Solitary mastocytoma mimicking granuloma faciale

randomized, placebo-controlled study. Arthritis Rheum2009; 60: 976–986.

3 Kavanaugh A, Mease P, Krueger GG, et al. Golimumab,a new human, TNF-alpha antibody, administeredsubcutaneously every four weeks in psoriatic arthritispatients: 104-week efficacy and safety results of therandomized, placebo-controlled GO-REVEAL study. AnnRheum Dis 2009; 68(Suppl. 3): 136.

4 Xu Z, Vu P, Lee H, et al. Population pharmacokinetics ofgolimumab an anti-tumor necrosis factor-a humanmonoclonal antibody, in patients with psoriatic arthritis.J Clin Pharmacol 2009; 49: 1056–1070.

5 Smolen JS, Kay J, Doyle MK, et al. Golimumab in patientswith active rheumatoid arthritis after treatment with tumornecrosis factor-a inhibitors (GO-AFTER study): amulticenter, randomized, double-blind, placebo-controlled,phase III trial. Lancet 2009; 374: 210–221.

6 Inman RD, Davis JC Jr, van der Heijde D, et al. Efficacyand safety of golimumab in patients with ankylosingspondylitis: results of a randomized, double-blind,placebo-controlled, phase III trial. Arthritis Rheum 2008;58: 3402–3412.

7 Kavanaugh A, van der Heijde D, McInnes I, et al.Golimumab in psoriatic arthritis: one-year clinicalefficacy, radiographic, and safety results from a phase III,randomized, placebo-controlled trial. Arthritis Rheum2012; 64: 2504–2517.

8 Kavanaugh A, McInnes IB, Mease PJ, et al. Clinicalefficacy, radiographic and safety findings through 2 yearsof golimumab treatment in patients with active psoriaticarthritis: results from a long-term extension of therandomized, placebo-controlled GO-REVEAL study. AnnRheum Dis 2013; 72: 1777–1785.

9 Karlsson JA, Kristensen LE, Kapetanovic MC, et al.Treatment response to a second or third TNF-inhibitor inRA: results from the South Swedish Arthritis TreatmentGroup Register. Rheumatology (Oxford) 2008; 47: 507–513.

Solitary mastocytoma mimicking granuloma faciale

Editor,As a localized variant of mastocytosis, cutaneous masto-cytoma is characterized by excessive mast cell accumula-tion within the skin, without extracutaneous organinvolvement.1 It usually presents at birth as an oval, yel-low–brown nodule or plaque, with no site predilection.1

If lesions are scratched, blistering may occur. Microscopi-cally, mastocytoma is usually characterized by a densediffuse or nodular mast cell infiltrate that may reach thesubcutaneous tissue. An accompanying sparse to mildmixed inflammatory infiltrate may also be present.2,3

Here, we report, to the best of our knowledge, the firstcase of cutaneous mastocytoma microscopically resem-bling granuloma faciale (GF).A 13-year-old boy presented with a single scalp lesion

of five months duration. The lesion was generally asymp-

tomatic, except when traumatized, which led to an itch-ing sensation. The subject was otherwise healthy. Physicalexamination revealed a solitary, 1.0 9 1.5-cm, well-demarcated, erythematous nodule over the scalp. Biopsyshowed a well-defined grenz zone with a diffuse dermalinflammatory infiltrate consisting of a mixture of lympho-cytes, eosinophils, neutrophils, plasma cells, histiocytes,and multinucleated giant cells (Fig. 1). Papillary dermaltelangiectases, extravasated red blood cells, and leukocy-toclasia were also seen; however, there was no evidenceof necrotizing vasculitis. These features were suggestive ofGF; however, the presence of numerous interstitial mastcells and a spindle cell proliferation raised the possibilityof cutaneous mastocytoma (Fig. 1). Giemsa stain con-firmed the metachromatic nature of mast cells, andCD117 immunohistochemical stain confirmed the densedermal mast cell infiltrate within the dense inflammatoryinfiltrate (Fig. 2). Cutaneous mastocytoma was diagnosed.Routine laboratory tests of blood, urine, liver, and renalfunction were normal, as was serum tryptase level.Granuloma faciale is an uncommon benign inflamma-

