Bathing Trunk Research Articles

A BEST classification system of large to giant congenital melanocytic nevi based on expert consensus and distribution characteristics.

Large to giant congenital melanocytic nevi (LGCMN) significantly decrease patients' quality of life, but the inaccuracy of current classification system makes their clinical management challenging. To improve and extend the existing LGCMN 6B/7B classification systems by developing a novel LGCMN classification system based on a new phenotypic approach to clinical tool development. Three hundred and sixty-one LGCMN cases were categorized into four subtypes based on anatomic site: bonce (25.48%), extremity (17.73%), shawl (19.67%) and trunks (37.12%) LGCMN. A 'BEST' classification system of LGCMN was established and validated by a support vector machine classifier combined with the 7B system. The most common LGCMN distributions were on bonce and trunks (bathing trunk), whereas breast/belly and body LGCMN were exceptionally rare. Sexual dimorphism characterized distribution, with females showing a wider range of lesions in the genital area. Nearly half of the patients with bathing trunk LGCMN exhibited a butterfly-like distribution. Approximately half of the LGCMN with chest involvement did not have nipple-areola complex involvement. Abdomen, back and buttock involvement was associated with the presence of satellite nevi (r = 0.558), and back and buttock involvement was associated with the presence of nodules (r = 0.364). The effective quantification of a standardized anatomical site provides data support for the accuracy of the 6B/7B classification systems. The simplified BEST classification system can help establish a LGCMN clinical database for exploration of LGCMN aetiology, disease management and prognosis prediction.

Mycosis Fungoides Palmaris et Plantaris Progressing to Complete Early-Stage Disease Improved with Phototherapy

Mycosis fungoides is the most common form of cutaneous T cell lymphoma. In this article, the authors describe a case of a 53-year-old female who presented with multiple pruritic, slightly erythematous and hyperpigmented, variably shaped macules, and patches and plaques, with scaling on the bilateral hands and feet. The lesions resembled various benign inflammatory dermatoses, such as contact dermatitis and superficial dermatophytosis, and were treated as such until hyperpigmented, variably shaped macules and patches appeared on the legs and with a bathing trunk distribution. Histopathologic and immunohistochemical findings were consistent with early mycosis fungoides. The patient has improved with full body narrowband ultraviolet B phototherapy, combined with a psoralen ultraviolet A soak for the hands and feet. This case highlights the importance for physicians to recognise that mycosis fungoides may occur with different presentations, and may not present in its classic form. The patient’s quality of life may be improved with early diagnosis.

Congenital Melanocytic Nevus: An Unusual Presentation

The overgrowth of melanocytes produces congenital melanocytic nevus. They are commonly present since birth, and the common terms used to describe them are called 'bathing trunk,' 'coat-sleeve,' or 'stocking naevi,' and they are usually found over thighs and back. The giant congenital nevus of size larger than twenty centimeters is highly pigmented and usually hairy. It is more commonly found in females compared to males. It is well recognized that the lesion's displeasing nature might have psychological and social ramifications, negatively affecting the patient's self-esteem, particularly females. Although congenital melanocytic nevus is mostly clinically diagnosed, the histological investigation is required to confirm the diagnosis and rule out malignant changes. The likelihood of transforming the giant nevus to malignant melanoma accounts for approximately five percent; fifty percent of melanomas develop before age two, and eighty percent develop before age seven. The risk of transformation to malignant melanoma is early in giant melanocytic nevus cases and adolescence in small and medium-sized nevi cases. Other complications like transformation to neurocutaneous melanosis consisting of the spread of melanoma to the central nervous system and leptomeninges are also dreadful. Hence, early removal is recommended. Here, we have reported a rare case of a six-year-old patient with a medium-sized nevus on their face with hairy tufts in between. On biopsy, the diagnosis was made as a junctional nevus. After which, the patient was managed with subsequent lesion excision and split skin grafting with an uneventful postoperative period. Our case report aims to report a rare case of congenital melanocytic nevus and to determine the importance of timely diagnosis and intervention. Keywords: , , , ,

Rapid Melanoma Death of an Adult Male with Congenital Bathing Trunk Nevus despite Initiation of Combination Immunotherapy

Dear Editors: Giant congenital melanocytic naevus (GCMN)-associated melanoma in adults is very rare [...]

