Correspondence
Ana Miguelez, MD Department of Dermatology University Hospital of La Princesa Madrid Spain Joaquın Due~ nas, MD Department of Pediatrics Son Espases University Hospital Palma de Mallorca Spain Daniel Hervas, MD Juan A. Hervas, MD, PhD University Institute of Health Sciences (IUNICS) University of the Balearic Islands Palma de Mallorca Spain E-mail: [email protected] Francisco Salva, PhD Department of Microbiology Son Espases University Hospital Palma de Mallorca Spain Ana Martın-Santiago, MD Department of Dermatology Son Espases University Hospital Palma de Mallorca Spain
References 1 Heegaard ED, Brown KE. Human parvovirus B19. Clin Microbiol Rev 2002; 15: 485–505. 2 Katta R. Parvovirus B19: a review. Dermatol Clin 2002; 20: 333–342. 3 Hashimoto H, Yuno T. Parvovirus B19-associated purpuric–petechial eruption. J Clin Virol 2011; 52: 269–271. 4 Yamamoto T, Nishioka K. Flagellate erythema. Int J Dermatol 2006; 45: 627–631. 5 Ziemer M, Goetze S, Juhasz K, et al. Flagellate dermatitis as a bleomycin-specific adverse effect of cytostatic therapy. Am J Clin Dermatol 2011; 12: 68–76. 6 Poppe LM, Anders D, Kneitz H, et al. Flagellate dermatitis caused by shiitake mushrooms. An Bras Dermatol 2012; 87: 463–465. 7 Watanabe T, Tsuchida T. Flagellate erythema in dermatomyositis. Dermatology 1995; 190: 230–231. et al. 8 Santonja C, Nieto-Gonz alez G, Santos-Briz A, Immunohistochemical detection of parvovirus B19 in
ª 2014 The International Society of Dermatology
gloves and socks papular purpuric syndrome: direct evidence for viral endothelial involvement. Report of three cases and review of the literature. Am J Dermatopathol 2011; 33: 790–795.
Intensification therapy with golimumab: a new treatment strategy for moderate–severe refractory psoriasis
Moderate–severe psoriasis is a disabling entity that requires treatment with systemic drugs and in some cases with biological products. Unfortunately, these regimens can lose efficacy with time. Several options can be utilized to obtain the best result for the patient.1 A 55-year-old man with hypertension and obesity, who was a chronic smoker and drinker, presented with moderate–severe plaque psoriasis with psoriatic arthritis (PsA). He had been treated with acitretin, methotrexate (MTX), psoralen and ultraviolet A therapy (PUVA), cyclosporine A, mycophenolate mofetil, efalizumab, infliximab, etanercept, adalimumab, and ustekinumab with and without MTX. All of these treatments had been withdrawn for adverse effects or the loss of efficacy at the cutaneous and/or joint level. The subject had last been treated with golimumab at a dose of 50 mg s.c. per month, along with weekly s.c. injections of MTX 10 mg and had achieved a Disease Activity Score-28 (DAS28) index of 0, but had maintained a Psoriasis Area and Severity Index (PASI) score of 24 (Fig. 1a). We decided to increase this subject’s therapy with golimumab to 50 mg s.c. administered every 15 days and achieved a PASI score of 0 after two months, with no adverse effects (Fig. 1b). The patient remained clear of symptoms until September 2012, when the efficacy of the treatment was observed to decrease. The patient reported a PASI score of 7 at six months after the increase in golimumab therapy, which he has maintained to the present. A 61-year-old overweight woman with type 1 diabetes mellitus, hypertension, and dyslipidemia presented with moderate–severe plaque psoriasis with PsA. She was treated with narrowband ultraviolet B (UVB) phototherapy, PUVA therapy, MTX, etanercept, adalimumab, infliximab, and ustekinumab with and without MTX, all of which were withdrawn as a result of adverse effects or loss of efficacy at the cutaneous and/or joint level. The subject’s last treatment had been golimumab administered s.c. at a dose of 50 mg per month, along with associated with weekly s.c. injections of MTX 15 mg. She reported a DAS28 score of 0 and a PASI score of 11. This patient’s therapy was intensified so that golimumab, associated with MTX, was administered at a dose of 15 mg s.c. every week (Fig. 2a). After two months of treatment with this regimen, the patient achieved a PASI score of 0 (Fig. 2b). At six months, a slight loss of efficacy was International Journal of Dermatology 2014, 53, e578–e596
e585
e586
Correspondence
(a)
(b)
Figure 1 (a) Scaly, erythematous plaques are apparent on the anterior surfaces of both legs in a 55-year-old man who presented with a Psoriasis Area and Severity Index (PASI) score of 24. (b) At 2 months after the increase in golimumab dose, the patient demonstrated scaling and xerosis without psoriatic lesions and achieved a PASI score of 0
(a)
(b)
