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Confluent and Reticulated Papillomatosis

Confluent and Reticulated Papillomatosis of Gougerot and Carteaud

Confluent and reticulated papillomatosis (CARP) is a dermatosis presented by papillomatous papules and hyperpigmented scaly macules coalescing into centrally confluent plaques with a reticular peripheral pattern. It is an uncommon condition and mostly occurs on the trunk.


Presentation

Confluent and reticulated papillomatosis (CARP), also known as Gougerot-Carteaud syndrome is a rare disorder first described by Gougerot and Carteaud in 1927 [1]. It occurs in all age groups, races, and genders. Usually, patients are asymptomatic. However, they may experience mild pruritus. The lesions are most commonly observed on the trunk [2]. Physical findings are limited to the skin.

CARP lesions begin as papules of 1 to 2 millimeters in diameters that are slightly hyperkeratotic to warty. They enlarge to form 4 to 5 millimeters lesions which coalesce and form confluent plaques centrally with a reticulated periphery. Skin markings are preserved, or in the neck and axillae, they may be exaggerated. It has been described as keratotic and vertically rippled plaques, and punctate pigmented papillomatosis [3] [4]. Rarely, atrophic macules may also be seen. A fine powdery scale is produced upon scraping of the lesions. Dermoscopy reveals an ill-defined brownish pigmentation, covered with white scales and a pattern labeled "sulci and gyri" [5]. The color of lesions in CARP are characterized by an early erythematous appearance that later evolves into a grayish brown color [6].

CARP lesions initially develop in the skin of epigastric or intermammary region, and over a period of weeks to months spread over the lower abdomen, breast, pubic area, and the flanks. Exceptions to this can occur, as in one case the lesions were confined to the cheeks and in another, they were confined to the pubic area [7] [8]. The mucous membranes are spared but the involvement of the face, proximal extremities, and forehead may be seen [9] [10].

Physician
  • Physicians encountering darkly pigmented individuals with hypopigmented plaques unresponsive to antifungals should have a high clinical suspicion for the hypopigmented variant of CARP.[ncbi.nlm.nih.gov]
  • Gougerot, Henri, French physician, 1881-1955. Gougerot and Blum disease - an eruption of lichenoid papules on the legs.[medical-dictionary.thefreedictionary.com]
  • Confluent and reticulated papillomatosis (CRP) described in 1927 by Goujerot and Carteaud is a rare disease that is often not recognised by physicians or misdiagnosed.[therapeutique-dermatologique.org]
  • Get your health question answered instantly from our pool of 18000 doctors from over 80 specialties Doctors waiting to answer your question MD MD MD MD Dr.Raju General & Family Physician Exp 11 years MD MD ...and 18,000 more Doctors from across the world[healthcaremagic.com]
  • Davis et al. (9) stated that CRP was not fully recognized by physicians or even by dermatologists: only 39 patients in 32 years at the Mayo Clinic have been precisely evaluated.[medicaljournals.se]
Pathologist
  • Madhavi Naik, Consultant Pathologist, St Theresa's Hospital, Bengaluru, India. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Scheinfeld N.[ijpd.in]
Developmental Delay
  • The syndrome displays distinctive clinical findings represented by developmental delay, intellectual disability, epilepsy and autistic behaviour. Skin pigmentation has been observed in several cases (1).[medicaljournals.se]
Epilepsy
  • The syndrome displays distinctive clinical findings represented by developmental delay, intellectual disability, epilepsy and autistic behaviour. Skin pigmentation has been observed in several cases (1).[medicaljournals.se]
Melena
  • March 1, 2018 A 49-year-old man presented to the emergency department with hematemesis and 2 episodes of melena. Examination findings included resting tachycardia and melenic stool. Blood pressure was 95/50 mm Hg.[consultant360.com]
Tachycardia
  • Examination findings included resting tachycardia and melenic stool. Blood pressure was 95/50 mm Hg. Multiple raised, soft, bluish 0.3 to 1 cm lesions were noted on the trunk and extremities.[consultant360.com]
Hyperpigmentation
  • We report three teenaged Caucasian patients with confluent and reticulated papillomatosis whose presentation was atypical due to the absence of hyperpigmentation and presence of a fine white scale.[ncbi.nlm.nih.gov]
  • Thus, there is the possibility that the skin hyperpigmentation associated with 15q tetrasomy syndrome is being diagnosed as CRP.[medicaljournals.se]
  • Histologic Features A basket-weave horn Papillomatosis of epidermis with deep depressions Thin epidermis and rete ridges Fusion of adjacent rete ridges forming X and Y configurations Slight hyperpigmentation of the basal layer[dxpath.com]
  • The rash actually resolved within 2 weeks with only some residual post-inflammatory hyperpigmentation.[theketorash.com]
  • […] papillomatosis Figure: 3 Confluent and reticulate papillomatosis Management Several antibiotics, especially minocycline and azithromycin, have been found to be effective when give for a period of 6-8 weeks Minocycline has occasionally been associated with hyperpigmentation[pcds.org.uk]
Skin Lesion
  • There was no family history of the same skin lesions. The histology showed epidermal papillomatosis and mild acanthosis with a marked hyperkeratosis.[ncbi.nlm.nih.gov]
  • Case Report: A 13 year-old overweight female adolescent was admitted to Dermatology outpatient clinic for asymptomatic brownish skin lesions. At physical examination she presented brown plaques spread out on the breast, neck and armpits.[revistas.rcaap.pt]
Photosensitivity
  • Other hypothesis include photosensitivity, genetic factor, amyloidosis cutis and endocrinopathy.[ncbi.nlm.nih.gov]
  • According to some authors there is could be a link with vitamin A deficiency, genetic factors, photosensitivity and cutaneous amyloidosis. 4)Site: The lesions are located in the inter-scapular, epigastric and intermammary regions, sparing, in general,[histopathology-india.net]
  • However, she discontinued the medication after 1 month because of concerns of photosensitivity, and the rash worsened. She is currently evaluating her treatment options. References: 1. Gougerot H, Carteaud A. Papillomatose pigmentee innominee.[consultant360.com]
Follicular Plugging
  • Clinically, one case had an atypical clinical presentation, and microscopically follicular plugging was seen in the majority of cases.[ncbi.nlm.nih.gov]
Seizure
  • He had experienced frequent generalized seizures since 8 years of age, which were treated with clobazam and carbamazepine.[medicaljournals.se]
Generalized Seizure
  • He had experienced frequent generalized seizures since 8 years of age, which were treated with clobazam and carbamazepine.[medicaljournals.se]

