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Dermal melanocytoses (DMs) comprise a heterogeneous group of benign lesions, located on skin and mucous membranes, characterized by dendritic melanocytes in the dermis. Although they share common histopathological features, some variants may present only as bluish or grey patches, some only as papules/nodules/plaques and others may show combination of all of these lesions. Despite the fact that blue naevus (BN) is typically characterized with papulonodular lesions, its variants may show all of the aforementioned presentations. Mongolian spot, naevus of Ota and naevus of Ito are patchy DMs distinguished by their specific localizations. Apart from these classical forms, many atypical variants without unique clinicopathological characteristics have been described in the literature making the nomenclature of DMs more complicated. However, congenital dermal melanocytosis and acquired dermal melanocytosis seem to be crucial umbrella terms that encompass all patchy DMs in atypical locations. Papules or subcutaneous nodules on patchy lesions and association of epidermal pigmentation presenting as brownish patches may be encountered as rare features of DMs. On the other hand, delayed-onset subcutaneous nodules may be typical presentations of melanoma in patchy DMs; therefore, they deserve special attention. Large plaque-type BN with subcutaneous cellular nodules is a newly described entity, harbouring clinical features of various DMs together and has a high risk of melanoma. The whole spectrum of dermal dendritic melanocytic proliferations is discussed including novelties and controversial issues.

Background: The frequency of clinicoepidemiological variants of Kaposi's sarcoma (KS) differs markedly throughout the world. The iatrogenic variant is mainly associated with the use of immunosuppressive therapy. Aims: We aimed to investigate the distribution of KS variants in our practice and elucidate the underlying causes of iatrogenic KS. Methods: Consecutive KS patients seen in a single tertiary center were grouped according to the tumor variants and iatrogenic KS patients were evaluated about associated conditions. Results: Among 137 patients, classic variant was the most frequent presentation (n = 88), followed by iatrogenic (n = 37) variant. Among the iatrogenic group, ten were transplant recipients. In 16 iatrogenic KS patients, systemic corticosteroid was used, in four for myasthenia gravis (MG) and in three for rheumatoid arthritis. In three patients, KS developed under topical corticosteroid (TC) treatment. Among iatrogenic KS patients, ten of them had a second primary neoplasm and one had congenital immunodeficiency syndrome. Conclusions: Our study revealed one of the highest rates for iatrogenic KS (27%) reported in the literature. Besides well-known causes, relatively frequent association with MG was remarkable. Usage of different forms of TCs was the cause of KS in a few cases.

A limited number of cases have been reported showing the togetherness of mycosis fungoides (MF) and pityriasis lichenoides (PL) which are two rare diseases. As we observed PL et varioliformis acuta development soon after MF in a pediatric patient, were reviewed the related cases in the literature and determined that relationship could be in four different possible ways: the clinical presentation of MF with PL-like lesions, coexistence of MF and PL lesions, PL’s pioneering role in MF and as observed in our case, the occurrence of PL in a patient followed-up with a diagnosis of MF. Considering this wide spectrum of the relationship between two diseases, their pathogenesis may be partly associated.

Secondary localized cutaneous amyloidosis is a histopathological finding seen in the dermis, in various benign, premalignant, and malignant skin conditions, without clinical significance. The real incidence is not known. We aimed to investigate the phenomenon of secondary localized cutaneous amyloidosis in Bowen�s disease and Bowenoid papulosis. We retrospectively evaluated the data of all cases with histopathological confirmation of Bowen�s disease and Bowenoid papulosis between 2006 and 2017 in our Dermatovenereology and/or Pathology departments. Secondary localized cutaneous amyloidosis was observed in three patients with Bowen�s disease (3/52; 5.8%) and in three patients with Bowenoid papulosis (3/18; 16.7%). Herein, we present the demographic, clinical and histopathological features of these six cases of secondary localized cutaneous amyloidosis in detail. Although the occurrence of secondary localized cutaneous amyloidosis in epithelial tumors is a well-known phenomenon, its incidence has not been previously reported in Bowen�s disease and Bowenoid papulosis. Therefore, our results indicating a high incidence may be particularly important for Bowenoid papulosis, as its association with secondary localized cutaneous amyloidosis has only been shown in one case before. Moreover, in three of six cases, we histologically observed areas of regression with a marked prominence of amyloid deposition. Remarkably, two of these patients had a history of topical application of destructive agents which reveals a possible etiologic relationship between secondary localized cutaneous amyloidosis and cellular apoptosis/necrosis induced by these external agents.