Figure 2 (a) Scaly, erythematous plaques are observed on the lumbosacral region in a 61-year-old woman who presented with a Psoriasis Area and Severity Index (PASI) score of 11. (b) At 2 months after the increase in golimumab dose, the patient demonstrated residual hyperpigmented macules on the back and reported a PASI score of 0
observed and the patient reported a PASI score of 2, which has remained stable to date with no adverse effects. Golimumab is an immunoglobulin G1K-type human monoclonal antibody. It has a half-life of 12.5 hours and is administered s.c. at a dose of 50 mg per month.2–4 It was licensed in 2009 by the US Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the treatment of rheumatoid arthritis,5 ankylosing spondylitis,6 and PsA.2 The clinical trial GO-REVEAL, conducted in patients with PsA, reported that a better rate of achievement of a PASI 75 response (75% reduction in score on the PASI) occurred in the group treated with s.c. golimumab at 100 mg per month than in patients treated with s.c. golimumab at 50 mg per month, with PASI 75 responses International Journal of Dermatology 2014, 53, e578–e596
achieved by 58% vs. 40% of patients at 14 weeks7 and 76% vs. 69% of patients at 104 weeks, regardless of whether MTX had been administered.8 The 50-mg increase in golimumab resulted in an average improvement of 22% in PASI scores after 12 weeks. Golimumab is equally as safe as all other anti-tumor necrosis factor-a (TNF-a) drugs approved for PsA, and no differences were observed between the two groups in occurrence of severe adverse effects.8 Based on the results of the GO-REVEAL trial, we decided to increase our patients’ doses of golimumab. After two months, both patients achieved PASI scores of 0 without any adverse effects. However, both patients experienced a slight loss of efficacy at six months after the increase in treatment. The gradual loss of efficacy of anti TNF-a drugs is a well-known phenomenon, particularly in patients who have been previously treated with drugs from the same group.9 Nevertheless, our patients maintained reductions of 70 and 80%, respectively, in PASI scores.
Jose Luis Torregrosa Calatayud, MD Juan Garcıas Ladaria, MD Jose Luis S anchez Carazo, PhD Amparo Perez-Ferriols, MD Vicente Oliver Martınez, MD Vıctor Alegre de Miquel, MD Department of Dermatology Consorcio General Hospital University of Valencia 46014 Valencia Spain E-mail: [email protected] Javier Calvo Catal a, MD Department of Rheumatology Consorcio General Hospital University of Valencia 46014 Valencia Spain E-mail: [email protected] Conflicts of interest: none.
References 1 Smith CH, Anstey AV, Barker JNWN, et al. British Association of Dermatologists guidelines for biologic interventions for psoriasis 2009. Br J Dermatol 2009; 161: 987–1019. 2 Kavanaugh A, McInnes I, Mease P, et al. Golimumab, a new human tumor necrosis factor a, administered every four weeks as a subcutaneous injection in psoriatic arthritis: twenty-four-week efficacy and safety results of a ª 2014 The International Society of Dermatology
Correspondence
3
4
5
6
7
8
9
randomized, placebo-controlled study. Arthritis Rheum 2009; 60: 976–986. Kavanaugh A, Mease P, Krueger GG, et al. Golimumab, a new human, TNF-alpha antibody, administered subcutaneously every four weeks in psoriatic arthritis patients: 104-week efficacy and safety results of the randomized, placebo-controlled GO-REVEAL study. Ann Rheum Dis 2009; 68(Suppl. 3): 136. Xu Z, Vu P, Lee H, et al. Population pharmacokinetics of golimumab an anti-tumor necrosis factor-a human monoclonal antibody, in patients with psoriatic arthritis. J Clin Pharmacol 2009; 49: 1056–1070. Smolen JS, Kay J, Doyle MK, et al. Golimumab in patients with active rheumatoid arthritis after treatment with tumor necrosis factor-a inhibitors (GO-AFTER study): a multicenter, randomized, double-blind, placebo-controlled, phase III trial. Lancet 2009; 374: 210–221. Inman RD, Davis JC Jr, van der Heijde D, et al. Efficacy and safety of golimumab in patients with ankylosing spondylitis: results of a randomized, double-blind, placebo-controlled, phase III trial. Arthritis Rheum 2008; 58: 3402–3412. Kavanaugh A, van der Heijde D, McInnes I, et al. Golimumab in psoriatic arthritis: one-year clinical efficacy, radiographic, and safety results from a phase III, randomized, placebo-controlled trial. Arthritis Rheum 2012; 64: 2504–2517. Kavanaugh A, McInnes IB, Mease PJ, et al. Clinical efficacy, radiographic and safety findings through 2 years of golimumab treatment in patients with active psoriatic arthritis: results from a long-term extension of the randomized, placebo-controlled GO-REVEAL study. Ann Rheum Dis 2013; 72: 1777–1785. Karlsson JA, Kristensen LE, Kapetanovic MC, et al. Treatment response to a second or third TNF-inhibitor in RA: results from the South Swedish Arthritis Treatment Group Register. Rheumatology (Oxford) 2008; 47: 507–513.
