REPORT
CONFLUENT AND RETICULATED PAPILLOMATOSIS: CLINICAL, LIGHT AND ELECTRON MICROSCOPIC STUDIES MIHOKOJIMISOW, M.D., PH.D.,
OREST TALPASH, M.D., E.R.C.P.C., AND KOWICHI JIMBOW, M.D., PH.D., F . R . C . P . C .
This report presents a case that clinically and histologically shares many features in common with those of CRP and shows electron microscopic findings of the skin lesions.
Abstract A case of a 17-year-old Japanese-Canadian boy with a 2-year history of skin lesions relevant to confluent and reticulated papillomatosis (CRP) is reported with electron microscopic findings. He had scattered skin lesions of brownish-colored,
Case Report
rough-surfaced, fine-scaled patches and plaques distributed
The patient is a 17-year-old Japanese-Canadian boy who has a 2-year history of reticular pigmented lesions on his trunk. His past medical history is unremarkable. There is not anyone who shares similar clinical features in his family members. Skin examination revealed brownish, flat-surfaced papules, which formed confluent patches and plaques with reticulate network on the nape and shoulders, the lateral aspects of the trunk, as well as the lower trunk and the gluteal, antecubital, and popliteal areas. The middle parts of the chest, back, and axillary areas were almost spared of the lesions. These reticulated papules had fine scales on their surface (Figs. 1 and 2). Peripheral blood and biochemical markers were within normal limits. Serum lipids and cholesterol sulfate were also within normal values.
on the nape, shoulders, and both flanks, extending to the chest and back. Importantly, they showed reticular or confluent arrangement. There was no family history of the same skin lesions. The histology showed epidermal papillomatosis and mild acanthosis with a marked hyperkeratosis. Electron microscopically, the skin lesions consisted of the following abnormal findings: (1) a marked alteration of cornified cell structures showing snake coil-like, or triangle-like stacks, (2) a marked increase in the number of lamellar granules in the granular layer, (3) an increased number of melanosomes in the horny layers, and (4) no significant fine structural changes of epidermal melanocytes. This is the first electron microscopic report on CRP suggesting that the pathophysiology of this disease is related to abnormal keratinocyte differentiation.
Confluent and reticulated papillomatosis (CRP) is a rare disease, which has a unique clinical feature and shows hyperkeratosis and papillomatosis histologically. This disease was first described by Gougerot and Carteaud in 1927.' It is characterized by greyish-brown hyperkeratotic patches and plaques with reticulated borders that evolve from the coalescence of initially erythematous papules, and later to hyperpigmented, slightly verrucous lesions. The intermammary region is usually affected first with subsequent spread to the breast and abdomen. The interscapular area, neck, and axilla also may be involved. The eruption often begins during puberty and more commonly affects women. The majority of cases are sporadic, but there are occasional reports of its occurrence in two or more members of a family. From the Division of Dermatology and Cutaneous Sciences, University of Alberta Faculty of Medicine, Edmonton, Alberta, Canada. Address for correspondence: Mihoko Jimbow, M.D., Ph.D., Dermatology and Cutaneous Sciences, Faculty of Medicine, 260G Heritage Medical Research Centre, University of Alberta, Edmonton, Alberta, Canada T6G 2S2.
Figure 1. Skin manifestation ol a 17-year-old boy with CRP. Note reticulated brownish plaques and papules distributed on the lateral trunk. Low power view of the lateral trunk. 480
Confluent and Reticulated PapiUomatosis Jimbow, Talpash, and Jimbow
Figure 2.
packed within individual corneocytes (horny-layered cells). The findings of Figure 4 would likely correspond to the area where we observed the hyperkeratosis with basket-weave pattern under the light microscopy. Apparently, there was a marked increase in the number of stacked corneocytes in the horny layer. There were also occasionally the remnants of desmosome-attachment plaques as well as melanosomes. These melanosomes in corneocytes showed partial degradation and fragmentation and did not have any apparent membrane structures consistent with the outer membrane of individual melanosomes, as could be seen in the melanocyte or lysosomal membrane in keratinocytes (melanosome complex). The epidermal melanocytes, however, did not show significant alterations in their fine structures. Figure 5 may show a much more prominent and abnormal finding regarding the profile of the cornified cells in the horny layer. There was a marked alteration in the arrangements of cornified cells, which were intermingled and twisted randomly, like snake-coil. The other abnormal finding of the cornified cells involved the edge of the individual cells, which was often bizarre and sharp, forming a triangle shape. In both situations, the filamentous components were densely packaged with the cornified cells.
