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Reticulate pigmented anomaly of the flexures associating reticulate acropigmentation: One single entity
Reticulate pigmented anomaly of the flexures associating reticulate acropigmentation: One single ~ntity Franco Crovato, M.D.,* and Alfredo Rebora, M.D.** Lavagna (GE) and Genoa, Italy We describe a patient and her family in whom the clinical features of reticulate pigmented anomaly of the flexures, also known as Dowling-Degas disease, are associated with those of Kitamura's reticulate acropigmentation. This family is the second in which the concurrence of such rare genodermatoses is reported. It seems likely that Dowling-Degos disease, and Kitamura's reticulate acropigmentation are different clinical expressions of the same entity. (J AM ACAD DERMATOL 14:359-361, 1986.)
Kitamura's reticulate acropigmentation is a rare disease characterized by reticulate, brownish-pigmented macules on the dorsa of the hands and feet and by typical "pits" on the palms. It has been described in only a few white patients, whereas in Japan more than fifty cases have been reported. In 1983 we' observed a patient in whom the clinical and histopathologic features of Kitamura's reticulate acropigmentation were associated with those of the reticulate pigmented anomaly of the flexures, or Dowling-Degos disease, in which deeply pigmented macules arranged in a reticulate pattern affect the flexures, the inner aspects of thighs, the neck and rarely the wrists, the scrotum, and the face and scalp. Small comedo-like lesions are often present. 2 In the family of our patient the two diseases were variously distributed, suggesting that they are different clinical expressions of the same entity. 1,3 We found another family with the same association, which confirms our point of view. From the Division of Dermatology, Chiavari-Lavagna Hospital, Lavagna,* and the Department of Dermatology, University of Genoa.** Reprint requests to: Dr. Alfredo Rebora, Istituto di Dermatologia, Viale Benedetto XV, 7, 16132, Genoa, Italy.
Fig. 1. Small macules on the inner aspects of the thigh. Note comedo-like lesions (arrows).
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Fig. 2. Macules on the dorsum of the hand. CASE REPORT
The patient was a 36-year-old woman of southern Italian origin who, since her puberty, had complained of a progressively spreading hyperpigmentation of the flexures and hands. On examination she had several brownish-pigmented, small, reticulate macules on the inner aspects of her thighs, inframammary region, neck, and armpits, interspersed with small, comedo-like lesions (Fig. 1). On the dorsa of her hands, she exhibited smaller, slightly depressed macules of the same color arranged in a reticulate pattern (Fig. 2). On her palms, there were several interruptions of the epidermal ridge pattern. The patient disregarded her anomaly as a well-known trivial problem of members of her family, some of whom, incidentally, are living a thousand miles away. As a consequence we were not able to examine the entire family, but we observed similar lesions in the mother (Fig. 3) and in one aunt of the proposita; one brother was free of the lesions. Histopathologic findings in two biopsy specimens from the thigh and hand were substantially normal, revealing only a modest papillomatosis. The patient refused further biopsies. COMMENT
The presence of lesions of both diseases is indisputable. On clinical grounds, no other diagnosis may be made. For example, dyskeratosis congenita displays a number of anomalies (scarring alopecia, dental anomalies, leukoplakia, etc.) that were absent in our patient and that occur only in males. Furthermore, the Franceschetti-Jadassohn syndrome is characterized by discoloration of teeth, reduced eccrine glands, and palmar-plantar kera-
Journal of the American Academy of Dermatology
Fig. 3. Typical macules of Dowling-Degos disease on the inner aspects of the thigh of the patient's mother.
