Cameo Oxford, UK International IJD Blackwell 1365-4632 45 Publishing Journal Ltd, Ltd. of Dermatology 2003
Angioma serpiginosum: A case report Angiomaetserpiginosum: Agrawal cAMEO al. a case report
Sudha Agrawal, MD, Arun Agarwalla, MD, Arpana Rizal, MD, Arvind sinha, MD, and Krishna Debbarman, MD
From the Department of Dermatology and Pathology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal Correspondence Dr Sudha Agrawal Assistant Professor Department of Dermatology B. P. Koirala Institute of Health Sciences Dharan Nepal E-mail:
[email protected]
Case Report A 45-year-old male referred to the Dermatology Out Patients Department for evaluation of purpuric lesions over the trunk and extremities of 5 years’ duration. The lesions had started as asymptomatic, minute, reddish rashes on the extensor aspect of the left arm, which after approximately 2 years increased in number and distribution to involve the bilateral arms, forearms, trunk and lower limbs. He had a history of spontaneous regression of some of the lesions. There was no history of swelling and pain in the joints, recurrent fever, easy fatigability, palpitation or chest pain. Bowel and bladder habits were normal. Past history was noncontributory. There was no family history of similar skin lesions. His general physical and systemic examinations were within normal limits. The cutaneous examination revealed bilateral, confluent and scattered, nonblanching, erythematous to violaceous, punctae within large, erythamatous, blanching, macules on the upper (Fig. 1) and lower extremities, anterior chest and abdomen. In a few areas, it appeared as large sheets and livedoid patterns. The clinical diagnosis of purpura was made by the physician, and the patient underwent investigations such as a complete blood count, bleeding and coagulating profiles, a peripheral smear for cytology, a microscopic examination of urine and bone marrow aspiration cytology, which were found to be within normal limits. Considering the cutaneous examination findings, the clinical diagnosis of purpura was revised to angioma serpiginosum. The skin biopsy taken from the arm showed multiple areas of a lobulated proliferation of dilated capillaries in the dermal papillae and superficial reticular dermis (Fig. 2). The epidermis appeared normal and there was no inflammatory cells or © 2004 The International Society of Dermatology
Figure 1 Multiple, confluent and scattered, non-blanching,
erythematous to violaceous, punctae within large, erythamatous, blanching macules
hemosiderin in the dermis. The features were consistent with angioma serpiginosum. The facility of pulse dye laser is not available in Nepal and the patient was unable to afford the cost of therapy. Therefore, the patient was reassured about the nature of the disease. Discussion Angioma serpiginosum, a rare vascular naevoid disorder, was first described by Hutchinson in 1889.1 It classically begins in childhood and is more common in females (90%). Most cases are sporadic, but autosomal dominant inheritance has also been reported in the literature.2 The skin lesions are asymptomatic, characterized by grouped, erythematous to violaceous, punctate macules, and it sometimes forms large International Journal of Dermatology 2004, 43, 917– 918
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Agrawal et al.
skin shows clusters of dilated capillaries in the upper dermis. The epidermis appears normal and there is no associated inflammation or hemosiderin.8 The association of retinal and spinal nerve root angioma has also been reported in the literature.9 However, complete regressions of the eruptions are very rare, and individual puncta often disappear spontaneously. Pulse dye laser or diathermy may be effective for these patients.10,11 This case is reported for its rarity, late age of onset, and extensive cutaneous involvement. To the best of our knowledge, this is the first case report from Nepal. We also would like to emphasize that physicians should be aware of the condition and recognize the vascular disorders, which may simulate purpura and avoid unnecessary investigations. References
Figure 2 Microphotograph showing multiple areas of a
lobulated proliferation of dilated capillaries in the upper dermis (hematoxylin and eosin, ×10)
sheets or livedoid patterns. Usually, it appears on the lower limbs and buttocks, often unilateral initially but it may be more widespread.3 Infrequently, extensive involvement of the trunk and extremities have been reported.4 Gerbig et al. suggested that it might follow the lines of Blaschko.5 The progression of lesions usually ceases after a period of months and years but may recur in adult life. Frequently, there is a background of larger erythematous macules that are present as a result of dilatation of the subpapillary venous plexuses. The lesions may not blanch completely on pressure. Therefore, sometimes patients undergo a variety of hematological tests for investigations of purpura.6 Epiluminescence microscopy of the lesion can identify dilated capillaries and is beneficial in distinguishing angioma serpinginosum from purpuric dermatoses.7 Histopathology of the
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1 Barker LP, Sachs PM. Angioma serpiginosum. Arch Dermatol 1965; 92: 613 – 620. 2 Marriott PJ, Munro DD, Ryan T. Angioma serpiginosum: familial incidence. Br J Dermatol 1975; 93: 701– 706. 3 Champion RH. Disorders of blood vessels. In: Champion RH, Burton JL, Burns DA, et al., eds. Textbook of Dermatology, 6th edn. Oxford: Blackwell Scientific Publication, 1998: 1827 –1849. 4 Katta R, Wagner A. Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol 2000; 42: 384 – 385. 5 Gerbig AW, Zala L, Hunziker T. Angioma serpiginosum, a skin change along Blaschko lines? Hautarzt 1995; 46: 847 – 849. 6 Cox NH, Paterson WD. Angioma serpiginosum, a simulator of purpura. Postgrad Med J 1991; 67: 1065 –1066. 7 Ohnishi T, Nagayama T, Morita T, et al. Angioma serpiginosum. a report of 2 cases identified using epiluminescence microscopy. Arch Dermatol 1999; 135: 1366 –1368. 8 Kumakiri M, Katoh H, Miura Y. Angioma serpiginosum. J Cut Pathol 1980; 7: 410 – 421. 9 Gautier-Smith PC, Sandedrs MD, Sanderson KV. Ocular and nervous system involvement in angioma serpiginosum. Br J Ophthalmol 1971; 55: 433 – 443. 10 Polla LL, Tan OT, Garden JM, et al. Tunable pulsed dye laser for the treatment of benign cutaneous vascular ectasia. Dermatologica 1987; 174: 11–17. 11 Long CC, Lanigan SW. Treatment of angioma serpiginosum using a pulsed tunable dye laser. Br J Dermatol 1997; 136: 631– 632.
© 2004 The International Society of Dermatology
