Clinical and Experimental Dermatology
Unusual nodules around a surgical wound A. G. Matthews,1 C. A. Humphreys,2 A. J. Muinonen-Martin,1 M. Gangopadhyay2 and S. Holmes1 1
Department of Dermatology, Glasgow Royal Infirmary, Glasgow, UK; and 2Department of Histopathology, Southern General Hospital, Glasgow, UK
doi: 10.1111/ced.12419
Clinical findings
Histopathological findings
A 65-year-old woman presented with a 1-month history of dusky nodules forming around the scar left from a hemiarthroplasty that she had undergone 3 years previously. In the few weeks following initial presentation, further lesions appeared distally along the lateral aspect of the left thigh (Fig. 1). The hip replacement had been complicated by recurrent deep infection and displacement of the femoral head, resulting in chronic pain and poor mobility. The patient had been largely wheelchair-bound for several years. The skin lesions were asymptomatic, but occurred in association with an exacerbation of the patient’s chronic hip infection, which had required surgical intervention. Her other medical history of note included alcoholic liver disease, subdural haematoma and epilepsy. In view of the rapid onset and spread of the lesions, atypical infection and neoplasia were considered in the differential diagnosis. Microbiological tests, including tissue culture for fungi and acid-fast bacilli, were negative. The lesions were unchanged by treatment with broad-spectrum antibiotics; however, most regressed following removal of the patient’s hip prosthesis and aggressive debridement of the joint space.
Histological examination of an excised lesion revealed a dermal nodule, consisting of a mixed inflammatory cell infiltrate containing neutrophils, plasma cells, lymphocytes and histiocytes. Within the nodule, there was vascular proliferation, with red cell extravasation and haemosiderin deposition. There was focal fibroblast proliferation in the surrounding dermis. There were no granulomata. Stains for organisms and human herpes virus -8 (HHV-8) were negative (Fig. 2). What is your diagnosis?
Figure 1 Dusky nodules forming around the scar from hem-
iarthroplasty carried out 3 years previously.
Correspondence: Dr Allan Matthews, Department of Dermatology, Glasgow Royal Infirmary, 84 Castle Street, Glasgow, G4 0JS, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 16 March 2014
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2014) 39, pp847–849
847
D CP
CED
CPD • Clinicopathological case
D
CP
Clinicopathological case
(a)
(b)
(c)
(d)
Figure 2 (a) Proliferation of regular blood vessels surrounded by a mixed inflammatory cell infiltrate; (b) the vessels are lined by plump
endothelial cells and there is heavy haemosiderin deposition. (haematoxylin and eosin, original magnification (a) 9 100; (b) 9 200. (c) Immunostaining with CD31 highlights the vascular spaces within the lesion (original magnification (a) 9 40). (d) Perl stain confirms dense haemosiderin deposition (original magnification (a) 9 40).
Diagnosis Acroangiodermatitis [also known as pseudo-Kaposi sarcoma (pseudo-KS)].
Discussion Acroangiodermatitis is a type of benign cutaneous reactive vascular proliferation.1 Lesions appear as purplish nodules or plaques, clinically similar to KS. Acroangiodermatitis most commonly occurs on the lower legs in patients with chronic venous insufficiency (acroangiodermatitis of Mali) and in this context is probably under-reported.2 It has also been described in association with lower limb arteriovenous malformations (Stewart–Bluefarb syndrome), limb paralysis, lower limb amputation (especially when
848
Clinical and Experimental Dermatology (2014) 39, pp847–849
there is a poorly fitting suction-type prosthesis),3 iatrogenic arteriovenous fistulae (usually in patients on dialysis) and thrombophilias. The pathogenesis of acroangiodermatitis is not precisely understood. Most cases occur in situations wherein cutaneous vascular flow is compromised. Dermal vessel occlusion seems to trigger a hypoxia-driven reactive process, which culminates in hyperplasia of endothelial cells, pericytes and sometimes histiocytes. Microscopically, this is seen as lobular collections of thick-walled capillaries surrounded by varying degrees of inflammatory cells, extravasated erthythrocytes, haemosiderin deposition and fibrosis.4 These features were present in this case, although the classic lobular arrangement of capillaries was lacking. The most important clinical and histopathological differential diagnosis in cases of acroangiodermatitis is
ª 2014 British Association of Dermatologists
D CP Clinicopathological case
KS. In KS, vascular proliferation is independent of existing vessels, thus there is not usually a history of underlying vascular problems, and the topography of lesions is different. The presence of irregular ‘slit-like’ vascular channels containing red blood cells and surrounded by a spindled stroma, presence of the promontory sign and positive immunostaining for HHV-8 confirm KS histologically.5 Another histopathological differential diagnosis in cases of acroangiodermatitis is lobular capillary haemangioma (pyogenic granuloma). This was excluded clinically in this case. To our knowledge, this is the first reported case of acroangiodermatitis occurring in relation to a surgical wound on the upper leg. The patient had a major risk factor for this condition in that she had been poorly mobile for months. In addition, we hypothesize that the disordered anatomy of the hip joint and the presence of a large volume of tissue oedema beneath the skin led to a localized disruption of the cutaneous blood flow, resulting in the pattern of skin lesions observed. This theory is supported by the fact that most of the lesions regressed following surgical debridement of the hip joint space.