Solitary mastocytoma mimicking granuloma faciale
Editor, As a localized variant of mastocytosis, cutaneous mastocytoma is characterized by excessive mast cell accumulation within the skin, without extracutaneous organ involvement.1 It usually presents at birth as an oval, yellow–brown nodule or plaque, with no site predilection.1 If lesions are scratched, blistering may occur. Microscopically, mastocytoma is usually characterized by a dense diffuse or nodular mast cell infiltrate that may reach the subcutaneous tissue. An accompanying sparse to mild mixed inflammatory infiltrate may also be present.2,3 Here, we report, to the best of our knowledge, the first case of cutaneous mastocytoma microscopically resembling granuloma faciale (GF). A 13-year-old boy presented with a single scalp lesion of five months duration. The lesion was generally asympª 2014 The International Society of Dermatology
tomatic, except when traumatized, which led to an itching sensation. The subject was otherwise healthy. Physical examination revealed a solitary, 1.0 9 1.5-cm, welldemarcated, erythematous nodule over the scalp. Biopsy showed a well-defined grenz zone with a diffuse dermal inflammatory infiltrate consisting of a mixture of lymphocytes, eosinophils, neutrophils, plasma cells, histiocytes, and multinucleated giant cells (Fig. 1). Papillary dermal telangiectases, extravasated red blood cells, and leukocytoclasia were also seen; however, there was no evidence of necrotizing vasculitis. These features were suggestive of GF; however, the presence of numerous interstitial mast cells and a spindle cell proliferation raised the possibility of cutaneous mastocytoma (Fig. 1). Giemsa stain confirmed the metachromatic nature of mast cells, and CD117 immunohistochemical stain confirmed the dense dermal mast cell infiltrate within the dense inflammatory infiltrate (Fig. 2). Cutaneous mastocytoma was diagnosed. Routine laboratory tests of blood, urine, liver, and renal function were normal, as was serum tryptase level. Granuloma faciale is an uncommon benign inflammatory dermatosis that usually presents as asymptomatic or as multiple brown–red plaques, nodules, or papules, mostly on the face. However, extra-facial involvement such as that of the scalp can also occur.4 Its origin is unknown, but vascular injury is thought to contribute to the pathogenesis.4 Some consider it to be a manifestation of leukocytoclastic vasculitis, although vasculitic changes are not commonly seen. Classic histopathological features include the presence of a grenz zone (in up to 74% of cases), dermal telengiectasias (in up to 74% of cases), and a dermal mixed inflammatory infiltrate that can be diffuse in up to 38% of cases. The inflammatory infiltrate usually tends to comprise a mixture of lymphocytes, neutrophils, plasma cells, and/or eosinophils. Even multinucleated giant cells have been reported in 12.5% of cases.4 All of these microscopic features were actually present in our case. This might easily have led to a diagnostic misinterpretation were it not for the presence of a spindle cell proliferation and scattered mast cells within the infiltrate. Giemsa stain and CD117 immunohistochemical stain confirmed the diagnosis of cutaneous mastocytoma. Although mixed inflammatory infiltrate has been reported in association with cutaneous mastocytosis, we believe that a dense inflammatory infiltrate and skin involvement in a pattern strikingly reminiscent of GF have not been previously reported. Furthermore, the grenz zone is known to be one of the characteristic features of GF,4 although this feature has also been observed in other cutaneous inflammatory conditions, as well as in neoplastic benign and malignant entities. However, it has not been reported in association with cutaneous mastocytosis. International Journal of Dermatology 2014, 53, e578–e596
e587
Ana Miguelez, MD Department of Dermatology University Hospital of La Princesa Madrid Spain Joaquın Due~ nas, MD Department of Pediatrics Son Espases University Hospital Palma de Mallorca Spain Daniel Hervas, MD Juan A. Hervas, MD, PhD University Institute of Health Sciences (IUNICS) University of the Balearic Islands Palma de Mallorca Spain E-mail: [email protected] Francisco Salva, PhD Department of Microbiology Son Espases University Hospital Palma de Mallorca Spain Ana Martın-Santiago, MD Department of Dermatology Son Espases University Hospital Palma de Mallorca Spain
