Demographic, clinical and dermoscopic features of melanocytic nevi on the scalp.

BACKGROUND: The scalp is a special anatomical area and dermoscopic findings of this region may significantly differ from other body parts. OBJECTIVE: To investigate and compare the clinical and dermoscopic patterns of scalp melanocytic nevi in patients ≤15 years of age and above, and to analyse their relevance to demographic features, atypical mole syndrome (AMS) and total body nevus count (TBNC). METHODS: In this retrospective cohort study, the clinical data and dermoscopic images of patients with scalp melanocytic nevi were retrieved, reviewed and analysed. Demographic, clinical and dermoscopic features were compared in patients ≤15 years of age and above. RESULTS: A total of 196 scalp melanocytic nevi in 126 patients (female/male:64/62; ≤15/>15 years of age: 49/77) with a median age of 18.5 years (range 0-72) were evaluated. Statistically, the globular pattern was significantly higher in all age groups, and the papillomatous pattern was significantly lower in patients ≤15 years of age (P = 0.008 and P = 0.005, respectively). The eclipse pattern was significantly higher, and the homogenous pattern was significantly lower in patients ≤15 years of age with AMS (P = 0.003 and P = 0.014, respectively). Finally, patients ≤15 years of age with 50 to 100 TBNC had a higher eclipse pattern than those with 0 to 25 TBNC. CONCLUSION: The findings of this retrospective study might implicate that children with eclipse pattern of scalp melanocytic nevi might be 'moley' in the future with an impending risk of AMS. This hypothesis requires confirmation in future prospective studies on a larger cohort of patients.

Clear cell sarcoma of soft tissue with eccrine differentiation: A case report and review of the literature.

Clear cell sarcoma of soft tissue (CCSST) is a deep soft tissue tumor presenting in the extremities of young adults. Histopathologically, nests and sheets of polygonal cells with clear to eosinophilic cytoplasm separated by fibrous septa as well as occasional "wreath-like" giant cells are visualized. However, CCSST has been noted to have atypical histopathological features, such as epidermotropism or myxoid differentiation, or occurrence at unusual sites. Here, we present a case of eccrine ductal differentiation in CCSST. The patient, a 21-year-old woman, presented with a lump of 10-year duration sized 3 × 5 cm on the plantar surface of the fourth and fifth interdigital spaces. There had been an increase in size as well as pain and redness over 6 years. Besides the characteristic findings, there were ductal structures in continuity with the upper dermis indicative of ductal differentiation. The tumor stained positively for S100, HMB45, and succinic dehydrogenase; ducts stained positively for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). CCSST was confirmed with cytogenetic analysis showing the translocation associated with EWSR1-ATF1 fusion gene. Therefore, ductal differentiation is a unique finding that should be considered when evaluating for CCSST.

Secretan Syndrome: A Fluctuating Case of Factitious Lymphedema.

Secretan syndrome (SS) is a recurrent or chronic form of factitious lymphedema that usually affects the dorsal aspect of the hand and is accepted as a subtype of Munchausen syndrome. Secretan syndrome usually is induced by compression of the extremity by tourniquets, ligatures, cords, or similar equipment. This unconsciously motivated and consciously produced lymphedema is an expression of underlying psychiatric disease. Herein, we present a fluctuating case of SS involving the hand, with periods of severe lymphedema and those of complete amelioration, parallel to stressful and peaceful periods in the patient's personal life. We briefly review the current literature on SS to increase awareness among dermatologists, and we present information on the clinical portrait, diagnosis, and appropriate management of this peculiar and underreported disorder.

Histopathological characteristics and CD163 immunostaining pattern in fibrous papule of the face.

BACKGROUND: Signs of inflammation including epidermal interface changes, spongiosis, and dermal inflammation as well as pagetoid dyskeratosis are rarely described in fibrous papule (FP). We aimed to describe the inflammatory parameters, the rate of pagetoid dyskeratosis, along with CD163 immunohistochemical staining as an adjunctive diagnostic tool in FP. METHODS: Histopathology samples of all biopsy-proven FP cases were retrieved from archives and investigated for inflammatory parameters, presence of pagetoid dyskeratosis, as well as CD163, CD10, and CD34 immunostaining pattern of dermal spindle/stellate or multinucleate cells (graded from 0 to 4). RESULTS: Thirty-two cases of FP were identified. A high rate of inflammatory parameters including interface changes (20/32), spongiosis (31/32), and dermal lymphocytic inflammation (31/32) were detected. Pagetoid dyskeratosis was identified in eight out of 32 cases (25%). A grade 4 staining revealing a strong dendritic pattern was confirmed in all FP cases with CD163 immunohistochemistry including atypical variants such as granular FP, compared with CD10 (11/32) and CD34 (3/32). CONCLUSION: The dendritic cellular proliferation in FP may represent an inflammatory response to various stimuli; pagetoid dyskeratosis is a relatively common and underrecognized epidermal feature and CD163 immunostaining may be used as an adjunctive diagnostic tool in unusual histopathological subtypes.

