Correspondence Address:
Dibyendu Bikash Bhanja
Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, Kolkata, West Bengal
India

Full Text



Sir,

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare benign cutaneous hamartoma with two clinical forms - classical and solitary type.[1] Involvement of aesthetically concerned sites (eyelid, nose, clitoris) has been infrequently documented in the literature. Herein we report a case of solitary NLCS over the nipple in a primigravid woman.

A 29-year-old pregnant woman (26 weeks of gestation; P1G0) presented with an asymptomatic slowly progressive growth over the right nipple for the preceding 4 months. There was no history of any preceding trauma, discharge, bleeding, underlying breast lump or any symptom suggestive of neurological deficit, skeletal or ocular abnormality. Past medical history and family history were non-contributory. Examination revealed a solitary skin-coloured to hyperpigmented, soft, non-tender, pedunculated nodule (5 × 4 cm) with wrinkled and lobulated surface arising from the lateral aspect of the right nipple [Figure 1].{Figure 1}

A bunch of hair had been tightly tethered around the base of the lesion by the patient herself. Other mucocutaneous sites and systemic examination were unremarkable. Routine laboratory investigation including serology for human immune deficiency virus, viral hepatitis and syphilis was normal. Dermoscopy (DermLite DL4, 10x, contact/polarized mode) showed a cerebriform surface with honeycomb-like pigment network (yellow circle) and yellowish white structureless areas (red circle) [Figure 2].{Figure 2}

Excision biopsy followed by histopathological examination revealed orthokeratotic hyperkeratosis and papillomatosis along with lobules of mature adipocytes in the upper and mid-dermis, splitting the collagen bundles [Figure 3]. Based on clinical, corroborative dermoscopic and histopathological findings, a diagnosis of nevus lipomatosus cutaneous superficialis was established. No recurrence was noted over the six months follow-up period. The remaining period of gestation and puerperium was uneventful.{Figure 3}

NLCS represents a benign nevoid anomaly attributed to hamartomatous mature adipocytes collection in the papillary and reticular dermis. The precise etiopathogenesis remains poorly understood; adipose metaplasia during the course of degenerative changes in dermal connective tissue, development of mature adipocytes by mesenchymal perivascular cells and genetic factors have been implicated in the pathogenesis.[2] Classical NLCS usually occurs at birth or during first three decades of life. The solitary form usually occurs after the age of 20 years, presenting as a single dome-shaped or sessile papule or nodule without any site preference. Although solitary NLCS has no site predilection, less frequently it involves unusual site like scalp, eyelid, clitoris, or calves.[3] Involvement of the nipple, as seen in our case, has not been reported before.

Physiological skin changes in pregnancy include pigmentary alterations, dysregulation of connective tissue, vascular system, endocrine abnormalities, as well as changes in glandular function where eccrine and sebaceous function increases while apocrine function decreases.[4] Some undocumented hormonal influence(s) may explain the aberrant adipocyte proliferation.

A presumptive diagnosis of NLCS can be made through use of a dermoscope. The typical dermatoscopic description include cerebriform surface formed by gyri (ridges) and sulci (fissures), honeycomb-like pigment network, rimming of the cerebriform surface with “ground glass” white film, yellowish structureless areas with or without perifollicular distribution and comedo-like openings.[5]

Systemic abnormalities and malignant alterations have not been associated with solitary NLCS. Prompt surgical excision is necessary when it involves cosmetically sensitive areas to eliminate extensive reconstruction of the defect and to reduce postoperative scar formation.

In conclusion, this documentation adds to the sparse literature on NLCS. To the best of our knowledge, NLCS with onset during pregnancy and involving the nipple is being reported for the first time.

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

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