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Gigantic ichthyosis hystrix
646
Journal of the American Academy of Dermatology April 1997
Brief communications
REFERENCES 1. Brage ME, Simon MA. Evaluation, prognosis, and medical treatment considerations of metastatic bone tumors. Orthopedics 1992;15:589-96. 2. Frassica FJ, Gitelis S, Sim FH. Metastatic bone disease: general principles, pathophysiology, evaluation, and biopsy. In: Instructional course lectures (Mosby, Park Ridge, IL); 1992;41:293-300. 3. Nielsen OS, Munro AJ, Tannock W. Bone metastases: pathophysiology and management. J Clin Onco11991;9:50924.
4. Yoshida D, Chen MN, Awaya S, et al. Cranial metastasis of hepatocellular carcinoma in a female. Neurol Med Chit (Tokyo) 1993;33:839-44. 5. Averbuch SD. New bisphosphonates in the treatment of bone metastases. Cancer 1993;72:3443-52. 6. Komata T, Takahashi S, Konno K, et al. Metastatic leiomyosarcoma of the skull. Neurol Med Chit (Tokyo) 1994;34:
180-2. 7. Baldwin HE, Berck CM, Lynfield YL. Subcutaneous nodules of the scalp: preoperative management. J Am Acad Dermatol 1991;25:819-30.
Gigantic ichthyosis hystrix Jos6 Kriner, MD, and Leopoldo F. Montes, MD, MS, FRCPC Buenos Aires, Argentina
Ichthyosis hystrix OH), also known as hyperkeratosis monstruosa or sauriasis, is a rare genetic epidermal malformation. 1 The word hystrix means resemblance to the spines of the porcupine; the conclifton has also been referred to as porcupine disease. ~ Historically, six children of the Lambert family of Suffolk3 were well known in England as the "porcupine men." We describe a patient with this dermatosis who closely resembled members of the Lambert family. CASE R E P O R T A 36-year-old white man had a widespread eruption affecting mainly the extremities that began in early childhood. No other family members were affected. Examination disclosed disseminated hyperkeratotic lesions. The dorsum of the hands and anterior thighs contained hyperkeratotic plaques. The lesions on the posterior surface of the thighs seemed to be following Blaschko's lines supporting Lever's view (Fig. 1). The more slriking lesions were located over both legs. They consisted of elevated, rocky-hard, dark brown excrescences.
From the Division of Dermatology,Hospital Alvear and Hospital Israelita. Reprint requests: Jos6 Kriner, MD, Uruguay 1226, (1016) Buenos Aires, Argentina. J Am Acad Dermatol 1997;36:646-7. Copyright © 1997 by the American Academy of Dermatology,Inc. 0190-9622/97/$5.00 + 0 16154/78980
Fig. 1. Hyperkeratotic verrucous papules and plaques on the posterior surface of the thighs and legs.
Journal of the American Academy of Dermatology Volume 36, Number 4
Brief communications
647
These outgrowths resembled transversally cut logs (Fig. 2). The largest were from 2 to 3 cm in height and their base ranged from 1 to 2 cm in diameter. The palms and soles 'were thickened but did not show these excrescences. The scalp, face, nails, and genitalia were unaffected. HJstopathologic examination revealed marked hyperkeratosis and acanthosis. The granular layer was prominent and vacuolated with fragmentation of the keratohyalin granules. The stratum malpighii was edematous. There was a mild inflammatory infiltrate in the papillary dermis.
DISCUSSION
The term 114 embraces all ichthyoses that display striking hyperkeratotic verrucous dark b r o w n ridges. Four different types of I H have been described: L a m b e r t type, Curth-Macklin type, Rheydt type, and Bafverstedt type. 4 Our patient belongs to the L a m bert type because o f the resemblance to a porcupine and involvement o f the entire cutaneous surface, except the face, genitals, palms, and soles. L e v e r and Schaumburg-Lever, 5 while emphasizing the symmetric involvement, consider I H to be a f o r m of systematized linear epidermal nevus. According to Pinkus and Mehregan, 6 I H shows histologic changes identical to epidermolytic hyperkeratosis. These changes were present in our patient. Although systemic retinoids have produced limited and temporary response in this and other types o f ichthyoses, 7 they were not tried in this patient.