CRP, high power view of the abdomen.
RESULTS
Light Microscopic Findings The hematoxylin and eosin staining of the typical skin lesion (lateral trunk) showed mild papillomatosis and mild acanthosis with a decreased granular layer. The horny layer showed follicular plugging and prominent basket-weaved hyperkeratosis. There was no fungal element or spores seen with PAS staining (Fig. 3).
hi addition, there was a marked increase in the number of lamellar granules (bodies) or Odland's bodies in the granular layer (Fig. 6). There was, however, little change in the fine structure of the keratohyaline granules.
Electron Microscopic Findings Our electron microscopic study provided basic important information regarding the nature of hyperkeratosis and pigmentation that was characteristically seen by clinical and light microscopic observations. Figures 4 and 5 represent low power views of cornified cells in the horny layer. Thickened keratin materials were densely
DISCUSSION
/
The pathophysiology of CRP is still unknown. Because of histologic resemblance, some authors consider that this disorder represents a variant of acanthosis nigricans either of the inherited or of the idiopathic type.-
Figure 4. Multilayered corneocytes in the stratum corneum. Arrows indicate the fragmented melanosomes (MS) within the corneocytes. us, the remnant of desmosome-attachment plaques, (original magnification x 7500)
Figure 3. Hematoxylin and eosin staining of the hyperkeratotic plaque showing hyperkeratosis and papillomatosis with follicular plug, (original magnification x 400) 481
International Journal of Dermatology Vol. 31, No. 7, July 1992
Figure 5. Altered arrangement of multilayered corneocytes in the stratum corneum. They were intermingled and twisted randomly like a snake-coil (arrows). MS, melanosomes. (original magnification x 12,000)
Figure 6. Stratum corneum showing normal pattern of keratohyalin granules (KH), but increased number of lamellar granules (arrows), (original magnification: a, x 6000; b, x 18,000)
conditions of rapid cell turnover and increased desquamation, such as psoriasis," as well as in certain acantholytic states such as epidermolytic hyperkeratosis.'^ Hyperkeratosis in CRP might be related to hyperproliferation of the epidermis. A case of uv-induced CRP was recently reported, and an abnormal epidermal response to UV light was suggested to have caused the abnormal keratinization." An endocrine imbalance was suggested as another possible cause.''' In these cases, abnormal glucose tolerance or frank diabetes were detected. Pseudoacanthosis nigricans, either solitary or familial, with or without Pityrosporum organisms, may mimic CRP. In our case, none of these signs were identified. The brownish pigmentation is another unique feature of this disease. Our electron microscopic study showed that this increased melanin pigmentation is related to the increased number of melanosomes present in the hyperkeratotic horny layer, not related to the increased melanogenesis within the melanocyte. Abnormal pig-' mentation of Pityrosporum infection under electron microscopy revealed small, sometimes abnormal, melanosomes, an increased number of melanosomes in melanocytes, a decreased number of melanosomes in keratinocytes, and irregular melanosome distribution in keratinocytes. In addition, abnormal stage I melanosomes were reported.'^ In contrast, brownish pigmentation associated with an endocrinopathy, pseudoacanthosis nigricans, would be directly or indirectly related to the stimulation of melanocyte stimulating hormone (MSH), which results in an increased melanogenic activity of melanocytes and an increased dispersion of melanosomes within melanocytes, enhancing both movement and transfer of melanosomes to keratinocytes.'^ All of their findings may indicate that the abnormal melanin pigmentation in CRP is different from that seen in the above disease conditions.
