Acta Dermato-Venereologica 97-6 97-6CompleteContent | Page 26

756 SHORT COMMUNICATION Multiple Primary Acral Lentiginous Melanoma on the Feet Developing in Lesions of Nagashima-type Palmoplantar Keratoderma Akimasa ADACHI 1 , Mayumi KOMINE 1 , Takeo MAEKAWA 1 , Satoru MURATA 1 , Aiko SHIOHAMA 2,3 , Akiharu KUBO 2 and Mamitaro OHTSUKI 1 Department of Dermatology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi 329-0498, 2 Department of Dermatology, Keio University, and 3 KOSÉ Endowed Program for Skin Care and Allergy Prevention, Keio University School of Medicine, Tokyo, Japan. E-mail: [email protected] 1 Accepted Feb 20, 2017; Epub ahead of print Feb 22, 2017 The background of multiple primary melanomas (MPMs) occuring in a patient varies influenced by genetic and environmental factors. Palmoplantar keratoderma (PPK) could be one of such backgrounds. The first cases of Nagashima-type palmoplantar keratoderma (NPPK) were reported in the Japanese literature in 1977 and in the English literature in 1989 (1, 2), respectively. NPPK was suggested to be a distinct entity from other PPKs (3), and this was confirmed by the identification of the causative gene mutations in SERPINB7, a gene coding for a skin-specific serine protease inhibitor (4). Twenty cases of malignant melanoma (MM) in skin lesions of PPK have been reported, including the following types of PPK: NPPK, Mal de Meleda (MDM), Papillon-Lefèvre syndrome, and Unna-Thost PPK (5–7). In 2014, the first case of acral lentiginous melanoma (ALM) in skin lesions of NPPK was reported; however, the mutation in the SERPINB7 gene was not investigated (5). PPK with no specific diagnosis and no history of familial mela- noma. Laboratory tests were within the normal limits, including serum 5-S-cysteinyl-DOPA (5-S-CD) level, which is often used as a marker of disease progression in Japan. Abdominal ultraso- nographic test, whole-body computed tomography (CT), and gal- lium-67 scintigraphy showed no signs of metastasis. Surgery was performed with a 3-cm margin with full-thickness subcutaneous adipose tissue. A medial plantar flap was applied to the left heel and a full-thickness skin graft from his abdomen to both heels. Histopathological examination revealed that atypical spindle to epithelioid cells were proliferated in the spinous and basal layer of the epidermis in nests and in single cells (Fig. 1c–f). They infiltrated the deep dermis and subcutaneous adipose tissue (Fig. 1g, h), and along the ducts of sweat glands. The thickness of the tumours in the right and left heels were 3.0 and 3.4 mm, respec- tively, with negative surgical margins. Elective bilateral inguinal lymphadenectomy was performed and no residual tumour cells were detected. The patient was diagnosed with multiple primary CASE REPORT A 55-year-old Japanese man with bilateral black macu- les on both heels presented to our outpatient department in 1999. A macule had developed on his left heel 3 years prior to presentation; and on his right heel 2 months prior to presentation. Both macules were gradually in- creasing in size. Physical examination revealed poorly demarcated black macules, and a plaque on the right heel, with ulceration and hyperkeratosis, 35×30 mm in size, and a plaque on the left heel, 20×20 mm in size (Fig. 1a, b). The patient was diagnosed with bilateral ALM. The patient had had PPK as a child and had re- ceived radiotherapy to his palms and soles during his primary school years. He had unspecified kidney disease at the age of 15 years old for several months, which improved and he had no renal dysfunction after that. His family history had no consanguineous marriages. His mother and grandmother had similar symptoms of Fig. 1. The patient’s first melanomas on the feet. (a) Poorly demarcated black macule (35×30 mm) on the right heel in 1999, with hyperkeratosis and ulceration. (b) Black macule on the left heel (20×20 mm). (c–h) Histopathological findings of lesions on the right and left heel on haematoxylin and eosin staining. (c, d) Low-magnification image of the lesions on: (c) right and (d) left heels revealed basophilic nests located in the epidermis and deep dermis (original magnification ×40). (e–h) High-magnification image of the lesions on: (e, g) right and (f, h) left heels revealed atypical cells as single cells and nests in the epidermis (e, f) and infiltrated into the deep dermis (g, h) (original magnification ×200). doi: 10.2340/00015555-2640 Acta Derm Venereol 2017; 97: 756–758 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2017 Acta Dermato-Venereologica.