Linear syringocystadenoma papilliferum on the retroauricular area associated with nevus sebaceus.

Syringocystadenoma papilliferum is a rare cutaneous adnexal tumor that usually arises in the head and neck region. It may develop de novo or within a nevus sebaceus. Linear syringocystadenoma papilliferum is an uncommon variant of this benign tumor. We report a child with linear retroauricular distribution of syringocystadenoma papilliferum. A background nevus sebaceus was shown histologically. Total excision was curative with no recurrence. An association between the linear variant of syringocystadenoma papilliferum and nevus sebaceus has not been reported previously.

Multiple cutaneous neuromas and macular amyloidosis associated with medullary thyroid carcinoma.

Multiple cutaneous neuromas are rarely seen in dermatology practice. We report a case of multiple cutaneous neuromas, macular amyloidosis (MA), and medullary thyroid carcinoma (MTC) and discuss the interrelationship of the associated conditions. Multiple endocrine neoplasia 2 (MEN 2) is a hereditary syndrome that comprises MEN 2A, MEN 2B, and familial MTC. Germline mutations in the RET proto-oncogene is the underlying cause of the syndrome. MEN 2A and MEN 2B show some common endocrine manifestations including MTC and pheochromocytoma. There are reports of families with MA and MEN 2A. Multiple mucosal neuromas occur in 100% of patients with MEN 2B syndrome. Cutaneous neuromas are infrequently reported in MEN 2B syndrome. Our patient was a heterozygote carrier of GAG-->GAC mutation (Glu 768 Asp) in exon 13, codon 768 of the RET proto-oncogene. We speculate that our patient may represent an unusual presentation of MEN 2B or an overlap of MEN 2A and MEN 2B syndromes or a sporadic MTC case with unusual associations.

Acquired digital fibrokeratoma.

Acquired digital fibrokeratoma (ADF) is an uncommon benign tumor of fibrous tissue that presents as smooth, dome-shaped or fingerlike, flesh-colored papules that are mostly located on the distal extremities. As it can easily be misdiagnosed for other common benign lesions such as viral warts, which usually are not subject to routine histopathologic examination, it might be underreported. We report 13 patients with ADF diagnosed in our clinic in the past 4 1/2 years.

Childhood pemphigus vulgaris: five cases in 16 years.

Pemphigus vulgaris (PV) usually occurs in adults. There are only a few reports of large PV series concerning childhood cases. We report here five cases of PV in patients younger than 16 years. They were analyzed among 169 PV cases out of a total of 192 pemphigus patients diagnosed between 1988-2004. The ratio of childhood cases was 2.9% in our large PV series. This relatively high ratio of childhood patients suggests that PV should not be neglected in the differential diagnosis of bullous lesions in childhood. Four of the five cases were followed up between 2-4 years and all of these four cases showed at least one relapse. PV also seems to show a relapsing course in the pediatric age group like in adults.

Effective treatment with hydroxychloroquine in a case of annular elastolytic giant cell granuloma.

Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous and elastolytic skin disease of unknown pathogenesis. Therapy for AEGCG is controversial. The data about the effectiveness of chloroquine in the treatment of AEGCG are also variable. Here, we report a case of AEGCG with significant improvement after a total treatment period of 22 weeks with hydroxychloroquine. Although a possibility of spontaneous remission cannot be ruled out, we think that chloroquine can be considered as an effective treatment of this chronic disorder.

Lipoid proteinosis: a case series from Istanbul.

BACKGROUND: Lipoid proteinosis (LP) is a very rare genodermatosis. The literature on LP consists of case reports only. As we have observed 14 LP patients belonging to nine different families in the last 15 years in our practice, we decided to review all reported Turkish LP patients in this 15-year period, and noted 37 diagnosed cases. The reasons for this relatively large number of cases, the clinical features of the patients, and the associations of LP with other clinical conditions are described in this article. METHODS: Fourteen LP patients followed in our university clinic in Istanbul were scrutinized with regard to their demographic and clinical features. Diagnoses were established using clinical features, with histopathologic confirmation in 13 cases. RESULTS: All but one of the patients had a history of consanguinity, or at least a marriage of parents from the same village. Typical cutaneous signs of LP and hoarseness of the voice were observed in all patients. Two patients of the same pedigree had insulin-dependent diabetes mellitus (IDDM), two patients from two different pedigrees had short stature, one patient had multinodular toxic goiter, and one patient had celiac disease. CONCLUSION: LP is not rare in Turkey as consanguineous marriage is still a social problem, especially in some rural areas. The disease is not limited to a particular geographic region in Turkey. Short stature was observed in two cases from two different families, an association not reported previously; the association of LP with IDDM in one pedigree was thought to be coincidental.

[Tumors associated with nevus sebaceous]. / Mit Naevus sebaceus assoziierte Tumoren.

BACKGROUND: Nevus sebaceus (NS) is a congenital hamartomatous lesion, usually involving the scalp or the face. Various benign and malignant neoplasms can develop in association with NS, the most common being trichoblastoma, syringocystadenoma papilliferum, viral warts and basal cell carcinoma (BCC). PATIENTS AND METHODS: We retrospectively examined the clinical and histopathological records of 15 NS cases with an associated tumor which were diagnosed between 1996 and 2004. RESULTS: All cases were adults and 3 of them had more than one tumor. Six cases had BCC, which is a higher incidence of malignant tumor association with NS, than that reported in last years. Trichoblastoma was observed in 3 cases and was the most common benign tumor in our series. Filiform, hyperkeratotic, pigmented, pedunculated and/or eroded papulonodular changes were major clinical signs of secondary tumors on NS in our series. CONCLUSION: Clinical features are not sufficient enough to make an exact diagnosis of benign or malignant secondary tumors. Though prophylactic excision for NS is done less in last years, especially adult patients should closely be followed for any new changes evoking tumor development on this congenital lesion.