Erdheim-Chester disease (ECD) is a very rare chronic life-threatening systemic disorder with non-specific skin involvement. It was formerly included in the non-Langerhans cell histiocytosis group. However in the recent revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineage, ECD is classified in the “Langerhans-related group” due to the showed clonal mutations involving genes of RAS-RAF-MEK-ERK protein kinase pathways detected in more than 80% of the patients similar to Langerhans cell histiocytosis. The heterogenous manifestations of ECD are caused by infiltration of histiocytes on many organs such as skeleton, central nervous system (CNS), cardiovascular system (CVS), lungs, orbit, kidneys, pituitary glands and skin. Although most parts of the cardiovascular system can be affected, pericardium and perivascular parts of the large vessels are most commonly affected. Cardiovascular manifestations are the major cause of mortality in ECD. As there are no specific symptoms of ECD, awareness of its multisystemic involvement and multidisciplinary approach is very important in diagnosis. Although skin findings including xanthelasma-like eyelid lesions and xanthomatous papulonodular lesions are not specific, they may play important role in the early diagnosis of the ECD when co-existing with systemic findings of the disease. Some patients of ECD follow an indolent clinical course and active treatment is necessary for symptomatic disease, CNS involvement or evidence of organ dysfunction. There are many treatment options however all of these, except for IFN-α, have low level of evidence for survival benefit. Vemurafenib is a new therapeutic option targeting the pathogenesis of ECD.

Background/objectives Benign cephalic histiocytosis (BCH) is a rare form of non-Langerhans cell histiocytosis in infants and young children characterized by self-healing macules and papules occurring primarily in the head and neck region. So far there have been nearly 60 reported cases in the English-language literature. Methods In this retrospective study, we evaluated clinical features and follow-up data of 11 patients diagnosed with BCH between 2004 and 2016 in the Department of Dermatovenereology, Istanbul Medical Faculty. Results There were 5 girls and 6 boys (median age 24 months, range 9-72 months). The median age at the onset of lesions was 8 months (range 3-36 months). The lesions first appeared on the face in 10 patients and on the trunk in 1. Proximal parts of the extremities and trunk were also involved in nine patients (81.8%). Patients were categorized into two groups based on their clinical features; five had 20 to 30 predominantly red-brown dome-shaped papules and six had 50 to hundreds of yellow-brown or predominantly pinkish brown flat papules. Four patients were lost to follow-up. In seven patients with a mean follow-up of 5 years, four had nearly complete resolution and three showed remarkable regression without treatment. Conclusion With 11 additional cases from a single center, BCH seems to be an underrecognized disease. Its clinical presentation is not uniform. Considering that most of the patients in this series and those previously reported had extracephalic involvement, the term "cephalic" needs to be reevaluated.

Acrodermatitis enteropathica syndrome (AE) is a clinical entity that results in severe zinc deficiency. It can be genetic or acquired. Acquired AE has been reported in patients with chronic liver disease, malabsorption syndrome, sickle cell anemia, and chronic renal failure. We present a kidney transplant recipient with skin rash and watery diarrhea. The patient had low serum zinc levels, which quickly resolved after zinc supplementation. Skin biopsy showed cytoplasmic pallor and vacuolization and ballooning degeneration of keratinocytes within the superficial epidermis, which may have led to confluent necrosis of keratinocytes. Large amounts of keratinosome-derived lamellae were found in the intercellular spaces in the keratinized area, probably related to disturbance of keratinosome metabolism due to zinc deficiency.

