Silvana Bundino

Pyoderma gangrenosum associated with selective hereditary IgA deficiency.

A case of pyoderma gangrenosum is described in a girl aged 4. The condition was associated with selective IgA deficiency. The father and the 2 brothers suffered from the same deficiency (autosomal dominant transmission). Treatment with prednisolone and clofazimine produced an excellent clinical response.

Disseminated Porokeratosis Mibelli Treated with RO 10-9359

2 patients with widespread porokeratosis Mibelli (PM) were treated orally with RO 10-9359. The dose was 75 mg/day for 10 days, then 50 mg/day for 3 weeks. The drug produced a good improvement of condition with no serious side effect. Ultrastructural examination of a healed lesion showed the presence of a fine granular substance in the intercellular space of the spinous layer, most likely produced by keratinocytes; ultrastructural cellular alterations of PM were still evident and the lesion recurred after suspension of treatment.

Dermatofibrosarcoma protuberans. An ultrastructural study of five cases.

An ultrastructural study of five cases of dermatofibrosarcoma protuberans revealed that the basic cell was a spindle shaped fibroblast with a peculiar deeply lobulated nucleus. An origin from perineural and endoneural cells could not be established since basal lamina material was sparsely present in only one case and other differential features were missing. In one case histiocytic cells were found, but also in this case the basic cell was the fibroblast; so evidence of relationship with fibrous histiocytoma was not found.

Familial mandibuloacral dysplasia

A family is described in which two sisters and their two female fifth‐cousins were cases of mandibuloacral dysplasia. This syndrome is characterized by mandibular hypoplasia, delayed cranial suture closure, dysplastic clavicles, abbreviated terminal club‐shaped phalanges associated with acroosteolysis and atrophy of the skin over hands and feet. Furthermore, in this family alopecia and loss of the lower teeth were noted.

Ichthyosis linearis circumflexa Comèl and Netherton’s Syndrome; an Ultrastructural Study

An electron microscopic study of 3 cases of ichthyosis linearis circumflexa Comèl (ILC) reveals the presence of round bodies in the prickle cells: a characteristic feature of ILC. The horny and the granular layers are absent and are replaced by parakeratotic cells with condensed cytoplasm, which an amorphous material dissociates. The early formation of a vesiculopustule is detected. The importance of the dermal inflammation is considered and it is stated that ILC is not a purely epidermal disorder of keratinization.

Sea-blue histiocyte syndrome with cutaneous involvement. Case report with ultrastructural findings.

A patient with infiltration of the skin resulting in eyelid swelling and facial nodules was recognized as a case of sea-blue histiocyte syndrome with cutaneous involvement. Typical sea-blue histiocytes were found in the skin and confirmation was provided by electron microscopy. Hepatosplenomegaly, lung infiltrates and bone marrow involvement were the other symptoms. The relationship between sea-blue histiocyte syndrome and adult Niemann-Pick disease is also discussed.

Acrosyringial epidermolytic papulosis neviformis.

A 42-year-old woman showed multiple verrucous papules on her right ring finger. Histology revealed a hyperkeratotic plug and epidermal foci of epidermolytic hyperkeratosis exclusively involving the intraepidermal sweat duct units. We propose the term acrosyringial epidermolytic papulosis neviformis or epidermolytic sweat duct nevus to define this unique entity.

Multiple cutaneous fibromyxomas. A light and electron microscopic study

A 27‐year‐old woman developed many nodular lesions, ranging in diameter from 3 mm to 15 mm and clinically indistinguishable from dermatofibromas. Light microscopic examination showed large amounts of mucin, fibroblasts and histiocytes throughout the dermis. Electron microscopic study revealed wide electron lucent areas and long branching winding electron dense threads occupying the ground substance. While dilated cisternae of endoplasmic reticulum containing a finely granular material characterized the fibroblasts, banded structures, intracellular collagen and myelinoid figures were prominent in the histiocytes. The term fibromyxoma is introduced to designate this tumor.