Patient Presentation A 28-year-old African-American male presented with a 5-year history of mildly pruritic eruption on his trunk and proximal upper extremities. The rash improved somewhat with the continued use of selenium sulfide shampoo, but it tended to worsen during the winter months. We observed multiple, brownish-black and tan 2-mm to 5-mm papules with fine scaling, which were distributed on the chest, abdomen, back and proximal extremities. The papules showed areas of confluence centrally and formed a reticulate pattern on the periphery, as shown in the photos above. The patient wasn’t taking any oral medications at the time. In addition, his past medical and surgical histories, as well as his family history, were unremarkable. A KOH preparation was negative. A punch biopsy of the chest revealed prominent hyperkeratosis and parakeratosis along with mild to moderate irregular acanthosis. The epidermis also showed basilar hyperpigmentation. A mild superficial perivascular lymphocytic infiltrate was present. A PASF stain was negative. Previous treatment with topical steroids (Elocon and Cutivate ointments) in combination with emollients (Aquaphor and Moisturel) was unsuccessful. What’s Your Diagnosis? CRP was described in 1927 in the French literature by Henri Gougerot and Alexandre Carteaud as papillomatose pigmentée innominée or papillomatose pigmentée confluente et reticulée. It was later renamed confluent and reticulated papillomatosis by Fred Wise, who reported the first case in the United States. The eruption is chronic with exacerbations and remissions. Although CRP responds to treatment, it frequently recurs after therapy is discontinued. Patients with CRP are often asymptomatic, but may experience mild pruritus. Clinically, CRP consists of confluent, flat, brown papules localized to the intramammary and interscapular regions. Subsequent spread occurs over weeks to months to the abdomen and periphery of the chest and back, forming a characteristic reticulated pigmented pattern, which led to the name of the disorder. Lower abdomen, flanks, and pubic area may also be involved. Cases of CRP lesions confined to the cheeks or the pubic areas have also been reported. The mucous membranes are usually spared. Reports of familial occurrence exist, but the majority of cases are sporadic. A Glance at Pathogenesis and Etiology CRP and acanthosis nigricans have clinical and histologic similarities, although no definite etiology has been identified for CRP. Suggested mechanisms include endocrine disturbances, disorder of keratinization, abnormal host reaction to fungi or bacteria, hereditary disorder, or a variant of amyloidosis. At the time of its description, Gougerot suggested that CRP might be due to endocrine disorders. Subsequent reports outlined the common observation of CRP in patients with obesity, abnormal glucose tolerance, diabetes mellitus, Cushing’s disease, menstrual irregularities, thyroid disease, pituitary dysfunction, and hirsutism or hypertrichosis, therefore suggesting some form of association. Recent investigative reports showed that the epidermis showed slight acanthosis with papillomatosis, a reduced granular layer, enhanced numbers of keratinosomes, and a loose hyperkeratotic horny layer. A peculiar finding in the keratinocytes of the upper granular layer was the presence of perinuclear hollow spaces in some patients with CRP. Fine membranes were recognizable in these spaces, and their remainders were evident in the keratinized cells of the horny layer in the form of stacks of elongated membranes and membrane coils. According to Arnold and Anton-Lamprecht,1 these characteristic markers confirmed a disturbance of epidermal differentiation and keratinization for this disease. Using electron microscopy, Inaloz et al2 showed an increased number of transitional cells between the stratum granulosum and the stratum corneum. Immunohistochemical examinations revealed suprabasal keratin 16 (K16) expression throughout the epidermis with intense focal staining in the stratum granulosum and an increased number of cycling epidermal cells with Ki-67 binding in the basal layer and stratum malpighii in two brothers with CRP. Increased melanosomes in the stratum corneum probably account for pigmentary changes. A hypothesis of abnormal reaction to Pityrosporum infections has been put forward. This is mainly due to the clinical resemblance of the rash to that of pityriasis versicolor. However, KOH preparation of scrapings from lesions failed to be consistently positive. Others also have advanced this theory in light of the common finding of Pityrosporum yeast in CRP lesions. Since the observation of improvement of CRP lesions in patients taking antibiotics for other conditions, a plethora of reports showed treatment success with different antibiotic families leading to the concept that one or multiple bacteria might be etiologic agents. Those bacteria or its products (bacterial superantigens) could induce the epidermal proliferation changes of CRP.Scattered reports showed