effectively to clear this condition (5) . Our patient was successfully treated with minocycline (l00mg/die over 6 weeks) . The basis far the efficacy of minocycline is uncertain. This agent is effective against gram-positive and gram-negative bacteria and some mycobacteria, suggesting that a yet unknown bacterium ' plays a role in the pathogenesis of CRP. Fig. 2b. Small spores in the cornified layer (Hematoxylin-eosin stain< x 250) REFERENCES AUTHORS' ADDRESSES l. Gougerot H, Carteaud A. Papillomatose pigmentee innominee. Bul! Soc Fr Dermatol Syph 1927; 34: 719-21. 2. Broberg A, Faergemann]. A case of confluent and reticulate papillomatosis (Gougerot-Carteaud) with an unusual location. Acta Derm Venereol (Stockh) 1987; 68: 158-60. 3. Faergemann J. Lipophilic yeasts in skin diseases. Semin Dermatol 1985; 4: 173-84. 4. Lee MP, Stiller MJ, McClain STA, Shupack JL, Cohen DE. Confluent and reticulated papillomatosis: Response to high-dose oral isotretinoin therapy and reassessment of epidemiologic data. J Am Acad Dermatol 1994; 31: 327-31. 5. Baalbaki SA, MalakJA, Al-Khars MA. Confluent and reticulated papillomatosis. Treatment with etretinate. Arch Dermatol 1993; 129: 961-3 Regina Fink-Puches, MD, Department oj Dermatology, University oj Graz, Auenbruggerplatz 8, A-8036,Austria Josej Smolle, MD, associate projessor oj dermatology, same address H Peter Soyer, MD, associate projessor oj dermatology, same address acta dermatovenerologica A.P.A. Vol 8, 99, No 1 ----- ------------------------- 31