Workup

The diagnostic criteria for CARP is proposed as:

  1. Scaly brown macules and patches. Some appearing papillomatous and reticulated.
  2. Involvement of the neck, upper trunk, or flexural areas.
  3. No response to antifungal therapy, or negative fungal staining.
  4. A favorable response to antibiotics.

On potassium hydroxide (KOH) test, pityrosporum ovale or pityrosporum orbiculare spores and rarely hyphae may be found. A fungal culture may also reveal these microorganisms in some cases. On wood lamp examination, yellow fluorescence can occur if pityrosporum organisms are present. Electron microscopy can help detect alterations in the structure and arrangement of cornified cells in some lesions. An increase may be observed in transitional cell layer, the number of melanosomes in the stratum corneum, and the number of lamellar bodies in the stratum granulosum [11]. On immunohistochemical analysis, suprabasal keratin 16 expression and an intense focal staining in the stratum granulosum are revealed, with an increased number of epidermal cells with Ki-67 binding in the stratum malpighii.

Histopathological features of CARP include:

  1. Basket-weave hyperkeratosis.
  2. Papillomatosis.
  3. Focal acanthosis (limited to areas of rete pegs elongation).
  4. Raised basal melanin pigmentation.

Treatment
  • Although a variety of treatments for CARP exist, oral minocycline 50-100mg twice daily has been the preferred treatment.[ncbi.nlm.nih.gov]
  • Although a variety of treatments for CARP exist, oral minocycline 50–100mg twice daily has been the preferred treatment.[doi.org]
  • Management Pearls Treatment is for cosmetic reasons. • Overweight Treatment of confluent and reticulated papillomatosis with azithromycin. J Dermatol.[ekoservis-kuban.ru]

Prognosis
  • (Outcomes/Resolutions) The prognosis of Confluent and Reticulated Papillomatosis is good with adequate treatment.[dovemed.com]
  • Prognosis CRP usually responds to minocycline and azithromycin as first-line drugs, with remission being sustained for years. Recurrences are more common in those treated with other agents, occurring in up to 15 percent of such patients.[news-medical.net]
  • However, given the superior safety profile of minocycline and azithromycin, systemic retinoids are typically reserved for nonresponders to antibiotics. 28 Prognosis The prognosis of CRP is good with marked response ( 50% improvement) when minocycline[ncbi.nlm.nih.gov]
  • In one study of 10 Lebanese cases, the mean age at diagnosis was 19 years. [8] Prognosis Confluent and reticulated papillomatosis is a chronic disease characterized by exacerbations and remissions.[emedicine.medscape.com]

Etiology
  • Of unknown etiology, the condition is characterised by oval grayish-brown or erythematous papules that coalesce to form a reticular pattern, usually on the trunk, especially in the intermammary or interscapular region and on the neck.[ncbi.nlm.nih.gov]
  • Amyloidosis of the skin has also been proposed as an etiological agent. Genetic Causes While most cases of CRP are sporadic, some familial cases have been reported.[news-medical.net]