some amyloid deposit in patients with CRP, but the small sample of patients included in these reports makes this theory unlikely. Treating CRP Different treatments have been used for CRP with variable results. The most consistently effective treatment, and the only one evaluated by retrospective and prospective studies, has been oral antibiotics.3-5 Treating CRP with antibiotics occurred after improvement was noted in a patient taking furacycline for arthritis. Since then, CRP has been successfully treated with tetracyclines, macrolides and cephalosporins. The most consistent results are seen with minocycline. Antibiotics are used for their antibacterial, and possibly anti-inflammatory and anti-proliferative effects. Tetracyclines are weak antimetabolites and might have an effect on the epidermal hyperplasia after observed CRP. Based on our findings, we treated our patient with minocycline (Minocin) 100 mg b.i.d. We achieved complete clearance of lesions in 4 weeks. Since therapy, our patient has been in clinical remission for 3 months. Oral and topical forms of retinoids have also been successful for CRP. They mainly correct disorders of keratinization. Vitamin D analogues regulate skin cell production and development. Calcipotriol 0.005% ointment (Dovonex) applications, twice daily for 3 weeks;6,7 and more recently, topical tacalcitol used twice daily as opposed to the usual once daily, were successful in clearing CRP lesions.8 Other modalities include: antifungals, keratolytics, sodium thiosulphate, ammoniated mercury, oral contraceptives, thyroid extract, ultraviolet light, and propylene glycol. Topical corticosteroids aren’t usually effective.
How Would You Diagnose These Papules?
Patient Presentation A 28-year-old African-American male presented with a 5-year history of mildly pruritic eruption on his trunk and proximal upper extremities. The rash improved somewhat with the continued use of selenium sulfide shampoo, but it tended to worsen during the winter months. We observed multiple, brownish-black and tan 2-mm to 5-mm papules with fine scaling, which were distributed on the chest, abdomen, back and proximal extremities. The papules showed areas of confluence centrally and formed a reticulate pattern on the periphery, as shown in the photos above. The patient wasn’t taking any oral medications at the time. In addition, his past medical and surgical histories, as well as his family history, were unremarkable. A KOH preparation was negative. A punch biopsy of the chest revealed prominent hyperkeratosis and parakeratosis along with mild to moderate irregular acanthosis. The epidermis also showed basilar hyperpigmentation. A mild superficial perivascular lymphocytic infiltrate was present. A PASF stain was negative. Previous treatment with topical steroids (Elocon and Cutivate ointments) in combination with emollients (Aquaphor and Moisturel) was unsuccessful. What’s Your Diagnosis? CRP was described in 1927 in the French literature by Henri Gougerot and Alexandre Carteaud as papillomatose pigmentée innominée or papillomatose pigmentée confluente et reticulée. It was later renamed confluent and reticulated papillomatosis by Fred Wise, who reported the first case in the United States. The eruption is chronic with exacerbations and remissions. Although CRP responds to treatment, it frequently recurs after therapy is discontinued. Patients with CRP are often asymptomatic, but may experience mild pruritus. Clinically, CRP consists of confluent, flat, brown papules localized to the intramammary and interscapular regions. Subsequent spread occurs over weeks to months to the abdomen and periphery of the chest and back, forming a characteristic reticulated pigmented pattern, which led to the name of the disorder. Lower abdomen, flanks, and pubic area may also be involved. Cases of CRP lesions confined to the cheeks or the pubic areas have also been reported. The mucous membranes are usually spared. Reports of familial occurrence exist, but the majority of cases are sporadic. A Glance at Pathogenesis and Etiology CRP and acanthosis nigricans have clinical and histologic similarities, although no definite etiology has been identified for CRP. Suggested mechanisms include endocrine disturbances, disorder of keratinization, abnormal host reaction to fungi or bacteria, hereditary disorder, or a variant of amyloidosis. At the time of its description, Gougerot suggested that CRP might be due to endocrine disorders. Subsequent reports outlined the common observation of CRP in patients with obesity, abnormal glucose tolerance, diabetes mellitus, Cushing’s disease, menstrual irregularities, thyroid disease, pituitary dysfunction, and hirsutism or hypertrichosis, therefore suggesting some form of association. Recent investigative reports showed that the epidermis showed slight acanthosis with papillomatosis, a reduced granular layer, enhanced numbers of keratinosomes, and a loose hyperkeratotic horny layer. A peculiar finding in the keratinocytes of the upper granular layer was the presence