Clone and Contradistinction—Mycosis Fungoides

Mycosis fungoides is a peripheral T cell lymphoma engendered from mature, post-thymic T lymphocytes representing with cutaneous patches, plaques, tumours and erythroderma. Mycosis fungoides demonstrates a ‘bathing trunk’ distribution wherein gluteal region, trunk and proximal limbs appear incriminated. Mycosis fungoides enunciates enhanced expression of Th2 gene along with Th2 associated cytokine production and activation of nuclear factor kappa B (NFkB). Lesions depict a band-like infiltrate of atypical lymphoid cells within papillary dermis with focal fibroplasia and Pautrier’s micro-abscesses. Neoplastic cells display mature T cell phenotype and are immune reactive to CD45RO+, TCRβ+, CD2+, CD3+, CD4+, CD5+or CD7+. Photodynamic therapy, retinoids, targeted therapy, biologic therapy, radiation, chemotherapy or allogenic haematopoietic stem cell transplant may be adopted to treat mycosis fungoides.

Bathing trunk naevus

Giant congenital melanocytic naevus is a naevus measuring more than 20 cm in adulthood. Its incidence is 1:20,000 and is more common in girls. Bathing trunk naevus, a specific form of giant congenital melanocytic naevus is even rarer with incidence of 1:500,000. Giant congenital melanocytic naevus usually presents as a brown lesion, with flat or mamillated surface, well-defined borders and hypertrichosis. Dermoscopic examination of melanocytic naevi shows brown-blackish background, globules, reticular network and vascular structures. Although giant congenital melanocytic naevus is often diagnosed clinically, dermoscopy aids in detecting early changes of complications such as malignant melanoma. We report a case of bathing trunk naevus with multiple satellite lesions in a brown child with dermoscopic findings.

Neurocutaneous melanoma in association with giant congenital melanocytic nevi in a child (Touraine syndrome) – A dermatoradiological correlation

Touraine syndrome or neurocutaneous melanosis/melanoma is a rare melanophakomatosis characterized by extensive/multiple congenital melanocytic nevi associated with cerebral/meningeal melanosis or melanoma. We report a 12-year-old boy with a congenital giant melanocytic nevus on the bathing trunk distribution with scattered lesions on the face, neck, and legs. MRI brain revealed a melanoma in the right amygdala.

Distribution Patterns (7B Rule) and Characteristics of Large Congenital Melanocytic Nevi: A Retrospective Cohort Study in China.

Large congenital melanocytic nevus has a high risk of malignancy. However, few studies have summarized its characteristics, treatments, outcomes and malignancy incidence in Chinese patients. This paper reviews a retrospective cohort study evaluating 1,171 patients from Shanghai Ninth People's Hospital between 1 January 1989 and 31 August 2019 using electronic medical records and phone calls to collect clinical and pathological data in which 133 patients were diagnosed with a large congenital melanocytic nevus. Three patients relapsed, and none developed melanoma among the qualified patients. Besides, a new “7B” rule for distribution patterns of large congenital melanocytic nevi was proposed, including bonce, bolero, back, bathing trunk, breast/belly, body extremity, and body. The most common distribution pattern of large congenital melanocytic nevi was bonce, and all blue nevi distributed as bonce. Statistical analysis showed a significant difference (P = 0.0249) in the “7B” patterns between the melanocytic nevus and the neuronevus. In conclusion, the malignancy rate of large congenital melanocytic nevi is much lower in China than in other regions and people of other races. The pathology of large congenital melanocytic nevus may decide its “7B” distribution pattern.

Congenital Bathing Trunk Nevus with Meningomyelocele.

Congenital Bathing Trunk Nevus with Meningomyelocele.

Patterns of distribution of giant congenital melanocytic nevi (GCMN): The 6B rule

Garment-related terms have been used to describe the pattern of distribution of giant congenital melanocytic nevi (GCMN). We sought to describe patterns of distribution of GCMN and propose a classification scheme. Photographic records of patients with GCMN from the Hospital Clinic of Barcelona were analyzed and a classification based on observed GCMN distribution patterns was created. The classification was independently applied by 8 observers to cases found in the literature. The interobserver agreement was assessed. Among 22 patients we observed 6 repeatable patterns of distribution of GCMN, which we termed the "6B": bolero (involving the upper aspect of the back, including the neck), back (on the back, without involvement of the buttocks or shoulders), bathing trunk (involving the genital region and buttocks), breast/belly (isolated to the chest or abdomen without involvement of bolero or bathing trunk distributions), body extremity (isolated to extremity), and body (both bolero and bathing trunk involvement). Our literature search found 113 cases of GCMN, which we were able to classify into 1 of the 6B patterns with an overall kappa of 0.89. Some patterns occur infrequently with a dearth of images available for analysis. The anatomic distribution of GCMN occurs in 6 recognizable and repeatable patterns.