ZusammenfassungHintergrund und Ziel Als seltene Form der Mycosis fungoides (MF), ist die follikulotrope MF (FMF) durch ein breites Spektrum klinischer Symptome gekennzeichnet. Dazu gehoren, neben den vorherrschenden follikularen Lasionen, auch viele atypische Manifestationen. Das Ziel der vorliegenden Studie war eine klinische Bewertung von FMF-Patienten, unter besonderer Berucksichtigung von vernachlassigten dermatologischen Merkmalen. Patienten und Methodik Insgesamt wurden 27 FMF-Patienten aus dem 572 Patienten umfassenden MF-Register unserer Abteilung retrospektiv bezuglich ihrer Demographie sowie der klinischen Merkmale, Behandlungsformen, Nachsorge und Therapieergebnisse bewertet. Ergebnisse Neben den bekannten klinischen Symptomen der FMF fanden wir Lichen-spinulosus-artige Lasionen mit begleitender Hypopigmentierung (n = 3) und Alopezie (n = 2), infiltrierte/erhabene, erythematose Plaques im Gesicht, die zunachst als Lupus tumidus angesehen wurden (n = 2), pseudotumorale Lasionen, die klinisch eine MF im Tumorstadium vortauschten (n = 1), dauerhafte Exkoriationen (n = 1), erythematose, Rosazea-artige Papeln im Gesicht (n = 1) sowie kuppelformige, asymptomatische, mit Muzin gefullte (in der Histologie) Papeln/Knoten (n = 2), die andere krankheitsbedingte Lasionen uberlagerten. Es kamen mehrere Therapieansatze mit unterschiedlichem Ergebnis zur Anwendung. Acht (29,6 %) Patienten hatten FMF im Spatstadium. Schlussfolgerungen Das Bewusstsein fur vernachlassigte klinische Symptome kann wesentlich dazu beitragen, verspatete Diagnosen dieser aggressiven MF-Variante zu verringern.

SummaryBackground and Objective A rare variant of mycosis fungoides (MF), folliculotropic MF (FMF) is characterized by a broad clinical spectrum that primarily includes follicle-based lesions but also many atypical clinical manifestations. The objective of the present study was to conduct a clinical analysis of patients with FMF, with a particular focus on highlighting underrecognized dermatological features. Patients and Methods Overall, 27 FMF patients enrolled in our department';s MF registry, which includes 572 patients, were retrospectively reevaluated with regard to demographics, clinical features, treatment modalities, follow-up, and outcomes. Results Besides the well-known clinical features of FMF, we found lichen spinulosus-like lesions in association with hypopigmentation (n = 3) and alopecia (n = 2), infiltrated/elevated erythematous facial plaques initially considered to be lupus tumidus (n = 2), pseudotumoral lesions clinically mimicking tumor-stage MF (n = 1), persistent excoriations (n = 1) and erythematous facial papules mimicking rosacea (n = 1), as well as white dome-shaped asymptomatic papules/nodules filled with mucin (on histology) (n = 2) that overlay other disease-related lesions. Various therapeutic methods were used with variable results. Eight (29.6 %) patients had late-stage disease. Conclusions Awareness of underrecognized clinical manifestations may be key to reducing delayed diagnosis of this aggressive MF variant.

Lichen sclerosus (LS), also known as lichen sclerosus et atrophicus, is a chronic inflammatory mucocutaneous disease affecting the genital and/or extragenital areas. Although LS usually occurs alone, its coexistence in morphea patients has been reported in 5.7% and 38.0% (genital LS) of cases, in two series (1). A 74-year-old woman presented with a 6-month history of multiple asymptomatic, shiny , indurated, brownish large flat plaques located on the abdomen (Figure 1, a-b) and back, intermingled with slightly atrophic, white-colored, guttate, and patchy areas (Figure 1, d-e). Both punch biopsies of the sclerotic plaques on the back and abdomen showed findings consistent with morphea (Figure 1, c, f). Furthermore, the punch biopsy of a well-demarcated white plaque on the back revealed findings compatible with LS (Figure 1, f). Remarkably, there were also multiple white-colored lesions on the sites of pregnancy-induced striae distensae (SD) (Figure 2, a-b) on the lower abdomen and an old appendectomy scar (Figure 2, c). There was no anogenital involvement. A diagnosis of morphea-LS overlap was established and white lesions located on the surgical scar and SD were clinically evaluated as LS. Methotrexate (15 mg/week) achieved a partial regression of morphea plaques in three months. However, white LS lesions remained unchanged. Our patient presented with coexistence of LS and morphea on different sites of the trunk and on the same lesion. Additionally, one of the isolated LS lesions was located on a surgical scar. Occurrence of LS on skin grafts, irradiated areas, injection sites, or burn/surgical scars has been attributed to the Koebner phenomenon, also called isomorphic response, defined as "the formation of the skin lesions in the same morphology of the existing disease on the areas of various cutaneous injuries" (2). LS is classified under the Koebner category-III (occasional lesions) (2). However, in a case of morphea with features of LS that developed in 1 month following a herpes zoster infection has been suggested to represent "Wolf's isotopic response" (3), which was originally defined as "the occurrence of a new skin disease at the site of another, unrelated and already healed skin disorder" with a time interval between the first and second diseases ranging from months to several years (4). Remarkably, typical morphea plaques in our patient did not involve the surgical scar, in contrast to a cohort in which 16% of 329 patients developed initial morphea lesions at sites of prior (surgery) or ongoing/repetitive (chronic friction) skin trauma (5). SD appear on skin as atrophic linear bands mostly due to rapid weight changes, pregnancy, Cushing syndrome, or prolonged use of corticosteroids (6). The mechanism underlying the occurrence of several diseases on striae is still elusive. Blunt trauma occurring during the development of striae has been suggested to cause the Koebner phenomenon in patients with vitiligo, psoriasis, and lichen planus (7), but it has been suggested that the occurrence of leukemia cutis on SD in a patient reflects Wolf's isotopic response (8). Although chronic graft-versus-host disease, urticarial vasculitis, keloid, lupus erythematosus, diffuse normolipemic plane xanthoma, and drug-induced cutaneous eruptions have been reported to occur on striae (6,9), such an association with LS as in our patient has not been previously documented in the literature. Concomitant occurrence of LS patches on different previous lesions such as a surgical scar and SD in our patient raises the possibility of a common underlying mechanism. As mentioned above, the terms "Koebner phenomenon" or "Wolf's isotopic response" have been used to designate the development of some diseases in injured areas. However, Happle and Kluger (10) claimed in a recent statement that "there is no clear-cut criterion to distinguish isotopic response from Koebner phenomenon and all reactions of this kind represent examples of Koebner phenomenon", which seems to be the best way to describe the site-specific occurrence of LS lesions in our patient.