Fig. 2. Rocky-hard dark brown excrescenses on legs.
REFERENCES
1. Rook A, Wilkinson DS, Ebling FJG. Textbook of dermatology. Oxford: Blackwell Scientific Publications, 1979: 1277. 2. Stedman's Medical Dictionary. Baltimore: Williams & Willdns, 1972:615. 3. Traupe H. The ichthyoses: a guide to clinical diagnosis, genetic counseling and therapy. Berlin: Springer Verlag, 1989. 4. Braun-Falco O, Plewig G, Wolff HH, et al. Dermatology. Berlin: Springer-Verlag, 1991:518-9. 5. Lever WF, Schaumburg-Lever G. Histopathology of the skin. Philadelphia: JB Lippincott, 1975:452-3. 6. Pinkus H, Mehregan AH. A guide to dermatohistopathology. New York: Appleton-Century-Crofts, 1981:326-7. 7. Fitzpatrick TB, Eisen AZ, Wolff K, et al. Dermatology in general medicine. New York: McGraw-Hill, 1993: 859-60.
The effect of oral ibuprofen on patch test reactivity in subjects allergic to poison ivy/oak Elizabeth F. Sherertz, M D Winston-Salem, North Carolina From the Department of Dermatology, Bowman Gray School of Medicine. Presenttedin abstractformat the AmericanContactDermatitisSociety meeting, ! Washington, D,C., December 1993. Reprint~equests:ElizabethF. Sherertz,MD, Departmentof Dermatol-
ogy,BowmanGraySchoolof Medicine,MedicalCenterBoulevard, Winston-Salem, NC 27157. J Am Acad Dermatol 1997;36:647-9. Copyright © 1997 by the AmericanAcademyof Dermatology,Inc. 0190-9622/97155.00 + 0 16/54/79551
Journal of the American Academy of Dermatology April 1997
Brief communications
REFERENCES 1. Brage ME, Simon MA. Evaluation, prognosis, and medical treatment considerations of metastatic bone tumors. Orthopedics 1992;15:589-96. 2. Frassica FJ, Gitelis S, Sim FH. Metastatic bone disease: general principles, pathophysiology, evaluation, and biopsy. In: Instructional course lectures (Mosby, Park Ridge, IL); 1992;41:293-300. 3. Nielsen OS, Munro AJ, Tannock W. Bone metastases: pathophysiology and management. J Clin Onco11991;9:50924.
4. Yoshida D, Chen MN, Awaya S, et al. Cranial metastasis of hepatocellular carcinoma in a female. Neurol Med Chit (Tokyo) 1993;33:839-44. 5. Averbuch SD. New bisphosphonates in the treatment of bone metastases. Cancer 1993;72:3443-52. 6. Komata T, Takahashi S, Konno K, et al. Metastatic leiomyosarcoma of the skull. Neurol Med Chit (Tokyo) 1994;34:
180-2. 7. Baldwin HE, Berck CM, Lynfield YL. Subcutaneous nodules of the scalp: preoperative management. J Am Acad Dermatol 1991;25:819-30.
Gigantic ichthyosis hystrix Jos6 Kriner, MD, and Leopoldo F. Montes, MD, MS, FRCPC Buenos Aires, Argentina
Ichthyosis hystrix OH), also known as hyperkeratosis monstruosa or sauriasis, is a rare genetic epidermal malformation. 1 The word hystrix means resemblance to the spines of the porcupine; the conclifton has also been referred to as porcupine disease. ~ Historically, six children of the Lambert family of Suffolk3 were well known in England as the "porcupine men." We describe a patient with this dermatosis who closely resembled members of the Lambert family. CASE R E P O R T A 36-year-old white man had a widespread eruption affecting mainly the extremities that began in early childhood. No other family members were affected. Examination disclosed disseminated hyperkeratotic lesions. The dorsum of the hands and anterior thighs contained hyperkeratotic plaques. The lesions on the posterior surface of the thighs seemed to be following Blaschko's lines supporting Lever's view (Fig. 1). The more slriking lesions were located over both legs. They consisted of elevated, rocky-hard, dark brown excrescences.
