Photo Essay: An Array of Hyperpigmented Lesions

January 5, 2010

The areas of hyperpigmentation shown here on the trunk of a 15-year-old girl appeared 2 years earlier as 1- to 2-mm hyperpigmented papules with either flat or verrucous surfaces.

Confluent and Reticulated Papillomatosis
The areas of hyperpigmentation shown here on the trunk of a 15-year-old girl appeared 2 years earlier as 1- to 2-mm hyperpigmented papules with either flat or verrucous surfaces. The papules gradually enlarged and coalesced into confluent plaques with reticulated borders. Examination revealed diffusely distributed lesions over the neck and upper abdomen with a velvety, hyperkeratotic texture. The patient denied pain and pruritus. Previous application of topical ketoconazole had been ineffective.

These skin changes are characteristic of confluent and reticulated papillomatosis (Gougerot-Carteaud syndrome), which usually develops in postpubescent teenagers. Males and females are equally affected; evidence of a racial predilection is conflicting.1 Although this condition is very rare (with only 39 cases reported in the past 30 years2), it is probably underdiagnosed and/or underreported because of its benign nature.

Typical distributions may include the neck, back, axillae, breasts, and upper abdomen.1 Biopsy, which may be performed to confirm the diagnosis, reveals hyperkeratosis, papillomatosis, increased melanin deposition, and acanthotic projections deep into interpapillomatous valleys.1 However, the lesions may lack hyperpigmentation3 and histological papillomatosis,4 and they may present with a fine scale or associated mild pruritus.1

The differential diagnosis includes more common conditions, such as acanthosis nigricans and tinea versicolor. Acanthosis nigricans seldom presents on the trunk and lacks peripheral reticulation. Tinea versicolor produces a white-to-brown keratinaceous scale that reveals Malassezia in potassium hydroxide preparation; unlike confluent and reticulated papillomatosis, tinea responds to antifungal medication.

Although the cause remains unknown, some theories include faulty keratinization, bacterial infection, reaction to Pityrosporum yeast, and reaction to UV light. More than 80% of patients treated with minocycline or azithromycin respond completely, and 10% to 15% exhibit a partial response; successful treatment confirms the diagnosis.1,2 However, recurrence rates as high as 50% have been reported with the cessation of therapy.1,2 In recurrent cases, repeated antibiotic courses may be used because acquired antibiotic resistance has not been described.

This patient was treated with minocycline, 100 mg twice daily for 6 weeks. This treatment, first described in 1965 by Carteaud,5 has been supplanted by azithromycin, 500 mg 3 times weekly for 3 weeks, because of the latter’s benign adverse-effect profile. Other therapeutic options include topical or systemic retinoids and vitamin D derivatives, such as calcipotriol.1 Untreated confluent and reticulated papillomatosis follows a chronic course of plaque expansion and recession. Over years to decades, individual lesions may stabilize and remain permanently.

REFERENCES:1. Scheinfeld N. Confluent and reticulated papillomatosis: a review of the literature. Am J Clin Dermatol. 2006;7:305-313.
2. Davis MD, Weenig RH, Camilleri MJ. 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. 2006;154:287-293.
3. Treat JR, Barak OG, James WD. Nonpigmenting confluent and reticulated papillomatosis. Pediatr Dermatol. 2006;23:497-499.
4. Mutasim DF. Confluent and reticulated papillomatosis without papillomatosis. J Am Acad Dermatol. 2003;49:1182-1184.
5. Carteaud A. A case of Gougerot and Carteaud’s confluent and reticulated papulous papillomatosis, completely cleared up by antibiotics [in French]. Bull Soc Fr Dermatol Syphiligr. 1965;72:396-397.

(Case and photograph courtesy of Andrew M. Goldsweig, MD.)



Neurofibromatosis Type 1

Over the past few months, 8 uniformly tan macules of 0.5 to 2 cm had developed on the abdomen, back, and upper and lower extremities of an otherwise healthy 8-month-old girl. There had been no complications or lesions at birth. Her father, who had similar lesions as a child, was noted to have several nodules on his body.