Most authors, however, regard it as a disease entity because of its location and reticulated pattern. Based on a clinical resemblance of pityriasis versicolor and the occasional findings of yeast forms in some cases, as well as responses to treatment with topical and systemic antifungal agents, some authors have proposed that the CRP represents a peculiar host reaction to pityriasis versicolor.^"^ But in many other cases, including our patient, mycologic examinations revealed the absence of this organism. Furthermore, considering the common presence of the yeast as a nonpathogen, the role of Pityrosporum (Malassezia furfur) as the cause of CRP can be discounted. Some responses to treatment with oral and topical vitamin A and aromatic retinoid, etretinate, or isotretinoin made some authors support Miescher's concept of keratinization disorder as the basic defect in patients with CRP.''"*' In our case, the distribution of the lesion is different from the typical case of CRP because middle parts of trunk and axillary areas are almost spared from the lesion. The reticular pattern of the lesion and histologic findings are consistent with those of CRP. Importantly, our electron microscopic study showed two major findings of keratinocyte differentiation: (1) a marked alteration of cornified cell structures showing snake coll-like or triangle-like stacks, and (2) a marked increase in the number of lamellar granules in the granular layers. The latter finding is interesting to speculate the pathophysiology of hyperkeratosis in CRP. Lamellar granules can mediate cell cohesion of stratum corneum by releasing contents and lipids. The hydrolytic contents of the granules that accompany the release of lipids are, in part, responsible for their reorganization and subsequent assembly in the intercorneocyte spaces to form the intercellular lamellae in the stratum corneum.'''" They have been noted to be particularly numerous in 482
Confluent and Reticulated Papillomatosis .[imbow, Talpash, and Jimbow
REFERENCES
9.
Elias PM. Epidermal lipids, harrier function, and desquamation. J Invest Dermatol 1983; 80:44s-49s.
1.
Gougerot H, Carteaud A. Papillomatose pigmentee innominee. Bull Soc Fr Dermatol Syphiligr 1927; 34: 7f9-721.
10.
2.
Kesten BM, Jarnes HD. Pseudoatrophoderma colli, acanthosis nigricans and confluent and reticular papillomatosis. Arch Dermatol f 957; 75:525-542. Roherts SOB, Lachapelle JM. Confluent and reticulate papillomatosis (Gougerot-Carteaud) and pityrosporum orbiculare. Br J Dermatol 1969; 81:841-845.
Wertz PW, Downing DT. Epidermal lipids. In: Goldsmith LA, ed. Physiology, hiochemistry, and molecular hiology of the skin. Oxford University Press: New York, 1991:205.
11.
Zech H, Lagerholm B, von Weittstein D. Particles resemhling virus in psoriatic lesions. Virology 1961; 14: 489.
12.
Wilgram GF, Weinstock A. Advances in genetic dermatology. Arch Dermatol 1966; 94:456-479.
13.
Vassileva S, Pramatarov K, Poppova L. Ultraviolet light - induced confluent and reticulated papillomatosis. J Am Acad Dermatol 1989; 21:413-414.
14.
Hamilton D, Tavafoghi V, Shafer JG, Hamhrick GW Jr. Gonfluent and reticulated papillomatosis of Gougerot and Garteaud. J Am Acad Dermatol 1980; 2:401-410.
15.
Mosher DB, Fitzpatrick TB, Ortonne JP, Hori Y. Disorders of pigmentation. In: Fitzpatrick TB, et al., eds. Dermatology in general medicine. New York: McGraw Hill 1987:794.
16.
Jimbow M, Jimhow K. Pigmentary disorders in oriental skin. Glin Dermatol 1989; 7:11-27.
3.
4.
5. 6. 7.
8.
Yesudian P, Kamalam S, Razack A. Confluent and reticulated papillomatosis (Gougerot-Carteaud). Acta Derm Venereol (Stockh) 1973; 53:381-384. Kellet JK, Macdonald RH. Confluent and reticulated papillomatosis. Arch Dermatol 1985; 121:587-588. Miescher VG. Erythrokeratodermia paplUaris et reticularis: Dermatologica 1954; 108:303-309. Bruynzeel-koomen CAFM, de Wit RFE. Confluent and reticulated papillomatosis successfully treated with aromatic etretinate. Arch Dermatol 1984; 120:1236-1237. Hodge JA, Ray MG. Gonfluenr and reticulated papillomatosis: response to isotretinoin. J Am Acad Dermatol 1991; 16:654.
