ACROANGIODERMATITIS MIMICKING KAPOSI S SARCOMA IN A YOUNG PATIENT WITH ACQUIRED APLASTIC ANAEMIA

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1 Dermatology Case Report International Journal of Clinical And Diagnostic Research ISSN Volume 6, Issue 3, May-June 2018 ACROANGIODERMATITIS MIMICKING KAPOSI S SARCOMA IN A YOUNG PATIENT WITH ACQUIRED APLASTIC ANAEMIA Abstract Vidya D Kharkar 1, Khan Mohammed Oves 2*, Sunanda A. Mahajan 3 Acroangiodermatitis (AAD) of Mali is an uncommon benign chronic dermatosis, commonly associated with chronic venous insufficiency in elderly and stasis dermatitis, presenting as violaceous macules, patches progressing slowly to become soft, non-tender, papules, nodules or indurated plaques often involving bilateral lower extremity. These lesions closely mimic common conditions like stasis dermatitis, lipodermatosclerosis as well as more serious ones like Kaposi s sarcoma. Histopathology as well as immunohistochemistry is often cornerstone in diagnosis. Ours was a young patient of acquired aplastic anaemia presenting with multiple well defined hyperpigmented, violaceous to dusky colour patches and plaques over the bilateral legs lacking clinically obvious varicosities, which on biopsy and immunohistochemistry turned out to be AAD of Mali. Author Affiliations: Department of Dermatology, KEM and Seth G.S. Hospital, Mumbai. Keywords: Acroangiodermatitis, Kaposi s sarcoma, stasis dermatitis, aplastic anaemia *Corresponding Author: Dr. Khan Mohammed Oves, Daulat complex, Flat no 404, 4 th floor, PattheBapurao Marg Mumbai Mobile was first described by Earhart et al in 1974 [1, INTRODUCTION AAD (Pseudo-Kaposi sarcoma, AAD of Mali- Kuiper, gravitational purpura, stasis purpura.) 2].In 1965 Mali et al first reported Kaposi like lesions in a patient with chronic venous insufficiency. Later in 1967, Bluefarb and

2 Adams described this condition in a patient with congenital vascular malformation [3]. AAD usually seen in males presenting as an exaggerated stasis dermatitis that begins as violaceous macules, patches progressing to become papules, nodules and plaques, often involving one or bilateral lower extremities. Usually a benign condition, it has to be differentiated from its close yet atrocious mimicker Kaposi's sarcoma, especially in current era of HIV and AIDS. thrill and no lymphadenopathy. Systemic examination revealed no abnormalities. A provisional diagnosis of stasis dermatitis was made and patient was advised to avoid prolonged standing, elevate leg when resting along with systemic antihistamines, moderately potent topicalsteroids, emollients and was instructed to follow up in a month. CASE DETAILS A 19-year-old, male student was a diagnosed as a case of acquired aplastic anaemia a year ago and was being managed for the same bydepartment of haematology at our hospital. He was referred to us for complaints of multiple, flat and raised brown to dusky colour rash associated with itching over bilateral legs for three months. There was no history of trauma, pain on prolong standing, cramps, claudication or ulcers. Examination revealed multiple, well defined,hyperpigmented,violaceous to dusky colour patches and plaques over the medial and lateral aspects of bilateral legs(as shown in Figures 1, 2 and 3), with no clinically obvious varicosities along with pitting edema over bilateral legs. All peripheral pulses were well felt. There was no evidence of bruit or Figure 1: Well defined, hyperpigmented, violaceous to dusky colour plaque over lateral aspects of right leg. Figure 2: Well defined, violaceous plaque with few nodules over medial aspect of the right leg.

3 Figure 3: Well defined, scaly, violaceous plaque with few nodules over lateral aspect of left leg along with induration and oedema of left foot. On follow up, patient expressed little relief of his symptoms, presently some of his lesions evolved to become well defined brown to dusky colour indurated nodules (fig.4) over the medial aspect of left leg associated with woody, mildly painful edema of both legs. A differential diagnosis of stasis dermatitis with lipodermatosclerosis, Kaposi s sarcoma and AAD of Mali was considered. Blood parameters were, Hb-9.4gm%, WBC-4.25 x 10 3 /µl and platelet count-less than Patient was non-reactive for HIV, HbsAg and HCV. Colour venous doppler study of both legs revealed few dilated vessels along the distribution of long saphenous vein in left leg with competent perforators in both limbs without any evidence of deep vein thrombosis. To be definitive about the diagnosis a biopsy was performed after his platelets counts were stabilized which revealed, hyperplastic epidermis with numerous proliferating capillaries in the papillary and reticular dermis amidst hemosiderin deposition along with perivascular lymphocytic infiltrate at some places (as shown in figures 5). Figure 4: Well defined, hyperpigmented-violaceous patches and plaques along with few nodules and satellite lesions over medial aspect of left leg. Figure 5: 10x view, hematoxylin and eosin showing- Hyperplastic epidermis with proliferation of small vessels in the upper dermis, extravasated RBCs and hemosiderin within an edematous dermis.

