Influence of pemphigoid gestationis on pregnancy outcome: A case report and review of the literature.

Pemphigoid gestationis is considered to be a rare pregnancy exclusive bullous disease, which modifies the course of the pregnancy, with difficulties in the management of the pruritus and skin lesions as well as a possible change in the neonatal outcome. Differential diagnosis of skin lesions and pruritus in pregnancy is challenging, and complementary investigations such as skin biopsy or laboratory tests are indispensable. The correct diagnosis and proper treatment could change the natural course of a pregnancy at risk and could improve maternal and fetal morbidity. We present the case of a patient with pemfigoid gestationis with the aim to highlight: i) the management of this pregnancy-associated skin disorder which transfers this pregnancy into a category of high obstetrical risk pregnancy; ii) the particularities of the course of the pregnancy; and iii) the importance in the differential diagnosis of pregnancy dermatoses. The particularity of this case of pemphigoid gestationis was the acute fetal distress in the absence of intrauterine growth restriction that is frequently found in this pathology, and the management of a rare pregnancy skin condition that currently has no standard treatment.

Granulomatous slack skin - seven years follow-up of a case with features of "parapsoriasis en plaques", muscle involvement, granulomatous vasculitis, and necrobiotic changes.

Granulomatous slack skin (GSS) represents an extremely rare variant of mycosis fungoides with only 70 cases reported in the literature to date. It is characterized clinically by the occurrence of bulky, pendulous skinfolds, usually located in flexural areas and histologically by an infiltrate composed of small neoplastic T-lymphocytes joined by granulomatous inflammation with scattered multinucleated giant cells containing nuclei arranged in a wreath-like fashion. Since its first description, very rare cases of GSS with muscle involvement, large vessels involvement, or necrobiotic changes have been reported. We present an extraordinary case of GSS with all these unusual features developing in the lesions of the same patient. The long follow-up of seven years allowed us to document the evolution of each lesion. Some lesions appeared and evolved in a manner very reminiscent of those of "parapsoriasis en plaques", others were classical GSS lesions, and still others developed large ulcerated lesions. These ulcerated lesions consistently failed to respond to conventional wound therapy, skin directed therapy [retinoids + psoralen combined with ultraviolet A (PUVA)-therapy], and interferon-alpha therapy. Remarkably, the ulcers completely healed when systemic corticosteroids were added. We hence postulate that the ulcers appeared because of large vessel vasculitis rather than tumoral direct destruction.

Desmoplastic melanoma--challenges in the diagnosis and management of a rare cutaneous tumor.

Desmoplastic melanoma (DM) represents a distinctive rare variant of spindle cell melanoma with a predilection for chronically sun-exposed skin of the elderly. This neoplasm is notoriously difficult to diagnose, both clinically and histopathologically. Therefore, DM is deeply infiltrative at the time of presentation. Histologically, the tumor presents as a proliferation consisting of non-pigmented spindle cells arranged in poorly formed fascicles. The neoplastic cells have a deceptively bland appearance with slightly pleomorphic and hyperchromatic nuclei, inconspicuous nucleoli and low mitotic activity. DM can mimic a whole range of benign and malignant neoplasms with spindle cell and fibrous appearance. Even though S100 remains the first-choice marker for DM, currently, there is no reliable marker with both high sensitivity and specificity for its detection. However, emerging melanoma markers, such as SOX10, have shown promising results in the diagnosis of DM. An accurate diagnosis of DM should always be based on the integration of all the clinical, histological and immunohistochemical features. Once diagnosed, DM should be aggressively excised with at least 2 cm lateral margins and down to the fascia. We present a case of DM that appeared on a non sun-exposed site. The tumor recurred multiple times in spite of repeated surgery involving wide local excisions and histologically reported negative margins. Recurrences are almost always associated with the presence of neurotropism. In our case, the neurotropism was obvious only in the second recurrence. We highlight the difficulties encountered in the diagnosis and management of both the initial tumor and its recurrence.

Completely regressed primary cutaneous melanoma - difficulties in diagnosis and classification.

Complete regression of primary cutaneous melanoma is a very rare phenomenon. Only 49 cases of well-documented completely regressed primary cutaneous melanoma have been reported to date. The clinical picture and histological findings may vary considerably. The presence of regional lymphadenopathy represents a necessary requisite for the diagnosis of completely regressed primary cutaneous melanoma. However, some cases lie outside these criteria and are difficult to diagnose and classify. Moreover, completely regressed melanoma is not specifically referred to in the current AJCC (American Joint Commission on Cancer) melanoma staging system. We report three cases of completely regressed primary cutaneous melanoma. One of the cases presented with unquestionable clinical and histopathological findings of completely regressed primary cutaneous melanoma, but without concomitant regional lymph node metastasis. As expected, this patient eventually developed nodal metastatic disease. An extraordinary case of a completely regressed melanoma that appeared in association with a congenital melanocytic nevus is also documented. This case revealed a unique type of regression that affected only the melanoma. The nevus was left undisturbed by the immunological response.