of perinuclear hollow spaces in some patients with CRP. Fine membranes were recognizable in these spaces, and their remainders were evident in the keratinized cells of the horny layer in the form of stacks of elongated membranes and membrane coils. According to Arnold and Anton-Lamprecht,1 these characteristic markers confirmed a disturbance of epidermal differentiation and keratinization for this disease. Using electron microscopy, Inaloz et al2 showed an increased number of transitional cells between the stratum granulosum and the stratum corneum. Immunohistochemical examinations revealed suprabasal keratin 16 (K16) expression throughout the epidermis with intense focal staining in the stratum granulosum and an increased number of cycling epidermal cells with Ki-67 binding in the basal layer and stratum malpighii in two brothers with CRP. Increased melanosomes in the stratum corneum probably account for pigmentary changes. A hypothesis of abnormal reaction to Pityrosporum infections has been put forward. This is mainly due to the clinical resemblance of the rash to that of pityriasis versicolor. However, KOH preparation of scrapings from lesions failed to be consistently positive. Others also have advanced this theory in light of the common finding of Pityrosporum yeast in CRP lesions. Since the observation of improvement of CRP lesions in patients taking antibiotics for other conditions, a plethora of reports showed treatment success with different antibiotic families leading to the concept that one or multiple bacteria might be etiologic agents. Those bacteria or its products (bacterial superantigens) could induce the epidermal proliferation changes of CRP.Scattered reports showed some amyloid deposit in patients with CRP, but the small sample of patients included in these reports makes this theory unlikely. Treating CRP Different treatments have been used for CRP with variable results. The most consistently effective treatment, and the only one evaluated by retrospective and prospective studies, has been oral antibiotics.3-5 Treating CRP with antibiotics occurred after improvement was noted in a patient taking furacycline for arthritis. Since then, CRP has been successfully treated with tetracyclines, macrolides and cephalosporins. The most consistent results are seen with minocycline. Antibiotics are used for their antibacterial, and possibly anti-inflammatory and anti-proliferative effects. Tetracyclines are weak antimetabolites and might have an effect on the epidermal hyperplasia after observed CRP. Based on our findings, we treated our patient with minocycline (Minocin) 100 mg b.i.d. We achieved complete clearance of lesions in 4 weeks. Since therapy, our patient has been in clinical remission for 3 months. Oral and topical forms of retinoids have also been successful for CRP. They mainly correct disorders of keratinization. Vitamin D analogues regulate skin cell production and development. Calcipotriol 0.005% ointment (Dovonex) applications, twice daily for 3 weeks;6,7 and more recently, topical tacalcitol used twice daily as opposed to the usual once daily, were successful in clearing CRP lesions.8 Other modalities include: antifungals, keratolytics, sodium thiosulphate, ammoniated mercury, oral contraceptives, thyroid extract, ultraviolet light, and propylene glycol. Topical corticosteroids aren’t usually effective.
Patient Presentation A 28-year-old African-American male presented with a 5-year history of mildly pruritic eruption on his trunk and proximal upper extremities. The rash improved somewhat with the continued use of selenium sulfide shampoo, but it tended to worsen during the winter months. We observed multiple, brownish-black and tan 2-mm to 5-mm papules with fine scaling, which were distributed on the chest, abdomen, back and proximal extremities. The papules showed areas of confluence centrally and formed a reticulate pattern on the periphery, as shown in the photos above. The patient wasn’t taking any oral medications at the time. In addition, his past medical and surgical histories, as well as his family history, were unremarkable. A KOH preparation was negative. A punch biopsy of the chest revealed prominent hyperkeratosis and parakeratosis along with mild to moderate irregular acanthosis. The epidermis also showed basilar hyperpigmentation. A mild superficial perivascular lymphocytic infiltrate was present. A PASF stain was negative. Previous treatment with topical steroids (Elocon and Cutivate ointments) in combination with emollients (Aquaphor and Moisturel) was unsuccessful. What’s Your Diagnosis? CRP was described in 1927 in the French literature by Henri Gougerot and Alexandre Carteaud as papillomatose pigmentée innominée or papillomatose pigmentée confluente et reticulée. It was later renamed confluent and reticulated papillomatosis by Fred Wise, who reported the first case in the United States. The eruption is chronic with exacerbations and remissions. Although CRP responds to treatment, it frequently recurs after therapy is