José M. de Moragas

ACUTE FEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME)

Background. Sweets syndrome is well recognized and not infrequently diagnosed in Spain; however, the range of clinical and pathologic expression may not have been fully realized.

Cutaneous reactions secondary to heparin injections

Ten to 20 days after starting prophylactic doses of heparin in six patients, large, indurated, erythematous plaques appeared at the sites of the subcutaneous injections. Pruritus, local tenderness, and burning sensation or pain were present. No skin necrosis developed. Heparin was discontinued and topical corticosteroids were applied to the involved areas. Five to 9 days later the lesions had resolved completely. The reaction seen in the six patients is similar to the early erythematous phase of the so-called heparin necrosis. There is a spectrum of cutaneous changes ranging from mild erythematous pruritic areas to large symptomatic plaques and to heparin necrosis, following subcutaneous injections of heparin.

Immunofluorescence of epithelial skin tumors. I. Patterns of intercellular substance.

Epidermal tumors were studied for production of intercellular substance (ICS) present in normal epidermis. Antibody to ICS found in pemphigus serum was used and detected by immunofluorescent technique. ICS was absent in squamous cell carcinoma, and its absence was related to degree of neoplasia. Individual cell keratinization was related to ICS. Basal cell carcinomas had no ICS, similar to sweat gland tumors. Seborrheic keratosis and keratoacanthoma produced normal amounts of ICS. It appears possible to classify tumors by this technique and probably relate intercellular substance to tumor behavior.

Brooke-spiegler Syndrome Variant Segregation of Tumor Types with Mixed Differentiation in Two Generations

Brooke-Spiegler syndrome (BSS) is an autosomal dominantly inherited disease characterized by the development of multiple trichoepitheliomas and cylindromas. Other lesions have been reported to occur in patients with BSS, including parotid basal cell adenomas, milia, organoid nevi, basal cell carcinomas, and spiradenomas. Spiradenomas and cylindromas have so many features in common that they have been regarded as polar extremes belonging to a spectrum of cutaneous adnexal neoplasms. We report on a 61-year-old woman with multiple spiradenomas on the scalp and periauricular areas and her 28-year-old daughter, with multiple facial trichoepitheliomas. Occasional features of pilar and ductal differentiation were found in tissue specimens of tumors from the mother and daughter, respectively. Co-existence of multiple spiradenomas and trichoepitheliomas and segregation of the predominant type of tumor in different members of a family with BSS have been reported occasionally, but never, to our knowledge, in combination. The presence of mixed differentiation in tumor specimens from both patients provides additional evidence in support of the folliculosebaceous apocrine unit (FSAU) hypothesis. Mutations in genes regulating proliferation and differentiation of putative stem cells of the FSAU would give rise to different combinations of adnexal skin tumors as well as to other neoplasms.

Cutaneous angiomas in POEMS syndrome

POEMS syndrome is an unusual plasma cell dyscrasia with multisystemic manifestations featuring polyneuropathy, organomegaly, endocrinopathy, M protein and constant skin involvement. We have seen a 58-year-old white man presenting with generalized eruptive angiomas as a rare skin manifestation of this syndrome. We review the literature and discuss the pathogenesis of vascular proliferation in this unusual entity.

Immunofluorescence of epithelial skin tumors. II. Basement membrane

Antibodies to basement membrane in the sera of patients with bullous pemphigoid were used to study the basement membrane in epithelial tumors. Monospecific fluorescein conjugate of IgG was used to detect the antibody fixation. Squamous cell carcinoma of the skin was notable for absence of basement membrane related to anaplasia. Squamous cell carcinoma of the lip and vulva had well‐preserved basement membrane despite deep invasion of the subcutaneous tissue. In verruca, basement membrane surrounded individual cells of the germinative layer. Basement membrane was distinctly less pronounced in basal cell carcinoma. Keratoacanthoma and seborrheic keratosis have normal basement membrane. Normal epidermal differentiation appears related to basement membrane formation. Anaplasia, viral change as in verruca, and basal cell proliferation are respectively related to absence, increase, and defective basement membrane production.

Median raphe cyst of the penis with ciliated cells.

Cystic lesions occurring on the ventral surface of the penis have been classified as median raphe cysts of the penis. They are lined by pseudostratified, columnar or stratified squamous cell epithelium, mimicking the epithelial lining of the male urethra. Ciliated cysts of the human skin are unusual. Cystic lesions containing ciliated cells have been noted to occur in the chest, neck, or head, and bronchogenic origin has been the most accepted explanation for its origin. Other reports show the presence of ciliated cysts on the lower extremities, and the mechanism of formation is still a debated question. A case of median raphe cyst of the penis containing ciliated epithelium is presented. The existing literature about these cutaneous lesions is reviewed, including the possible mechanisms believed to be involved in its origin.

Multiple idiopathic mucosal neuromas: A minor form of multiple endocrine neoplasia type 2B or a new entity?

Multiple endocrine neoplasia type 2B (MEN 2B) is a rare autosomal dominant process characterized by medullary thyroid carcinoma, pheochromocytoma, and mucosal neuromas. A point mutation at codon 918 of the RET protooncogene has been observed in approximately 90% of patients and families with MEN 2B. Mucosal neuromas are the most consistent and distinctive feature (100% of patients) of MEN 2B and are considered pathognomonic. We describe a 35-year-old woman with mucosal neuromas of the lower lip and tongue that appeared in early childhood. Examination did not reveal other abnormalities. After a follow-up of more than 22 years with periodic clinical, biochemical, and radiologic studies, no evidence of MEN 2B has been detected. Analysis of the RET protooncogene exons 10, 11, and 16 did not demonstrate point mutation in the MEN 2B region (M918T).

REITER'S SYNDROME‐LIKE PATTERN IN AIDS‐ASSOCIATED PSORIASIFORM DERMATITIS

Background. The prevalence of psoriasiform dermatitis in HIV‐infected patients is similar to that in the general population, but its clinical severity and the immunosuppression in these patients pose special problems of therapeutic management. Furthermore, a distinctive clinical pattern has been reported in some cases. In order to assess these features in our clinical setting, we have done a retrospective study on the clinical records of all human immunodeficiency virus (HIV)‐positive patients with psoriasiform dermatitis requiring systemic treatment.

Papules in the auricular concha: lichen amyloidosus in a case of biphasic amyloidosis.

We present a patient with lichen amyloidosus on the ears and macular amyloidosis on the back. These diagnoses were supported by histological, histochemical and immunohistochemical studies. This is to the best of our knowledge the first reported case of a biphasic form of amyloidosis whose lichenoid counterpart consists of papules on the ears. This suggests that primary cutaneous localized amyloidosis may have peculiar clinical manifestations depending on the location of the lesion.