References 1 Heegaard ED, Brown KE. Human parvovirus B19. Clin Microbiol Rev 2002; 15: 485–505. 2 Katta R. Parvovirus B19: a review. Dermatol Clin 2002; 20: 333–342. 3 Hashimoto H, Yuno T. Parvovirus B19-associated purpuric–petechial eruption. J Clin Virol 2011; 52: 269–271. 4 Yamamoto T, Nishioka K. Flagellate erythema. Int J Dermatol 2006; 45: 627–631. 5 Ziemer M, Goetze S, Juhasz K, et al. Flagellate dermatitis as a bleomycin-specific adverse effect of cytostatic therapy. Am J Clin Dermatol 2011; 12: 68–76. 6 Poppe LM, Anders D, Kneitz H, et al. Flagellate dermatitis caused by shiitake mushrooms. An Bras Dermatol 2012; 87: 463–465. 7 Watanabe T, Tsuchida T. Flagellate erythema in dermatomyositis. Dermatology 1995; 190: 230–231. et al. 8 Santonja C, Nieto-Gonz alez G, Santos-Briz A, Immunohistochemical detection of parvovirus B19 in
ª 2014 The International Society of Dermatology
gloves and socks papular purpuric syndrome: direct evidence for viral endothelial involvement. Report of three cases and review of the literature. Am J Dermatopathol 2011; 33: 790–795.
Intensification therapy with golimumab: a new treatment strategy for moderate–severe refractory psoriasis
Moderate–severe psoriasis is a disabling entity that requires treatment with systemic drugs and in some cases with biological products. Unfortunately, these regimens can lose efficacy with time. Several options can be utilized to obtain the best result for the patient.1 A 55-year-old man with hypertension and obesity, who was a chronic smoker and drinker, presented with moderate–severe plaque psoriasis with psoriatic arthritis (PsA). He had been treated with acitretin, methotrexate (MTX), psoralen and ultraviolet A therapy (PUVA), cyclosporine A, mycophenolate mofetil, efalizumab, infliximab, etanercept, adalimumab, and ustekinumab with and without MTX. All of these treatments had been withdrawn for adverse effects or the loss of efficacy at the cutaneous and/or joint level. The subject had last been treated with golimumab at a dose of 50 mg s.c. per month, along with weekly s.c. injections of MTX 10 mg and had achieved a Disease Activity Score-28 (DAS28) index of 0, but had maintained a Psoriasis Area and Severity Index (PASI) score of 24 (Fig. 1a). We decided to increase this subject’s therapy with golimumab to 50 mg s.c. administered every 15 days and achieved a PASI score of 0 after two months, with no adverse effects (Fig. 1b). The patient remained clear of symptoms until September 2012, when the efficacy of the treatment was observed to decrease. The patient reported a PASI score of 7 at six months after the increase in golimumab therapy, which he has maintained to the present. A 61-year-old overweight woman with type 1 diabetes mellitus, hypertension, and dyslipidemia presented with moderate–severe plaque psoriasis with PsA. She was treated with narrowband ultraviolet B (UVB) phototherapy, PUVA therapy, MTX, etanercept, adalimumab, infliximab, and ustekinumab with and without MTX, all of which were withdrawn as a result of adverse effects or loss of efficacy at the cutaneous and/or joint level. The subject’s last treatment had been golimumab administered s.c. at a dose of 50 mg per month, along with associated with weekly s.c. injections of MTX 15 mg. She reported a DAS28 score of 0 and a PASI score of 11. This patient’s therapy was intensified so that golimumab, associated with MTX, was administered at a dose of 15 mg s.c. every week (Fig. 2a). After two months of treatment with this regimen, the patient achieved a PASI score of 0 (Fig. 2b). At six months, a slight loss of efficacy was International Journal of Dermatology 2014, 53, e578–e596
e585
e586
Correspondence
(a)
(b)
Figure 1 (a) Scaly, erythematous plaques are apparent on the anterior surfaces of both legs in a 55-year-old man who presented with a Psoriasis Area and Severity Index (PASI) score of 24. (b) At 2 months after the increase in golimumab dose, the patient demonstrated scaling and xerosis without psoriatic lesions and achieved a PASI score of 0
(a)
(b)
Figure 2 (a) Scaly, erythematous plaques are observed on the lumbosacral region in a 61-year-old woman who presented with a Psoriasis Area and Severity Index (PASI) score of 11. (b) At 2 months after the increase in golimumab dose, the patient demonstrated residual hyperpigmented macules on the back and reported a PASI score of 0
