Papillomatosis confluens et reticularis L83.x

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Dr. med. Martin Hahn

All authors of this article

Last updated on: 06.03.2024

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Synonym(s)

Confluent and reticulated papillomatosis; Confluent and reticulated papillomatosis of Gougerot and Carteaud; Confluent reticular papillomatosis; Gougerot-Carteaud's disease; Gougerot-Carteaud Syndrome; papillomatosis confluente et réticulée; Papillomatosis confluent reticular

History
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Gougerot and Carteaud, 1927

Definition
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Sporadic, rarely familial disease similar to acanthosis nigricans or combined with acanthosis nigricans with persistent, reticulate-striped brownish or brownish-yellowish smaller or larger patches or brown, slightly hyperkeratotic (not granular hyperkeratotic but rather velvety hyperkeratotic) reticulate papules and plaques. It is often considered a clinical variant of pityriasis versicolor, especially if it has not been treated for a long time. Sometimes the clinical picture presents itself in the context of a mild ichthyosis. To differentiate from other hyperpigmentations, it can be checked whether the plaques can be washed off with soap and water, in which case it is dermatosis neglecta, or whether the plaques can only be removed with alcohol, in which case it is terra firma-forme dermatosis (TFFD).

Etiopathogenesis
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Unexplained.

Genetically determined (familial occurrence is described) disorders of keratinization with association to acanthosis nigricans(pseudoacanthosis nigricans) are discussed.

Furthermore, reactive epidermis proliferations by Malassezia spp. (DD: atypical pityriasis versicolor) or gram-positive bacteria (Dietziaceae spp. from the order of Actinomycetales) are described.

Associations with diabetes mellitus, obesity as well as hormonal disorders (thyroid dysfunctions) are possible.

Manifestation
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Gynecotropy is discussed (?) ! Manifestation age: 10-36 years (average age in smaller studies is 19 years). Frequency peak shortly after puberty. Dark-skinned people are affected twice as often as light-skinned people.

Localization
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Mainly sternoepigastric area, intermammary region, also spreading over abdomen, neck, shoulder, back. Face remains free. Less frequently also in the genital area.

Clinical features
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Chronic persistent (duration of the disease before diagnosis about 3 years) skin disease with multiple, flat, gray to brown, up to 0.5 cm large, blurred, slightly scaly, velvety papules, which can confluent to larger (up to 10 cm), flat homogeneous plaques, but can also form reticular or striped patterns and can confluent to dirty brown areas in the intertriginous areas.

Histology
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Acanthosis with distinct papillomatosis. Severe plexiform orthohyperkeratosis. Bulky perivascular oriented lymphocytic infiltrate in the upper dermis. PAS staining is negative (no detection of Pityrosporon spp.).

Diagnosis
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Criteria varies according to Davies:

  • velvety, brown-yellow or brownish papules and plaques
  • filled flat plaques or net-like patterns
  • seborrhoic zones
  • proximal trunk, neck, also genital region
  • chronic course
  • negative fungus detection
  • no therapeutic success with antimycotics
  • no itching
  • Good response to minocycline/doxycycline
  • Histology not very specific.

Differential diagnosis
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External therapy
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Mild keratolytic measures with urea-containing external agents, e.g. a 5-10% anionic urea cream R102 or emulsion R104.

If pityrosporon species are detected, supplementary antimycotic therapy with a solution containing clotrimazole(e.g. Canesten solution, Cutistad, R055 ) or an antimycotic cream, e.g. ketoconazole cream(e.g. Nizoral) or bifonazole cream(e.g. Mycospor).

Internal therapy
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Good therapeutic success is achieved with Minocycline 2 times/day 50-100 mg p.o. (complete healing in 6-8 weeks/75% of cases) or Doxycyclin 2x/day 100mg p.o.

Therapy duration 6-8 weeks. Recurrences after discontinuation are possible.

Alternatively, a systemic therapy with acitretin or isotretinoin can be applied.

Note(s)
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It is to be assumed that the clinical picture of the "confluent and reticular papillomatosis" described by Gougerot and Carteaud does not conceal an etiopathogenetically uniform clinical picture. Rather microbial factors, a tendency to obesity, genetic factors like dark-skinnedness seem to lead to this mild cornification anomaly.

Case report(s)
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16-year-old obese female patient with non-pruritic HV on décolleté and neck since 2 years. Findings: Reticular, also striated, in places confluent to larger plaques, gray-brown papules, in places verrucous.

Histology: Discrete lymphocytic, superficial, interstitial dermatitis with papillomatosis and orthohyperkeratosis. Masses of bacteria in the stratum corneum. No mycelia or spores.

Mycology (native specimen): Neg.

Bacteriology: Staph. aureus, minocycline sensitive.

Laboratory: Inconspicuous.

Therapy: Frustrating external (keratolytic) therapy with urea, benzoyl peroxide and glucocorticoids at first. Internal therapy with minocycline (2 times/day 50 mg p.o.) for 6 weeks complete healing of all HV.

Literature
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  1. Açıkgöz G et al. (2014) Confluent and reticulated papillomatosis (Gougerot-Carteaud syndrome) in two brothers. Acta Dermatovenerol Croat 22:57-59.
  2. Berk DR (2010) Confluent and reticulated papillomatosis response to 70% alcohol swabbing. Arch Dermatol 147: 247-248
  3. Davis MD et al (2006) Confluent and reticulate papillomatosis (Gougerot-Carteaud syndrome): a minocycline-responsive dermatosis without evidence for yeast in pathogenesis. A study of 39 patients and a proposal of diagnostic criteria. Br J Dermatol 154:287-293
  4. Gougerot H, Carteaud A (1927) Papillomatosis pigmentée innominée. Bull Soc Fr Dermatol Syph 34: 719-721.
  5. Jankowska-Konsur A et al (2013) Confluent brownish papules and plaques on the neck, upper chest and back: a quiz. Confluent and reticulated papillomatosis of Gougerot and Carteaud. Acta Derm Venereol 93:493-494
  6. Jo S et al (2014) Updated diagnostic criteria for confluent and reticulated papillomatosis: a case report. Ann Dermatol 26:409-410
  7. Koguchi H et al (2013) Confluent and reticulated papillomatosis associated with 15q tetrasomy syndrome. Acta Derm Venereol 93: 202-203
  8. Löwenstein M (2006) Successful minocycline therapy of papillomatosis confluens et reticularis Gougerot-Carteaud. JDDG 7: 556-557
  9. Nishikawa T (2002) Comment on the contribution by Weigl CB et al. "Papillomatosis confluens et reticularis". Dermatologist 53: 369
  10. Tamraz H et al (2013) Confluent and reticulated papillomatosis: clinical and histopathological study of 10 cases from Lebanon. J Eur Acad Dermatol Venereol 27:e119-123.
  11. Wagner et al (2004) Pityriasis versicolor under the clinical presentation of papillomatosis confluens et reticularis. Act Dermatol 30: 114-119
  12. Weins AB et al (2021) Papillomatosis confluens et reticularis (Gougerot-Carteaud's disease) - a retrospective study of 25 patients. J Dtsch Dermatol Ges 19: 588-591.
  13. Wiesenborn A et al (2004) Papillomatosis confluens et reticularis. Dermatol 55: 976-978

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Last updated on: 06.03.2024