References 1 Rongioletti F, Rebora A. Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation. J Am Acad Dermatol 2003; 49: 887–96. 2 Rashkovsky I, Gilead L, Schamroth J, Leibovici V. Acroangiodermatitis: review of the literature and report of a case. Acta Derm Venereol 1995; 75: 475–8. 3 Sbano P, Miracco C, Risudo M, Fimiani M. Acroangiodermatitis (pseudo-Kaposi sarcoma) associated with verrucous hyperplasia induced by suction-socket limb prosthesis. J Cutan Pathol 2005; 32: 429–32. 4 Wassef M, Hunt SJ, Santa Cruz DJ, Barnhill RL. Vascular tumours and vascular malformations. In: Dermatopathology, 3rd edn (Barnhill RL, Crowson AN, Magro CM, eds). New York: McGraw-Hill, 2010: 811–18. 5 Patel RM, Goldblum JR, Hsi ED. Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 is useful in the diagnosis of Kaposi sarcoma. Mod Pathol 2004; 17: 456–60.
Learning points
•
Atypical patterns of acroangiodermatitis can occur in situations where there is localized disruption of blood flow through the skin. • Clinicopathological correlation is important for diagnosing acroangiodermatitis and distinguishing it from malignant conditions with a similar appearance such as KS or low-grade angiosarcoma. • The mainstay of treatment for acroangiodermatitis involves correction of any underlying vascular pathology to maximize afferent and efferent cutaneous blood flow.
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2014) 39, pp847–849
849
Unusual nodules around a surgical wound A. G. Matthews,1 C. A. Humphreys,2 A. J. Muinonen-Martin,1 M. Gangopadhyay2 and S. Holmes1 1
Department of Dermatology, Glasgow Royal Infirmary, Glasgow, UK; and 2Department of Histopathology, Southern General Hospital, Glasgow, UK
doi: 10.1111/ced.12419
Clinical findings
Histopathological findings
A 65-year-old woman presented with a 1-month history of dusky nodules forming around the scar left from a hemiarthroplasty that she had undergone 3 years previously. In the few weeks following initial presentation, further lesions appeared distally along the lateral aspect of the left thigh (Fig. 1). The hip replacement had been complicated by recurrent deep infection and displacement of the femoral head, resulting in chronic pain and poor mobility. The patient had been largely wheelchair-bound for several years. The skin lesions were asymptomatic, but occurred in association with an exacerbation of the patient’s chronic hip infection, which had required surgical intervention. Her other medical history of note included alcoholic liver disease, subdural haematoma and epilepsy. In view of the rapid onset and spread of the lesions, atypical infection and neoplasia were considered in the differential diagnosis. Microbiological tests, including tissue culture for fungi and acid-fast bacilli, were negative. The lesions were unchanged by treatment with broad-spectrum antibiotics; however, most regressed following removal of the patient’s hip prosthesis and aggressive debridement of the joint space.
Histological examination of an excised lesion revealed a dermal nodule, consisting of a mixed inflammatory cell infiltrate containing neutrophils, plasma cells, lymphocytes and histiocytes. Within the nodule, there was vascular proliferation, with red cell extravasation and haemosiderin deposition. There was focal fibroblast proliferation in the surrounding dermis. There were no granulomata. Stains for organisms and human herpes virus -8 (HHV-8) were negative (Fig. 2). What is your diagnosis?
Figure 1 Dusky nodules forming around the scar from hem-
iarthroplasty carried out 3 years previously.