Objective: To reveal the risk factors for lower-extremity cellulitis, to investigate the role of risk factors on the treatment, and to have knowledge about treatment efficiency and the optimal duration of therapy in patients on parenteral antibiotic therapy. Methods: Potential risk factors, therapies used during hospitalization, outcomes of therapies and follow-up data of 46 patients hospitalized in the Department of Dermatovenereology of Istanbul Medical Faculty of Istanbul University with lower-extremity cellulitis between years 2009 and 2014 were investigated retrospectively. Results: Tinea pedis (n=19), diabetes mellitus (n=12), by-pass surgery (n=6), and chronic lymphedema of the legs (n=5) were detected as main risk factors. Improvement with systemic therapy was seen in all patients except one patient who was lost to follow-up. Thirty nine of the patients (84.8%) were treated with parenteral ampicillin-sulbactam. The mean duration of parenteral antibiotic therapy was 17.7±12.4 (median: 14) days. In 11 patients (32.4%), recurrence was detected, during an average follow-up of 2 years. There was a significant correlation between tinea pedis and recurrence. Conclusion: Tinea pedis and chronic lymphedema were main risk factors for multiple attacks. Parenteral antibiotic treatment mostly used as the single drug regimen with ampicillin-sulbactam was highly effective. Relatively longer duration of treatment in our patients has been attributed to the facts that being a tertiary medical center, dealing mostly with therapy resistant and high risk patients of cellulitis or antibiotic resistance in our country.

Background/objectives The association between vitiligo and congenital melanocytic nevi remains incompletely understood. The objective of this study was to investigate the frequency of depigmentation, including vitiligo, in patients with a large congenital melanocytic nevus (LCMN), which is a rare melanocytic tumor variant. Methods We retrospectively reviewed the files of 92 patients with an LCMN, including photographic documentation regarding the presence of pigment loss on the nevus mass, around the nevus, around the satellites, and elsewhere on the body. Results Depigmentation was observed in 8 (8.7%) of 92 patients with an LCMN. Depigmented areas within the main nevus mass were observed in six patients, and adjacent or remote vitiligo was observed in four patients. One patient also demonstrated halo depigmentation around some satellite nevi. Conclusion The coexistence of an LCMN with vitiligo does not appear to be rare and may occur with a spectrum of clinical presentations.

Nevoid hyperkeratosis of the nipple and areola (NHNA) is a rare clinicopathological entity showing persistent and strictly localized hyperkeratotic lesions of the nipple, areola or both with unknown etiopathogenesis. A similar clinical appearance may also be seen in different diseases with specific histopathological features. There are a few anecdotal reports on the association of NHNA with mycosis fungoides (MF), but they do not describe a uniform condition. In this report, we present 3 patients with hyperkeratotic lesions of the nipple and areola associated with MF but showing different histopathological features. We also review similar cases in the literature and discuss possibilities concerning this association. Two of our cases represent the association between MF and NHNA without histopathological features of MF on the nipple-areola complex. The other case represents hyperkeratosis of the nipple and areola with specific histological and immunohistochemical features of MF. Hence, we would like to hypothesize that MF may involve the nipple and areola and have an appearance similar to NHNA. Intriguingly, however, NHNA may occasionally also be seen in association with MF. However, this peculiar association requires further explanation.