Malakoplakia
.
Malakoplakia is a rare chronic inflammatory disorder characterised hy the formation of granulomas in the hladder or other organs of the genitourinary tract, but other organs and tissues including testis, epididymis, lungs, bone, lymph nodes, and retroperitoneal tissue can also be affected. Clinically, the tumour-like nodules mimic a malignant neoplasm and the diagnosis can be made only by histological examination. Light microscopy shows the accumulation of macrophages with a characteristic morphology—von Hansemann macrophages. Large calcified structures (MichaelisGutmann bodies) are found in the cytoplasm of these macrophages. An infectious cause of malakoplakia is suggested by the electronmicroscopic finding of coliform bacteria in the phagolysosomes of macrophages. Little is known about the pathogenesis of the disease. Defective killing and impaired digestion of phagocytosed bacteria are suggested by the characteristic intracellular abnormalities of the macrophages. Lower than normal bactericidal activity against Escherichia coli and signs of lysomal abnormalities in monocytes were found in one patient. Treatment of malakoplakia usually aims to improve macrophage function, by means of cholinergic agonists (eg, bethanechol), and to eliminate bacteria with antimicrobial drugs. Cholinergic agonists raise intracellular cyclic C M P concentrations, which in turn stimulates synthesis of tumour necrosis factor; the latter enhances the microbicidal function of macrophages (unpublished). Such regimens rarely produce a sustained response, however, and the lesions c o n t i n u e to progress slowly. Failure of antimicrobial therapy could be due to poor penetration of the drug into the macrophages or its low activity in the phagolysosomes of the cells. Cure of malakoplakia by long-term antibiotic treatment has not been re-
ported. From van Furth R, van't Wout JW, Wertheimer PA, Zwartendijk J. Ciprofloxacin for treatment of malakoplakia. Lancet 1992; 339:148-149. 483
CONFLUENT AND RETICULATED PAPILLOMATOSIS: CLINICAL, LIGHT AND ELECTRON MICROSCOPIC STUDIES MIHOKOJIMISOW, M.D., PH.D.,
OREST TALPASH, M.D., E.R.C.P.C., AND KOWICHI JIMBOW, M.D., PH.D., F . R . C . P . C .
This report presents a case that clinically and histologically shares many features in common with those of CRP and shows electron microscopic findings of the skin lesions.
Abstract A case of a 17-year-old Japanese-Canadian boy with a 2-year history of skin lesions relevant to confluent and reticulated papillomatosis (CRP) is reported with electron microscopic findings. He had scattered skin lesions of brownish-colored,
Case Report
rough-surfaced, fine-scaled patches and plaques distributed
The patient is a 17-year-old Japanese-Canadian boy who has a 2-year history of reticular pigmented lesions on his trunk. His past medical history is unremarkable. There is not anyone who shares similar clinical features in his family members. Skin examination revealed brownish, flat-surfaced papules, which formed confluent patches and plaques with reticulate network on the nape and shoulders, the lateral aspects of the trunk, as well as the lower trunk and the gluteal, antecubital, and popliteal areas. The middle parts of the chest, back, and axillary areas were almost spared of the lesions. These reticulated papules had fine scales on their surface (Figs. 1 and 2). Peripheral blood and biochemical markers were within normal limits. Serum lipids and cholesterol sulfate were also within normal values.
on the nape, shoulders, and both flanks, extending to the chest and back. Importantly, they showed reticular or confluent arrangement. There was no family history of the same skin lesions. The histology showed epidermal papillomatosis and mild acanthosis with a marked hyperkeratosis. Electron microscopically, the skin lesions consisted of the following abnormal findings: (1) a marked alteration of cornified cell structures showing snake coil-like, or triangle-like stacks, (2) a marked increase in the number of lamellar granules in the granular layer, (3) an increased number of melanosomes in the horny layers, and (4) no significant fine structural changes of epidermal melanocytes. This is the first electron microscopic report on CRP suggesting that the pathophysiology of this disease is related to abnormal keratinocyte differentiation.
