Meltem Türkmen

Phakomatosis pigmentovascularis type IIb associated with Klippel-Trénaunay syndrome and congenital triangular alopecia.

kyphosis, and restriction of movement. Two had bilateral Achilles tendon rupture. Radiography of the dorsolumbar spine revealed typical intervertebral disc calcification. Darkening of urine on standing overnight was seen in each case. Palmar plantar pigmentation as a diagnostic clue to alkaptonuric ochronosis was first reported by Cherian. Similar case reports have since emerged from the Indian subcontinent. This is the first series highlighting the unique pattern of pigment deposition that was present in a consistently similar manner resembling marginal keratoderma in all five patients. DCPH and AKE are distinct variants of marginal acrokeratoderma, which occur as a result of a combination of long-term trauma and pressure. They are characterized by symmetric, acral, hyperkeratotic lesions,most frequentlyon thehands, andareassociated with abnormalities of the dermal connective tissue. DCPH typically consists of linear, firm plaques formed by the fusion of disk-like areas along the lines of transgredience of the fingers. AKE is a genodermatosis that involves the epidermis and manifests as horny papules favoring the thenar and hypothenar eminences. Histopathologically, DCPH is characterized by collagen degeneration, whereas epidermal changes with normal collagen and distorted elastic fibers distinguishAKE.The skin lesions inourpatients had combined features of both conditions but showed a greater resemblance toDCPHhistopathologically. In previous published reports of acral pigmentation in alkaptonuria, the histopathology has been similar to our cases, with changes mainly in the dermal collagen while the epidermis was relatively uninvolved. Pigment deposition in alkaptonuria is observed to favor stress-bearing sites, such as the dorsolumbar vertebrae and large joints. The occurrence of lesions on the palms and soles in our patients exposed to manual labor also seemed to be influenced by stress and frictional factors. Ochronotic pigment has been found to have a particular affinity for collagen fibers, which could explain its deposition in the pattern of DCPH. A positive family history has not been documented in previous reports. In conclusion, acral lesions in alkaptonuric ochronosis may resemble DCPH. This presentation should be considered as a cutaneous marker for alkaptonuria, in addition to the typical sites of involvement.

Facial angiofibromas of tuberous sclerosis: successful treatment with podophyllin

Editor Tuberous sclerosis is a rare dominantly inherited neurocutaneous syndrome characterized by hamartomas in many organs particularly skin, brain, kidney, heart, and eyes.1 Facial angiofibromas are one of the major cutaneous manifestations of tuberous sclerosis occurring in about 75% of the patients.2 They often appear at puberty and then remain unchanged. Clinically, they appear as 0.1–1.0 mm in diameter, reddish, dome-shaped smooth telangiectatic papules on the face. They may lead to cosmetic and psychological problems. Here, we report a patient who had multiple facial angiofibromas treated successfully with podophyllin 25% solution. A 27-year-old woman presented with a 15-year history of skin-coloured papules on the face. Dermatological examination revealed numerous pink to red, domed-shaped papules 0.5–2.0 mm in diameter symmetrically distributed on the cheeks, nasolabial folds, chin, forehead, and perioral area (Fig. 1). She also had multiple ash leaf macules on her trunk, Koenen tumours on the toes, and a shagreen patch in the lumbosacral region. Complete blood count, erythrocyte sedimentation rate, liver, renal function tests, chest X-ray, abdominal ultrasonography, thorax, and abdominal computerized tomography were normal. Asymptomatic cortical and subcortical tubers on bilateral frontal, occipital, and parietal regions detected at magnetic resonance imaging of brain. Podophyllin (25%) extract in benzoin tincture was applied only to the surface of facial angiofibromas by means of haematocrit pipet. She thoroughly washed her face with soap 4 hours after application. We observed flared erythema on the papules followed by superficial necrosis that healed within a few days. The application was repeated once monthly for 3 months while the papules were flattened (Fig. 2). There were no side effects except slight burning sensation. All treatment was well tolerated by the patient, and there was no recurrence at the end of a year of follow-up. The treatment of facial angiofibromas has been a problem because of the multiplicity of the lesions and the presence of the lesions at anatomically difficult sites such as nasolabial folds, chin, upper lip, nose, and periorbital areas. Several treatment modalities causing non-selective destruction of tissue such as curettage,3 dermabrasion,4 chemical peeling,3 cryotherapy,5 electrosurgery,6 excision,4 and lasers7–9 have been used for the treatment with

