Confluent and Reticulated Papillomatosis


Originally described confluent and reticulated papillomatosis under the name papillomatose pigmentée innominée and later renamed it papillomatose pigmentée confluente et réticulée.

A rare disease typically affecting young persons.

Characterized by grayish blue hyperkeratotic papules, usually located on the trunk.

The lesions tend to coalesce to form confluent plaques centrally and a reticular pattern peripherally.

May represent an endocrine disturbance.

May be a disorder of keratinization.

May be a result of an abnormal host reaction to fungi or bacteria.

May be a hereditary disorder.

May be a variant of amyloidosis.

A chronic eruption, associated with exacerbations and remissions.

Is responsive to treatment but frequently recurs after discontinuation of therapy.

Associated with abnormal keratinocyte differentiation and maturation, an increased transition cell layer and increased lamellar granules in the stratum granulosum, along with increased keratin 16, and Ki-67 expression.

Increased melanosomes in the stratum corneum may explain pigmentary changes.

The frequency is unknown, but is rare.

Racial prevalence is variable.

The proportion of women to men affected reported to be as high as 2.8:1, but the ratio is probably closer to 1.4:1.

The onset usually occurs shortly after puberty.

The mean patient age at onset from 18.5-21 years.

Patients are often asymptomatic, but they may experience mild pruritus.

Pigmented macules, patches, and plaques most commonly on the chest.

Grayish brown hyperkeratotic papules and plaques in a confluent pattern on the intermammary region.

Clinically a reticular pattern peripherally.

Lesions begin as slightly hyperkeratotic to warty papules that are 1-2 mm in diameter.

Lesions enlarge to 4-5 mm in diameter and coalesce to form a reticular pattern peripherally and confluent plaques centrally.

Skin markings are preserved and sometimes exaggerated, especially in the neck and the axillae, where the skin may be thickened.

Atrophic macules rarely may be seen.

Scraping of lesions produces a fine powdery scale or mealy deposit.

An autosomal dominant trait is characterized by many asymptomatic oval macules symmetrically distributed on axillae, groin, face, neck, arms, and trunk; scattered comedolike papules, and pitted acneiform scars.

Usually begins on the skin of the intermammary or epigastric region, spreading over a period of weeks or months to the breasts, the lower abdomen, the flanks, and the pubic area.

May be found in the interscapular region, from which it spreads to the shoulders, the nape of the neck, and the gluteal cleft.

May involve the face, forehead and the proximal extremities.

The mucous membranes are not involved.

Early lesions are erythematous and later become grayish brown.

Etiology includes an endocrine disturbance, a disorder of keratinization, and an abnormal host reaction to Pityrosporum organisms or bacteria.

Reports also exist of familial cases.

Possibily represents a variant of amyloidosis.

Common observation of endocrine disturbances including Cushing disease, menstrual irregularities, obesity, abnormal glucose tolerance or diabetes mellitus, thyroid disease, pituitary dysfunction, hirsutism or hypertrichosis?

In Japan, 76.5% of cases are associated with obesity or rapid weight gain.

In addition, lesions of confluent and reticulated papillomatosis have been noted to remit during pregnancy or with weight loss.

No single endocrine abnormality is seen; however, in many of the cases, no abnormality exists at all.

Confluent and reticulated papillomatosis maybe due to a defect in keratinization, as increased lamellar granules in the stratum granulosum is present, a finding seen in conditions of increased cell turnover and desquamation.

There are increased expression of involucrin, keratin 16, and Ki-67—protein markers for keratinocyte differentiation and maturation.

The defect of keratinization is indicated by the response of confluent and reticulated papillomatosis to topical and oral retinoids.

Also responds to topical analogues of vitamin D, agents that also regulate cell differentiation and inhibit keratinocyte proliferation.

Ultraviolet light exposure and avitaminosis are associated with its development, and they may trigger or exacerbate the underlying abnormality.

May represent an abnormal host reaction to Pityrosporum orbiculare, either in the yeast or the hyphal form.

Many cases, however, do not have any evidence of Pityrosporum infection, and fail to respond to antifungal therapy.

It is suggested a genetic or diet-induced abnormal keratinizing response may be be triggered by P orbiculare.

Reports of successful treatment with antibiotics of the tetracycline, macrolide, cephalosporin and steroid classes.

Successful treatment with antibiotics lead to the hypothesis that bacteria, perhaps within the hair follicle, are the etiologic agents.

There is no particular pattern or mode of inheritance.

Differential Diagnoses include:

Acanthosis Nigricans

Amyloidosis, Macular

Dermatopathia Pigmentosa Reticularis

Dyskeratosis Congenita

Epidermal Nevus Syndrome

Epidermodysplasia V2242uciformis

Erythrokeratodermia Variabilis

Keratosis Follicularis (Darier Disease)

Naegeli-Franceschetti-Jadassohn Syndrome

Pityriasis Rubra Pilaris

Pseudoatrophoderma Colli

Seborrheic Keratosis


Tinea Versicolor

KOH examination of skin scrapings may find Pityrosporum orbiculare or Pityrosporum ovale spores and rarely hyphae.

P orbiculare or P ovale may be cultured in some cases.

Under Wood lamp examination yellow fluorescence occurs when Pityrosporum organisms are present.

The epidermis shows compact hyperkeratosis, often associated with the presence of Pityrosporum yeast.

Other histological features are less consistent and include a decreased or absent granular cell layer, papillomatosis, and a stratum spinosum that varies from acanthotic to atrophic.

A disorder of the skin that results in cosmetic disfigurement, with no adverse systemic effects.

No treatment is necessary other than for eradication of the rash.

The most consistent therapeutic results may be seen with minocycline.

Other treatments include keratolytics; intramuscular, multiple forms of vitamin A, sodium thiosulphate, ammoniated mercury, oral contraceptives, oral and topical retinoids, thyroid extract, ultraviolet light, propylene glycol, antibiotics, antimycotics, calcipotriene, and topical mupirocin.

The hyperpigmentation can be removed using 70% alcohol swabbing.

Surgical treatment is not unsuccessful.

Lesions may regress with weight reduction.

Miconazole topical damages fungal cell wall membrane by inhibiting biosynthesis of ergosterol, which increases permeability causing nutrients to leak out and resulting in fungal cell death.

Ketoconazole a broad-spectrum antifungal agent; inhibits synthesis of ergosterol, causing cellular components to leak and resulting in fungal cell death.

Retinoid-like agents correct disorders of keratinization.

Retinoid-like agents Inhibit microcomedo formation and eliminates existing lesions.

It is a chronic disease characterized by exacerbations and remissions, and discontinuation of successful therapy usually results in recurrence of the confluent and reticulated papillomatosis.

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