M. M. S. Nico

Malformations of the index nails

We report a 52‐year‐old woman with micronychia of the index fingers. Radiographic examination revealed a Y‐shaped bifurcation of the distal phalanx of both index fingers. She was diagnosed with congenital onychodysplasia of the index fingers (COIF) or Iso–Kikuchi syndrome. COIF is a rare condition characterized by a variety of nail dysplasia of the index fingers. Five criteria characterize COIF: congenital occurrence, unilateral or bilateral index finger involvement, variability in nail appearance, hereditary involvement and frequently associated bone abnormalities. Micronychia, polyonychia, anonychia, hemionychrogryphosis and malalignment are the observed index finger defects. Most cases have been described in Japan, and to our knowledge, this is the first case of COIF reported in South America.

The lip in lupus erythematosus

Lupus erythematosus (LE) frequently compromises the skin, lips and oral mucosa. There is a large body of medical and dental literature about the cutaneous and mucosal lesions of LE, but very little has been written specifically about labial lupus. The lip has a peculiar anatomical and histological architecture, and LE lesions at this site may have some distinct features. This article reviews the existing data and adds some new concepts to the issue of labial lupus in all of its presentations, comparing such lesions to the well‐established characterization of cutaneous LE (in acute, subacute and chronic lesions), and highlighting some special clinical features that may enable a better diagnosis and differential diagnosis of lip diseases.

Oral ulcers on the vestibular sulci

A 16-year-old white Brazilian man presented with a main complaint of painful oral lesions of 5 months’ duration. He also reported weight loss of 3 kg in the past 3 months and a 1-year history of occasional bilateral hip arthralgia Otherwise he was generally well, and personal and familiar antecedents were unremarkable. Physical examination of the oral cavity revealed two vegetating ulcers orientated along both mandibular vestibular sulci, with symmetrical distribution (Fig. 1a,b). Routine blood investigation detected microcytic anaemia, reduced serum albumin level and elevated C-reactive protein and erythrocyte sedimentation rate.

Divided naevus of the penis: two paediatric cases with dermoscopic findings.

Divided or kissing naevi are located on adjacent parts of the body that are separated during embryogenesis. Divided naevus of the penis (DNP) is exceedingly rare, with < 15 cases reported in the English language literature. Divided penile naevi affect the glans penis and inner foreskin, which are anatomical structures believed to have a common embryological origin. We report the clinical, dermoscopic and histopathological findings of two children with DNP. To our knowledge, this is the first report of patients with DNP seen in South America, and the first in which dermoscopic findings are discussed; these included a large globular pattern in one patient, and homogeneous, streaked and globular patterns in the other.

Violaceous and lichenoid papules on the trunk of a 61‐year‐old woman

A 61-year-old Brazilian woman presented with a 3-year history of pruritic papules. Physical examination revealed numerous flat-topped, infiltrated, violaceous, slightly scaly papules (Fig. 1a,b). Lesions were grouped in clusters on the arms, back, forehead and scalp. Cervical lymphadenopathy was present, and the patient also had bilateral eye hyperaemia. A tuberculin test was negative. Chest radiography showed bilateral hilar lymphadenopathy (Fig. 2a). Ophthalmological examination revealed uveitis in both eyes. Additional laboratory studies included urinalysis, peripheral blood count, blood chemistry and blood calcium level, which were all within normal ranges.

Oral ulcers in an immunosuppressed 5-year-old boy

A 5-year-old white boy presented with a 15-day historyof oral ulcers associated with pain and difficulty ineating. His medical history was significant for hearttransplant 2 years previously, to treat idiopathic myo-cardiopathy. He had been on prednisone, tacrolimusand mycophenolate mofetil for the past 16 months toprevent transplant rejection.Physical examination revealed multiple shallow anddeep ulcerations on the buccal mucosa and dorsum ofthe tongue (Fig. 1a,b), the largest 30 mm in diameter,covered with grey pseudomembranes and surroundedby an erythematous halo. There were no palpablecervical nodes and the rest of the physical examinationwas unremarkable.

