Congenital subungual nevus

A 4-month-old infant with a congenital, darkly pigmented, macular lesion involving the subungual and periungual areas of the right ring finger is described. The lesion was excised when the infant was 6 months old in order to rule out a congenital pigmented nevus as well as a congenital acrolentiginous melanoma. Histopathologic examination revealed a compound nevus. Nevi in this location are very rare in Caucasians at any age and should probably be excised to rule out malignant melanoma.     

Two cases of infantile subungual pigmented nevi with Hutchinson's sign

Two cases of subungual pigmented nevi with periungual melanotic freckles, namely Hutchinson's sign, are presented. They are a 4-year-old female and a 11-month-old male; in neither case did the mother notice the lesion at birth. Histologically, both cases were characterized by the presence of well-circumscribed small nests of nevus cells in the periungual melanotic areas; diagnoses of junctional nevus and compound nevus were made, respectively. However, atypical proliferations of melanocytes at the dermo-epidermal junction of the nail matrix and nail bed were observed in some areas. The measurement of the DNA content of melanocytes by means of microfluorometry revealed normal diploid pattern in the former case, but the latter case contained a few aneuploid cells, suggesting that the proliferating melanocytes might have malignant potentials. Infantile subungual pigmented nevi with Hutchinson's sign must be examined thoroughly and watched carefully.     

Subungual blue nevus with combined phenotypic features

Blue nail dyschromia may represent melanocytic, vascular, or other etiologies. A case of a subungual blue nodule is presented, with a pseudo-clubbed nail. On histopathologic examination, there was a combined subungual blue nevus, with features of a common blue nevus and a pigmented epithelioid melanocytoma. This unusual presentation is reviewed, with a discussion of blue nail dyschromia and subungual blue nevi.     

Malignant Melanoma In situ Arising in the Nail Unit of a Child

A very rare case of malignant melanoma in situ of the nail unit of a child is presented. Clinically, a pigmented streak was present on the finger nail of a 3-year-old girl, and the lesion increased in size and in darkness of the color associated with periungual pigmentation in the following two years. Histopathologically, sections showed proliferation of atypical melanocytes, arranged mostly in single units but some in nests, in and above the basal layer of the epithelium and admixed with long dendrites and a few mitotic figures. Pigmented lesions of nail units of children often show fading or loss of pigmentation clinically; however, a biopsy should be done when they show augmentative changes which clinically suggest malignancy, because subungual malignant melanoma can exist even in children, and proper biopsy can detect it in its early stages.     

Trichilemmal cyst with homogeneous blue pigmentation on dermoscopy

A 61-year-old woman was referred to our dermoscopy unit for a pigmented lesion that had been present on her left arm for 8 years. The patient did not notice any enlargement or change in colour. On dermoscopy, homogeneous blue pigmentation was seen. The lesion was excised with the pre-operative diagnosis of melanoma, blue naevus and dermatofibroma. Histopathological examination showed a trichilemmal cyst in the mid-dermis. Although homogeneous blue pigmentation on dermoscopy is the hallmark of blue naevus, it may be seen in metastatic melanoma and exceptionally in hemosiderotic and cellular types of dermatofibroma. Trichilemmal cyst should be borne in mind also in the dermoscopic differential diagnosis.     

Giant congenital melanocytic naevus associated with Dandy–Walker malformation, lipomatosis and hemihypertrophy of the leg

A 30-year-old woman presented with congenital hyperpigmented plaques and multiple tumoral lesions. Her left leg was hypertrophic, although the bones were normal. Dermatological examination revealed hyperpigmented macules and plaques with hair on the legs, abdominal and lumbar areas (covering > 60% of the total body surface) and multiple naevi on the face, arms, back and chest. Multiple soft masses of various sizes, some of which colocalized with hyperpigmented plaques, were seen on the trunk and legs. Malignant melanoma was excluded by histopathological examinations of multiple biopsy specimens obtained from the pigmented lesions. Histopathological examination of one of the soft masses showed that it was a lipoma. Cranial magnetic resonance imaging and computed tomography scans showed an enlarged fouth ventricule and vermis hypoplasia (Dandy–Walker malformation; DWM). Neurocutaneous melanosis is a rare combined abnormality of the skin and central nervous system. A few case reports relating to the coexistence of giant congenital melanocytic naevus, lipomatosis and hemihypertrophy have been published in the literature. We report this very rare case of giant melanocytic naevus with lipomatosis, hemihypertrophy of the leg, and DWM.     