toderma, none of which had occurred in our patient, and dermatopathia pigmentosa reticularis and Dohi' s acropigmentation have hypopigmented lesions. Histopathologic findings differed from the typical aspect of Kitamura's reticulate acropigmentation and Dowling-Degos disease. Normal skin features can be seen, however, in the illustration presented by Griffiths 4 and in the patient with Kitamura's reticulate aeropigmentation reported by Woodley et aI,5 and they are probably to be considered as an occasional finding. More biopsies would perhaps detect the typical digitations of Dowling-Degos disease and Kitamura's reticulate acropigmentation. The association of Dowling-Degas disease with Kitamura's reticulate acropigmentation may be suspected in other cases reported in the literature. The five patients described by Becker and Reuter' as having familial pigmentary anomaly and the patient of Montgomery and Reuter,? with the same diagnosis but later repudiated as such,6 probably are genuine cases of associated Dowling-Degos disease and Kitamura's reticulate acropigmentafion. Rhone et aI, * as quoted by Dr. Ichiro Kikucki (personal communication, 1984), described a Japanese family with Kitamura's reticulate acropigmentation but displaying lesions that resembled Dowling-Degos disease; in addition, the patient with Kitamura's reticulate acropigmentation re*Hirone T, Yamasaki T, Nagai T: A father and her daughter with Kitamura's aeropigmentation. Rinsho Dennatol 5:774, 1963.
Volume 14 Number 2, Part 2 February, 1986
cently reported by Bajaj and Gupta8 had lesions of the neck and breast that recall Dowling-Degas disease. We believe that our original hypothesis-that the reticulate pigmentation of the flexures and reticulate acropigmentation are a single entity-may be strongly supported by these new findings.
REFERENCES 1. Crovato F, Desirello G, Rebora A: Is Dowling-Degas disease the same disease as Kitamura's reticulate acropigmentation? Br J Dermatol109:105-110, 1983.
Reticulate pigmented anomaly
2. Wilson-Jones E, Grice K: Reticulate pigmented anomaly of the flexures. Arch Dermatol 114:1150-1157, 1978. 3. Rebora A, Crovato F: The spectrum of Dowling-Degos disease. Br J DermatolllO:627-630, 1984. 4. Griffiths WAD: Reticulate acropigmentation of Kitamura. Br J Dermatol 95:437-443, 1976. 5. Woodley DT, CarD I, Wheeler CE: Reticulate acropigmentation of Kitamura. Arch Dermato1 115:760-761, 1979. 6. Becker SW, Reuter MJ: A familial pigmentary anomaly. Arch Dermatol Syphilol 40:987-998, 1939. 7. Montgomery H, Reuter MJ: Xerodenna pigmentosum. Arch Dermatol Syphilol 26:256-267, 1932. 8. Bajaj AK, Gupta SC: Reticulate acropigmentation of Kitamura. Dermatologica 168:247-249, 1984.
Necrotizing fasciitis associated with porphyria cutanea tarda * Kenneth R. Kranz, Captain, MC, USA,** Oliver M. Reed, Captain, MC, USA,*** and Ronald E. Grimwood, Lieutenant Colonel, MC, USA*** Aurora, CO A case is presented of a 31-year-old white woman with necrotizing fasciitis. She had had a previous diagnosis of porphyria clltanea tarda that was manifested in part by bullous lesions on her forearms and hands. A soft tissue infection developed in her left hand and forearm in an area of bullous disease. After a delay of 8 days the diagnosis of necrotizing fasciitis was made. She recovered following surgical debridement, extensive fasciotomy, broadspectrum antibiotic coverage, and hyperbaric oxygen therapy. (J AM ACAD DERMATOL 14:361-367, 1986.)
Necrotizing fasciitis is a rare soft-tissue infection characterized by widespread fascial necrosis with relative sparing of skin and underlying muscle. It is frequently associated with severe systemic toxicity and is usually rapidly fatal unless promptly recognized and aggressively treated. The most freFrom the Department of Medicine** and the Department of Medicinel Dennatology Service,*** Fitzsimons Army Medical Center. Reprint requests to: Dr. Ronald E. Grimwood, Ohio State University, 456 Clinic Dr., Room 4731, Columbus, OR 43210. *The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.