observed and the patient reported a PASI score of 2, which has remained stable to date with no adverse effects. Golimumab is an immunoglobulin G1K-type human monoclonal antibody. It has a half-life of 12.5 hours and is administered s.c. at a dose of 50 mg per month.2–4 It was licensed in 2009 by the US Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the treatment of rheumatoid arthritis,5 ankylosing spondylitis,6 and PsA.2 The clinical trial GO-REVEAL, conducted in patients with PsA, reported that a better rate of achievement of a PASI 75 response (75% reduction in score on the PASI) occurred in the group treated with s.c. golimumab at 100 mg per month than in patients treated with s.c. golimumab at 50 mg per month, with PASI 75 responses International Journal of Dermatology 2014, 53, e578–e596
achieved by 58% vs. 40% of patients at 14 weeks7 and 76% vs. 69% of patients at 104 weeks, regardless of whether MTX had been administered.8 The 50-mg increase in golimumab resulted in an average improvement of 22% in PASI scores after 12 weeks. Golimumab is equally as safe as all other anti-tumor necrosis factor-a (TNF-a) drugs approved for PsA, and no differences were observed between the two groups in occurrence of severe adverse effects.8 Based on the results of the GO-REVEAL trial, we decided to increase our patients’ doses of golimumab. After two months, both patients achieved PASI scores of 0 without any adverse effects. However, both patients experienced a slight loss of efficacy at six months after the increase in treatment. The gradual loss of efficacy of anti TNF-a drugs is a well-known phenomenon, particularly in patients who have been previously treated with drugs from the same group.9 Nevertheless, our patients maintained reductions of 70 and 80%, respectively, in PASI scores.
Jose Luis Torregrosa Calatayud, MD Juan Garcıas Ladaria, MD Jose Luis S anchez Carazo, PhD Amparo Perez-Ferriols, MD Vicente Oliver Martınez, MD Vıctor Alegre de Miquel, MD Department of Dermatology Consorcio General Hospital University of Valencia 46014 Valencia Spain E-mail: [email protected] Javier Calvo Catal a, MD Department of Rheumatology Consorcio General Hospital University of Valencia 46014 Valencia Spain E-mail: [email protected] Conflicts of interest: none.
References 1 Smith CH, Anstey AV, Barker JNWN, et al. British Association of Dermatologists guidelines for biologic interventions for psoriasis 2009. Br J Dermatol 2009; 161: 987–1019. 2 Kavanaugh A, McInnes I, Mease P, et al. Golimumab, a new human tumor necrosis factor a, administered every four weeks as a subcutaneous injection in psoriatic arthritis: twenty-four-week efficacy and safety results of a ª 2014 The International Society of Dermatology
Correspondence
3
4
5
6
7
8
9
randomized, placebo-controlled study. Arthritis Rheum 2009; 60: 976–986. Kavanaugh A, Mease P, Krueger GG, et al. Golimumab, a new human, TNF-alpha antibody, administered subcutaneously every four weeks in psoriatic arthritis patients: 104-week efficacy and safety results of the randomized, placebo-controlled GO-REVEAL study. Ann Rheum Dis 2009; 68(Suppl. 3): 136. Xu Z, Vu P, Lee H, et al. Population pharmacokinetics of golimumab an anti-tumor necrosis factor-a human monoclonal antibody, in patients with psoriatic arthritis. J Clin Pharmacol 2009; 49: 1056–1070. Smolen JS, Kay J, Doyle MK, et al. Golimumab in patients with active rheumatoid arthritis after treatment with tumor necrosis factor-a inhibitors (GO-AFTER study): a multicenter, randomized, double-blind, placebo-controlled, phase III trial. Lancet 2009; 374: 210–221. Inman RD, Davis JC Jr, van der Heijde D, et al. Efficacy and safety of golimumab in patients with ankylosing spondylitis: results of a randomized, double-blind, placebo-controlled, phase III trial. Arthritis Rheum 2008; 58: 3402–3412. Kavanaugh A, van der Heijde D, McInnes I, et al. Golimumab in psoriatic arthritis: one-year clinical efficacy, radiographic, and safety results from a phase III, randomized, placebo-controlled trial. Arthritis Rheum 2012; 64: 2504–2517. Kavanaugh A, McInnes IB, Mease PJ, et al. Clinical efficacy, radiographic and safety findings through 2 years of golimumab treatment in patients with active psoriatic arthritis: results from a long-term extension of the randomized, placebo-controlled GO-REVEAL study. Ann Rheum Dis 2013; 72: 1777–1785. Karlsson JA, Kristensen LE, Kapetanovic MC, et al. Treatment response to a second or third TNF-inhibitor in RA: results from the South Swedish Arthritis Treatment Group Register. Rheumatology (Oxford) 2008; 47: 507–513.