Nail involvement in autosomal recessive congenital ichthyosis

Autosomal recessive congenital ichthyosis (ARCI) is a clinically and genetically heterogeneous category of nonsyndromic ichthyosis. Nail changes in ARCI are generally frequent but have been rarely reported and studied in the literature. This stimulated us to conduct a study to describe nail changes in ARCI using a combined literature review and prospective examination from March 2019 to August 2019 (6 months) in the Dermatology Department of Habib Thameur Hospital, Tunis, Tunisia. A total of 25 patients with ARCI had a clinical and dermatoscopic review. The mean age was 19.8 years (range, 1-43), with a female predominance (17 women [68%] and 8 men [32%]). Seventy-two percent had nail unit changes involving more than one nail, none had single nail disease, 64% had involvement of fingernails, and 68% had involvement of toenails, with cases including periungual hyperkeratosis (64%), xanthonychia (40%), pachyonychia (40%), macrolunula (36%), digital clubbing (32%), and onychomycosis (24%). Rarer findings included pseudoainhum, transverse leukonychia, longitudinal melanonychia, and subungual hemorrhages, each in one patient (4%). There was a statistically significant increased frequency of nail changes in adults over children (P = .001). Nail abnormalities statistically associated with ARCI were macrolunula, periungual hyperkeratosis, xanthonychia, and pachyonychia. A comprehensive review of the literature was performed, creating the first comprehensive review addressing nail disease in ARCI.     

Dermoscopy of subungual haemorrhage: its usefulness in differential diagnosis from nail‐unit melanoma

Background Subungual haemorrhages are characterized by well‐circumscribed dots or blotches with a red to red–black pigmentation, but some cases can be difficult to distinguish from subungual melanoma by the naked eye alone. Dermoscopy has proven to be a useful, noninvasive tool in the diagnosis of pigmented lesions in the nail; however, few dermoscopic studies of subungual haemorrhages have been reported. Objectives To investigate characteristic dermoscopic patterns of subungual haemorrhages, and to find distinctive features that can differentiate them from nail‐unit melanomas. Methods Patients with a confirmed diagnosis of either subungual haemorrhage or nail‐unit melanoma at a tertiary university hospital were included in the study. Clinical features and dermoscopic patterns were evaluated. Results Sixty‐four patients with a total of 90 lesions of subungual haemorrhage were enrolled in the study. The majority of cases (84%) showed combinations of more than one colour, while 16% had only one colour. The most common colour of the subungual haemorrhages was purple–black, in 37% of cases. A homogeneous pattern was observed in 92% of cases, globular patterns in 42% and streaks in 39%. Peripheral fading and periungual haemorrhages were found in 54% and 22% of cases, respectively. Destruction or dystrophy of the nail plate was observed in 16% of cases. In the 16 cases of nail‐unit melanomas, Hutchinson sign, longitudinal irregular bands or lines, triangular shape of bands, vascular pattern, and ulcerations were found in 100%, 81%, 25%, 6% and 81% of cases, respectively. In contrast, these features were not found in subungual haemorrhages. Conclusions Dermoscopy provides valuable information for the diagnosis of subungual haemorrhage and aids in the differential diagnosis from nail‐unit melanoma.     