Correspondence: Dr Allan Matthews, Department of Dermatology, Glasgow Royal Infirmary, 84 Castle Street, Glasgow, G4 0JS, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 16 March 2014
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2014) 39, pp847–849
847
D CP
CED
CPD • Clinicopathological case
D
CP
Clinicopathological case
(a)
(b)
(c)
(d)
Figure 2 (a) Proliferation of regular blood vessels surrounded by a mixed inflammatory cell infiltrate; (b) the vessels are lined by plump
endothelial cells and there is heavy haemosiderin deposition. (haematoxylin and eosin, original magnification (a) 9 100; (b) 9 200. (c) Immunostaining with CD31 highlights the vascular spaces within the lesion (original magnification (a) 9 40). (d) Perl stain confirms dense haemosiderin deposition (original magnification (a) 9 40).
Diagnosis Acroangiodermatitis [also known as pseudo-Kaposi sarcoma (pseudo-KS)].
Discussion Acroangiodermatitis is a type of benign cutaneous reactive vascular proliferation.1 Lesions appear as purplish nodules or plaques, clinically similar to KS. Acroangiodermatitis most commonly occurs on the lower legs in patients with chronic venous insufficiency (acroangiodermatitis of Mali) and in this context is probably under-reported.2 It has also been described in association with lower limb arteriovenous malformations (Stewart–Bluefarb syndrome), limb paralysis, lower limb amputation (especially when
848
Clinical and Experimental Dermatology (2014) 39, pp847–849
there is a poorly fitting suction-type prosthesis),3 iatrogenic arteriovenous fistulae (usually in patients on dialysis) and thrombophilias. The pathogenesis of acroangiodermatitis is not precisely understood. Most cases occur in situations wherein cutaneous vascular flow is compromised. Dermal vessel occlusion seems to trigger a hypoxia-driven reactive process, which culminates in hyperplasia of endothelial cells, pericytes and sometimes histiocytes. Microscopically, this is seen as lobular collections of thick-walled capillaries surrounded by varying degrees of inflammatory cells, extravasated erthythrocytes, haemosiderin deposition and fibrosis.4 These features were present in this case, although the classic lobular arrangement of capillaries was lacking. The most important clinical and histopathological differential diagnosis in cases of acroangiodermatitis is
ª 2014 British Association of Dermatologists
D CP Clinicopathological case
KS. In KS, vascular proliferation is independent of existing vessels, thus there is not usually a history of underlying vascular problems, and the topography of lesions is different. The presence of irregular ‘slit-like’ vascular channels containing red blood cells and surrounded by a spindled stroma, presence of the promontory sign and positive immunostaining for HHV-8 confirm KS histologically.5 Another histopathological differential diagnosis in cases of acroangiodermatitis is lobular capillary haemangioma (pyogenic granuloma). This was excluded clinically in this case. To our knowledge, this is the first reported case of acroangiodermatitis occurring in relation to a surgical wound on the upper leg. The patient had a major risk factor for this condition in that she had been poorly mobile for months. In addition, we hypothesize that the disordered anatomy of the hip joint and the presence of a large volume of tissue oedema beneath the skin led to a localized disruption of the cutaneous blood flow, resulting in the pattern of skin lesions observed. This theory is supported by the fact that most of the lesions regressed following surgical debridement of the hip joint space.
References 1 Rongioletti F, Rebora A. Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation. J Am Acad Dermatol 2003; 49: 887–96. 2 Rashkovsky I, Gilead L, Schamroth J, Leibovici V. Acroangiodermatitis: review of the literature and report of a case. Acta Derm Venereol 1995; 75: 475–8. 3 Sbano P, Miracco C, Risudo M, Fimiani M. Acroangiodermatitis (pseudo-Kaposi sarcoma) associated with verrucous hyperplasia induced by suction-socket limb prosthesis. J Cutan Pathol 2005; 32: 429–32. 4 Wassef M, Hunt SJ, Santa Cruz DJ, Barnhill RL. Vascular tumours and vascular malformations. In: Dermatopathology, 3rd edn (Barnhill RL, Crowson AN, Magro CM, eds). New York: McGraw-Hill, 2010: 811–18. 5 Patel RM, Goldblum JR, Hsi ED. Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 is useful in the diagnosis of Kaposi sarcoma. Mod Pathol 2004; 17: 456–60.
Learning points
•
Atypical patterns of acroangiodermatitis can occur in situations where there is localized disruption of blood flow through the skin. • Clinicopathological correlation is important for diagnosing acroangiodermatitis and distinguishing it from malignant conditions with a similar appearance such as KS or low-grade angiosarcoma. • The mainstay of treatment for acroangiodermatitis involves correction of any underlying vascular pathology to maximize afferent and efferent cutaneous blood flow.
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2014) 39, pp847–849
849