Exanthematous drug eruption due to valsartan

Objective: Valsartan is an angiotensin II receptor blocker (ARB) used for treatment of hypertension. The well-known adverse effects of valsartan are dizziness, headache and cough. Valsartan-related cutaneous side effects have been reported previously in a limited number of case reports. Materials and methods: A 47-year-old man admitted with diffuse, itchy erythematous maculopapular eruption all over the body. He has been taking 160 mg valsartan daily for 10 days before onset of the eruption. On the third day of valsartan therapy, erythema had appeared over the face and spread throughout the whole body within a week. Histopathologic examination of the lesions showed lymphocyte exocytosis, spongiosis, necrotic keratinocytes in the epidermis, and mixed inflammatory cell infiltrates including perivascular eosinophils in the dermis. The patient was diagnosed as drug reaction due to valsartan with historical, clinical and histopathologic features. Discussion and conclusion: Most common antihypertensive agents including diuretics, beta blockers, calcium-channel blockers, angiotensin-converting enzyme inhibitors have many cutaneous side effects. However, there are a few reports about the cutaneous side effects of ARBs. Physicians should be aware of the cutaneous side effects of this commonly used agent and valsartan should be considered as a triggering factor of an exanthematous drug reactions.

Folliculotropic T-cell lymphocytosis as an associated finding in mycosis fungoides.

© 2008 The Authors JEADV 2009, 23, 570–620 Journal compilation

A distinct expression profile separates Turkish and Australian melanocytic naevi

logical, pathological, and cytogenetic analysis of three cases. Skeletal Radiol. 2012; 41; 237–244. 13. Aouad R, Matar N, Sader-Ghorra C, Haddad A. Pathology quiz case 1. Oncocytic lipoadenoma of the parotid gland. Arch. Otolaryngol. Head Neck Surg. 2008; 134; 446–448. 14. Chahwala Q, Siddaraju N, Singh N, Goneppanavar M, Basu D. Fine needle aspiration cytology of oncocytic lipoadenoma of the parotid gland: report of a rare case. Acta Cytol. 2009; 53; 437–439. 15. Ilie M, Hofman V, Pedeutour F, Attias R, Santini J, Hofman P. Oncocytic lipoadenoma of the parotid gland: immunohistochemical and cytogenetic analysis. Pathol. Res. Pract. 2010; 206; 66–72. 16. Klieb HB, Perez-Ordonez B. Oncocytic lipoadenoma of the parotid gland with sebaceous differentiation. Study of its keratin profile. Virchows Arch. 2006; 449; 722–725. 17. McNeil ML, Bullock MJ, Trites JR, Hart RD, Taylor SM. Oncocytic lipoadenoma of the parotid gland with sebaceous differentiation in a 73-year-old male. J. Otolaryngol. Head Neck Surg. 2010; 39; E48–E50. 18. Mitsimponas KT, Agaimy A, Schlittenbauer T, Nkenke E, Neukam FW. Oncocytic lipoadenoma of the parotid gland: a report of a new case and review of the literature. Int. J. Clin. Exp. Pathol. 2012; 5; 1000–1006. 19. Pusiol T, Franceschetti I, Scialpi M, Piscioli I. Oncocytic sialolipoma of the submandibular gland with sebaceous differentiation: a new pathological entity. Indian J. Pathol. Microbiol. 2009; 52; 379–382. 20. Tokyol C, Dilek FH, Aktepe F, Aycicek A, Altuntas A. Oncocytic lipoadenoma of the parotid gland: a case report with fine needle aspiration cytology findings. Kulak Burun Bogaz Ihtis. Derg. 2010; 20; 146–149. 21. Chi CL, Kuo TT, Lee LY. Oncocytic lipoadenoma: a rare case of parotid gland tumor and review of the literature. J. Pathol. Transl. Med. 2015; 49; 144–147. 22. Lau SK, Thompson LD. Oncocytic lipoadenoma of the salivary gland: a clinicopathologic analysis of 7 cases and review of the literature. Head Neck Pathol. 2015; 9; 39–46. 23. Parmar HV. Oncocytic lipoadenoma of submandibular gland: a case report. J. Clin. Diagn. Res. 2015; 9; ED05–ED06. 24. Fletcher C, Bridge J, Hogendoorn P, Mertens F eds World Health Organization Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon: IARC Press, 2013.

A case of drug hypersensitivity due to azathioprine

© 2008 The Authors JEADV 2009, 23 , 702–738 Journal compilation