Neurocutaneous melanosis presented with giant congenital melanocytic nevi and primary intracranial melanoma

Neurocutaneous melanosis is a rare neurocutaneous syndrome defi ned by the presence of multiple and /or giant congenital cutaneous nevi and melanocytic deposits in the central nervous system with the infi ltration of leptomeninges. The major medical concern with giant congenital cutaneous nevi is high risk of developing cutaneous melanoma, leptomeningeal melanoma, and neurocutaneous melanosis. Neurological symptoms usually develop before 2 years of age, but rare in the second or third decades of life. A 24-year-old-man with giant hairy, pigmented nevus on his extremities, back, “bathing trunk” variety presented with onset of neurological symptoms only in second decade of life. Magnetic resonance imaging of brain revealed a large expansive lesion in the left parieto-occipital lobe with high intensity on T1- sequence, and was heterogeneous on T2-sequence with iso- and hyper intense areas. Post-surgical pathological diagnosis revealed characteristics of malignant melanoma. Our patient represents a rare association between neurocutaneous melanosis with giant congenital cutaneous nevi and development of primary intracranial melanoma with neurological symptoms appearing in adult life. The onset of neurological symptoms only in third decade of life in our patient illustrates the importance of recognizing the likelihood of neurocutaneous melanosis in children with giant congenital nevus with or without neurological symptoms.Journal of Kathmandu Medical College, Vol. 4(1) 2015, 29-33

Age related changes in brain MR appearance in the course of neurocutaneous melanosis

BackgroundPatients with giant congenital melanocytic nevi (GCMN) have increased risk of neurocutaneous melanosis (NCM). Brain MRI is mandatory in GCMN. ObjectivesTo present the age related changes in brain MRI in children with NCM during several-year follow-up. MethodsIn 7 NCM children following data were analyzed: main nevus localization, surgical treatment phase, histology of excised nevi, time of NCM diagnosis, number of MRI studies, neurological symptoms, changes of melanin deposits in brain during follow-up. ResultsFour children had bathing trunk nevus, two main nevus on back, one on head. Surgical excision is finished in 3 patients, 4 are in the course of multistage surgery. In one melanoma arising in GCMN was diagnosed. Six patients are neurologically healthy, one suffers from seizures. Age of patients at first MRI ranged from 12days to 7 years. All patients underwent more than one MRI. Follow-up period ranges from 2.5 to 8.5 years. Most of T1-hyperintense foci in brains regressed with time. In one case new lesion appeared, in one more lesions were visible on second MRI. ConclusionsIn all patients with NCM on subsequent MRI image changes were found with respect to original location and size of melanin deposits in CNS. Changes of NCM appearance on MRI with advancing age of patients reflect natural history of this disorder. Theory of gradual disappearance of intracerebral melanin deposits with age seems to be more likely than theory of their worse visualization on MRI with time.

Lipomatous hairy bathing trunk nevus with multiple satellite nevi

Lipomatous hairy bathing trunk nevus with multiple satellite nevi

Mexican Patient with Refractory Mycosis Fungoides Successfully Treated with Pralatrexate at Low Doses: Case Report