Istanbul Universitesi Istanbul Tip Fakultesi, Dermatoloji Anabilim Dali, Istanbul, Turkiye @Telif Hakki 2015 Turk Dermatoloji Dernegi Makale metnine www. turkdermatolojidergisi.com web sayfasindan ulasilabilir. @Copyright 2015 by Turkish Society of Dermatology Available on-line at www.turkdermatolojidergisi.com Melike Umarova Aslan, Istanbul Universitesi Istanbul Tip Fakultesi, Dermatoloji Anabilim Dali, Istanbul, Turkiye E-posta: melikeumarova@hotmail.com Gelis Tarihi/Submitted: 27.08.2014 Kabul Tarihi/Accepted: 02.12.2014 Yazisma Adresi/ Correspondence: Objective: PUVA therapy is mostly used longer than other diseases in mycosis fungoides (MF) patients. We aimed to investigate the chronic side effects of PUVA therapy, including skin cancer to disclose the actual risk in Turkish population. Methods: We screened all MF patients treated with PUVA at the Department of Dermatology and Venereology of Istanbul Medical Faculty and included patients in whom PUVA has first initiated between 1994 and 2006 and administered in one or several courses and who were followed up more than five years with last visit in 2012. Results: Fifty patients with MF had a mean follow-up duration of 10.2±2.8 years. Six patients (12%) had PUVA lentigines. Two of them (4%) developed skin cancer (basal cell carcinoma, Bowen’s disease) during their follow-up. The mean sessions (295.5±7.8 sessions) and total UVA doses (1452 J/cm2) in these patients with skin cancer were higher than those who did not develop skin cancer (128.7±73 sessions and 515±31 J/ cm2, respectively). There was a significant relationship between the PUVA lentigines and development of cutaneous malignancy (p=0.01). Ozgun Arastirma / Original Investigation 12 Turk J Dermatol 2015; 1: 12-7 • DOI: 10.4274/tdd.2413 13 Umarova Aslan ve ark. PUVA Tedavisinde Deri Kanseri Riski. Turk J Dermatol 2015; 1: 12-7 Conclusion: Cutaneous malignancies were detected in 4% of MF patients followed up approximately 10 years. Due to this low risk, we conclude that PUVA therapy is relatively safe in patients with MF in our country. However, UVA should not be applied in high total doses and patients should be followed-up lifelong.

Malignant melanoma (MM) shows rapid increase in the incidence and it is the most common cause of death due to skin cancer. On the other hand, MM can completely be removed in patients noticing the lesions early and applying to physicians on time. Thus the life expectancy of these patients is not altered. Although MM can occur in every individual, it is more common in patients with some of the established risk factors such as fair skin, increased sun exposure, genetic basis (CDKN2A mutation), and existence of increased number of melanocytic nevi and/or dysplastic nevi. A specialized care of patients with risk factors and their regular follow-up is critical to lower the mortality and morbidity in MM. Early diagnosis of MM prevents metastasis and decreases mortality rate. Nowadays, there are many newly developed imaging methods to help the clinicians before biopsy. However, the suspicion of MM should initially be based upon major clinical findings of the lesion subject to changes during the evolution of the tumor. In this review, risk factors of MM will be summarized and clues for early diagnosis of this dangerous tumor will be discussed.