tory dermatosis that usually presents as asymptomatic oras multiple brown–red plaques, nodules, or papules,mostly on the face. However, extra-facial involvementsuch as that of the scalp can also occur.4 Its origin isunknown, but vascular injury is thought to contribute tothe pathogenesis.4 Some consider it to be a manifestationof leukocytoclastic vasculitis, although vasculitic changesare not commonly seen. Classic histopathological featuresinclude the presence of a grenz zone (in up to 74% ofcases), dermal telengiectasias (in up to 74% of cases), anda dermal mixed inflammatory infiltrate that can be diffusein up to 38% of cases. The inflammatory infiltrate usuallytends to comprise a mixture of lymphocytes, neutrophils,plasma cells, and/or eosinophils. Even multinucleatedgiant cells have been reported in 12.5% of cases.4

All of these microscopic features were actually presentin our case. This might easily have led to a diagnosticmisinterpretation were it not for the presence of a spin-dle cell proliferation and scattered mast cells within theinfiltrate. Giemsa stain and CD117 immunohistochemicalstain confirmed the diagnosis of cutaneous mastocytoma.Although mixed inflammatory infiltrate has beenreported in association with cutaneous mastocytosis, webelieve that a dense inflammatory infiltrate and skininvolvement in a pattern strikingly reminiscent of GFhave not been previously reported. Furthermore, thegrenz zone is known to be one of the characteristic fea-tures of GF,4 although this feature has also beenobserved in other cutaneous inflammatory conditions, aswell as in neoplastic benign and malignant entities.However, it has not been reported in association withcutaneous mastocytosis.

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Although the evidence as to the underlying pathogene-sis of this microscopic presentation remains inconclusive,one logical explanation refers to the effects of severalcytokines (interleukin-4 [IL-4], IL-5, tumor necrosis fac-tor-a, CC chemokine macrophage chemotactic protein-1,and the CXC chemokine IL-8) produced by mast cells inrecruiting and activating other cells in the inflammatorymicroenvironment, including lymphocytes, neutrophils,and eosinophils.5

Lamah Bourji, MD

Mazen Kurban, MD

Ossama Abbas, MD

Department of DermatologyAmerican University of Beirut Medical CenterBeirutLebanonossamaabbas2003@yahoo.com

References1 Valent P. Diagnostic evaluation and classification of

mastocytosis. Immunol Allergy Clin North Am 2006; 26:515–534.

2 Akiyama M. A clinical and histological study of urticariapigmentosa: relationships between mast cell proliferationand the clinical and histological manifestations. JDermatol 1990; 17: 347–355.

3 Dunst KM, Huemer GM, Zelger BG, et al. A new variantof mastocytosis: report of three cases clinicopathologicallymimicking histiocytic and vasculitic disorders. Br JDermatol 2005; 153: 642–646.

4 Ortonne N, Wechsler J, Bagot M, et al. Granulomafaciale: a clinicopathologic study of 66 patients. J AmAcad Dermatol 2005; 53: 1002–1009.

5 Krishnaswamy G, Ajitawi O, Chi DS. The humanmast cell: an overview. Methods Mol Biol 2006; 315:13–34.

Nodular hidradenoma of the wrist with spontaneous

regression in a 7-month-old infant

Nodular hidradenoma, referred to as clear cell hidraden-oma and eccrine acrospiroma, is a common cutaneousneoplasm that presents mainly in adults.1 Nodular hid-radenoma is rarely known to present in an infant, andthe spontaneous regression of nodular hidradenoma ininfancy has not been reported in the English literature.Recently, authors experienced an interesting case of nod-

(a) (b)

Figure 1 (a) Histopathology of a biopsy from a scalp lesion in a 13-year-old boy showed a well-defined grenz zone with adiffuse dermal mixed inflammatory infiltrate consisting of lymphocytes, eosinophils and neutrophils. (b) A spindle cellproliferation and scattered interstitial mast cells were also detected. [Hematoxylin and eosin stain; original magnification (a)94, (b) 940]

(a) (b)

Figure 2 (a, b) CD117 immunohistochemical stain confirmed the diagnosis of cutaneous mastocytoma. [Original magnification(a) 94, (b) 920]

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International Journal of Dermatology 2014, 53, e578–e596 ª 2014 The International Society of Dermatology

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