Neurofibromatosis type 1 (NF1), formerly known as von Recklinghausen disease, mainly involves the skin and CNS. It has a prevalence of 1 in 3500 and is inherited in an autosomal dominant fashion.1 Hyperpigmented macules, or caf au lait spots, are the first clinical feature and may be present at birth or appear during infancy. Nearly 80% of patients with NF1 have 5 caf au lait spots by 1 year of age.2,3 These spots are benign; however, they may be of cosmetic concern.3 They are associated with several other diseases, including McCune-Albright syndrome, tuberous sclerosis, and Fanconi anemia.

The diagnosis of NF1 requires 2 or more of the following criteria:
• Six or more caf au lait spots equal to or greater than 5 mm in prepubertal patients (15 mm in postpubertal patients).
• Two or more neurofibromas of any type or 1 plexiform neurofibroma.
• Freckling in the axillary (Crowe sign) or inguinal regions.
• Optic glioma.
• Two or more Lisch nodules (iris hamartomas).
• A distinctive osseous lesion.
• A first-degree relative with NF1, according to the aforementioned criteria.1

Those clinical features that are not evident at birth may develop as the child ages. Children with NF1 may also have learning disabilities.2,3 Thus, all affected children require periodic monitoring to minimize potentially severe outcomes.1

Neurofibromas, benign tumors composed mainly of Schwann cells and fibroblasts, can arise in any peripheral nerve. Discrete cutaneous neurofibromas are often flesh-colored or pink, soft to the touch, and typically asymptomatic. They may be either sessile or pedunculated. Discrete subcutaneous neurofibromas are usually firm and may be tender. These 2 types of neurofibromas appear during puberty and increase in size and number. Nodular plexiform and diffuse plexiform neurofibromas may become clinically evident in adulthood and occasionally progress to malignant tumors.2

focuses on early detection of disease manifestations, symptomatic treatment, and genetic counseling. During yearly evaluations, document the patient’s neurodevelopmental progress and the presence of any new neurofibromas or skin lesions, blood pressure changes, eye symptoms, and skeletal anomalies. Referral to a specialist (dermatologist, neurologist, orthopedist, or ophthalmologist) may be warranted.1REFERENCES:1. Hersh JH; American Academy of Pediatrics Committee on Genetics. Health supervision for children with neurofibromatosis. Pediatrics. 2008;121:633-642.
2. Friedman JM. Neurofibromatosis 1: clinical manifestations and diagnostic criteria. J Child Neurol. 2002; 17:548-554.
3. Brill CB. Neurofibromatosis. Clinical overview. Clin Orthop Relat Res. 1989;245:10-15.

 (Case and photograph courtesy of Joshua S. Black, MD and Barbara Wilson, MD.)



Postinflammatory Hyperpigmentation
Photos A and B
show the right side of the neck of a 12-year-old girl who had tinea corporis. The infection (A) resolved after 3 weeks of daily application of ketoconazole cream; however, an oval area of hyperpigmentation remained and was still apparent 6 months later (B).

Inflammation from allergic reactions, infection, or trauma can result in either hypopigmentation or hyperpigmentation of the involved area. Increased pigment production responsible for postinflammatory hyperpigmentation could possibly be a consequence of poor blood supply in the area. No treatment is required.

For patients who request treatment for cosmesis of hyperpigmented skin, hypopigmenting agents, such as hydroquinone, can be used to lighten the darkened area, although results vary and some agents may even worsen the lesions. A combination cream that contains hydroquinone, tretinoin, and fluocinolone acetonide (Tri-Luma cream) has proved to be more effective than other single agents.1 Topical tretinoin or azelaic acid may also be used as monotherapy. Chemical peels using trichloroacetic acid and alpha hydroxy acids are somewhat effective; however, these treatments have been known to cause postinflammatory hyperpigmentation or hypopigmentation. Laser therapy has shown mixed results. Use of sunscreens that block UV-A and UV-B light can prevent further pigmentation. Cosmetics may be used to camouflage the pigmented lesions.

REFERENCE:1. Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Treatment. 4th ed. St Louis: Mosby; 2004:692.

(Case and photograph courtesy of Dr Joseph P. Bark.)