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Clustered unilateral trichoepitheliomas indicate Type 1 segmental manifestation of multiple familial trichoepithelioma

mended, but the patient refused it. APD is a rare dermatosis characterized by cyclical cutaneous eruptions. It usually affects premenopausal women, and occurs 3–10 days prior to the onset of menstrual flow, and resolves 2–3 days into menses when progesterone levels decrease. The pathogenesis of APD still remains unknown. It is supposed that the skin lesions occur via a type 1 hypersensitivity reaction, due to occurrence of APD in some patients after taking exogenous progesterone. However, this mechanism is not enough to explain the pathogenesis of patients such as our case, who have never taken exogenous progesterone. Antibodies against progesterone may stimulate dermatitis, leading to a type 3 hypersensitivity reaction. This mechanism may not be valid for some patients such as our case, because DIF staining is negative. IDT with progesterone may result with immediate (within 30 min) and delayed (24–48 h later) reactions. These two reactions may occur simultaneously, as with our patient, and may be indications of type 1 and type 4 hypersensitivity reactions. We believe that hypersensitivity reactions may play a combined role in the pathogenesis of APD. APD does not have a specific skin lesion associated with it, and it may be mistaken for various skin manifestations such as urticaria, eczema, erythema multiforme, fixed drug eruption and rarely, vesiculobullous eruptions. Vesicular eruption, including papules, pustules and bullous lesions, have been observed in small numbers of patients; however, only a few of these patients presented with vesicles alone, while others were evaluated as bullous erythema multiforme, dishydrotic eczema or erosive stomatitis. Histopathological findings usually reflect the dermatosis that is clinically observed, as in our case. Diagnosis of APD may not be easy if it is not suspected at first. Detailed medical history documenting the relation between skin lesions and menstrual cycle is the most important step for diagnosis. IDT or intramuscular challenge test with progesterone should be carried out to confirm the diagnosis.