IntroductionPrimary cutaneous lymphomas (PCL) are defined as non-Hodgkin lymphomas. They are found in the skin without an extra primary cutaneous location at the moment of diagnosis1. Mycosis fungoides (MF) and Sézary Syndrome (SS) are the most common subtypes of cutaneous T-cell lymphomas, MF represents less than 1% of the total number of non-Hodgkin lymphomas1,2,3; however, it is the most common cutaneous lymphoma2. It usually has an indolent course and good prognosis when identified in its early stages3. Therefore, treatment should be reaching the optimal benefit with minimizing the toxicity as much as possible. The therapeutic approaches for patients with PCL depend on the disease stage; skin-directed therapies are generally used, with systemic treatments reserved for patients with relapsed or more extensive disease. However durable remissions off-therapy are uncommon in MF/SS and, historically, more aggressive first-line approaches have not resulted in improved outcomes, but are associated with increased toxicity3. The moderate response rates and frequent lack of durable responses to current therapies for PCL underline the need for additional effective and tolerable treatments. This case represent the first Mexican patient treated successfully with low doses of pralatrexate as monotherapy, achieving almost complete response.CaseThe patient is a 40 years old male with history of skin tumoral lesions that previously was diagnosed as primary anaplastic lymphoma, however, in a skin biopsy and immunohistochemistry it was identified Mycosis fungoides.The classical type of MF has 4 stages: patch, plaque, tumoral and erythroderma or Sézary syndrome. Of them, the most common is the patch/plaque MF, which presents with extremely pruritic, erythematous macules and patches with telangiectasias and atrophy in the "bathing trunk" distribution2. In this case initially the patient presented with plaque and tumoral lesions in 45% of body surface area. Mostly in thorax, face and arms, highly symptomatic and aesthetically unpleasant.He was initially managed with PUVA and topical corticosteroids, followed by CHOP-R with bad response to both treatments. The treatment was changed to bortezomib and interferon with limited response. Then it was decided to change treatment to pralatrexate at low doses (according to Mexican guidelines of NHL treatment) 15 mg/m2 as monotherapy in cycles of 10 weeks (6 weeks on treatment and 4 off treatment). After the initial dosage of pralatrexate the patient manifested decrease of the symptoms, by the end of the first cycle the improvement was observed in approximately 20% of body surface area. The adverse reactions presented were fatigue, paresthesia at the site of administration and nausea, all of them were mild and not required additional treatment. Currently the patient has completed 3 cycles of pralatrexate showing improvement of cutaneous involvement in 35% of body surface area.DiscussionNone of the current therapeutic options available for patients with PCL are curative. Many patients are treated sequentially and suffer from frequent morbidity because of the burden of their disease and the cumulative toxicity of therapy3.Published papers have identified an effective PCL dosing regimen for pralatrexate with a safety profile that is acceptable for continuous long-term use4. In this case this regimen was adapted to the patient and it represents the first experience in Mexico using pralatrexate for Mycosis fungoides. The patient has shown favorable and rapid response to therapy, diminishing symptoms and disease activity. Pralatrexate appears to be a promising agent for the treatment of patients with refractory MF. 1.Yamashita T, Abbade LP, Marques ME, Marques SA. Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update. An Bras Dermatol 2012; 87: 817-282.Prince HM, Whittaker S, Hoppe RT. How I treat mycosis fungoides and Sézary syndrome. Blood 2009; 114: 4337-533.Al Hothali GI. Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach. Int J Health Sci (Qassim) 2013; 7: 220-394.Horwitz SM, Kim YH, Foss F, Zain JM, Myskowski PL, Lechowicz MJ, et al. Identification of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T-cell lymphoma. Blood 2012; 119: 4115-22 DisclosuresLopez:Mundipharma: Other: Subsidies for the costs of travel to the ASH annual meeting. Off Label Use: Pralatrexate is approved at a dose of 30mg/m2. In this case I administer a low dose (15 mg/m2) to improve tolerance and maintain in a long term the treatment..

Woman with Giant Lesion in the Trunk

Introduction The congenital melanocytic nevus is a type of melanocytic nevus found in infants at birth. This type of birthmark occurs in an estimated 1% of infants worldwide. It may be divided into the Small, medium-sized congenital nevocytic nevus and giant congenital melanocytic nevus also known as “Bathing trunk nevus,”. We report a case in a 42-year-old woman.

Giant congenital melanocytic nevi: selected aspects of diagnostics and treatment.