quent site of infection is an extremity, 1-6 with other areas being the trunk, 1.3-6 the neck, 6 the face,3,7 and the perineum and genitalia. 1,2,4,6.8.10 There appears to be no age or sex predilection. The introduction of bacteria into the subcutaneous space can occur through any compromise of the protective integument. Necrotizing fasciitis has developed following a variety of lesions, including minor trauma,I-3.4,6 skin abscess,I,2,6 needle sites in narcotics addicts,I,6 and frostbite,l as well as after compound fractures 1 or insect bite4 and in surgical wounds. II .12 In some individuals, no identifiable focal lesion is found. I.3.6 Grossman et aP3 reviewed the clinical and lab-
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Kitamura's reticulate acropigmentation is a rare disease characterized by reticulate, brownish-pigmented macules on the dorsa of the hands and feet and by typical "pits" on the palms. It has been described in only a few white patients, whereas in Japan more than fifty cases have been reported. In 1983 we' observed a patient in whom the clinical and histopathologic features of Kitamura's reticulate acropigmentation were associated with those of the reticulate pigmented anomaly of the flexures, or Dowling-Degos disease, in which deeply pigmented macules arranged in a reticulate pattern affect the flexures, the inner aspects of thighs, the neck and rarely the wrists, the scrotum, and the face and scalp. Small comedo-like lesions are often present. 2 In the family of our patient the two diseases were variously distributed, suggesting that they are different clinical expressions of the same entity. 1,3 We found another family with the same association, which confirms our point of view. From the Division of Dermatology, Chiavari-Lavagna Hospital, Lavagna,* and the Department of Dermatology, University of Genoa.** Reprint requests to: Dr. Alfredo Rebora, Istituto di Dermatologia, Viale Benedetto XV, 7, 16132, Genoa, Italy.
Fig. 1. Small macules on the inner aspects of the thigh. Note comedo-like lesions (arrows).
359
360
Crovato and Rebora
Fig. 2. Macules on the dorsum of the hand. CASE REPORT
The patient was a 36-year-old woman of southern Italian origin who, since her puberty, had complained of a progressively spreading hyperpigmentation of the flexures and hands. On examination she had several brownish-pigmented, small, reticulate macules on the inner aspects of her thighs, inframammary region, neck, and armpits, interspersed with small, comedo-like lesions (Fig. 1). On the dorsa of her hands, she exhibited smaller, slightly depressed macules of the same color arranged in a reticulate pattern (Fig. 2). On her palms, there were several interruptions of the epidermal ridge pattern. The patient disregarded her anomaly as a well-known trivial problem of members of her family, some of whom, incidentally, are living a thousand miles away. As a consequence we were not able to examine the entire family, but we observed similar lesions in the mother (Fig. 3) and in one aunt of the proposita; one brother was free of the lesions. Histopathologic findings in two biopsy specimens from the thigh and hand were substantially normal, revealing only a modest papillomatosis. The patient refused further biopsies. COMMENT
The presence of lesions of both diseases is indisputable. On clinical grounds, no other diagnosis may be made. For example, dyskeratosis congenita displays a number of anomalies (scarring alopecia, dental anomalies, leukoplakia, etc.) that were absent in our patient and that occur only in males. Furthermore, the Franceschetti-Jadassohn syndrome is characterized by discoloration of teeth, reduced eccrine glands, and palmar-plantar kera-
Journal of the American Academy of Dermatology
Fig. 3. Typical macules of Dowling-Degos disease on the inner aspects of the thigh of the patient's mother.
toderma, none of which had occurred in our patient, and dermatopathia pigmentosa reticularis and Dohi' s acropigmentation have hypopigmented lesions. Histopathologic findings differed from the typical aspect of Kitamura's reticulate acropigmentation and Dowling-Degos disease. Normal skin features can be seen, however, in the illustration presented by Griffiths 4 and in the patient with Kitamura's reticulate aeropigmentation reported by Woodley et aI,5 and they are probably to be considered as an occasional finding. More biopsies would perhaps detect the typical digitations of Dowling-Degos disease and Kitamura's reticulate acropigmentation. The association of Dowling-Degas disease with Kitamura's reticulate acropigmentation may be suspected in other cases reported in the literature. The five patients described by Becker and Reuter' as having familial pigmentary anomaly and the patient of Montgomery and Reuter,? with the same diagnosis but later repudiated as such,6 probably are genuine cases of associated Dowling-Degos disease and Kitamura's reticulate acropigmentafion. Rhone et aI, * as quoted by Dr. Ichiro Kikucki (personal communication, 1984), described a Japanese family with Kitamura's reticulate acropigmentation but displaying lesions that resembled Dowling-Degos disease; in addition, the patient with Kitamura's reticulate acropigmentation re*Hirone T, Yamasaki T, Nagai T: A father and her daughter with Kitamura's aeropigmentation. Rinsho Dennatol 5:774, 1963.