Solitary mastocytoma mimicking granuloma faciale
Editor, As a localized variant of mastocytosis, cutaneous mastocytoma is characterized by excessive mast cell accumulation within the skin, without extracutaneous organ involvement.1 It usually presents at birth as an oval, yellow–brown nodule or plaque, with no site predilection.1 If lesions are scratched, blistering may occur. Microscopically, mastocytoma is usually characterized by a dense diffuse or nodular mast cell infiltrate that may reach the subcutaneous tissue. An accompanying sparse to mild mixed inflammatory infiltrate may also be present.2,3 Here, we report, to the best of our knowledge, the first case of cutaneous mastocytoma microscopically resembling granuloma faciale (GF). A 13-year-old boy presented with a single scalp lesion of five months duration. The lesion was generally asympª 2014 The International Society of Dermatology
tomatic, except when traumatized, which led to an itching sensation. The subject was otherwise healthy. Physical examination revealed a solitary, 1.0 9 1.5-cm, welldemarcated, erythematous nodule over the scalp. Biopsy showed a well-defined grenz zone with a diffuse dermal inflammatory infiltrate consisting of a mixture of lymphocytes, eosinophils, neutrophils, plasma cells, histiocytes, and multinucleated giant cells (Fig. 1). Papillary dermal telangiectases, extravasated red blood cells, and leukocytoclasia were also seen; however, there was no evidence of necrotizing vasculitis. These features were suggestive of GF; however, the presence of numerous interstitial mast cells and a spindle cell proliferation raised the possibility of cutaneous mastocytoma (Fig. 1). Giemsa stain confirmed the metachromatic nature of mast cells, and CD117 immunohistochemical stain confirmed the dense dermal mast cell infiltrate within the dense inflammatory infiltrate (Fig. 2). Cutaneous mastocytoma was diagnosed. Routine laboratory tests of blood, urine, liver, and renal function were normal, as was serum tryptase level. Granuloma faciale is an uncommon benign inflammatory dermatosis that usually presents as asymptomatic or as multiple brown–red plaques, nodules, or papules, mostly on the face. However, extra-facial involvement such as that of the scalp can also occur.4 Its origin is unknown, but vascular injury is thought to contribute to the pathogenesis.4 Some consider it to be a manifestation of leukocytoclastic vasculitis, although vasculitic changes are not commonly seen. Classic histopathological features include the presence of a grenz zone (in up to 74% of cases), dermal telengiectasias (in up to 74% of cases), and a dermal mixed inflammatory infiltrate that can be diffuse in up to 38% of cases. The inflammatory infiltrate usually tends to comprise a mixture of lymphocytes, neutrophils, plasma cells, and/or eosinophils. Even multinucleated giant cells have been reported in 12.5% of cases.4 All of these microscopic features were actually present in our case. This might easily have led to a diagnostic misinterpretation were it not for the presence of a spindle cell proliferation and scattered mast cells within the infiltrate. Giemsa stain and CD117 immunohistochemical stain confirmed the diagnosis of cutaneous mastocytoma. Although mixed inflammatory infiltrate has been reported in association with cutaneous mastocytosis, we believe that a dense inflammatory infiltrate and skin involvement in a pattern strikingly reminiscent of GF have not been previously reported. Furthermore, the grenz zone is known to be one of the characteristic features of GF,4 although this feature has also been observed in other cutaneous inflammatory conditions, as well as in neoplastic benign and malignant entities. However, it has not been reported in association with cutaneous mastocytosis. International Journal of Dermatology 2014, 53, e578–e596
e587
