Defining the mimics and clinico-histological diagnosis criteria for mycosis fungoides to minimize misdiagnosis

Hyperkeratosis of the nipple and areola is an uncommon dermatosis without well-defined etiology, which occurs mostly in young women and may only be a cosmetic problem. In 1938, Levy-Frankel classified this disease into three variants; type I involves hyperkeratosis with an epidermal nevus, and has rarely been reported. We report a case of a 23-year-old woman with long-term bilateral pigmentation and thickening of the nipples and areolae, accompanied with dark brown, flat lesions with a linear distribution on her left forearm. The verrucous plaques were asymptomatic and could be scratched off by the patient herself. Consequently, she did not seek medical care for more than ten years. The prevalence of this condition is likely underestimated because many affected individuals are not sufficiently motivated to seek medical attention.

Bilateral hyperkeratosis of the nipples and areolae with linear nevus: a rare case report and review of the literature.

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.

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The Dilemma of Coexisting Nevoid Hyperkeratosis of the Nipple and Areola in Mycosis Fungoides: A Report of Three Cases.

A 20-year-old woman was referred to our dermatology outpatient clinic in August 2015 due to the gradual development of multiple, pinhead size papules on both nipples and areolae during the past nine months. The patient was previously healthy, with no personal or familial history of atopic dermatitis or any other skin disease. She reported a three-year history of oral contraceptive use but had not taken any other medications. Physical examination revealed symmetrical moderate verrucous thickening of the areolae as well as black, well-demarcated, 0,5–1 mm papules on the areolae and nipples of both breasts (Figure 1 ). Symptoms such as tenderness to touch, pain, itching or bleeding had not been observed but the lesions had slowly increased in size. No other skin lesions, in particular no patches or plaques, were present. A 4 mm punch biopsy was taken from the left areola. Histopathologic examination showed orthokeratotic hyperkeratosis with follicular plugging as well as slight to moderate spongiosis of the epidermis and a superfi cial lymphocytic infi ltrate. Filiform elongation of the rete ridges and hyperpigmentation of basal keratinocytes as well as exocytosis were noted. In addition, intraepidermal aggregates of lymphocytes resembling Pautrier‘s microabscesses were observed without further single-cell epidermotropism or alignment along the dermoepidermal junction (Figure 2 ). Immunohistochemical staining of lymphocytes revealed expression of CD3 with an elevated CD4/CD8 ratio and partial loss of CD7 expression (Figure 3 ). Further work-up involved PCR-based T-cell receptor (TCR) gamma and beta clonality testing using the BIOMED-2 protocol. Identical TCR gene rearrangement was detected, indicating clonal expansion of lymphocytes. Based on clinical signs and their histopathological correlation, hyperkeratosis of the nipple and areola (HKNA) was diagnosed. However, due to the histological and molecular pathological fi ndings an unusual presentation of mycosis fungoides could not be totally excluded; ultrasound examination of the lymph nodes was therefore undertaken, but this revealed no abnormalities. After topical treatment with adapalene and a cream containing 3 % salicylic acid in petrolatum once a day, the skin lesions resolved completely within four weeks. The results of biannual follow-up visits with full body and lymph node examination have been unremarkable for a period of two and a half years. Hyperkeratosis of the nipple and areola is a rare disorder characterized by hyperpigmented, verrucous or fi lamentary, keratotic thickening of the nipple and/or the areola. Men are affected less frequently than women, who often develop this condition in the second or third decade of life [ 1–3 ] . The frequency of occurrence, especially during puberty and pregnancy, and the incidence of the condition in men who

Hyperkeratosis of the nipple and areola: a histopathologic pitfall.

Breastfeeding has been demonstrated to have many benefits for both mother and child. The diagnosis and management of Candida in the breastfeeding dyad are difficult because diagnosis is most often based on subjective signs and symptoms. Few studies have attempted to confirm the diagnosis with biological tests. A 26-year-old breastfeeding mother presented with stabbing breast pain, accompanied by erythematous, hyperkeratotic areola, and nipples. Histopathological results and direct mycological examination, followed by culture and auxanogram, revealed Candida albicans as an etiological agent of mastitis; the case was managed successfully by antifungal medication. Candida mastitis in lactating women is an under-recognized and under-treated cause of breast pain, hyperkeratosis over areola, and discharge of nipple should be assessed by biological tests. The hope is that with an increased awareness of this infection, more mothers will be appropriately treated and will thus be more likely to continue breastfeeding.

