Olympia Rudra

Child and mother with unusual facies: Trichorhinophalangeal syndrome type I revisited

Sir, A 10‐year‐old girl presented to us with fine, sparse scalp hair since early childhood. On examination, frontotemporal alopecia was observed with fine, sparse hair on other part of the scalp. Eyebrows were sparse on the lateral side in comparison to medial aspect. Bulbous nose, everted ears, and large lips with mild receding chin were noticeable [Figure 1a]. Her mother also had similar clinical presentation [Figure 1b]. Clinical examination of both revealed angulation of the digits of hands and feet with brachydactyly and koilonychia [Figure 2]. Developmental milestone, intelligence, height, and weight of the patient were normal. Radiologic examination of the limbs showed angulation of the phalanges [Figure 3]. Systemic examination and routine hematological and urinalysis were noncontributory. A pedigree chart of their family showed similar history was present in her maternal grandfather and great grandfather. However, her maternal uncle and aunt did not have any of these clinical findings [Figure 4]. Based on the history and clinical findings, we diagnosed it as a case of trichorhinophalangeal syndrome (TRPS) type I.

Epidermoid cyst containing molluscum bodies in a 12-year-old Indian boy: A chance phenomenon or inducer?

Indian Journal of Paediatric Dermatology | Volume 18 | Issue 2 | April-June 2017 140 or subcutaneous nodule with a surface punctum through which cheesy material can be expressed. It commonly affects adult men or women. Chest, back, head and neck region are the most common sites of involvement.[2] On the other hand, MC is a viral infection caused by a poxvirus, subgenus molluscipox virus (MCV), which comprises four genetically distinct viral subtypes. It is characterized by shiny, pearly white, hemispherical, umbilicated papule over skin and mucosa. MC is more common among children. It is common in the head and neck region, followed by flexural areas and the genitalia.[5] Extensive lesions can occur in immunocompromised patients, especially in HIV, patients receiving immunosuppressive therapy, and in atopics.[5,6] However, our patient was neither atopic nor had history of use of any topical or systemic medication. After entering through the basal epidermis, the MCV starts cellular proliferation and forms lobulated epidermal growths which further compress the papillae and, thus, fibrous septae develop between the lobules. Ultimately, cells at the center of the lesion get destroyed and appear as large hyaline bodies containing eosinophilic, cytoplasmic masses of virus material.[5] Spontaneous regression usually occurs within 1 year due to cell‐mediated immune response. Antibody to the virus is present in almost 60% of patients, which suggest the role of humoral immunity Sir, A 12‐year‐old otherwise healthy boy presented to us with an untreated asymptomatic raised lesion on his shoulder since last 2 years. Initially, the lesion was small which gradually increased to attain the present size. There was no history of atopy. No similar history was present in the family. Cutaneous examination revealed a soft to firm, nontender sessile nodule of 1.5 cm diameter on his shoulder [Figure 1a]. The lesion was free from underlying structures. There was no erythema, scaling, or erosion on the surface of the lesion. However, a punctum was seen over the nodule. Rest of the cutaneous or systemic examination was noncontributory. Routine blood examinations were within normal limits. Screening for HIV was negative. Histopathological examination (HPE) of the excised nodule, stained by hematoxylin and eosin [Figure 1b‐d], revealed multiple big to small cystic structures in the dermis. The overlying epidermis was normal with irregular proliferation of rete pegs. The cysts were lined by single‐layered granular cells of surface epithelium and contained keratin debris, hair shaft remnants, and eosinophilic globular structures suggestive of molluscum bodies. Molluscum bodies were also seen in the walls of the lining epithelium. There was a sparse inflammatory cell infiltrate consisting of eosinophils, lymphocytes, and histiocytes surrounding the cystic structures. Based on the clinical with HPE findings, a diagnosis of epidermoid cyst (EC) containing molluscum bodies was made.

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 …

Benign lymphangioendothelioma: A report of a rare vascular hamartoma in a young Indian child

Sir, An 8-year-old boy presented to us with a raised asymptomatic cutaneous lesion over his left leg for the past one and half years. It started as a bluish, slightly elevated spot which gradually increased to attain its present size and shape. There was no preceding history of trauma or use of any medication. There was no overt history of external bleeding or oozing. Cutaneous examination revealed an ill-defined, oblong-shaped plaque (4 cm × 3 cm size) with a bluish hue situated over the anterior aspect of the left thigh. It was studded with a few dark-blue and reddish papules. Focal crusting over the surface was also noted [Figure 1]. The lesion was non-tender and soft in consistency, and there was no lesional thrill or bruit. No other cutaneous lesion was present elsewhere in his body. Hair, nail, and mucosae were normal. General and systemic examinations were noncontributory. Routine blood investigations were within normal limits. Assuming the case to be a vascular malformation, the lesion was completely excised a year back, and regular follow-up showed no recurrence until date. Histopathological examination (hematoxylin and eosin stain) [Figures 2-4] of the excised specimen revealed compact hyperkeratosis with irregular acanthosis. Dilated thin-walled spaces lined by intermittent flat endothelial cells resembling lymphatic channels were seen in the upper and mid-dermis. Similar, but more slit-like, structures were seen in the deeper dermis along with dilated blood vessels containing red blood cells.

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)

Generalized bullous drug eruption to faropenem - hitherto unreported

References 1. Daoud MS, Pittelkow MR. Lichen planus. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick’s Dermatology in General Medicine. 8th ed. New York: McGraw-Hill; 2012. p. 296-312. 2. Weedon D. Weedon’s Skin Pathology. 3rd ed. London: Churchill Livingstone Elsevier; 2010. 3. Hanau D, Sengel D. Perforating lichen planus. J Cutan Pathol 1984;11:176-8. 4. Gutte R, Khopkar U. Perforating lichen planus. Indian J Dermatol Venereol Leprol 2011;77:515-7. 5. Gutte R, Khopkar U. Predominant palmoplantar lichen planus: A diagnostic challenge. Indian J Dermatol 2014;59:343-7.

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.

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.