Confluent and reticulated papillomatosis (CRP) is a rare disease, which has a unique clinical feature and shows hyperkeratosis and papillomatosis histologically. This disease was first described by Gougerot and Carteaud in 1927.' It is characterized by greyish-brown hyperkeratotic patches and plaques with reticulated borders that evolve from the coalescence of initially erythematous papules, and later to hyperpigmented, slightly verrucous lesions. The intermammary region is usually affected first with subsequent spread to the breast and abdomen. The interscapular area, neck, and axilla also may be involved. The eruption often begins during puberty and more commonly affects women. The majority of cases are sporadic, but there are occasional reports of its occurrence in two or more members of a family. From the Division of Dermatology and Cutaneous Sciences, University of Alberta Faculty of Medicine, Edmonton, Alberta, Canada. Address for correspondence: Mihoko Jimbow, M.D., Ph.D., Dermatology and Cutaneous Sciences, Faculty of Medicine, 260G Heritage Medical Research Centre, University of Alberta, Edmonton, Alberta, Canada T6G 2S2.
Figure 1. Skin manifestation ol a 17-year-old boy with CRP. Note reticulated brownish plaques and papules distributed on the lateral trunk. Low power view of the lateral trunk. 480
Confluent and Reticulated PapiUomatosis Jimbow, Talpash, and Jimbow
Figure 2.
packed within individual corneocytes (horny-layered cells). The findings of Figure 4 would likely correspond to the area where we observed the hyperkeratosis with basket-weave pattern under the light microscopy. Apparently, there was a marked increase in the number of stacked corneocytes in the horny layer. There were also occasionally the remnants of desmosome-attachment plaques as well as melanosomes. These melanosomes in corneocytes showed partial degradation and fragmentation and did not have any apparent membrane structures consistent with the outer membrane of individual melanosomes, as could be seen in the melanocyte or lysosomal membrane in keratinocytes (melanosome complex). The epidermal melanocytes, however, did not show significant alterations in their fine structures. Figure 5 may show a much more prominent and abnormal finding regarding the profile of the cornified cells in the horny layer. There was a marked alteration in the arrangements of cornified cells, which were intermingled and twisted randomly, like snake-coil. The other abnormal finding of the cornified cells involved the edge of the individual cells, which was often bizarre and sharp, forming a triangle shape. In both situations, the filamentous components were densely packaged with the cornified cells.
CRP, high power view of the abdomen.
RESULTS
Light Microscopic Findings The hematoxylin and eosin staining of the typical skin lesion (lateral trunk) showed mild papillomatosis and mild acanthosis with a decreased granular layer. The horny layer showed follicular plugging and prominent basket-weaved hyperkeratosis. There was no fungal element or spores seen with PAS staining (Fig. 3).
hi addition, there was a marked increase in the number of lamellar granules (bodies) or Odland's bodies in the granular layer (Fig. 6). There was, however, little change in the fine structure of the keratohyaline granules.