Candida albicans Mastitis in Breastfeeding Woman: An Under Recognized Diagnosis.

Mycosis fungoides is one of the great imitators in dermatology; it can mimic many dermatoses. Nevoid hyperkeratosis of the nipple and areola is a rare idiopathic disease with typical clinical features of verrucous thickening and brownish discoloration of the nipple, areola or both. Here, a 16-year-old male patient with mycosis fungoides mimicking nevoid hyperkeratosis of the nipple and areola has been reported. To our knowledge, this is the first atypical MF patient to have presented with a NHNA-like lesion. Although the clinical appearance of nevoid hyperkeratosis of the nipple and areola is highly characteristic for diagnosis, histopathological examination is recommended, especially in cases with atypical features such as unexpected age, male gender and unilateral location.

Mycosis fungoides mimicking nevoid hyperkeratosis of the nipple and areola in an adolescent.

Prurigo pigmentosa is a distinctive inflammatory dermatosis characterized by recurrent, pruritic erythematous papules and reticulate hyperpigmentation. The eruption typically shows a symmetrical distribution on the trunk and tends to localize mostly on the neck, upper back, clavicular region and chest. The pathogenesis of prurigo pigmentosa is still unknown however it has been suggested that ketosis related to diabetes mellitus, dieting or anorexia nevrosa; friction, sensitivity to sunlight, and contact allergy might be a triggering factor. There are some prurigo pigmentosa cases reported to be associated to contact allergens like para-amino compounds, trichlorophenol, chromium and nickel in the literature. We report a Turkish young female prurigo pigmentosa patient with nickel sensitivity and we discuss the role of contact allergens in the etiology of prurigo pigmentosa.

Prurigo pigmentosa: A Case with Nickel Sensitivity

Robotic-assisted laparoscopic appendicovesicostomy

Objective: While robot-assisted laparoscopic surgery (RALS) is spreading rapidly all over the world, In pediatric surgery,
this spread is slower than in adult surgery for many reasons. In this study, we discuss our initial experiences with
pediatric robotic surgical cases.
Material and Methods: Robotic-assisted endoscopic surgery between November 2017 and April 2022 was
retrospectively reviewed. The medical records of the patients were reviewed retrospectively for some demographical
features, surgical details, and follow-up time.
Results: Of the 48 children, 27 were male and 21 were female. The youngest of the cases was 10 months old,
while the oldest was 17 years old (mean 7.72±5.6, median 7). The shortest follow-up period after surgery was 5
months, while the longest follow-up was 60 months. A total of 63 surgical procedures were performed on 48 pediatric
patients. 17 pyeloplasty (left 10, right 7), 9 Lich Gregoir operations (right 6, left 3), 4 ureteral reimplantation and tapering
procedures due to left ureterovesical junction obstruction (UJO), 3 Hemi nephrectomy, one appendicovesicostomy, One
right ureteral reimplantation, 11 Nissen fundoplication, 10 gastrostomy and one gastrojejunostomy, two ovarian sparing
surgeries for ovarian cyst were performed. One urachal remnant excision, one adrenal mass excision, and one thoracic
outlet mass excision were performed.
Conclusion: This is the first study that includes a wide range of pediatric robotic surgical procedures in our country.
Robotic-assisted laparoscopic procedures in pediatric surgery can be safely performed for many surgical pathologies. We
think at the beginning some simple surgical procedures and then turning to the more complex procedures after at least
15 cases will be more appropriate.

https://dergipark.org.tr/en/download/article-file/2889479

Özlem Ekici

Papers

Mycosis fungoides mimicking nevoid hyperkeratosis of the nipple and areola in an adolescent.

Prurigo pigmentosa: A Case with Nickel Sensitivity

Robotic-assisted laparoscopic appendicovesicostomy

Robotic Assisted Endoscopic Surgery Practices in Pediatric Surgery, Single Center Experience