BackgroundTreatment of giant melanocytic nevi (GMN) remains a multidisciplinary challenge. We present analysis of diagnostics, treatment, and follow-up in children with GMN to establish obligatory procedures in these patients.Material/MethodsIn 24 children with GMN, we analyzed: localization, main nevus diameter, satellite nevi, brain MRI, catecholamines concentrations in 24-h urine collection, surgery stages number, and histological examinations. The t test was used to compare catecholamines concentrations in patient subgroups.ResultsNine children had “bathing trunk” nevus, 7 had main nevus on the back, 6 on head/neck, and 2 on neck/shoulder and neck/thorax. Brain MRI revealed neurocutaneous melanosis (NCM) in 7/24 children (29.2%), symptomatic in 1. Among urine catecholamines levels from 20 patients (33 samples), dopamine concentration was elevated in 28/33, noradrenaline in 15, adrenaline in 11, and vanillylmandelic acid in 4. In 6 NCM children, all catecholamines concentrations were higher than in patients without NCM (statistically insignificant). In all patients, histological examination of excised nevi revealed compound nevus, with neurofibromatic component in 15 and melanoma in 2. They remain without recurrence/metastases at 8- and 3-year-follow-up. There were 4/7 NCM patients with more than 1 follow-up MRI; in 1 a new melanin deposit was found and in 3 there was no progression.ConclusionsEarly excision with histological examination speeds the diagnosis of melanoma. Brain MRI is necessary to confirm/rule-out NCM. High urine dopamine concentration in GMN children, especially with NCM, is an unpublished finding that can indicate patients with more serious neurological disease. Treatment of GMN children should be tailored individually for each case with respect to all medical/psychological aspects.

Neurocutaneous Melanosis: An Illustrative Patient

We present a newborn with an unremarkable birth history who presented with a giant congenital melanocytic nevus with a “bathing trunk” distribution, accompanied by multiple satellite nevi (Fig 1). Cranial magnetic resonance imaging revealed multiple hyperintense lesions of the brain parenchyma and leptomeninges (Fig 2), confirming the diagnosis of neurocutaneous melanosis. At 13 months, he developed a partial seizure with secondary generalization. He had been asymptomatic until then. Antiepileptic treatment with valproic acid was started. Subsequently he has had occasional seizures that have required antiepileptic readjustment but has otherwise been well. Currently he is 3 years of age and has been seizure-free for 18 months with only mild developmental delay and no apparent progression of his neurocutaneous melanosis. Discussion Congenital melanocytic nevi are proliferations of benign melanocytes that arise during embryogenesis. Neurocutaneous melanosis is a complication of congenital melanocytic nevi and consists of proliferation of melanocytes in the central nervous system and leptomeninges as well as in the skin. It occurs in 2.5-45% of patients with congenital melanocytic nevi, 1 and the risk factors include large congenital melanocytic nevus, multiple satellite nevi, and more than two medium-sized congenital melanocytic nevi. 2-4 Neurocutaneous melanosis can be either symptomatic (3-10% of infants and children with congenital melanocytic nevi 2 ) or asymptomatic. Symptoms usually present in the first 2 years of life as developmental delay and/or seizures, 2 and the prognosis of symptomatic individuals is poor. Neurocutaneous melanosis is best

A rare case of bullous mastocytosis in a child associated with bathing trunk nevus

A rare case of bullous mastocytosis in a child associated with bathing trunk nevus

Congenital giant melanocytic nevi – tip of the iceberg

Congenital Giant Melanocytic Nevus (CGMN), pigmented lesion present since birth, occurs in 1 % of infants worldwide. Fifteen percent of CGMN are localized in the head and neck region and it can also have a bathing trunk distribution. It grows proportionally to the size of the body as the child matures and grows with variation in colour and surface texture. A 29 years old female presented to the Gynecology Out Patient Department for infertility. She also had multiple large nevi of varying sizes present since birth. The lesion was distributed all over the body. She also complained of sudden appearance of swellings at the back which were later excised and the histopathological examination showed the presence of neural nevus involving the dermis and subcutaneous tissue. This case is being reported for its rarity, the higher risk for melanoma transformation, its association with meningeal melanosis and few benign / malignant tumors.

Giant bathing trunk nevus in a child: Case report with review of literature

Giant pigmented nevi are congenital melanocytic nevi that are of great cosmetic distress for the patient and cause an equal psychological stress to the parents. Here we report a case of a 4 year old female child who presented to us with a large black patch over her back extending upto upper half of her buttocks with multiple satellite lesions all over the body, since her birth. Their management is a challenge for the treating dermatologist owing to their large size and a propensity to recur. There have been reports of malignant change in upto 2% to 15% of the cases. However, there have been recent advances in the field with many new therapeutic modalities available.