Volume 14 Number 2, Part 2 February, 1986
cently reported by Bajaj and Gupta8 had lesions of the neck and breast that recall Dowling-Degas disease. We believe that our original hypothesis-that the reticulate pigmentation of the flexures and reticulate acropigmentation are a single entity-may be strongly supported by these new findings.
REFERENCES 1. Crovato F, Desirello G, Rebora A: Is Dowling-Degas disease the same disease as Kitamura's reticulate acropigmentation? Br J Dermatol109:105-110, 1983.
Reticulate pigmented anomaly
2. Wilson-Jones E, Grice K: Reticulate pigmented anomaly of the flexures. Arch Dermatol 114:1150-1157, 1978. 3. Rebora A, Crovato F: The spectrum of Dowling-Degos disease. Br J DermatolllO:627-630, 1984. 4. Griffiths WAD: Reticulate acropigmentation of Kitamura. Br J Dermatol 95:437-443, 1976. 5. Woodley DT, CarD I, Wheeler CE: Reticulate acropigmentation of Kitamura. Arch Dermato1 115:760-761, 1979. 6. Becker SW, Reuter MJ: A familial pigmentary anomaly. Arch Dermatol Syphilol 40:987-998, 1939. 7. Montgomery H, Reuter MJ: Xerodenna pigmentosum. Arch Dermatol Syphilol 26:256-267, 1932. 8. Bajaj AK, Gupta SC: Reticulate acropigmentation of Kitamura. Dermatologica 168:247-249, 1984.
Necrotizing fasciitis associated with porphyria cutanea tarda * Kenneth R. Kranz, Captain, MC, USA,** Oliver M. Reed, Captain, MC, USA,*** and Ronald E. Grimwood, Lieutenant Colonel, MC, USA*** Aurora, CO A case is presented of a 31-year-old white woman with necrotizing fasciitis. She had had a previous diagnosis of porphyria clltanea tarda that was manifested in part by bullous lesions on her forearms and hands. A soft tissue infection developed in her left hand and forearm in an area of bullous disease. After a delay of 8 days the diagnosis of necrotizing fasciitis was made. She recovered following surgical debridement, extensive fasciotomy, broadspectrum antibiotic coverage, and hyperbaric oxygen therapy. (J AM ACAD DERMATOL 14:361-367, 1986.)
Necrotizing fasciitis is a rare soft-tissue infection characterized by widespread fascial necrosis with relative sparing of skin and underlying muscle. It is frequently associated with severe systemic toxicity and is usually rapidly fatal unless promptly recognized and aggressively treated. The most freFrom the Department of Medicine** and the Department of Medicinel Dennatology Service,*** Fitzsimons Army Medical Center. Reprint requests to: Dr. Ronald E. Grimwood, Ohio State University, 456 Clinic Dr., Room 4731, Columbus, OR 43210. *The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.
quent site of infection is an extremity, 1-6 with other areas being the trunk, 1.3-6 the neck, 6 the face,3,7 and the perineum and genitalia. 1,2,4,6.8.10 There appears to be no age or sex predilection. The introduction of bacteria into the subcutaneous space can occur through any compromise of the protective integument. Necrotizing fasciitis has developed following a variety of lesions, including minor trauma,I-3.4,6 skin abscess,I,2,6 needle sites in narcotics addicts,I,6 and frostbite,l as well as after compound fractures 1 or insect bite4 and in surgical wounds. II .12 In some individuals, no identifiable focal lesion is found. I.3.6 Grossman et aP3 reviewed the clinical and lab-
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