Osteoporosis, osteosclerosis, and lytic bone lesions have been observed in patients with systemic mastocytosis (SM). We examined bone mineral density (BMD) biochemical turnover markers and serum tryptase levels in SM, which is considered a rare disease.Seventeen adult patients (5 females, 12 males; median age: 33 years, range: 20-64) with mastocytosis were included in this study. We investigated the value of quantitative ultrasound (QUS) of the calcaneus in the assessment of BMD in SM patients, as well as BMD of the lumbar spine (L1-L4), femoral neck, and distal radius using dual energy x-ray absorptiometry (DXA) and plasma tryptase levels, biochemical markers of bone turnover.At lumbar spine L1-L4, the femoral neck, and the distal radius or as calcaneus stiffness, 12 of 17 patients had T-scores of less than -1 at least at 1 site, reflecting osteopenia. Three of 17 patients had T-scores showing osteoporosis (T-score-2.5). There was no relationship between DXA and bone lesion severity. We also found a significant positive correlation between tryptase levels and disease severity, as well as between disease severity and pyridinoline (p0.01 by Spearman's test).DXA and calcaneal QUS may not be appropriate techniques to assess bone involvement in SM patients because of the effects of osteosclerosis. This study further shows that the osteoclastic marker pyridinoline is helpful in patients with severe disease activity and sclerotic bone lesions to show bone demineralization.Amaç: Sistemik mastositozlu (SM) hastalarda osteoporoz, osteoskleroz ve litik kemik lezyonları görülebilir. Bu çalışmada nadir bir hastalık olan SM’de, kemik yoğunluk ölçümü (BMD), serumda kemik ‘turnover’ belirteçleri ve triptaz düzeyi araştırılmıştır. Gereç ve Yöntemler: Çalışmaya 5’i kadın, 12’si erkek olmak üzere median yaşları 33 (20-64) olan toplam 17 hasta dahil edilmiştir. Hastaların BMD’leri lomber vertebra (L1-L4), femur boynu ve distal radiustan dual enerji x-ray absorbitesi (DXA) yöntemi, kalkaneustan ise kantitatif ultrasonografi (USG) yöntemi kullanılarak ayrı ayrı hesaplanmıştır. Serum triptaz düzeyleri ile biyokimyasal kemik ‘turnover’ belirteçlerileri arasındaki ilişki araştırılmıştır. Bulgular: Hastaların 12 tanesinde osteopeni (incelenen kemik alanlarının en az birinde T skoru-1), 3 tanesinde ise osteoporoz (T skoru-2,5) saptanmıştır. Kemik lezyonlarının şiddeti ile DXA sonuçları arasında korelasyon gösterilememiştir. Triptaz ve pridinolin seviyeleri ile hastalık şiddeti arasında pozitif korelasyon bulunmuştur (p0,01). Sonuç: Osteosklerotik kemik lezyonları sebebiyle SM’li hastalarda kemik tutulumu değerlendirmesi için DXA ve kalkaneus USG uygun teknikler değildir. Osteoklastik aktivite belirteçleri olan pridinolin, şiddetli hastalık aktivitesi ve kemik sklerozlarını değerlendirmede faydalıdır.

Docetaxel is a commonly used chemotherapeutic agent and can cause skin side effects including hand-foot syndrome. Hand-foot syndrome is mostly limited to palmoplantar regions, but may sometimes require discontinuation of the drug because of the severity of the lesions. In this report, we present three patients admitted to our dermatovenereology department for the lesions at the dorsal side of the hand, an unusual location for this side effect, while receiving docetaxel treatment in the oncology clinic. Erythematous, scaly patches on the dorsum of the hands were the common feature in the three patients. There were accompanying plantar involvement in one of the patients, and nail findings (onycholysis, melanonychia striata) in the other two patients. Premedication with corticosteroid before chemotherapy in these patients could not prevent the dermatologic side effects. The lesions healed after discontinuation of docetaxel and use of symptomatic topical drugs. The cutaneous side effects of docetaxel may reduce the quality of life and even lead to discontinuation of the drug as seen in our cases. The occurrence of lesions on both hand dorsums in our patients suggests that hand-foot syndrome induced by docetaxel is not always limited to palmoplantar regions.

Lipoid proteinosis (LP) is a rare autosomal recessive disease characterized by deposition of hyaline material in skin and mucosae. Epilepsy, as an extracutaneous manifestation associated with typical mesial temporal calcifications, has already been identified, but its characteristics and long-term prognosis have not been thoroughly investigated. We included 7 consecutive patients with LP with typical intracranial calcifications out of 16 patients with ECM1 mutations and investigated the semiologic features, ictal and interictal EEG findings, and long-term prognosis of epilepsy in this genodermatosis. Four of them had seizures (57.1%), and focal seizures with motionless staring were the most common seizure phenotype, originating from bilateral mesial temporal areas, but interictal spikes were scant. Auras were observed in three patients, mostly as epigastric sensation and deja vu, which indicated mesial temporal lobe origin. Three patients with homozygous mutations in sixth and seventh exons of the ECM1 gene had a drug-resistant course at the end of long-term follow-up. Molecular genetic testing showed a rare compound heterozygous mutation in one patient, which was also associated with seizures but without drug-resistance. Our findings indicated a spectrum for epilepsy with a desperate drug-resistant course for decades in most patients with LP, which is still an underrecognized disease by neurologists.