Disseminated blue naevus and malignant blue naevus associated with excessive aromatase syndrome

We report a case of a 17-year-old boy who had a giant congenital blue naevus with multiple satellite pigmented lesions. Later the patient developed melanoma arising in the pre-existing lesion. He also had gynaecomastia and was diagnosed as having aromatase excess syndrome. To our knowledge, the association of these two rare conditions has not been previously reported. Further studies should be performed to investigate this unusual combination, which may have a genetic, endocrine or local cutaneous link leading to its occurrence.     

Benign melanocytic proliferative nodule within a congenital naevus

A neonate presented with a deeply pigmented papule within a medium-sized congenital naevus. Histologically, this proved to be a benign proliferative nodule in a congenital naevus. This case is presented to highlight the occurrence of this lesion, the main differential of which is the rare entity of true congenital melanoma.     

Uncommon presentation of pigmented paraungual basal cell carcinoma on the first toe treated with total excision

Basal cell carcinomas (BCCs) are the most common types of skin neoplasm. The finger, toe, and nail unit are uncommon and rare locations of BCC. Only a few patients with foot BCC have been reported, and ungual BCC is even less frequent. We present a patient with a BCC on the left first toe. Clinically, it appeared as a brown‐colored plaque with an irregular border on the nail fold and dorsum of the left thumb. Histopathological findings were consistent with the pigmented type of BCC. Surgical approach with subsequent full thickness mesh graft led to a complete remission. Follow‐up at 10 months revealed a functionally and cosmetically acceptable outcome. The clinical presentation of nail unit BCC is variable, and nonspecific manifestations occur more frequently. Our case stated the necessity of histopathologic examination in ulcerous lesions resistant to topical treatment.     

Onychomycosis due to Scopulariopsis brevicaulis: clinical features and response to systemic antifungals

Summary Six cases of Scopulariopsis onychomycosis, including four patients with onychomycosis exclusively caused by Scopulariopsis brevicaulis and two patients with a mixed nail infection (S. brevicaulis + Tricophyton rubrum and S. brevicaulis + T. interdigitale), are reported. Four patients presented with a typical distal subungual onychomycosis characterized by subungual hyperkeratosis and onycholysis of the distal nail plate. In two patients. Scopulariopsis infection produced a total dystrophic onychomycosis associated with painful periungual inflammation. Three patients were treated with four pulses of itraconazole. 400 mg daily for 1 week a month, and three patients with terbinafine, 250 mg daily for 4 months. The mycological examination 8 months after discontinuation of treatment showed that one patient was mycologically cured whereas the remaining five patients still carried S. brevicaulis in their nails. The clinical examination at the end of the follow-up period showed a complete cure of the nail abnormalities in only one patient.     

Extensive proliferative nodules in a case of giant congenital naevus

The rare presence of proliferative nodules in cases of giant congenital naevus can, in some cases, be potentially misdiagnosed as neonatal melanoma. We report here a case of multiple, proliferative nodules found in a giant congenital naevus lesion in a female neonatal patient diagnosed with neurocutaneous melanosis. Our initial clinical observations of this case suggested the possibility of primary cutaneous neonatal melanoma or skin metastasis from a melanoma in the meninges or elsewhere in the central nervous system. However, histological examination revealed no sign of melanoma, abnormal mitosis, necrosis or any malignant change. Pagetoid arrays of naevus cells in the junctional zone and myxoid changes present in a significant portion of the dermis led to the diagnosis of proliferative nodules. Distinct histological patterns seen in the proliferative nodules in our neonatal patient were useful to differentiate between benign pigmented nodular lesions in a giant congenital naevus and malignant melanoma, and reduced the chance of misdiagnosis.     

Periungual lesions in pyoderma gangrenosum

Pyoderma gangrenosum (PG) is a progressive cutaneous necrosis of unknown origin. We report a case of PG presenting with periungual lesions. A 57-year-old woman was on treatment with ciclosporin A for PG. During tapering of the ciclosporin A dose, proliferating periungual lesions developed on the third and fourth finger of the left hand, the fourth finger of the right hand, and on the right great toe and the left fifth toe. All lesions appeared within a 4-week period. These abnormalities were ulcerated, involved about one-third of the distal part of the lateral nail folds including the part of nail fold bordering on the free edge of the nails, and were very painful. The skin biopsy was consistent with that seen in PG. Increasing the ciclosporin A dose led to significant improvement in the periungual lesions within the next few weeks and complete resolution within 6 months.     