Electron Microscopic Findings Our electron microscopic study provided basic important information regarding the nature of hyperkeratosis and pigmentation that was characteristically seen by clinical and light microscopic observations. Figures 4 and 5 represent low power views of cornified cells in the horny layer. Thickened keratin materials were densely
DISCUSSION
/
The pathophysiology of CRP is still unknown. Because of histologic resemblance, some authors consider that this disorder represents a variant of acanthosis nigricans either of the inherited or of the idiopathic type.-
Figure 4. Multilayered corneocytes in the stratum corneum. Arrows indicate the fragmented melanosomes (MS) within the corneocytes. us, the remnant of desmosome-attachment plaques, (original magnification x 7500)
Figure 3. Hematoxylin and eosin staining of the hyperkeratotic plaque showing hyperkeratosis and papillomatosis with follicular plug, (original magnification x 400) 481
International Journal of Dermatology Vol. 31, No. 7, July 1992
Figure 5. Altered arrangement of multilayered corneocytes in the stratum corneum. They were intermingled and twisted randomly like a snake-coil (arrows). MS, melanosomes. (original magnification x 12,000)
Figure 6. Stratum corneum showing normal pattern of keratohyalin granules (KH), but increased number of lamellar granules (arrows), (original magnification: a, x 6000; b, x 18,000)
conditions of rapid cell turnover and increased desquamation, such as psoriasis," as well as in certain acantholytic states such as epidermolytic hyperkeratosis.'^ Hyperkeratosis in CRP might be related to hyperproliferation of the epidermis. A case of uv-induced CRP was recently reported, and an abnormal epidermal response to UV light was suggested to have caused the abnormal keratinization." An endocrine imbalance was suggested as another possible cause.''' In these cases, abnormal glucose tolerance or frank diabetes were detected. Pseudoacanthosis nigricans, either solitary or familial, with or without Pityrosporum organisms, may mimic CRP. In our case, none of these signs were identified. The brownish pigmentation is another unique feature of this disease. Our electron microscopic study showed that this increased melanin pigmentation is related to the increased number of melanosomes present in the hyperkeratotic horny layer, not related to the increased melanogenesis within the melanocyte. Abnormal pig-' mentation of Pityrosporum infection under electron microscopy revealed small, sometimes abnormal, melanosomes, an increased number of melanosomes in melanocytes, a decreased number of melanosomes in keratinocytes, and irregular melanosome distribution in keratinocytes. In addition, abnormal stage I melanosomes were reported.'^ In contrast, brownish pigmentation associated with an endocrinopathy, pseudoacanthosis nigricans, would be directly or indirectly related to the stimulation of melanocyte stimulating hormone (MSH), which results in an increased melanogenic activity of melanocytes and an increased dispersion of melanosomes within melanocytes, enhancing both movement and transfer of melanosomes to keratinocytes.'^ All of their findings may indicate that the abnormal melanin pigmentation in CRP is different from that seen in the above disease conditions.
Most authors, however, regard it as a disease entity because of its location and reticulated pattern. Based on a clinical resemblance of pityriasis versicolor and the occasional findings of yeast forms in some cases, as well as responses to treatment with topical and systemic antifungal agents, some authors have proposed that the CRP represents a peculiar host reaction to pityriasis versicolor.^"^ But in many other cases, including our patient, mycologic examinations revealed the absence of this organism. Furthermore, considering the common presence of the yeast as a nonpathogen, the role of Pityrosporum (Malassezia furfur) as the cause of CRP can be discounted. Some responses to treatment with oral and topical vitamin A and aromatic retinoid, etretinate, or isotretinoin made some authors support Miescher's concept of keratinization disorder as the basic defect in patients with CRP.''"*' In our case, the distribution of the lesion is different from the typical case of CRP because middle parts of trunk and axillary areas are almost spared from the lesion. The reticular pattern of the lesion and histologic findings are consistent with those of CRP. Importantly, our electron microscopic study showed two major findings of keratinocyte differentiation: (1) a marked alteration of cornified cell structures showing snake coll-like or triangle-like stacks, and (2) a marked increase in the number of lamellar granules in the granular layers. The latter finding is interesting to speculate the pathophysiology of hyperkeratosis in CRP. Lamellar granules can mediate cell cohesion of stratum corneum by releasing contents and lipids. The hydrolytic contents of the granules that accompany the release of lipids are, in part, responsible for their reorganization and subsequent assembly in the intercorneocyte spaces to form the intercellular lamellae in the stratum corneum.'''" They have been noted to be particularly numerous in 482
Confluent and Reticulated Papillomatosis .[imbow, Talpash, and Jimbow
REFERENCES
9.
Elias PM. Epidermal lipids, harrier function, and desquamation. J Invest Dermatol 1983; 80:44s-49s.
1.
Gougerot H, Carteaud A. Papillomatose pigmentee innominee. Bull Soc Fr Dermatol Syphiligr 1927; 34: 7f9-721.
10.
2.
Kesten BM, Jarnes HD. Pseudoatrophoderma colli, acanthosis nigricans and confluent and reticular papillomatosis. Arch Dermatol f 957; 75:525-542. Roherts SOB, Lachapelle JM. Confluent and reticulate papillomatosis (Gougerot-Carteaud) and pityrosporum orbiculare. Br J Dermatol 1969; 81:841-845.