discontinued. Patients with CRP are often asymptomatic, but may experience mild pruritus. Clinically, CRP consists of confluent, flat, brown papules localized to the intramammary and interscapular regions. Subsequent spread occurs over weeks to months to the abdomen and periphery of the chest and back, forming a characteristic reticulated pigmented pattern, which led to the name of the disorder. Lower abdomen, flanks, and pubic area may also be involved. Cases of CRP lesions confined to the cheeks or the pubic areas have also been reported. The mucous membranes are usually spared. Reports of familial occurrence exist, but the majority of cases are sporadic. A Glance at Pathogenesis and Etiology CRP and acanthosis nigricans have clinical and histologic similarities, although no definite etiology has been identified for CRP. Suggested mechanisms include endocrine disturbances, disorder of keratinization, abnormal host reaction to fungi or bacteria, hereditary disorder, or a variant of amyloidosis. At the time of its description, Gougerot suggested that CRP might be due to endocrine disorders. Subsequent reports outlined the common observation of CRP in patients with obesity, abnormal glucose tolerance, diabetes mellitus, Cushing’s disease, menstrual irregularities, thyroid disease, pituitary dysfunction, and hirsutism or hypertrichosis, therefore suggesting some form of association. Recent investigative reports showed that the epidermis showed slight acanthosis with papillomatosis, a reduced granular layer, enhanced numbers of keratinosomes, and a loose hyperkeratotic horny layer. A peculiar finding in the keratinocytes of the upper granular layer was the presence of perinuclear hollow spaces in some patients with CRP. Fine membranes were recognizable in these spaces, and their remainders were evident in the keratinized cells of the horny layer in the form of stacks of elongated membranes and membrane coils. According to Arnold and Anton-Lamprecht,1 these characteristic markers confirmed a disturbance of epidermal differentiation and keratinization for this disease. Using electron microscopy, Inaloz et al2 showed an increased number of transitional cells between the stratum granulosum and the stratum corneum. Immunohistochemical examinations revealed suprabasal keratin 16 (K16) expression throughout the epidermis with intense focal staining in the stratum granulosum and an increased number of cycling epidermal cells with Ki-67 binding in the basal layer and stratum malpighii in two brothers with CRP. Increased melanosomes in the stratum corneum probably account for pigmentary changes. A hypothesis of abnormal reaction to Pityrosporum infections has been put forward. This is mainly due to the clinical resemblance of the rash to that of pityriasis versicolor. However, KOH preparation of scrapings from lesions failed to be consistently positive. Others also have advanced this theory in light of the common finding of Pityrosporum yeast in CRP lesions. Since the observation of improvement of CRP lesions in patients taking antibiotics for other conditions, a plethora of reports showed treatment success with different antibiotic families leading to the concept that one or multiple bacteria might be etiologic agents. Those bacteria or its products (bacterial superantigens) could induce the epidermal proliferation changes of CRP.Scattered reports showed some amyloid deposit in patients with CRP, but the small sample of patients included in these reports makes this theory unlikely. Treating CRP Different treatments have been used for CRP with variable results. The most consistently effective treatment, and the only one evaluated by retrospective and prospective studies, has been oral antibiotics.3-5 Treating CRP with antibiotics occurred after improvement was noted in a patient taking furacycline for arthritis. Since then, CRP has been successfully treated with tetracyclines, macrolides and cephalosporins. The most consistent results are seen with minocycline. Antibiotics are used for their antibacterial, and possibly anti-inflammatory and anti-proliferative effects. Tetracyclines are weak antimetabolites and might have an effect on the epidermal hyperplasia after observed CRP. Based on our findings, we treated our patient with minocycline (Minocin) 100 mg b.i.d. We achieved complete clearance of lesions in 4 weeks. Since therapy, our patient has been in clinical remission for 3 months. Oral and topical forms of retinoids have also been successful for CRP. They mainly correct disorders of keratinization. Vitamin D analogues regulate skin cell production and development. Calcipotriol 0.005% ointment (Dovonex) applications, twice daily for 3 weeks;6,7 and more recently, topical tacalcitol used twice daily as opposed to the usual once daily, were successful in clearing CRP lesions.8 Other modalities include: antifungals, keratolytics, sodium thiosulphate, ammoniated mercury, oral contraceptives, thyroid extract, ultraviolet light, and propylene glycol. Topical corticosteroids aren’t usually effective.
