Confluent and reticulated papillomatosis of Gougerot and Carteaud: report of three cases

Abstracts

A papilomatose confluente e reticulada de Gougerot e Carteaud é dermatose rara, de etiologia obscura. Há diversas opções terapêuticas - retinóides tópicos e sistêmicos, antibióticos orais, entre outros - a maioria com resultados discretos. Os autores descrevem três casos da doença, em adultos, dois do sexo feminino e um do sexo masculino, com lesões características - pápulas que se tornavam confluentes no centro e reticuladas na periferia, formando placas de limites imprecisos, ligeiramente descamativas - e discutem aspectos etiológicos e terapêuticos da doença.

Dermatopatias; Dermatopatias; Hiperpigmentação; Hiperpigmentação; Papiloma; Papiloma; Terapêutica

CASE REPORT

Confluent and reticulated papillomatosis of Gougerot and Carteaud: report of three cases

Leonardo Mello Ferreira^I; Lucia Martins Diniz^II; Carlos Jaques Mazzei Ferreira^III

^IGraduate Studies under course, Service of Dermatology, EMESCAM

^IIAssistant Professor, Service of Graduate Studies in Dermatology, EMESCAM. Specialist in Dermatology, Brazilian Society of Dermatology

^IIISpecialist in Dermatology, Brazilian Society of Dermatology

Mailing Address

ABSTRACT

Confluent and reticulated papillomatosis of Gougerot and Carteaud is a rare dermatosis of unknown etiology. There are numerous therapeutic options - topical retinoids and systemic retinoids, oral antibiotics, etc, and most present poor results. The authors describe three cases of the disease in two female and one male adult subjects, with characteristic lesions- papules become confluent in the center and reticulated in the periphery- and discuss etiological and therapeutical aspects of the disease.

Keywords:Skin diseases; Skin diseases/etiology; Hyperpigmentation; Hyperpigmentation/diagnosis; Papilloma; Papilloma/pathology; Therapeutics

INTRODUÇÃO

Confluent and reticulated papillomatosis (CRP) was described by French dermatologists Gougerot and Carteaud in 1927.¹ The etiology remains obscure, but the main hypotheses that try to explain its onset are keratinization disorder or abnormal response to skin biota microorganisms, which is caused, in this case, by lipophilic yeast genus Malassezia. ^2,3

It is a rare dermatosis which is more frequently found in women and melanodermic subjects, with higher incidence between the ages of 10 and 35 years.

Subjects with the disease have a trend to obesity and seborrheic skin ¹. Some authors have tried to associate it with endocrinopathies or atopia. There were reports of some cases in siblings, but they seem to be sporadic. 4 It is clinically characterized by rugosity, flat, not very salient papules, of variable color, hypochromic, pinkish or light brow, measuring up to 5mm. Papules become confluent in the center and reticulated in the periphery, forming plaques of imprecise limits, slightly desquamative. Papules may take a pseudo-atrophic aspect in some rare occasions. Lesions are located in inter-scapular, epigastric and intermammary regions. In more extensive cases, they may affect the back of the neck, lateral neck region, shoulders, axilla and pubic region ^1,2.

Subjects with the disease have a trend to obesity and seborrheic skin ¹. Some authors have tried to associate it with endocrinopathies or atopia. There were reports of some cases in siblings, but they seem to be sporadic. ⁴ It is clinically characterized by rugosity, flat, not very salient papules, of variable color, hypochromic, pinkish or light brow, measuring up to 5mm. Papules become confluent in the center and reticulated in the periphery, forming plaques of imprecise limits, slightly desquamative. Papules may take a pseudo-atrophic aspect in some rare occasions. Lesions are located in inter-scapular, epigastric and intermammary regions. In more extensive cases, they may affect the back of the neck, lateral neck region, shoulders, axilla and pubic region ^{1, 2}.

