Megha Agarwal

Becker's nevus syndrome: a report of a rare disease with unusual associations

Becker’s nevus is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis. Becker’s nevus syndrome (BNS) is a phenotype characterized by the basic presence of Becker’s nevus with ipsilateral hypoplasia of the breast or other skin, and skeletal and/or muscular disorders. This syndrome generally originates at birth, intensifies significantly in adolescence, and is one of the syndromes that comprise epidermal nevus syndrome. We describe a case of BNS with a few unique and rare associations.

Perforating lichen planus in an adolescent boy: A rare phenomenon

variant of this disease with features of both LP and transepidermal elimination in histopathology. A very few cases of perforating LP have been reported till date in the literature. A 14-year-old Indian boy presented with gradually progressive pruritic lesions over both lower legs for 6 months. On examination, we found multiple, hyperpigmented, keratotic papules and plaques over the anterior, posterior, and lateral sides of distal parts of both lower extremities [Figure 1a and b]. The mucosae, nail, hair, and systemic examinations were noncontributory. There was no history of any drug intake before the eruption. The patient also had no history of jaundice in the past. A provisional diagnosis of lichenoid dermatosis was considered. A 4-mm punch biopsy was performed including the keratotic central part of a lesion. On histopathological examination, we found hyperkeratosis, focal hypergranulosis, irregular acanthosis, basal cell degeneration, Civatte bodies, and dense band-like lymphocytic infiltrate mixed with histiocytes in the upper dermis with features of transepidermal elimination [Figure 2a and b]. The wide perforating channel was filled with dense lymphohistiocytic infiltrate [Figure 3a and b]. On the basis of these clinical and histopathological findings, the diagnosis of perforating LP was made. LP is an immune-mediated disorder classically presenting as faintly erythematous to violaceous, polygonal, flat-topped papules usually distributed symmetrically and bilaterally over the extremities. Many variations in the clinical presentations according to the morphology, configuration, or distribution have also been described. The classical epidermal changes of LP include hyperkeratosis, wedge-shaped hypergranulosis, and irregular elongation of rete ridges in sawtooth pattern. There is basal cell damage, and multiple apoptotic cells (colloid-hyaline bodies or Civatte bodies) are seen in the dermoepidermal junction. Eosinophilic colloid bodies are found in the papillary dermis. There is a band-like dense lymphocytic infiltrate mixed with histiocytes in the papillary dermis. [2] Perforating LP is a rare variant of LP which clinically presents as keratotic papules and plaques. On histopathology, there is transepidermal elimination with other features of LP. There are very few cases of perforating LP reported in the literature. Hanau and Sengel [3] reported a case in a 52-year-old woman in 1984. Histopathology of that case showed typical features of LP with an area of perforation of epidermis with a rectilinear channel containing hyaline bodies, inflammatory cells, melanophages, and fibrillar material. [4] described a case of perforating LP in a 38-year-old man with histological features of LP 3. Tsuji T, Sawada H. Eccrine angiomatous …

Linear atrophoderma of moulin over face: An exceedingly rare entity

Sir, A 16-year-old girl presented to us with asymptomatic hyperpigmented lesions over the left side of her chin since the past 6 months. According to the patient, it started as a small black discoloration of the skin which gradually increased in size. There was no history of preceding trauma, redness or tightness of the skin, associated systemic complaints, or family history of similar illness. Cutaneous examination revealed three broad unilateral linear hyperpigmented atrophic lesions, with depressed margins along Blaschko’s lines without any sign of inflammation or induration [Figure 1a and b]. The surface of the atrophic lesions was wrinkled. A 4 mm punch biopsy was taken from the margin of the lesion. Histopathologic examination (HPE) with hematoxylin and eosin staining showed epidermal atrophy along with dense melanin deposition along the basal layer with apparently normal subcutaneous tissue [Figure 2]. Sparse perivascular and periappendageal lymphocytic infiltrate with slight thickening of collagen bundles was present in the dermis. There was no evidence of sclerosis or atrophy of the appendages [Figure 3]. The difference with the normal epidermis could be seen in the HPE [Figure 4]. Verhoeff–van Gieson stain showed normal elastic tissue [Figure 5]. On the basis of the Figure 2: Epidermal atrophy along with dense melanin deposition along the basal layer with apparently normal sub cutaneous tissue (H and E, ×40)