From the Division of Dermatology,Hospital Alvear and Hospital Israelita. Reprint requests: Jos6 Kriner, MD, Uruguay 1226, (1016) Buenos Aires, Argentina. J Am Acad Dermatol 1997;36:646-7. Copyright © 1997 by the American Academy of Dermatology,Inc. 0190-9622/97/$5.00 + 0 16154/78980
Fig. 1. Hyperkeratotic verrucous papules and plaques on the posterior surface of the thighs and legs.
Journal of the American Academy of Dermatology Volume 36, Number 4
Brief communications
647
These outgrowths resembled transversally cut logs (Fig. 2). The largest were from 2 to 3 cm in height and their base ranged from 1 to 2 cm in diameter. The palms and soles 'were thickened but did not show these excrescences. The scalp, face, nails, and genitalia were unaffected. HJstopathologic examination revealed marked hyperkeratosis and acanthosis. The granular layer was prominent and vacuolated with fragmentation of the keratohyalin granules. The stratum malpighii was edematous. There was a mild inflammatory infiltrate in the papillary dermis.
DISCUSSION
The term 114 embraces all ichthyoses that display striking hyperkeratotic verrucous dark b r o w n ridges. Four different types of I H have been described: L a m b e r t type, Curth-Macklin type, Rheydt type, and Bafverstedt type. 4 Our patient belongs to the L a m bert type because o f the resemblance to a porcupine and involvement o f the entire cutaneous surface, except the face, genitals, palms, and soles. L e v e r and Schaumburg-Lever, 5 while emphasizing the symmetric involvement, consider I H to be a f o r m of systematized linear epidermal nevus. According to Pinkus and Mehregan, 6 I H shows histologic changes identical to epidermolytic hyperkeratosis. These changes were present in our patient. Although systemic retinoids have produced limited and temporary response in this and other types o f ichthyoses, 7 they were not tried in this patient.
Fig. 2. Rocky-hard dark brown excrescenses on legs.
REFERENCES
1. Rook A, Wilkinson DS, Ebling FJG. Textbook of dermatology. Oxford: Blackwell Scientific Publications, 1979: 1277. 2. Stedman's Medical Dictionary. Baltimore: Williams & Willdns, 1972:615. 3. Traupe H. The ichthyoses: a guide to clinical diagnosis, genetic counseling and therapy. Berlin: Springer Verlag, 1989. 4. Braun-Falco O, Plewig G, Wolff HH, et al. Dermatology. Berlin: Springer-Verlag, 1991:518-9. 5. Lever WF, Schaumburg-Lever G. Histopathology of the skin. Philadelphia: JB Lippincott, 1975:452-3. 6. Pinkus H, Mehregan AH. A guide to dermatohistopathology. New York: Appleton-Century-Crofts, 1981:326-7. 7. Fitzpatrick TB, Eisen AZ, Wolff K, et al. Dermatology in general medicine. New York: McGraw-Hill, 1993: 859-60.
The effect of oral ibuprofen on patch test reactivity in subjects allergic to poison ivy/oak Elizabeth F. Sherertz, M D Winston-Salem, North Carolina From the Department of Dermatology, Bowman Gray School of Medicine. Presenttedin abstractformat the AmericanContactDermatitisSociety meeting, ! Washington, D,C., December 1993. Reprint~equests:ElizabethF. Sherertz,MD, Departmentof Dermatol-
ogy,BowmanGraySchoolof Medicine,MedicalCenterBoulevard, Winston-Salem, NC 27157. J Am Acad Dermatol 1997;36:647-9. Copyright © 1997 by the AmericanAcademyof Dermatology,Inc. 0190-9622/97155.00 + 0 16/54/79551