The main differential diagnosis is with versicolor pityriasis, but others should also be considered: acanthosis nigricans, cutaneous amyloidosis, different keratinization disorders, and some forms of seborrheic dermatitis ^{1, 2}.

Histopathological examination is not very characteristic and there are subtle affections, correlated with clinical presentation of hyperkeratosis, normally orthokeratotic, irregular papillomatosis, acanthosis predominantly in interpapillary areas and hypogranulosis. There may also be focal atrophy of stratum spinosum, hyperpigmentation of basal layer, without affection to number of melanocytes. On the dermis, there may be chromatophores on the papillary region, discreet mononuclear superficial perivascular infiltrate, mild ectasis of vessels, and small to moderate papillary edema ^1,2,4-6.

The histopathological differential diagnoses include acanthosis nigricans, variants of seborrheic keratosis, non-inflammation epidermal nevus, Dowling-Degos disease and Becker nevus ⁴.

Electron microscopy shows increase in transitional cell layer between stratum granulosum and stratum corneum. This fact may define CRP as a disorder of keratinization of skin focal area, without defining whether it would be primary or secondary ⁶. There are different therapeutic approaches, such as topical agents (keratolytic, agents derived from vitamins A and D, antimicrobials) and systemic agents (antibiotics and retinoids), but most present inconsistent outcomes ³.

CASE REPORT

Case 1: Female 25-year-old African descendant, married, housewife, living in Serra, state of Espirito Santo (ES). Two years before, she noticed hypochromic and hyperchromic spots, slightly desquamative, on the mentalis region, neck and anterior and posterior regions of the chest (Figures 1 and 2).

She had undergone different treatment approaches for versicolor pityriasis with systemic antifungal agents (ketoconazole, itraconazole, fluconazole) and topical antifungal, such as selenium sulfite, without favorable outcomes.

After the diagnostic hypothesis of CRP was made, desquamation from the chest spots was collected and analyzed under direct mycological exam, whose result was negative. The analysis of the skin under Wood's glass did not show fluorescence and the histopathological exam showed few non-specific affections, such as compact and focal hyperorthokeratosis, spongiosis and mild acanthosis (Figure 3).

Case 2: Female 22-year-old Caucasian woman, single, clerk, living in Rio Bananal, state of ES. Since the age of 16, she had presented asymptomatic lesions on the trunk, with aspect of light brown or hypochromic plaques after desquamation, with improvement and worsening periods (Figures 4 and 5).

Histopathological exam of the lesions showed epidermis with hyperkeratosis and elongation of interpapillary cones. On the upper dermis, there was presence of scattered inflammatory lymphohistiocytarian infiltrate and some perivascular melanophages.

Case 3: Male 25-year-old African descendant patient, single, farm worker, living in Jaguare - state of ES. Since the age of 20, he had presented some lesions distributed on the trunk, anterior and posterior regions, forming a V shape, of keratotic aspect, light brown color, alternating with hypochromic maculae after desquamation. Histopathological exam of the lesion showed epidermis with hyperkeratosis, hypogranulosis, acanthosis and upper dermis with mild lymphohistiocytarian inflammatory infiltrate.

The three cases had lesions and progression compatible with the diagnosis of Gougerot and Carteaud CRP. Case 1 started treatment with glycolic acid at 12% cream, and improvement was observed 3 months later. Case 2 has been taking oral 20mg isotretinoin for 2 months, with improvement of the case, and case 3 is not taking any treatment.

DISCUSSION

Gougerot and Carteaud CRP is probably an underdiagnosed affection ⁴, amounting to a total of hundred cases reported in the literature, including those three of the Brazilian publication. Confirming literature data, the mean age of patients with the dermatosis in the three cases was 20 years, two were African descendants and had bone mass index higher than 25, were female and presented seborrheic skin. Conversely, there was no concomitant disease in any of the three cases.