Wertz PW, Downing DT. Epidermal lipids. In: Goldsmith LA, ed. Physiology, hiochemistry, and molecular hiology of the skin. Oxford University Press: New York, 1991:205.
11.
Zech H, Lagerholm B, von Weittstein D. Particles resemhling virus in psoriatic lesions. Virology 1961; 14: 489.
12.
Wilgram GF, Weinstock A. Advances in genetic dermatology. Arch Dermatol 1966; 94:456-479.
13.
Vassileva S, Pramatarov K, Poppova L. Ultraviolet light - induced confluent and reticulated papillomatosis. J Am Acad Dermatol 1989; 21:413-414.
14.
Hamilton D, Tavafoghi V, Shafer JG, Hamhrick GW Jr. Gonfluent and reticulated papillomatosis of Gougerot and Garteaud. J Am Acad Dermatol 1980; 2:401-410.
15.
Mosher DB, Fitzpatrick TB, Ortonne JP, Hori Y. Disorders of pigmentation. In: Fitzpatrick TB, et al., eds. Dermatology in general medicine. New York: McGraw Hill 1987:794.
16.
Jimbow M, Jimhow K. Pigmentary disorders in oriental skin. Glin Dermatol 1989; 7:11-27.
3.
4.
5. 6. 7.
8.
Yesudian P, Kamalam S, Razack A. Confluent and reticulated papillomatosis (Gougerot-Carteaud). Acta Derm Venereol (Stockh) 1973; 53:381-384. Kellet JK, Macdonald RH. Confluent and reticulated papillomatosis. Arch Dermatol 1985; 121:587-588. Miescher VG. Erythrokeratodermia paplUaris et reticularis: Dermatologica 1954; 108:303-309. Bruynzeel-koomen CAFM, de Wit RFE. Confluent and reticulated papillomatosis successfully treated with aromatic etretinate. Arch Dermatol 1984; 120:1236-1237. Hodge JA, Ray MG. Gonfluenr and reticulated papillomatosis: response to isotretinoin. J Am Acad Dermatol 1991; 16:654.
Malakoplakia
.
Malakoplakia is a rare chronic inflammatory disorder characterised hy the formation of granulomas in the hladder or other organs of the genitourinary tract, but other organs and tissues including testis, epididymis, lungs, bone, lymph nodes, and retroperitoneal tissue can also be affected. Clinically, the tumour-like nodules mimic a malignant neoplasm and the diagnosis can be made only by histological examination. Light microscopy shows the accumulation of macrophages with a characteristic morphology—von Hansemann macrophages. Large calcified structures (MichaelisGutmann bodies) are found in the cytoplasm of these macrophages. An infectious cause of malakoplakia is suggested by the electronmicroscopic finding of coliform bacteria in the phagolysosomes of macrophages. Little is known about the pathogenesis of the disease. Defective killing and impaired digestion of phagocytosed bacteria are suggested by the characteristic intracellular abnormalities of the macrophages. Lower than normal bactericidal activity against Escherichia coli and signs of lysomal abnormalities in monocytes were found in one patient. Treatment of malakoplakia usually aims to improve macrophage function, by means of cholinergic agonists (eg, bethanechol), and to eliminate bacteria with antimicrobial drugs. Cholinergic agonists raise intracellular cyclic C M P concentrations, which in turn stimulates synthesis of tumour necrosis factor; the latter enhances the microbicidal function of macrophages (unpublished). Such regimens rarely produce a sustained response, however, and the lesions c o n t i n u e to progress slowly. Failure of antimicrobial therapy could be due to poor penetration of the drug into the macrophages or its low activity in the phagolysosomes of the cells. Cure of malakoplakia by long-term antibiotic treatment has not been re-
ported. From van Furth R, van't Wout JW, Wertheimer PA, Zwartendijk J. Ciprofloxacin for treatment of malakoplakia. Lancet 1992; 339:148-149. 483
![[Confluent and reticulated papillomatosis of Gougerot-Carteaud: about a case].](/assets/img/hold.png)