Papular elastorrhexis: clinical perspectives.

First described by Bordas in 1987, papular elastorrhexis (PE) is a rare elastic fiber disorder of the skin characterized by multiple, discrete, asymptomatic, firm, nonfollicular, monomorphous, 1-5 mm, circumscribed, hypopigmented, oval to round papules, symmetrically distributed on the chest, abdomen, back, shoulders, arms, and thighs. The onset of the condition is usually in the first or second decade of life. PE appears to be an exceedingly rare entity, with 33 cases reported in the literature until now. However, the disorder might be underestimated probably because of its subtlety, asymptomatic course, and benign nature of clinical alterations, which can easily be confused with other dermatoses such as acne scars. Clinical and histopathological differential diagnosis of PE is broad and includes papular acne scars, eruptive collagenoma, disseminated lenticular dermatofibrosis (as a component of Buschke-Ollendorff syndrome), white fibrous papulosis of the neck, pseudoxanthoma elasticum, pseudoxanthoma elasticum-like papillary dermal elastolysis, middermal elastolysis, and perifollicular elastolysis. Treatment of PE is a matter of debate and no reliable curative option exists.

Immunoreactivity of Wilms tumor 1 (WT1) as an additional evidence supporting hemangiomatous rather than inflammatory origin in the etiopathogenesis of angiolymphoid hyperplasia with eosinophilia.

BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular proliferative disorder mainly located in the periauricular region. The etiopathogenesis of ALHE is unknown, and it is still controversial as to whether the entity represents a benign vascular neoplasm or an inflammatory process. AIM: Recently, the intracytoplasmic staining pattern of Wilms tumor 1 (WT1) on immunohistochemistry has highlighted true vascular neoplasms, such as microvenular hemangioma, tufted angioma, and spindle cell hemangioma, which has made it helpful to distinguish ALHE from vascular malformations, as there is a negative staining pattern in the other entities. We aimed to investigate the immunoreactivity of ALHE specimens for WT1 as well as glucose transporter protein 1 (GLUT1) immunohistochemistry, an important and sensitive marker for the diagnosis of infantile hemangioma, which recently has been described to label other hemangiomas, such as verrucous hemangioma. MATERIAL AND METHODS: Clinical data and histopathological specimens from patients diagnosed with ALHE were reviewed, and immunohistochemical staining and microscopic analysis for WT-1 and GLUT1 were performed. RESULTS: Intracytoplasmic endothelial staining of WT1 was detected in 19 of 20 ALHE specimens. GLUT1 was not detected in any ALHE specimen. CONCLUSIONS: We conclude that ALHE may represent a true hemangioma (i.e., benign vascular neoplasia) characterized by an eosinophil- and lymphocyte-rich inflammatory component as opposed to the reactive inflammatory dermatosis with a positive intracytoplasmic staining pattern for WT1. As far as we are aware, WT1 staining for ALHE has not been described to date.

Meyerson Phenomenon as a Component of Melanoma in situ.