Polymorphic keratotic nodules in an immunocompetent man

A 25-year-old male labourer presented to our institution with multiple, asymptomatic, keratotic bumps over his face, right arm, and trunk. He reported that they had started developing 6 months previously; initially a red swelling appeared over his face and subsequently, three cutaneous lesions appeared over his abdomen, chest, and right arm. There was no family history for such lesions and he did not have a history of high risk sexual behaviour. A solitary, erythematous nodule (3 cm × 3 cm) with a verrucous surface had developed on his face (fi gure A). He had a skin-coloured keratotic plaque (4 cm × 4 cm) situated over the right side of his abdomen. Another two erythematous keratotic plaques (each 1 cm × 1 cm) were situated over his chest and arm. The lesions were non-tender and free from underlying structures. He had no lesions on other areas of his body including the nasal and oral cavities, genitalia, and the perianal area. Examination of the patient’s organ systems did not reveal anything unusual. Results of routine laboratory investigations, chest radiography, and abdominal ultrasonography were normal. ELISA for HIV infection was non-reactive. The lesions were excised by radiofrequency surgery in phases. Histopathological examination of all the excised biopsy specimens revealed multiple thick-walled sporangia with numerous spores and chronic infl ammatory infi ltrate (fi gure B, C). On the basis of the clinicopathological features, a diagnosis of primary cutaneous rhinosporidiosis was made. The lesions completely healed within 3 weeks with mild residual scarring. The patient was then prescribed dapsone gel (5%) locally twice daily for 3 months to prevent relapse. The patient was followed up for 12 months after surgery without any sign of recurrence. Rhinosporidiosis is a chronic granulomatous, infective disease, mainly caused by Rhinosporidium seeberi. Although it is endemic in Sri Lanka, India, and South America, cases have been reported globally. It is transmitted by direct contact with spores through dust, infected clothing, fi ngers, swimming in stagnant water, and autoinoculation. Although nasal mucosa is the most commonly aff ected site, other mucosal surfaces and rarely other parts of the body such as skin, viscera, and brain can be involved. Defi nitive diagnosis can be made by histopathological examination. Surgical excision or electrodessication, or both, are the most common methods of treatment of cutaneous rhinosporidiosis.

Suppurative boggy swelling over scalp.

An 8-year-old boy came to us with a painful, purulent swelling on his scalp for the preceding 3 weeks. Before presenting to us, the patient was treated outside with co-amoxyclav for 7 days without any improvement. Examination revealed an erythematous, 3 × 3 cm, slightly tender, boggy swelling with focal hair loss over the scalp. It was studded with multiple bright yellow pustules (Fig.1). The hairs over the plaque were dull, matted and easily pluckable. There was no lymphadenopathy or any other skin lesion elsewhere in his body. Systemic examination was normal. W hat

Subacute nodular skin lesions.

1 Left facial nerve. An ‘upper motor neuron’ lesion, implying damage to the fibres between the cerebral cortex and the facial nerve nucleus in the pons, would lead to paralysis of the lower half of the face on the contralateral side. Unilateral complete facial weakness involving the forehead, eyelids and lower face implies damage at the level of the facial nerve nucleus or the nerve itself (‘lower motor neuron’). While the congenital absence of the facial nerve nucleus (partial Moebius syndrome) would have a static clinical course (with no improvement), an acquired brainstem pathology is unlikely to cause an isolated facial nuclear lesion. With structural lesions in the brainstem (e.g. stroke), neonates would present with severe neurological manifestations including hemiplegia, apnea and feeding difficulties, in addition to facial weakness. Given the absence of other neurological signs and the gradual recovery in the immediate post‐natal period, the patient’s clinical presentation is highly consistent with a lesion of the left facial nerve. 2 No further investigations are necessary at this point. Neonatal facial neuropraxia is an infrequent cranial neuropathy with an incidence of 0.8–7.5 cases/1000 births. Risk factors include birth weight ≥3500 grammes, primiparity and forceps delivery. Neuroimaging has limited utility in a neonate with an isolated facial neuropraxia and should be reserved for patients with a high clinical suspicion for a pontine lesion or a syndromic presentation. Another condition that has been rarely associated with neonatal facial neuropathy related to forceps delivery is hereditary neuropathy with liability to pressure palsies (HNPP), which is transmitted in an autosomal dominant manner. A family history of recurrent compressive neuropathies (not present in our patient) should prompt consideration of HNPP. Other clues may be obtained by examination of the parents which may reveal presence of pes cavus and/or absent ankle reflexes in either parent. When clinical suspicion exists, diagnosis of HNPP can be confirmed by nerve conduction studies and genetic evaluation. 3 Supportive therapy, good prognosis. Acquired facial neuropraxia in neonates is associated with an excellent outcome. More than 90% infants who develop facial neuropraxia related to forceps delivery show spontaneous recovery within 4months. Given the excellent prognosis, interventions such as corticosteroids and surgery are not recommended.

Erythema necroticans exhibiting transepidermal migration of lepra bacilli as a probable source of infection to other family members