Severe and relapsing upper lip enlargement in a 10-year-old boy (Discussion and Diagnosis)

The Case Presentation can be found on page 1758 DISCUSSION Analysis of the patient’s upper lip biopsy revealed the presence of well-defined, non-caseating granulomas, and oedema on the lamina propria. The findings of a granulomatous lip inflammation leading to macrocheilia, associated with plicated tongue, as in the case herein presented, are features suggestive of Melkersson–Rosenthal syndrome (MRS). Even more, the pathological aspect of the lip lesion discarded the possibility of contact dermatitis in this case, so patch test was not considered necessary. This entity is an infrequent idiopathic inflammatory disorder characterized by the classical triad of recurrent or persistent orofacial swelling, plicated tongue and facial nerve palsy (FNP) (1,2). It primarily affects young adults at the end of the second decade of life, but it is an extremely rare disease in childhood with approximately 35 cases reported in the literature (3–5). Manifestations of MRS at infancy, as well as occurrences in adults, are reported to be variable as some present with the full clinical triad of the syndrome, while others are only monoor olygosymptomatic. Cheilitis granulomatosa (also named Miescher’s chronic granulomatous cheilitis) clinically consists of a painless enlargement mainly of the upper lip, with the presence of non-necrotizing granulomas as well as oedema and perivascular lymphocytic infiltration on histopathology (6). It has been generally regarded as an entity within the spectrum of the signs of orofacial granulomatosis, and it may represent a monosympomatic or olygosymptomatic form of MRS. This finding is one of the first and most common manifestations of MRS, occurring in approximately 75% of patients (5). Its development in children is a rarity (4), and its course does not differ from that in adults (4). Plicated tongue is reported to occur only in 30% of MRS in childhood. The presence or absence of this sign is not significant for the establishment of the diagnosis because according to the review of Ziem et al., (4) it is not present in the initial phases of the disease in most children, but it can develop during the posterior course. FNP, of the peripheral type, occurs in about 30% of the MRS cases. It may be unior bilateral, complete or incomplete and frequently has a recurrent course (4,7). Facial palsy may precede the attacks of oedema by several months or years or starts simultaneously, but it more commonly develops later. The incidence and course of childhood FNP in MRS does not differ from adults. Although it constitutes the most recognized neurological sign of MRS, its occurrence is not obligatory, as in the case of our patient (7). As incomplete expression of MRS is common, full confirmation of the diagnosis requires clinicopathological correlation. Common microscopic findings include oedema, non-caseating granulomas, lymphangiectasia, perivascular mononuclear infiltration of variable intensity and fibrosis (2,8,9). In the case examined herein, the classical aspect of non-caseating granuloma was observed. In some areas of the specimens analysed, a marked oedema and lymphangiectasia were also evident. A detailed medical history is also important in the establishment of MRS diagnosis, as cheilitis granulomatosa is a finding occasionally associated with tuberculosis, leprosy and mainly with sarcoidosis and Crohn’s disease (10). The possibility of sarcoidosis and Crohn’s disease was considered highly unlikely in our patient as no gastrointestinal or respiratory complaints were reported and medical history was uneventful. Fungal and Acta Paediatrica ISSN 0803–5253

An ulcerated plaque on the hard palate

A 37-year-old Brazilian man presented with a 1-year history of a palatal lesion that bled occasionally. He reported having been previously treated with several intralesional corticosteroid injections. Physical examination revealed an irregular ulcerated plaque extending from the midline to the palatal gingiva on the right side of the hard palate. The lesion was friable and appeared to be accompanied by bone resorption on palpation. Exposure of teeth roots was also seen (Fig. 1a). Panoramic and occlusal radiography of the oral cavity showed bone resorption of the hard palate. Computerized tomography revealed only lymphoid hypertrophy of rhynopharynx and tonsils. Additional laboratory studies included urinalysis, peripheral blood count and blood chemistry, all within normal ranges.

A destructive ulcer on the lower lip

A 47-year-old white woman presented with a 1-year history of recurrent and nonhealing ulcer on the lower lip. She reported previous treatment with benzylpenicillin injections, with no effect. The rest of her medical history was noncontributory. Physical examination revealed an extensive and destructive ulcer with irregular, indurated and keratotic raised margins on the left side of the lower lip vermilion. The ulcer was associated with considerable loss of tissue (Fig. 1). There was no regional lymphadenopathy. Laboratory studies included urinalysis, blood count and blood chemistry, the results of which were all within the normal range.

Severe and relapsing upper lip enlargement in a 10‐year‐old boy (Case Presentation)

The Discussion and Diagnosis can be found on page 1906. CASE A 10-year-old Afro-Brazilian boy complained of a painless and persistent upper lip enlargement of 3 years of evolution (Fig. 1) that had a relapsing course. Personal and familial history was otherwise unremarkable. Oral examination revealed a diffuse and rubbery enlargement of the upper lip and plicated tongue. Remaining physical examination was normal. A lip biopsy was performed, and histopathological findings are depicted in Figure 2. Laboratorial examinations (erythrocyte and white cell count, sedimentation rate, antinuclear antibodies, urianalysis and allergic patch tests) revealed no abnormalities.