4 To differentiate this from Kaposi s sarcoma, immunohistochemical staining usingcd34 (cluster of differentiation 34) was performed that highlighted only the endothelial cells sparing the perivascular cells (as shown in figures 6 and 7). Correlating clinic-histopathological scenario a conclusive diagnosis of AAD of Mali was made. DISCUSSION: AAD is an uncommon chronic dermatosisis considered to be reactive angiodysplasia of cutaneous vasculature often associated with chronic venous insufficiency, vascular anomalies like Klippel-Trenaunay syndrome, stump dermatosis in amputees [4], paralysed limbs, acquired iatrogenic arteriovenous (AV) fistula in patients undergoing haemodialysis and rarely with hereditary coagulation defects [5]. De novo cases without venous insufficiency have also been reported [6]. Variants of AAD include (charti) [4]. Figure 6: Immunohistochemical stain using CD34, scanner view - demonstrating distinct staining of endothelial cells sparing perivascular cells. Types 1. Stewart-Bluefarb syndrome Description congenital AV malformation with multiple shunts 2. Mali type exaggerated stasis dermatitis often seen in elderly Figure 7: Immunohistochemical stain using CD34, 40x view- demonstrating distinct staining of endothelial cells sparing perivascular cells. 3. Dermiteocre of Favre or AAD in first pregnancy 4. AAD with chronic renal failure Chart I: Variants of AAD gravity purpura at sites of venous varicosities In patients placed with an AV shunt for hemodialysis

5 Exact etiopathogenesis remains unclear. Vascular endothelial growth factor induced, neovascularisation and fibroblast proliferation may play a role [7]. Hypoxia in distal areas stimulates proliferation of endothelial cells. Mast cells may also play a role [8]. Clinically lesions tend to simulate common conditions like gravitational dermatitis, pigmented purpuricdermatoses, chromoblastomycosis as well as more serious one like Kaposi s sarcoma. Histopathologically, lesions closely resemble Kaposi s sarcoma. Features that distinguish AAD from Kaposi s sarcoma include, former is associated with proliferation of pre-existing vasculature, however later is associated with vascular proliferation independent from the underlying normal vasculature [9].The pattern of growth is lobular in AAD whereas it is irregular in Kaposi s sarcoma. Unlike Kaposi s sarcoma, AAD is associated with absence ofvascular slits, spindle cells, proliferating fusiform cells and atypical cells. Vessels of AAD do not express HHV-8 and lack CD 34 expression in perivascular cells [10]. In cases of ambiguity with conventional microscopy, electron microscopy can be useful. Treatment dwells in correction of the underlying pathology for e.g. elimination of shunts and embolization for small fistulae in cases associated with AV malformations [3]. Different modalities that have been tried with varying results include compression bandages, stockings or pumps that help in relieving of symptoms in cases associated with circulatory disturbances. Other therapies include oral erythromycin 500 mg QID,dapsone 50 mg BID for 3 months along with compression therapy [5],topical corticosteroids and laser ablation using as pulsed-dye laser [1]. CONCLUSION AAD is an infrequently encountered dermatosis, in current era of HIV and AIDS a dermatologist ought to be careful to distinguish this from its more aggressive mimicker, Kaposi s sarcoma. AAD of Mali is generally seen in elderly and in patients suffering from stasis dermatitis, however our case was a young patient with acquired aplastic anaemia with no clinically obvious varicosities. Our understanding is aplastic anaemia is associated with reduced production of haemoglobin, thereby impeding oxygen carrying capacity of blood creating conditions of hypoxia which may have furthered

6 proliferation of vessels and lead to development of AAD; this hypothesis however needs further evaluation. Various associations have been cited in literature, to best of our knowledge there has been limited reports about development of AAD of Mali following acquired aplastic anaemia in young. Conflict of Interest Statement- There is no conflict of interest. REFERENCES: 1) Rongioletti F, Alfredo R. Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation. J Am acad Dermatol 2003; 49: ) Kapdagli H, Gündüz K., Öztürk G, Kandiloglu G. Pseudo Kaposi s sarcoma (Mali type). Int J of Dermatol 1998; 37, ) Agrawal S, Rizal A, Agrawal C, Agrawal A. Pseudo-Kaposi s sarcoma (Bluefarb-Stewart type). Int J Dermatol 2005;44: ) Mehta AA, Pereira RR, Nayak CS, Dhurat RS. Acroangiodermatitis of Mali: A rare vascular phenomenon. Indian J Dermatol Venereol Leprol. 2010;76: ) Heller M, Karen JK, Fangman W. Acroangiodermatitis. Dermatol Online J. 2007;13:2. 6) Hung NA, Strack M, Van Rij A, North CJ, Blennerhassett JB. Spontaneous acroangiodermatitis in a young woman. Dermatol Online J. 2004;10:8. 7) Philips T. Acroangiodermatitis of Mali. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Bolognia Textbook of Dermatology. 2 nd ed. Spain: Mosby Elsevier publishing; p ) Ikeda, Kano E, Baba S, Suzuki H. Mast cells in pseudo- Kaposi s sarcoma lesions. J EurAcad Dermatol Venereol2001;15: [PMID: ] 9) Calonje E. Vascular Tumours: Tumours and tumour-like conditions of blood vessels and lymphatics. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, Xu X, editors. Lever's Histopathology of the Skin. 10th ed. Philadelphia: Lippincott Williams and Wilkins; pp ) Kanitakis J, Narvaez D, Claudy A. Expression of the CD34 antigen distinguishes Kaposi s sarcoma from pseudo-kaposi s sarcoma (acroangiodermatitis) Br Journal Dermatol. 1996;134(1):44 6.