Epidemiology
  • Our review of the literature on confluent and reticulated papillomatosis refutes previously accepted epidemiologic data.[ncbi.nlm.nih.gov]
  • The epidemiology of CRP is not well defined. A recent case series from the Mayo Clinic of 39 patients demonstrated an average age at presentation of 15 years.1 At the first visit, the eruption had been present for a mean of 3.1 years.[healio.com]
  • Confluent and reticulated papillomatosis: response to high-dose oral isotretinoin therapy and reassessment of epidemiologic data. J Am Acad Dermatol. 1994 Aug; 31(2/2): 327–31. PubMed Angeli-Besson C, Koeppel MC, Jacquet P, Andrac L, Sayag J.[dermnetnz.org]
  • Epidemiology Frequency The frequency of confluent and reticulated papillomatosis internationally is unknown. It is considered to be rare.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology
  • This is the first electron microscopic report on CRP suggesting that the pathophysiology of this disease is related to abnormal keratinocyte differentiation.[ncbi.nlm.nih.gov]
  • To the best of our knowledge, her transient post-partum clearance has not been reported anywhere else in the literature and we believe it may offer a clue in the pathophysiologic process of CARP.[consultant360.com]

Prevention
  • The macrolides, on the other hand, regulate immune activation via IL-8 suppression and preventing the neutrophil oxidative burst.[news-medical.net]
  • General skin care is advised by most healthcare providers typically as a preventive measure for any skin condition.[dovemed.com]
  • Minocycline inhibits migration of neutrophils. prevents release of reactive oxygen species& inhibits matrix-metalloproteinase. (10)(11)(12) in both cases lesions were completely cleared in 3 weeks suggesting that the bacterial infection might be the cause[jemds.com]
  • Atlanta: Centers for Disease Control and Prevention, September 1995. ‎ Página 51 - Stone KM, Reiff-Eldridge R, White AD, Cordero JF, Brown Z, Alexander ER, Andrews EB.[books.google.es]
  • Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public[emedicine.staging.medscape.com]

References

Article

  1. Gougerot H, Carteaud A. Papillomatose pigmentée innominée. Bull Soc Fr Dermatol Syphil. 1927;34(Nov 10):719-721.
  2. Tamraz H, Raffoul M, Kurban M, Kibbi AG, Abbas O. Confluent and reticulated papillomatosis: clinical and histopathological study of 10 cases from Lebanon. J Eur Acad Dermatol Venereol. 2013;27(1):e119-23.
  3. Min ZS, Tan C, Xu P, Zhu WY. Confluent and reticulated papillomatosis manifested as vertically rippled and keratotic plaques. Postepy Dermatol Alergol. 2014;31(5):335-7.
  4. Ibekwe PU, Ukonu BA. Punctate, pigmented, and verrucous papillomatosis: a rare variant of Gougerot-Carteaud syndrome. Int J Dermatol. 2016;55(7):806-808. doi: 10.1111/ijd.13097.
  5. Bernardes Filho F, Quaresma MV, Rezende FC, Kac BK, Nery JA, Azulay-Abulafia L. Confluent and reticulate papillomatosis of Gougerot-Carteaud and obesity: dermoscopic findings. An Bras Dermatol. 2014;89(3):507-9.
  6. Bhagwat PV, Tophakhane RS, Shashikumar BM, Noronha TM, Naidu V. Three cases of Dowling Degos disease in two families. Indian J Dermatol Venereol Leprol. 2009;75(4):398-400.
  7. Faergemann J, Fredriksson T, Nathorst-Windahl G. One case of confluent and reticulate papillomatosis (Gougerot-Carteaud). Acta Derm Venereol. 1980;60(3):269-71.
  8. Broberg A, Faergemann J. A case of confluent and reticulate papillomatosis (Gougerot-Carteaud) with an unusual location. Acta Derm Venereol. 1988;68(2):158-60.
  9. Lee D, Cho KJ, Hong SK, Seo JK, Hwang SW, Sung HS. Two cases of confluent and reticulated papillomatosis with an unusual location. Acta Derm Venereol. 2009;89(1):84-5.
  10. Kim BS, Lim HJ, Kim HY, et al. Case of minocycline-effective confluent and reticulated papillomatosis with unusual location on forehead. J Dermatol. 2009;36(4):251-3.
  11. Jimbow M, Talpash O, Jimbow K. Confluent and reticulated papillomatosis: clinical, light and electron microscopic studies. Int J Dermatol. 1992;31(7):480-3.

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Last updated: 2019-06-28 09:21