,,Blaue`` Variante eines Naevus spilus

Summary We report on a 29-year-old female patient with an unusual pigmented lesion of the face. Clinically the lesion looked like a pigment patch of the naevus spilus type, while histological examination revealed the presence of dermal melanocytosis and multiple common blue naevi with a discrete lentiginous component in addition. The melanocytic nature of the infiltrate was ascertained by immunohistochemistry analysis using S 100 protein and HMB 45 antibodies. We interpret this lesion as agminated blue naevi in association with lentigo simplex, an unusual variant of speckled lentiginous naevus.      

Pigmented nail with atypical melanocytic hyperplasia

We report two cases showing black discoloration of the thumb nail which were histologically found to be acral lentiginous melanoma (ALM) in situ. A pigmented subungual lesion is more frequently malignant than benign and it is generally believed that diagnosis of subungual melanoma during the radial-growth phase is very difficult. Our cases are particularly interesting because atypical melanocytic hyperplasia was confined to the epidermis despite the lesion being present for a long time.     

Pigmented nail streak associated with Bowen's disease of the nail matrix

We described a 59-year-old male physician with Bowen's disease occurring on the nail matrix of his right 5th finger. The rapid growth of the pigmented nail streak accompanied by nail deformity led us to consider the possibility of subungual melanoma clinically. Histologic features, however, were compatible with those of Bowen's disease accompanied by melanocytes with melanin-rich long dendrites in the nail matrix. We speculate that his occupational exposure to X-rays for 25 years played an important role in the pathomechanism of the present case.     

Melanoma arising from a plaque‐type blue naevus with subcutaneous cellular nodules of the scalp

Plaque‐type blue naevus (PTBN) is a very rare variant of blue naevus (BN). The potential malignancy of subcutaneous cellular nodules (SCN) in PTBN was discovered in 2012, and there is currently no clear consensus on prognostic factors or management guidelines of such lesions. PTBN on the scalp have not been described in the literature. We report the clinical, histopathological and immunohistological features of a 50‐year‐old man who presented with a 30‐year history of scalp PTBN, with malignant proliferation of nodular elements and fatal outcome 8 years later. This case suggests that long‐term monitoring of patients with PTBN is required. Early surgical removal of such lesions should be considered, especially in the presence of any case of enlargement or change.     

Dermal melanocytosis of the scalp associated to intracranial melanoma: malignant blue nevus, neurocutaneous melanosis, or neurocristic cutaneous hamartoma?

Intracranial invasion of cellular blue nevus is extremely rare, and its malignant transformation is even less common. The differential diagnosis includes neurocutaneous melanosis and neurocristic cutaneous hamartoma. A 50-year-old female presented with intracranial melanoma in contiguity with a congenital blue nevus on the scalp. The patient showed a wide pigmented lesion on the scalp that had grown in the last few years over the congenital blue nevus. Magnetic resonance imaging revealed an intracranial tumor lying contiguous to the nevus. Despite aggressive surgery, the tumor relapsed and the patient developed systemic metastases. We report a rare case of cellular blue nevus showing an unexpected aggressive behavior with extensive extra- and intracranial expansion and distant metastases.     

甲周纤维瘤继发甲脱落一例

患者,女,57岁.半年前无明显诱因右手中指甲右侧缘出现肿物,指甲逐渐开裂.肿物组织病理示:(右手中指肿物)损害中央伴有成纤维细胞和胶原纤维束,呈垂直走向;(右手中指双层甲)甲板平整,甲下大量角化物,表皮角化过度、灶性角化不全.诊断为甲周纤维瘤继发甲脱落.予以外科手术切除甲周疣状肿物及上层甲板.