Many questions still persist about this dermatosis, especially in relation to etiology and management. Some hypotheses try to explain CRP etiology as vitamin A deficiency, genetic factors, photosensitivity, endocrine abnormalities, cutaneous amyloidosis, tissue reaction to skin colonization by lipophilic yeasts genus Malassezia, Staphylococcus or Propionibacterium acnes, and above all, keratinization defects ^{4, 7-9}.

As the etiopathogenesis remains unknown, there are many different proposed treatment approaches, such as oral antibiotics (minocycline, doxicycline, fusidic acid, clarithromycin, erythromycin and azythromycin), selenium sulfite, calcipotriol, radiotherapy, ultraviolet light, thyroid extract, 5-fluorouracil, hydroquinone, progesterone, coal tar, salicylic acid, urea, liquid nitrogen, dermoabrasion, topical retinoids (retinoic acid and tazarotene) and systemic retinoids (isotretinoin, etretinate) ^{3, 7}.

Response to retinoids support the theory of a disease caused by keratinization disorder. The use of oral isotretinoin 1-2 mg/Kg/day has been advocated and produced some favorable outcomes within two months. Patients in cases 2 and 3 were initially treated with oral and topical antifungal agents and presented partial improvement within the first 15 to 30 days. The same clinical improvement was observed by Shimizu et al., favoring the hypothesis of the existence of an infectious agent that triggers the process. It would also explain the good response obtained with the use of oral minocycline (200 mg/day for 10 weeks, reaching a response within 2 weeks), even though the antianabolic and antiinflammatory effects of tetracyclines may contribute to improve the clinical presentation ^{7, 8}. It seems reasonable to assume that Gougerot and Carteaud CPR have multifactorial etiopathogenesis, similarly to other diseases such as acne vulgaris, rosacea and seborrheic dermatitis, which would help us understand the therapeutic response to agents with very different action mechanisms.

REFERENCES

• 1.  Bopp C, Bakos L. Papilomatose papulosa confluente e reticulada. An Bras Dermatol. 1969;44:89-105.
• 2.  Dias MFRG, Azulay RD. Ceratoses foliculares. In: Azulay RD, Azulay DR, editores. Dermatologia. 3 ed. Rio de Janeiro: Guanabara Koogan; 2004. p.452-5.
• 3.  DiGiovana JJ. Ichthyosiform dermatoses. In: Freedberg IM, Eizen AZ, Wollf K, Austin KF, Goldsmith LA, Kats S, et al. Dermatology in General Medicine. 6 ed. New York: Mc Graw-Hill; 1999.p.481-505.
• 4.  Berger CM. Clinical pathological challenge: confluent and reticulated papillomatosis of Gougerot and Carteaud. Am J Dermatophatol. 2003;25:179-80.
• 5.  Soares MA, Rodrigues TS, Kede MPV, Nascimento LV. Papilomatose confluente e reticulada de Gougerot- Carteaud. An Bras Dermatol. 1992;67:287-8.
• 6.  Lee SH, Choi EH, Lee WS, Kang WH, Bang D. Confluent and reticulated papillomatosis: a clinical, histopathologi cal, and electron microscopic study. J Dermatol. 1991;18:725-30.
• 7.  Bowman PH, Davis LS. Confluent and reticulated papillo matosis: response to tazarotene. J Am Acad Dermatol. 2003;48(5 Suppl):S80-1.
• 8.  Shimizu S, Han-Yaku H. Confluent and reticulated papillomatosis responsive to minocycline. Dermatology. 1997;194:59-61.
• 9.  Montemarano CPTAD, Hengge M, Sau COLP, Welch MAJM. Confluent and reticulated papillomatosis: response to minocycline. J Am Acad Dermatol. 1996;34:253-6.

*

Trabalho realizado na Escola de Medicina da Santa Casa de Misericórdia de Vitória (Emescam) - Vitória (ES), Brasil.

Publication Dates

• Publication in this collection
  23 June 2009
• Date of issue
  Feb 2009

History

• Accepted
  13 June 2007
• Received
  30 Aug 2005