infection to other family members A 40-year-old Indian man presented with recurrent crops of painful, ulcerative skin lesions mainly over the extremities and trunk for the last six months. It was associated with high-grade, intermittent fever, myalgia, and polyarthralgia. There were multiple, tender, ulcerated nodules and indurated plaques, many of them exhibiting seropurulent exudation. Nodular infiltrations over both pinna were also present (Fig. 1). Both the hands were mildly swollen and tender. The sensation was slightly diminished bilaterally and symmetrically in his hands and feet. Both of his ulnar nerves and median nerves were grossly thickened and tender. Apart from fever, systemic examination was non-contributory. Ocular and other mucosal examination was normal. Gram staining and bacterial culture of the swabs taken from the exudative ulcerative lesions were non-contributory. Ziehl–Neelsen stain of smear prepared from the seropurulent fluid revealed presence of acid-fast bacilli. A slit skin smear also showed acid-fast bacilli (bacteriological index 5+). A complete hemogram showed neutrophilia with raised erythrocyte sedimentation rate. C reactive protein level was also raised. Biochemical panels and routine urine examination was normal. Incisional biopsies were taken both from a plaque without any ulceration and also from the indurated areas of an ulcerated plaque. Histopathological examination of both the biopsy specimens (Figs. 2 and 3) showed similar features of flat epidermis, upper dermal edema with scattered cellular infiltrate comprising of polymorphs and lymphocytes, perineural and peri-appendageal granulomatous inflammation (comprising of lymphocytes, epithelioid cells, neutrophils, and foam cells), and lobular panniculitis (comprising of mononuclear cells, histiocytes, neutrophils, and eosinophils), and features of small vessel vasculitis in the dermis and subcutaneous tissue. Fite stain (Fig. 4) demonstrated clumps of predominantly solid staining and a few fragmented and granular acid-fast bacilli in the subcutaneous tissue, dermis, and epidermis. One noteworthy finding was the presence of numerous lepra bacilli (both intracellular and extracellular) in the malpighian layer and stratum corneum suggestive of their transepidermal migration. Based on the clinical and histopathological features, a diagnosis of erythema necroticans was made. We started multidrug therapy for leprosy as recommended by World Health Organization along with a course of prednisolone, started as 40 mg/day and gradually tapered over a period of three months. Within a couple of months, the skin lesions almost completely healed. After six months of the initial presentation of the patient to us, his elder son and mother presented with hypopigmented anesthetic patches, which were further confirmed clinically and histopathologically as borderline tuberculoid leprosy. Erythema nodosum leprosum, the main presenting feature of a type-2 reaction in leprosy, may complicate the course of borderline lepromatous or lepromatous leprosy. Severe erythema nodosum leprosum may become vesicular or bullous and may eventually break down, which is termed as erythema nodosum necroticans or erythema

Spontaneous sublingual hematoma due to warfarin: An emergency presenting to the dermatologist

Sir, A 47-year-old man presented to us with a painless red swelling of his tongue causing difficulty in speech and swallowing for 2 days. He had been on oral warfarin (4 mg/day) for atrial fibrillation for 6 months before this. His international normalized ratio (INR) was monitored weekly with a target of 2–3. There was no history of recent trauma or external bleeding. Physical examination revealed a tense, tender, red submucosal hematoma involving the floor of the mouth and ventral lingual surface bilaterally [Figures 1 and 2]. The tongue was pushed slightly upward and the patient could protrude his tongue only with difficulty. Vital parameters were normal and systemic examination was non-contributory.

Keratotic papules and scars in a young boy

DOI: 10.4103/0019‐5154.190112 A 7-year-old, Indian boy, born out of consanguineous parentage, presented with multiple scars on his face for the past 2 years and a few raised pruritic skin eruptions over different parts of his body for the same duration. His grandfather reportedly had similar kind of lesions. On examination, we found multiple well-defined, shallow, atrophic scars over his face. Some of these scars were arranged in a linear fashion. In addition, multiple skin-colored, discrete keratotic papules with a central adherent keratinous plug were located over the face, dorsum of hands, lower legs, and feet [Figures 1 and 2]. The skin was otherwise normal in appearance. Examination of the nails and mucosae showed no abnormality. Systemic examination was unremarkable. Routine laboratory investigations were within normal limits. We did 4-mm lesional punch biopsies from two representative lesions and histopathological examination was done subsequently.

Dermatoses among children from celebration of “Holi,” the spring festival, in India: A cross-sectional observational study

Background: “Holi” is a spring festival celebrated primarily in the Indian subcontinent and also abroad by expatriate Indians. It is a festival of colors, traditionally celebrated by mutual application of colors in different forms on a particular day of the year. These colors frequently comprise a range of synthetic dyes which have harmful effects on the skin and mucosae. Children take part in this colorful festival with much enthusiasm and vigor, making them prone to develop different “Holi”-related dermatoses. Our objective was to find out the different patterns of “Holi”-related dermatoses in a group of pediatric patients. Methodology: This was a cross-sectional descriptive study carried out over a period of 6 years (2010–2015). Consecutive patients of pediatric age group who attended dermatology outpatient department (OPD) with different dermatoses following application of “Holi” color were included in this study. Results: A total of 63 patients (mean age 11 years; range 1–16 years) were evaluated with a female to male ratio of 1.3:1. Itching is the predominant presenting symptom followed by burning sensation, dryness, scaling, oozing, and loss of hair. Examination revealed that eczematous lesion was the most common (69.8%) reaction pattern followed by xerosis, desquamation, excoriation, erythema, morbilliform eruption, erosion, alopecia, ulceration, acute paronychia, and hyperpigmentation. The face was the most common (76.4%) site of affection. Conclusion: A sizable number of patients of pediatric age group may be affected by “Holi”-related dermatoses necessitating precautionary measures.