Meyerson phenomenon (MP) is characterized by a symmetrical area of erythema and scales encircling a central lesion, which is most commonly a banal melanocytic nevus. Herein, we describe an unusual case with MP representing an eczematized response to a melanoma in situ and review the literature covering this entity. A 56-year-old man presented with a 6-month history of a pruritic, pigmented lesion on the trunk. The patient had no other significant medical history and no notable family history of similar lesions. Physical examination revealed an irregular, hyperpigmented plaque, 1 cm in diameter, with a surrounding halo of erythematous, scaly areas on the right abdominal region (Figure 1, a). On dermatoscopical examination, an irregular, broadened pigment network, radial streaming, and a focal blue-white veil, encircled by a homogenous, erythematous zone was observed (Figure 1, b). Based on clinical and dermatoscopical findings, a presumptive diagnosis of MP occurring on an early melanoma was made and the lesion was excised with a 5 mm safety margin. Histopathological examination of the excised material revealed a central intraepidermal atypical, confluent melanocytic proliferation with angular, hyperkeratotic, and irregular nuclei and a prominent fixation artifact around the cells (Figure 1, c). Human melanoma black (HMB-45) immunostaining highlighted the confluence of the neoplastic melanocytic proliferation. Lymphohistiocytic infiltration with melanophages was also identified in the upper dermis. An interesting feature was the presence of subacute spongiotic dermatitis around the melanocytic lesions (i.e. parakeratosis, serum/crusting, spongiosis, lymphocyte exocytosis, and acanthosis). Immunohistochemical staining with the Langerhans cell marker, CD1a, revealed an increased cell population in the perilesional, erythematous halo (Figure 1, d). A diagnosis of MP existing on melanoma in situ was established with clinical and histopathological findings. No recurrence of the eczematized components or melanocytic lesions was identified despite 1-year follow-up. Also called halo dermatitis and halo eczema, MP presents as an eczematized, perilesional plaque around various lesions, mainly of banal melanocytic nevi (1). Occurrence of halo dermatitis around a melanocytic nevus was first described by Meyerson in 1971. Other cutaneous disorders with MP include those of dysplastic nevus, melanoma, seborrheic keratosis, stucco keratosis, nevus sebaceous, dermatofibroma, vascular malformations, nevus flammeus, molluscum contagiosum, basal cell carcinoma, squamous cell carcinoma, and keloid formation (2-7). History of atopy and atopic dermatitis is observed in a subset of patients, which was absent in our case. Rarely, patients with Behçet's disease, severe sunburn, and cessation of interferon therapy have also been associated with this entity. MP is described to be more frequent in young males and has a tendency to occur in summer. As far as we are aware, there are only two cases of melanoma with features of MP in the literature. Rodin et al. presented a case report showing features of MP around a melanoma in situ arising on a dysplastic nevus (8). By way of comparison, a pre-existing precursor dysplastic nevus was not identified in our case. Dermatoscopic features including scar-like depigmentation and negative pigment network also differed from our case which featured a broadened pigment network, radial streaming, and blue-white veil. The other case report was of a 50-year-old man, presenting with an atypical melanocytic lesion several years in duration showing an erythematous halo (9). Histopathological examination was consistent with a Clark level 2 superficial spreading melanoma, which was cured with excision with no recurrence despite long-term follow-up. As far as we are aware, our case report is the first to describe de novo melanoma in situ without dermal invasion or a precursor dysplastic nevus. The etiopathology of MP is unclear; however it is considered to be immune-mediated. Up-regulation of intercellular adhesion molecule-1 on keratinocytes and dermal endothelial cell surfaces has been shown, suggesting the involvement of adhesion molecules in pathogenesis (10). We hypothesize that increased Langerhans cell population, as observed in our case, results in a delayed immune response reminiscent of an eczematous process in MP. Excision of the central lesion has been reported to precipitate the resolution of dermatitis, as in our case, in which recurrence of the erythematous, scaly eruption was not observed after removal of the central lesion despite a 1-year follow-up period. Some authors recommend pre-treatment of the lesion with topical corticosteroids to suppress the eczematous process in the adjacent skin. Coexistence of MP around a melanocytic nevus (Meyerson nevus) with halo nevus and progression of Meyerson nevus to halo nevus has also been reported. We suggest that melanoma may occur as a component of MP, and careful dermatoscopic examination is essential to differentiate between pigmented lesions with a perilesional erythematous halo.

Diagnostic utility of Ki-67 and Cyclin D1 immunostaining in differentiation of psoriasis vs. other psoriasiform dermatitis.

BACKGROUND: Differentiation of psoriasis from non-psoriasis psoriasiform dermatitis (NPPD) may be difficult for dermatopathologists, as lack of distinctive histopathological features in a subset of cases may cause confusion in diagnosis. OBJECTIVE: As the prototype of psoriasiform dermatitis, psoriasis is a hyperproliferative skin disorder with increased epidermal turnover compared with NPPD, we investigated the role of proliferation markers, Ki-67 and Cyclin D1 as diagnostic tools to differentiate psoriasis from other psoriasiform dermatitis. METHODS: Histopathological specimens of psoriasis (n = 35) and NPPD (n = 36, 14 pityriasis rubra pilaris, 12 pityriasis rosea and 10 lichen simplex) cases were reviewed and immunohistochemically stained for Ki-67 and Cyclin D1. Ki-67 and Cyclin D1 positive cells were counted for suprabasal, and total epidermal immunostaining per mm(2). RESULTS: Suprabasal and total epidermal cell counts for Ki-67 were found to be significantly higher in the psoriasis group compared with the NPPD group (p < 0.05). An important and interesting feature was the presence of a cut-off value for the suprabasal/total epidermal cell count ratio of 75% for Ki-67 immunostaining, which was higher in all patients having psoriasis (range, 77.1% - 92.4%) and lower in all NPPD cases (range, 21.0% - 73.3%). However, suprabasal Cyclin D1 cell counts were higher in the psoriasis group compared with the NPPD group (p < 0.05), total epidermal Cyclin D1 cell counts were not statistically significant in either group (p = 0.167), and a cut-off value for suprabasal/total epidermal cell count ratio to distinguish these two entities was not detected using this immunostain. CONCLUSIONS: We suggest that Ki-67 is a more sensitive marker than Cyclin D1 in terms of having a cutoff value of 75% for the suprabasal/total epidermal immunoreactive cell count ratio, which we believe could be useful for dermatopathologists in differentiating psoriasis from other psoriasiform dermatitis.